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INDEX




                                                         INTRODUCTION
                                                         RADIOLOGICAL    PATHOLOGY
                                                          OF PILOCYTIC ASTROCYTOMAS




INTRODUCTION

Astrocytomas are, by far, the largest category of primary neoplasms of the brain. There
are two primary patterns of growth seen in astrocytomas: diffuse and circumscribed. The
diffusely infiltrating astrocytomas have been known since the early days of brain surgery
and neuropathology. The circumscribed group of astrocytomas has only recently received
widespread acceptance, although one subtype-pilocytic astrocytoma (PA)-has been well
described for decades. Several of the subtypes of astrocytoma more recently added to the
WHO classification are characterized by a circumscribed pattern of growth.
   Pilocytic Astrocytoma

PA is the prototype for low-grade (benign) circumscribed astrocytoma. 30 This tumor
represents approximately 2% to 6% of all primary brain tumors. In some series, they are
described as the most common tumor of the cerebellum in childhood. 13, 14,15 In other series,
they are less frequent than medulloblastoma and account for little more than 7% of all
neoplasms in patients younger than age 20. Patients with these tumors present primarily
during childhood with the peak ages of presentation between 5 and 15 years old. 1,3,5,6,7,8,10
Tumors of the chiasm and other locations of the diencephalon may present at younger ages.
Within the diencephalon, PA may present in the orbital portions of the optic nerves, within
the hypothalamus, and within the thalamus. Most of the optic nerve gliomas that occur in
neurofibromatosis type 1 (von Recklinghausen type) are PA. Most series report an equal
incidence in both sexes; others indicate a slight preponderance of female patients in a ratio
less than 4:3. 7,8,9



                                     Common anatomical location 1,3,5,6,7,8,10
                                     Cerebral hemisphere (18%)
                                     Cerebellum (55%)
                                     Brain stem (17%)
                                     Hypothalamic, chiasmal (2%)



In the Egyptian study of metwally 30 most of the tumors occurred in the deep parietal
region (64%) and the cerebellum was involved next in frequency. (see table 1) In all the
reviewed literature the cerebellum was the primary site of involvement in patients younger
than the age of ten while in older patients pilocytic astrocytomas occur more frequently
supratentorially1,3,5,6,7,8,10 and this is consistent with the results of Metwally 30 since patients
in his study with supratentorial (parietal) tumors were older, and greater in number, than
patients with cerebellar tumors and subsequently the percentage of tumor occurrence,
anatomically, was higher in the supratentorial (deep parental) zone, compared with the
cerebellar area in this series. All patients in the Egyptian study of metwally 30 were males
and this is unlike most of the reviewed studies. 1,3,5,6,7,8,10
Figure 1. A pilocytic astrocytoma, notice the peripherally located hypercellular part (mural
nodule [arrows]) and the multicystic appearance of the tumors.

It is interesting that the clinical picture, in pilocytic astrocytomas patients, is characterized
by mild clinical disability and paucity of clinical signs despite the fact that tumors are
frequently large enough. This can be explained by the benign nature of this neoplasm that
apparently resulted in a very slow rate of growth. These tumors widely, and very slowly,
expand neural tissues without neural destruction or interruption of normal function. 11,12,13
The very slow rate of growth of these tumors allows the brain to move functionality from
one region to anther and this process of brain remapping, which has been shown to occur
at all ages, is partially responsible for the relatively late appearance of symptoms and
paucity of clinical signs on presentation in pilocytic astrocytomas patients. 11,12,13

Table 1. Distribution of pilocytic astrocytomas in Egypt 30

Anatomical localization                              %                      Mean age
Deep frontoparietal                                  64%                    13 years
Cerebellar hemispherical                             18                     6 years
Hypothalamus, 3rd ventricular                        9                      9 years
Brain stem, pontine                                  9                      9 years
RADIOLOGICAL PATHOLOGY OF THE JUVENILE PILOCYTIC ASTROCYTOMA

The capillaries may be abnormal and  Grossly,     PA    is    a   well-circumscribed      yet
can be coiled (angiomatous) and      unencapsulated mass. The lesion grows primarily by
thick-walled. Apparently the blood-  expansion rather than the infiltration characteristic of
brain barrier is not well formed in  most astrocytomas. In many cases, lesions are easily
these tumors. The proteinaceous fluidseparated from the adjacent uninvolved cerebellar
that accumulates as both microcysts  folia. Most PA have a significant grossly visible cystic
and macrocysts probably leaks from   component. In many cases, the tumor has the
the abnormal vessels. Mitosis and    classically described cyst with nodule morphology-in
necrosis are distinctly uncommon.    which neoplasm is confined to a nubbin of tissue
Despite this, occasional PA show     embedded in the wall of a fluid-filled cavity. In these
microscopic hemorrhages or brownish  cases, the cyst fluid is surrounded by nonneoplastic
staining.                            compressed or gliotic tissue. Calcification can be seen
                                     in 25% of cases. Microscopically, there is a biphasic
pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes alternating
with looser regions that may have microcysts. One distinctive feature is the presence of
eosinophilic curvilinear Rosenthal fibers within the dense regions. The capillaries may be
abnormal and can be coiled (angiomatous) and thick-walled. Apparently the blood-brain
barrier is not well formed in these tumors. The proteinaceous fluid that accumulates as
both microcysts and macrocysts probably leaks from the abnormal vessels. Mitosis and
necrosis are distinctly uncommon. Despite this, occasional PA show microscopic
hemorrhages or brownish staining.

Table 2. The mural nodule is composed of two main parts as follows* 30

Part      Appearance               Histopathology
Part I    Dense          compact Composed of elongated bipolar hairlike (pilocytic)
          appearance             astrocytes with high nuclear to cytoplasmic ratio and
                                 with minimal extracellular fluid. This part is relatively
                                 vascular.
Part II   Loose appearance         Relatively acellular and composed mainly of microcysts
                                   and enlarged extracellular fluid filled spaces. This part
                                   is relatively avascular.

* The spatial distribution of each part within the mural nodule will determine the
neuroimaging appearance of the mural nodule.
Figure 2. Cystic (pilocytic) brain stem
glioma.




Many astrocytomas, particularly in the cerebellum, hypothalamus, and optic pathways of
children, exhibit a typical histologic appearance previously termed polar spongioblastoma and
now universally referred to as juvenile pilocytic astrocytoma. These tumors frequently contain
both macrocysts (as in cystic cerebellar astrocytoma) or microcysts. Rosenthal fibers, strongly
eosinophilic coalescences of neurofibrillary elements, are characteristic of juvenile pilocytic
astrocytoma, but may be found in other forms of tumor, particularly as a glial reaction
surrounding craniopharyngioma. Endothelial proliferation is common in these tumors and has
none of the ominous connotations in this context that it implies in other forms of astrocytomas.




Figure 3. Pilocytic astrocytoma with hair-like cells

From the pathological point of view pilocytic (hair cells) astrocytomas are composed of two
main parts, a fluid-filled large cyst and a projecting mural nodule. The neoplastic cells are
confined to the mural nodule and the cyst walls are composed of non-neoplastic
compressed or gliotic neural tissues. Pilocytic astrocytomas do not have true capsule, yet
they can easily be separated from the surrounding tissues. Calcification can be seen in 25%
of cases. 1,3,5,6,7,8,10 These tumors frequently have microcysts and macrocysts.
Microscopically, there is a biphasic pattern of dense areas with elongated bipolar hairlike
(pilocytic) astrocytes alternating with looser regions that may have microcysts. One
distinctive feature is the presence of eosinophilic curvilinear Rosenthal fibers and strongly
eosinophilic coalescences of neurofibrillary elements within the dense regions. 1,3,5,6,7,8,10 The
capillaries may be abnormal and can be coiled (angiomatous) and thick-walled. Apparently
the blood-brain barrier is not well formed in these tumors. The proteinaceous fluid that
accumulates as both microcysts and macrocysts probably leaks from the abnormal vessels.
Necrosis, mitotic activity, endothelial proliferation are selectively absent in pilocytic
astrocytomas. Pilocytic astrocytomas are very slowly growing tumors, with long
premonitory symptoms before clinical presentation, that selectively grow by expansion
rather by infiltration of the surrounding neural tissues that is more characteristic of diffuse
astrocytomas. 1,3,5,6,7,8,10,11,12,13,14,15




Figure 4. This figure shows a gross specimen of a pilocytic astrocytoma of the posterior
fossa. Identify the cerebellar hemispheres, the pons and the fourth ventricle. The fourth
ventricle is nearly obliterated due to the large cystic tumor in the midline. Note the white
nodule to one side of the cyst. This is the actual tumor. Many pilocytic astrocytomas in the
posterior fossa will have an associated cyst and a contrast enhancing "mural" nodule.
Pilocytic astrocytomas are one of the most common pediatric brain tumors and most occur
in the posterior fossa, but in children with Neurofibromatosis type I, they may occur in the
optic tracts.

Juvenile pilocytic astrocytomas tend to be well circumscribed and to grow slowly with long
periods of premonitory symptoms before presentation. This pattern is especially true of tumors
that arise in the cerebellum. Tumors of the anterior third ventricle tend to be well-defined
superiorly but diffusely infiltrating the optic mechanisms and hypothalamus inferiorly. The
course of these tumors is normally benign but may be unpredictable thereby making treatment
decisions extremely difficult. The presence of a juvenile pilocytic astrocytoma that extends into
the subarachnoid space is common.

Pilocytic astrocytomas differ from the more common diffuse astrocytomas from the
pathological, nosological, radiological, genetic and prognostic point of view.
1,3,5,6,7,8,10,11,12,13,14,15
                              From the pathological point of view diffuse astrocytomas are neoplasms
of widely varying potential that are unencapsulated, poorly marginated and diffusely
infiltrate into the surrounding brain. These diffuse astrocytomas appear to form a
continuum of both biological and histological aggression. They vary from lesions with
almost normal cytology (grade II astrocytomas) through intermediate stages (grade III,
anaplastic astrocytomas) and up to the most aggressive of all human brain tumors (grade
IV astrocytomas or glioblastoma multiforme). 11,12,13




                                             Figure 5. This figure shows a gross specimen
                                             of a pilocytic astrocytoma of the posterior
                                             fossa. Identify the cerebellar hemispheres, the
                                             pons and the fourth ventricle. The fourth
                                             ventricle is nearly obliterated due to the large
                                             cystic tumor in the midline.




Figure 6. Histopathological and gross pathological picture of the pilocytic astrocytoma with
the characteristic microcysts and a large mural nodule

From the nosological point of view, and according to the WHO classification of brain
tumors 13, pilocytic astrocytomas are ranked as grade I benign gliomas while diffuse
astrocytomas are ranked as grade II, grade III (anaplastic astrocytomas) and grade IV
(glioblastoma multiforme). The following pathological differences are present between
diffuse and pilocytic astrocytomas. 11,12,13

      Diffuse astrocytomas, unlike pilocytic astrocytomas, have a peculiar tendency to
       change its grade over time and the condition is age dependant. 11,12,13 A change in
       the grade of diffuse astrocytoma is more likely to occur in the older age group. In
       older age group (over the age of 40 years) diffuse low grade astrocytomas (grade II
       astrocytoma according to WHO) have a bad prognosis because they have a great
       tendency for anaplastic transformation (to grade III or grade IV astrocytoma
       according to WHO), 11,12,13 while at a younger age group anaplastic transformation
of diffuse low grade astrocytomas (grade II astrocytoma according to WHO) is
       extremely uncommon, 11,12,13,14,15 also the probability for diffuse low grade
       astrocytomas to have a highly malignant component (i.e, grade III or IV mixed with
       grade II) is higher in the older age group. 11,12,13 On the other hand pilocytic
       astrocytomas (grade I astrocytoma according to WHO) never change its grade over
       time. 1,3,5,6,7,8,10,11,12,13,14,15
      Diffuse astrocytomas (grade II, III or grade IV astrocytomas) grow by infiltration of
       the nearby neural tissues (commonly in the form of remote neoplastic cells radiating
       from the mother tumor) and so they are poorly marginated and, practically,
       complete surgical resection is not possible and some neoplastic cells are almost
       invariably left behind after surgical resection. On the other hand pilocytic
       astrocytomas grow by expansion and so they are well circumscribed and
       subsequently complete surgical resection is possible. 11,12,13
      Diffuse astrocytomas, unlike pilocytic astrocytomas, are highly cellular neoplasms
       with cells that range from normal appearing astrocytes (grade II) to cells with
       marked pleomorphism and hyperchromatic nuclei ( grade III, and IV). 11,12,13 On
       the other hand pilocytic astrocytomas are histopathologically composed of scanty
       elongated cells, Rosenthal fibers and microcysts and this combination constitutes the
       classic of pilocytic astrocytomas. Cells are only confined to the mural nodule in
       pilocytic astrocytomas and subsequently the mural nodule is the only neoplastic part
       of the tumor. 11,12,13
      Pilocytic astrocytomas are truly benign gliomas while diffuse astrocytomas are, at
       best, of low grade malignancy. 1,3,5,6,7,8,10,11,12,13,14,15

Radiologically pilocytic astrocytomas differ from diffuse astrocytomas in the following
points

   1. Pilocytic astrocytomas are typically "cystic tumors with a mural nodule" and with
      prominent mass effect, while diffuse astrocytomas with a lower grade (grade II) are
      typically solid tumors with minimal or no mass effect that appear homogeneously
      hypodense on CT scan, hyperintense on the MRI T2 images and hypointense on the
      T1 MRI images. 1,2,3,5,6,7,11,12,13,26 The neuroimaging appearance of diffuse
      astrocytomas is due to increased cell count. 11,12,13,27 The mural nodule of pilocytic
      astrocytomas might or might enhance while diffuse low grade astrocytomas usually
      do not enhance on postcontrast scan. 1,2,3,5,6,7,11,12,13,27,28,29 Diffuse low grade
      astrocytomas (grade II) diffusely expand the affected part of the brain with poor
      margin, while pilocytic astrocytomas are well circumscribed rounded or oval
      tumors. 11,12,13,27,28
   2. Although central necrosis in highly malignant glioblastoma multiforme might,
      morphologically, create the appearance of a cyst with a projecting mural nodule,
      however this can easily be differentiated from pilocytic astrocytomas by the fact that
      the walls of the cyst in pilocytic astrocytomas, being composed of non-neoplastic
      compressed neural tissues, never enhance while the walls of the cyst in glioblastomas
      with central necrosis invariably enhance because it is composed of viable tumor
      tissues. 11,12,13 Wall enhancement is characteristic of glioblastomas with central
      necrosis and when it is observed radiologically should shift the tumor grade from
the most benign pilocytic astrocytoma to the most malignant glioblastoma
      multiforme, see figure 8. 11,12,13 The presence of significant edema and short history
      before clinical presentation favor the diagnosis of glioblastoma multiforme. In
      general glioblastoma multiforme occurs at an older age compared with pilocytic
      astrocytoma. 11,12,13,27,28 The presence of blood products on CT scan or MRI is
      characteristic of the highly malignant glioblastoma multiforme.




Figure 7. Two CT scan studies showing juvenile pilocytic astrocytomas in left cerebral
hemisphere, A and the cerebellum, B. Notice the wall calcification in (B). Notice that the
mural nodule has a hypodense core and a hyperdense rim in A (pattern I), and is diffusely
hyperdense in B (pattern II). The cystic component of the tumors is diffusely hypodense.




Figure 8. A,B Precontrast and postcontrast CT scan studies showing parietal pilocytic
astrocytoma, notice that only the mural nodule was enhanced on postcontrast scan A, while
the cyst wall remained unenhanced. For comparison a postcontrast CT scan study of a case
with glioblastoma and with central necrosis is presented (C). Notice that in glioblastoma
both the cyst wall and the mural nodule are enhanced.
On imaging studies, the presence of a cystic
Juvenile pilocytic astrocytomas are usually well defined
                                             component in these lesions is suggestive of
on both CT and magnetic resonance imaging (MRI). In
                                             pilocytic astrocytomas. 30 The lesions may
the cerebellum, they arise either within the vermis or
cerebellar hemispheres. They tend to have a large single
                                             appear on both CT and MR as a classic cyst
cyst with a "mural nodule". They can, however, have
                                             with nodule mass. Purely solid masses are
multiple cysts or be completely solid. Tumors without
                                             not common. Complex shapes (e.g.,
cysts are more frequently the diffuse (nonpilocytic)
                                             multiloculated) are frequent and may create
astrocytomas. Before contrast enhancement, the
                                             a misleading appearance. With contrast
appearance of the tumor is similar to cerebellar tissue.
                                             infusion, both on CT and on MR, PA almost
invariably demonstrate prominent enhancement. The pattern may be a classic cyst with
nodule, (only the mural nodule enhances brightly while the cyst wall, being composed of
nonneoplastic compressed neural tissues, usually does not enhance beyond the edge of
nodule) although variable degrees of wall enhancement also occur. More complex patterns
of enhancement may appear to suggest necrosis and a high-grade neoplasm. Although this
is a low-grade (WHO grade I) tumor, in certain examples, especially those presenting in the
cerebral hemispheres, the prominent surrounding vasogenic edema may create a
disturbing appearance.




                                                           Figure 9. A, Precontrast MRI T1, and MRI
                                                           T2 (B,C) showing cerebellar pilocytic
                                                           astrocytoma, Notice that the mural nodule is
                                                           isointense, relative to the normal cerebellar
                                                           tissues, on the T1 image hypointense,
                                                           relative to the cyst, on the T2 MRI images,
                                                           (B,C). The cyst is hypointense on the T1
                                                           image and hyperintense on the T2 images



In neuroimaging studies of juvenile pilocytic astrocytomas, the acellular cystic part
generally appears as a low signal intensity on T1-weighted sequences, as a high signal
intensity on T2-weighted sequences and diffusely hypodense on CT scan studies. The mural
nodule, which is the neoplastic part of the tumor, have four neuroimaging patterns of CT
density and MRI T1, T2 signal intensities as follows:

Table 3. Neuroimaging patterns of the mural nodule 30 (see Fig 16)

Pattern      Comment
Pattern I    The mural nodule has a large central core and a peripheral thin rim. The core
             is hypointense on precontrast T1 MRI studies, hypodense on precontrast CT
             scan studies and hyperintense on the MRI T2 images. The peripheral rim is
             hyperintense on the precontrast MRI T1 images, and hyperdense on
             precontrast CT scan studies (even though not calcified) and hypointense on
             the MRI T2 studies. Because the peripheral thin rim has CT density/MRI
             signal intensity different from the central core of the mural nodule and from
             the tumor cystic cavity, it separates the mural nodule from the cystic cavity on
             neuroimaging studies making it easier to differentiate between the cystic
             component and the mural nodule. CT Density measurement reveals no
             evidence of calcification in all cases with this pattern. In this pattern the
             peripheral thin rim is hypercellular/vascular while the central core of the
             mural nodule is relatively acellular/avascular and composed of microcysts.
             After contrast enhancement only the hypercellular peripheral rim of the
             mural nodule enhances, while the acellular central core does not enhance.
             Mural nodule with this pattern is larger in size. See Fig 7A
pattern II   The nodule appears diffusely hyperdense on precontrast CT scan and
             diffusely hypointense on MRI T2 images. CT Density measurement reveals no
             evidence of calcification in all cases with this pattern. In this pattern the mural
             nodule has a compact, dense appearance and is diffusely
             hypercellular/vascular and enhances diffusely and brightly after contrast
             injection. Mural nodule with this pattern is smaller in size. See Fig 7B and Fig
             13
pattern III The nodule appears diffusely hypodense on precontrast CT scan, diffusely
            hypointense on precontrast MRI T1 images, and diffusely hyperintense on
            MRI T2 images. Differentiation between the mural nodule and the cystic part
            of the tumor might not be easy. The nodule in pattern III is relatively diffusely
            acellular and has a looser appearance. Contrast enhancement in this pattern is
            poor. See Fig 10b
Pattern IV The nodule appears irregularly hyperdense on precontrast CT scan and
           hypointense on MRI T2 images. CT Density measurement reveals evidence of
           patchy calcification of the mural nodule in all cases with this pattern. See Fig.
           14

In the author experience the prevailing radiological pattern of the mural nodule is pattern
I.

Microscopically, the mural nodule is composed of a biphasic pattern of dense areas with
elongated bipolar hairlike (pilocytic) astrocytes (with high nuclear to cytoplasmic ratio and
with minimal extracellular fluid), these astrocytes are arranged peripherally in most tumor
nodules, and alternating with looser regions that are rich in fluid filled microcysts, these
regions are arranged centrally in most tumor nodules. The hypercellular peripheral rim
(with cells that have a high nuclear to cytoplasmic ratio with minimal extracellular fluid)
appear hyperintense on the precontrast T1 images, hypointense on the MRI T2 studies and
hyperdense on precontrast the CT scan studies, while the relatively acellular fluid filled
microcystic core appear hypointense on the precontrast T1 studies, hyperintense on the
MRI T2 studies and hypodense on the precontrast CT scan studies. The topographic
distribution of the hypercellular part, peripherally, and the microcystic acellular part,
centrally, within the mural nodule is responsible for the production of the patten I. Should
the hypercellular part predominate the histopathological architecture of the mural nodule,
pattern II is the expected result. Should the relatively acellular fluid filled microcystic part
predominate the histopathological architecture of the mural nodule, pattern III is the
expected result. Should the mural nodule become calcified, pattern IV is the expected
result.30




Figure 10. A, CT scan showing pattern I of the mural nodule, B, CT scan showing pattern
II of the mural nodule, C, MRI T2 image showing pattern III of the mural nodule, the
nodule is not showing because it is isointense to the cystic cavity. D,E MRI T1,T2 showing
pattern I of the mural nodule. F, MRI T2 image showing pattern II of the mural nodule,
the nodule is hypointense relative to the cystic cavity.
Pilocytic tumors are sometime wholly solid (noncystic) and composed of elongated bipolar
hairlike (pilocytic) astrocytes (with high nuclear to cytoplasmic ratio and with minimal
extracellular fluid). The tumor in this case appears hyperintense on the precontrast T1
images, hypointense to isointense on the MRI T2 studies and hyperdense on precontrast
CT scan studies, with dense postcontrast enhancement. In the author experience
perilesional edema is common in solid tumors. 30



                                               Figure 11. A solid pilocytic astrocytoma,
                                               The tumor is hyperdense on noncontrast
                                               CT scan (A), with dense postcontrast
                                               enhancement. (B). The tumor contains
                                               some cystic spaces and is surrounded by
                                               edema. This is probably the initial stage in
                                               the natural evolution of the tumor.



After the infusion of an intravenous contrast agent, the solid hypercellular components of
the mural nodule tend to enhance brightly and to appear as a distinct, well-defined mass.
Contrast enhancement is prominent is mural nodule with pattern II tissue, while in mural
nodule with pattern I tissues enhancement, though present in the hypercellular/vascular
peripheral thin rim, might not be appreciated visually (enhancement can be appreciated if
the CT density of the peripheral rim is taken before and after contrast injection).
Enhancement is prominent in purely solid tumors. The cyst wall, being composed of
nonneoplastic compressed neural tissues, usually does not enhance with contrast material.
Figure 12. A,B, Precontrast MRI T1, and MRI T2 (C)
                                 showing frontal pilocytic astrocytoma. The cyst is
                                 hypointense on the T1 images and hyperintense on the T2
                                 images. The mural nodule has pattern III and cannot be
                                 seen as it is isointense to the cystic cavity.




In general pilocytic astrocytomas have heterogenous histopathological composition
(biphasic pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes
alternating with looser regions that may have microcysts) and subsequently the tumor
might be completely solid and occasionally the tumor might be cystic with a small dense
hypercellular peripheral mural nodule (pattern II). Sometimes the mural nodule itself
might have a large cystic core with a peripheral hypercellular thin cover (patten I).
Although the share taken by the cystic (acellular/avascular) and the solid
(hypercellular/vascular) in the histopathological composition of the pilocytic astrocytomas
might vary (resulting in heterogenous appearance of the tumors in neuroimaging study
both in precontrast and in postcontrast studies), however the neuroimaging appearance of
pilocytic astrocytomas simply reflects the natural evolution of the tumors. 30

A pilocytic astrocytoma usually starts as hypercellular solid tumor with elongated bipolar
hairlike (pilocytic) astrocytes with high nuclear to cytoplasmic ratio and with minimal
extracellular fluid (purely solid tumors). Solid tumors are vascular and their capillaries
may be abnormal and can be coiled (angiomatous) and thick-walled. Apparently the blood-
brain barrier is not well formed in these tumors and proteinaceous fluid probably leaks
from the abnormal vessels and accumulates in the tumors as microcysts, first, and
macrocysts later on. With progressive enlargement of the macrocysts (microcysts enlarge
and coalesce forming a single large cyst), the viable tumor tissues are progressively
compressed into a smaller, dense and hypercellular peripheral mural nodule (pattern II).
Progressive leakage of proteinaceous fluid within the core of the mural nodule will result in
progressive enlargement of the mural nodule, the core of which will be cystic with a thin
outer cover of viable tumor tissues (pattern I). Although the typical appearance of a
pilocytic tumor is a large single cyst with a mural nodule, however the spatial distribution
of the solid (hypercellular/vascular) and the cystic components within the tumors can vary,
also the share taken by the solid the cystic parts in the histopathological make-up of the
tumors might vary. These histopathological variations might result in tumors that have
quite atypical appearance with irregular cystic and solid parts and with irregular or patchy
contrast enhancement. 30




Color plate 1. A pilocytic astrocytoma commonly starts as a solid mas (1), however due to
defective blood brain barrier in the newly formed blood vessels proteinaceous fluid
probably leaks and accumulates inside the tumor as microcysts, first (2), and macrocysts
later on (3). With progressive enlargement of the macrocysts (microcysts enlarge and
coalesce forming a single large cyst), the viable tumor tissues are progressively compressed
into a smaller, dense and hypercellular peripheral mural nodule (pattern II) against a large
cyst (3). Progressive leakage of proteinaceous fluid within the core of the mural nodule will
result in progressive enlargement of the mural nodule, the core of which will be cystic with
a thin outer cover of viable tumor tissues (pattern I) (4,5,6). (Blue = cystic parts and brown
= solid parts of the tumor)

If the CT scan discloses that both the wall of the cyst and the solid component of the
neoplasm enhance with intravenous injection of contrast material, MRI scan commonly
disclose that the tumor is more extensive than the CT scan suggested and the surgical
specimens disclose highly malignant gliomas (anaplastic astrocytoma or glioblastoma).
In general calcification is commonly present in pilocytic astrocytomas (28% 30),
Calcification might be present in the mural nodule (see figure 14) or in the cyst wall (see
figure 7).



                                                               Figure     13.     Pilocytic
                                                               astrocytoma. Axial and
                                                               sagittal   T1     -weighted
                                                               gadolinium-enhanced MR
                                                               images show a classic cyst
                                                               with nodule morphology
                                                               (with pattern II tissues).
                                                               Notice that the wall of the
                                                               cyst does not enhance
                                                               beyond the edge of nodule.



The appearance of cystic astrocytomas of the brain stem is very similar to that of cystic
astrocytomas of the cerebellum. The CT scans/MRI disclose only the mural nodule
enhanced with contrast. Cystic astrocytomas are often associated with a large cyst that
excavates much of the brain stem. These tumors are commonly found in the cerebral
peduncle or pons, and both CT scan and MRI give satisfactory imaging.




Figure 14. A, Precontrast CT scan showing frontal pilocytic tumor with a calcified mural
nodule (pattern IV), and (B) cystic cerebellar pilocytic tumor with a large, calcified mural
nodule (pattern IV).
After intravenous infusion of contrast material, the tumor will normally enhance brightly and
thoroughly. Two patterns seem to exist in the primarily cystic varieties. Most commonly, the
mural nodule enhances brightly and the cyst wall does not enhance. In this case, pathologic
assessments show that the cyst wall is composed of compressed cerebellar tissue and that the
tumor is confined to the mural nodule. In some tumors, the entire wall of the cyst enhances. This
apparently means that there has been degeneration within the center of the tumor, and viable
tumor completely surrounds the cyst. In this case, the pathological diagnosis is glioblastoma
multiforme and the entire cyst wall must be resected.

                                                              Figure 15. A, postcontrast CT
                                                              scan study showing a large
                                                              parietal pilocytic astrocytoma.
                                                              Neither the mural nodule nor
                                                              the cyst wall was enhanced. B.
                                                              postcontrast CT scan study
                                                              showing a large hypothalamic
                                                              pilocytic astrocytoma with wall
                                                              enhancement.




Figure 16. The stages of the natural evolution of pilocytic astrocytoma. The initial solid
stage with some microcysts is illustrated in (A). The second stage is illustrated in (B) where
the tumor is composed of a large cyst with a single densely enhanced non-cystic small
mural nodule (pattern II). The third stage is illustrated in (C) The mural nodule has
enlarged and is composed or a cystic hypodense central core and a peripheral rim of viable
tumor tissue (pattern I). The mural nodule ultimately becomes cystic.

From the genetic point of view pilocytic astrocytoma is different from diffuse astrocytomas
in the following points

      The analyses of the genetic lesions of pilocytic astrocytoma have targeted the TP53
       gene on chromosome 17. Investigations have not confirmed a critical role for
alterations of this gene in the development of these tumors, however. In one series,
    the cytogenetic analysis of 14 pilocytic tumor cultures did not identify a specific
    pattern of chromosomal aberration. (15) Patients with tumors characterized by
    normal stem line karyotypes had the most favorable outcomes. The presence of
    clonal structural abnormalities and the presence of markers were associated with a
    high risk of early recurrence. (15)
   On the other hand genetic lesions associated with the development and malignant
    transformation of diffuse astrocytomas have been well described in the cytogenetic
    literature. (16, 17, 18, 19) To date, three distinct clinical, histologic, and genetic
    patterns of glioblastoma multiforme have been characterized. In younger patients,
    most diffuse astrocytomas are believed to begin as low-grade astrocytoma, with
    progression to glioblastoma multiforme through a stepwise acquisition of genetic
    lesions. These secondary glioblastoma multiforme often contain areas of well-
    differentiated residual tumor. (20, 21) The most frequent chromosomal abnormality
    identified in diffuse astrocytomas is the abnormal gain of chromosome 7 with an
    associated loss of one of the sex chromosomes. Additionally, allelic loss or mutation
    of 17p, resulting in critical alterations of the TP53 gene, has been targeted as an
    essential step in the early development of glioma. (22, 23) Mutant TP53, identified in
    at least one third of all astrocytomas, may contribute to the formation of these
    tumors by inhibiting programmed cell death. glioblastoma multiforme in older
    patients are usually primary-that is, they occur as glioblastoma multiforme from
    their inception, without progression from a lower- grade tumor. 16, 17, 18, 19, In this
    group, the development of glioblastoma multiforme involves a parallel sequence of
    genetic alterations, including amplifications and deletions, that up-regulate growth
    factor receptors and drive cell proliferation. 16, 17, 18, 19, 24, 25
Figure 17. MRI
                                                                              T1, T2 images
                                                                              showing      cystic
                                                                              pontine pilocytic
                                                                              astrocytoma with
                                                                              a large -pattern I-
                                                                              cystic       mural
                                                                              nodule



      Management of juvenile pilocytic astrocytoma

Because pilocytic astrocytomas grow by expansion rather by infiltration of the nearby
neural structure (infiltration results in tumor cells being found histologically radiating
diffusely from the mother tumor to the surrounding normal neural structures), they
remain circumscribed and can be separated from normal neural tissues 11,12,13,27,28,29, thus
allowing complete surgical removal without leaving behind any residual tumor cells and it
is because of this that postoperative radiotherapy or chemotherapy are not indicated
11,12,13,27,28,29
                  and probably even contraindicated because Burger and Fuller 28 reported a
pilocytic astrocytoma recurring after 28 years as a glioblastoma multiforme in a child who
received postoperative radiotherapy and they attributed this rare occurrence to the
probable teratogenic effect of radiotherapy. The prognosis in pilocytic astrocytoma is good
with a five year survival rate reaching up to 95% to 100% in many studies after complete
surgical removal. 27,28,29 Recurrence was attributed in most studies to incomplete surgical
removal in technically difficult anatomical sites such as patients with hypothalamic
neoplasm.11,12,13,27,28,29,30

Because of the very good prognosis of this neoplasm it is important to be familiar with its
clinical and neuroimaging pictures. The presence of a cystic component in these lesions is
suggestive. The lesions may appear on both CT and MR as a classic cyst with a nodular
mass. Purely solid masses are not common. Complex shapes (e.g., multiloculated) can occur
and may create a misleading appearance. 1,3,5,6,7,8,10,11,12,13,14,15 With contrast infusion, both
on CT and on MR, the mural nodule might or might not enhance, however the cyst wall
does not enhance. Histopathological confirmation is invariably needed for the ultimate
diagnosis of this neoplasm. Although the word astrocytoma is typed in the name of this
neoplasm, however it should not be equated with the more common diffuse astrocytoma.
"Overgrading" occurs when the generic name "astrocytoma" is applied to pilocytic
astrocytomas, so it is very important to distinguish between diffuse astrocytoma and
pilocytic astrocytoma as there is a real chance that pilocytic astrocytomas are cured by
surgery alone once the neoplasms are completely removed. 30



REFERENCES

1. Cottingham SL, Boesel CP, Yates AJ: Pilocytic astrocytoma in infants: A distinctive
histologic pattern. J Neuropathol Exp Neurol 55:654, 1996

2. Ashby LS, Coons SW, Scheck AC, et al: Cytogenetic abnormalities in juvenile pilocytic
astrocytomas (JPA) and patient prognosis. Neurooncology 1:S3, 1999

3. Lee YY, van Tassel P, Bruner JM, et al: Juvenile pilocytic astrocytomas: CT and MR
characteristics. AJNR Am j Neuroradiol 10:363-370, 1989

4. Brown MT, Friedman HS, Oakes Wj, et al: Chemotherapy for pilocytic astrocytomas.
Cancer 71:3165, 1993

5. Clark GB, Henry JM, McKeever PE: Cerebral pilocytic astrocytoma. Cancer 56:1128,
1985

6. Forsyth PA, Shaw EG, Scheithauer BW, et al: Supratentorial pilocytic astrocytomas. A
clinicopathologic, prognostic, and flow cytometric study of 51 patients. Cancer
72:1335,1993

7. Mamelak AN, Prados MD, Obana WG, et al: Treatment options and prognosis for
multicentric juvenile pilocytic astrocytoma. J Neurosurg 81:24, 1994

8. Palma L, Guidetti B: Cystic pilocytic astrocytomas of the cerebral hemispheres. Surgical
experience with 51 cases and long-term results. J Neurosurg 62:811 - 815, 1985

9. Rollins-NK; Nisen-P; Shapiro-KN: The use of early postoperative MR in detecting
residual juvenile cerebellar pilocytic astrocytoma. AJNR-Am-J-Neuroradiol. 19(1): 151-6,
1998

10. Garcia DM, Fulling KH: Juvenile pilocytic astrocytomas of the cerebrum in adults. A
distinctive neoplasm with favorable prognosis. j Neurosurg 63:382 -386, 1985

11. Luh GY, Roger Bird C: Imaging of brain tumors in the pediatric population.
Neuroimaging clinics of north America, 9, 4:691-716, 1999
12. Ricci PE: Imaging of adult brain tumors. Neuroimaging clinics of north America, 9,
4:651-669, 1999

13. Smirniotopoulos JG:The new WHO classification of brain tumors. Neuroimaging
Clinics of north America, 9, 4:595-613, 1999

14. Gajjar A, Sanford RA, Heideman R, et al: Low-grade astrocytoma: A decade of
experience at St. Jude children's research hospital. J Clin Oncol 15:2792-2799, 1997

15. Epstein F, Wisoff JH: Surgical management of brain stem tumors of childhood and
adolescence. Neurosurgery clinics of North America, 1, 1:111-121, 1990

16. Bigner SH, Mark J, Burger PC, et al: Specific chromosomal abnormalities in malignant
human gliomas. Cancer Res 88:405-411, 1988

17. Finlay JL, Goins SC: Brain tumors in children: Advances in diagnosis. Am j Pediatr
Hematol Oncol 9:246-255, 1987

18. Frankel RH, Bayona W, Koslow M, et al: P53 mutations in human malignant gliomas:
Comparison of loss of heterozygosity with mutation frequency. Cancer Res 52:1427-1433,
1992

19. Fults D, Brockmeyer D, Tullous MW, et al: p53 mutation and loss of heterozygosity on
chromosomes 17 and 10 during human astrocytoma progression. Cancer Res 52:674-679,
1992

20. Jenkins RB, Kimmel DW, Moertel CA, et al: A cytogenetic study of 53 human gliomas.
Cancer Genet Cytogenet 39:253 -279, 1989

21. Kimmel DW, O'Fallon JR, Scheithauer BW, et al: Prognostic value of cytogenetic
analysis in human cerebral astrocytomas. Ann Neurol 31:534-542, 1992

22. McComb RD, Burger PC: Pathologic analysis of primary brain tumors. Neurol Clin
3:711-728,1985

23. Rasheed BK, Bigner SH: Genetic alterations in glioma and medulloblastoma. Cancer
Metastasis Rev 10: 289-299, 1991

24. Lang FF, Miller DC, Koslow M et al: Pathways leading to glioblastoma multiforme: A
molecular analysis of genetic alterations in 65 astrocytic tumors. j Neurosurg 81:427-436,
1994

25. Watanabe K, Tachibana 0, Sato K, et al: Overexpression of the EGF receptor and p53
mutations are mutually exclusive in the evolution of primary and secondary glioblastomas.
Brain Pathol 6:217-224, 1996
26. Ashby LS, Coons SW, Scheck AC, et al: Cytogenetic abnormalities in juvenile pilocytic
astrocytomas (JPA) and patient prognosis. Neurooncology 1:S3, 1999

27. Afra D, Muller W, Solwik F :Supratentorial lober pilocytic astrocytoma: report of 45
operated cases including 9 recurrence. Acta neurochir (Wien) 81:90-93, 1986

28. Burger PC, Fuller GN : Pathology-trends and pitfalls in histological diagnosis,
immunopathology and application of oncogene research. Neurologic clinics, Vol. 2, No. 2,
pp 249-271, 1991

29. Rekate HL, Rakfal SM: Low grade astrocytoma in children. Neurologic clinics, Vol. 2,
No. 2, pp 423-440, 1991

30. Metwally MYM: The pilocytic astrocytoma, a clinico-radiological study with
histopathological correlation. The Egyptian J Neurol Psychiatr Neurosurg Vol 38 (1)279-
289, 2002

Professor Yasser Metwally
Professor of neurology, Ain Shams University, Cairo, Egypt

http://yassermetwally.com

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Topic of the month: Radiological pathology of pilocytic astrocytoma

  • 1. INDEX  INTRODUCTION  RADIOLOGICAL PATHOLOGY OF PILOCYTIC ASTROCYTOMAS INTRODUCTION Astrocytomas are, by far, the largest category of primary neoplasms of the brain. There are two primary patterns of growth seen in astrocytomas: diffuse and circumscribed. The diffusely infiltrating astrocytomas have been known since the early days of brain surgery and neuropathology. The circumscribed group of astrocytomas has only recently received widespread acceptance, although one subtype-pilocytic astrocytoma (PA)-has been well described for decades. Several of the subtypes of astrocytoma more recently added to the WHO classification are characterized by a circumscribed pattern of growth.
  • 2. Pilocytic Astrocytoma PA is the prototype for low-grade (benign) circumscribed astrocytoma. 30 This tumor represents approximately 2% to 6% of all primary brain tumors. In some series, they are described as the most common tumor of the cerebellum in childhood. 13, 14,15 In other series, they are less frequent than medulloblastoma and account for little more than 7% of all neoplasms in patients younger than age 20. Patients with these tumors present primarily during childhood with the peak ages of presentation between 5 and 15 years old. 1,3,5,6,7,8,10 Tumors of the chiasm and other locations of the diencephalon may present at younger ages. Within the diencephalon, PA may present in the orbital portions of the optic nerves, within the hypothalamus, and within the thalamus. Most of the optic nerve gliomas that occur in neurofibromatosis type 1 (von Recklinghausen type) are PA. Most series report an equal incidence in both sexes; others indicate a slight preponderance of female patients in a ratio less than 4:3. 7,8,9 Common anatomical location 1,3,5,6,7,8,10 Cerebral hemisphere (18%) Cerebellum (55%) Brain stem (17%) Hypothalamic, chiasmal (2%) In the Egyptian study of metwally 30 most of the tumors occurred in the deep parietal region (64%) and the cerebellum was involved next in frequency. (see table 1) In all the reviewed literature the cerebellum was the primary site of involvement in patients younger than the age of ten while in older patients pilocytic astrocytomas occur more frequently supratentorially1,3,5,6,7,8,10 and this is consistent with the results of Metwally 30 since patients in his study with supratentorial (parietal) tumors were older, and greater in number, than patients with cerebellar tumors and subsequently the percentage of tumor occurrence, anatomically, was higher in the supratentorial (deep parental) zone, compared with the cerebellar area in this series. All patients in the Egyptian study of metwally 30 were males and this is unlike most of the reviewed studies. 1,3,5,6,7,8,10
  • 3. Figure 1. A pilocytic astrocytoma, notice the peripherally located hypercellular part (mural nodule [arrows]) and the multicystic appearance of the tumors. It is interesting that the clinical picture, in pilocytic astrocytomas patients, is characterized by mild clinical disability and paucity of clinical signs despite the fact that tumors are frequently large enough. This can be explained by the benign nature of this neoplasm that apparently resulted in a very slow rate of growth. These tumors widely, and very slowly, expand neural tissues without neural destruction or interruption of normal function. 11,12,13 The very slow rate of growth of these tumors allows the brain to move functionality from one region to anther and this process of brain remapping, which has been shown to occur at all ages, is partially responsible for the relatively late appearance of symptoms and paucity of clinical signs on presentation in pilocytic astrocytomas patients. 11,12,13 Table 1. Distribution of pilocytic astrocytomas in Egypt 30 Anatomical localization % Mean age Deep frontoparietal 64% 13 years Cerebellar hemispherical 18 6 years Hypothalamus, 3rd ventricular 9 9 years Brain stem, pontine 9 9 years
  • 4. RADIOLOGICAL PATHOLOGY OF THE JUVENILE PILOCYTIC ASTROCYTOMA The capillaries may be abnormal and Grossly, PA is a well-circumscribed yet can be coiled (angiomatous) and unencapsulated mass. The lesion grows primarily by thick-walled. Apparently the blood- expansion rather than the infiltration characteristic of brain barrier is not well formed in most astrocytomas. In many cases, lesions are easily these tumors. The proteinaceous fluidseparated from the adjacent uninvolved cerebellar that accumulates as both microcysts folia. Most PA have a significant grossly visible cystic and macrocysts probably leaks from component. In many cases, the tumor has the the abnormal vessels. Mitosis and classically described cyst with nodule morphology-in necrosis are distinctly uncommon. which neoplasm is confined to a nubbin of tissue Despite this, occasional PA show embedded in the wall of a fluid-filled cavity. In these microscopic hemorrhages or brownish cases, the cyst fluid is surrounded by nonneoplastic staining. compressed or gliotic tissue. Calcification can be seen in 25% of cases. Microscopically, there is a biphasic pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes alternating with looser regions that may have microcysts. One distinctive feature is the presence of eosinophilic curvilinear Rosenthal fibers within the dense regions. The capillaries may be abnormal and can be coiled (angiomatous) and thick-walled. Apparently the blood-brain barrier is not well formed in these tumors. The proteinaceous fluid that accumulates as both microcysts and macrocysts probably leaks from the abnormal vessels. Mitosis and necrosis are distinctly uncommon. Despite this, occasional PA show microscopic hemorrhages or brownish staining. Table 2. The mural nodule is composed of two main parts as follows* 30 Part Appearance Histopathology Part I Dense compact Composed of elongated bipolar hairlike (pilocytic) appearance astrocytes with high nuclear to cytoplasmic ratio and with minimal extracellular fluid. This part is relatively vascular. Part II Loose appearance Relatively acellular and composed mainly of microcysts and enlarged extracellular fluid filled spaces. This part is relatively avascular. * The spatial distribution of each part within the mural nodule will determine the neuroimaging appearance of the mural nodule.
  • 5. Figure 2. Cystic (pilocytic) brain stem glioma. Many astrocytomas, particularly in the cerebellum, hypothalamus, and optic pathways of children, exhibit a typical histologic appearance previously termed polar spongioblastoma and now universally referred to as juvenile pilocytic astrocytoma. These tumors frequently contain both macrocysts (as in cystic cerebellar astrocytoma) or microcysts. Rosenthal fibers, strongly eosinophilic coalescences of neurofibrillary elements, are characteristic of juvenile pilocytic astrocytoma, but may be found in other forms of tumor, particularly as a glial reaction surrounding craniopharyngioma. Endothelial proliferation is common in these tumors and has none of the ominous connotations in this context that it implies in other forms of astrocytomas. Figure 3. Pilocytic astrocytoma with hair-like cells From the pathological point of view pilocytic (hair cells) astrocytomas are composed of two main parts, a fluid-filled large cyst and a projecting mural nodule. The neoplastic cells are confined to the mural nodule and the cyst walls are composed of non-neoplastic compressed or gliotic neural tissues. Pilocytic astrocytomas do not have true capsule, yet they can easily be separated from the surrounding tissues. Calcification can be seen in 25% of cases. 1,3,5,6,7,8,10 These tumors frequently have microcysts and macrocysts. Microscopically, there is a biphasic pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes alternating with looser regions that may have microcysts. One distinctive feature is the presence of eosinophilic curvilinear Rosenthal fibers and strongly eosinophilic coalescences of neurofibrillary elements within the dense regions. 1,3,5,6,7,8,10 The
  • 6. capillaries may be abnormal and can be coiled (angiomatous) and thick-walled. Apparently the blood-brain barrier is not well formed in these tumors. The proteinaceous fluid that accumulates as both microcysts and macrocysts probably leaks from the abnormal vessels. Necrosis, mitotic activity, endothelial proliferation are selectively absent in pilocytic astrocytomas. Pilocytic astrocytomas are very slowly growing tumors, with long premonitory symptoms before clinical presentation, that selectively grow by expansion rather by infiltration of the surrounding neural tissues that is more characteristic of diffuse astrocytomas. 1,3,5,6,7,8,10,11,12,13,14,15 Figure 4. This figure shows a gross specimen of a pilocytic astrocytoma of the posterior fossa. Identify the cerebellar hemispheres, the pons and the fourth ventricle. The fourth ventricle is nearly obliterated due to the large cystic tumor in the midline. Note the white nodule to one side of the cyst. This is the actual tumor. Many pilocytic astrocytomas in the posterior fossa will have an associated cyst and a contrast enhancing "mural" nodule. Pilocytic astrocytomas are one of the most common pediatric brain tumors and most occur in the posterior fossa, but in children with Neurofibromatosis type I, they may occur in the optic tracts. Juvenile pilocytic astrocytomas tend to be well circumscribed and to grow slowly with long periods of premonitory symptoms before presentation. This pattern is especially true of tumors that arise in the cerebellum. Tumors of the anterior third ventricle tend to be well-defined superiorly but diffusely infiltrating the optic mechanisms and hypothalamus inferiorly. The course of these tumors is normally benign but may be unpredictable thereby making treatment decisions extremely difficult. The presence of a juvenile pilocytic astrocytoma that extends into the subarachnoid space is common. Pilocytic astrocytomas differ from the more common diffuse astrocytomas from the pathological, nosological, radiological, genetic and prognostic point of view. 1,3,5,6,7,8,10,11,12,13,14,15 From the pathological point of view diffuse astrocytomas are neoplasms
  • 7. of widely varying potential that are unencapsulated, poorly marginated and diffusely infiltrate into the surrounding brain. These diffuse astrocytomas appear to form a continuum of both biological and histological aggression. They vary from lesions with almost normal cytology (grade II astrocytomas) through intermediate stages (grade III, anaplastic astrocytomas) and up to the most aggressive of all human brain tumors (grade IV astrocytomas or glioblastoma multiforme). 11,12,13 Figure 5. This figure shows a gross specimen of a pilocytic astrocytoma of the posterior fossa. Identify the cerebellar hemispheres, the pons and the fourth ventricle. The fourth ventricle is nearly obliterated due to the large cystic tumor in the midline. Figure 6. Histopathological and gross pathological picture of the pilocytic astrocytoma with the characteristic microcysts and a large mural nodule From the nosological point of view, and according to the WHO classification of brain tumors 13, pilocytic astrocytomas are ranked as grade I benign gliomas while diffuse astrocytomas are ranked as grade II, grade III (anaplastic astrocytomas) and grade IV (glioblastoma multiforme). The following pathological differences are present between diffuse and pilocytic astrocytomas. 11,12,13  Diffuse astrocytomas, unlike pilocytic astrocytomas, have a peculiar tendency to change its grade over time and the condition is age dependant. 11,12,13 A change in the grade of diffuse astrocytoma is more likely to occur in the older age group. In older age group (over the age of 40 years) diffuse low grade astrocytomas (grade II astrocytoma according to WHO) have a bad prognosis because they have a great tendency for anaplastic transformation (to grade III or grade IV astrocytoma according to WHO), 11,12,13 while at a younger age group anaplastic transformation
  • 8. of diffuse low grade astrocytomas (grade II astrocytoma according to WHO) is extremely uncommon, 11,12,13,14,15 also the probability for diffuse low grade astrocytomas to have a highly malignant component (i.e, grade III or IV mixed with grade II) is higher in the older age group. 11,12,13 On the other hand pilocytic astrocytomas (grade I astrocytoma according to WHO) never change its grade over time. 1,3,5,6,7,8,10,11,12,13,14,15  Diffuse astrocytomas (grade II, III or grade IV astrocytomas) grow by infiltration of the nearby neural tissues (commonly in the form of remote neoplastic cells radiating from the mother tumor) and so they are poorly marginated and, practically, complete surgical resection is not possible and some neoplastic cells are almost invariably left behind after surgical resection. On the other hand pilocytic astrocytomas grow by expansion and so they are well circumscribed and subsequently complete surgical resection is possible. 11,12,13  Diffuse astrocytomas, unlike pilocytic astrocytomas, are highly cellular neoplasms with cells that range from normal appearing astrocytes (grade II) to cells with marked pleomorphism and hyperchromatic nuclei ( grade III, and IV). 11,12,13 On the other hand pilocytic astrocytomas are histopathologically composed of scanty elongated cells, Rosenthal fibers and microcysts and this combination constitutes the classic of pilocytic astrocytomas. Cells are only confined to the mural nodule in pilocytic astrocytomas and subsequently the mural nodule is the only neoplastic part of the tumor. 11,12,13  Pilocytic astrocytomas are truly benign gliomas while diffuse astrocytomas are, at best, of low grade malignancy. 1,3,5,6,7,8,10,11,12,13,14,15 Radiologically pilocytic astrocytomas differ from diffuse astrocytomas in the following points 1. Pilocytic astrocytomas are typically "cystic tumors with a mural nodule" and with prominent mass effect, while diffuse astrocytomas with a lower grade (grade II) are typically solid tumors with minimal or no mass effect that appear homogeneously hypodense on CT scan, hyperintense on the MRI T2 images and hypointense on the T1 MRI images. 1,2,3,5,6,7,11,12,13,26 The neuroimaging appearance of diffuse astrocytomas is due to increased cell count. 11,12,13,27 The mural nodule of pilocytic astrocytomas might or might enhance while diffuse low grade astrocytomas usually do not enhance on postcontrast scan. 1,2,3,5,6,7,11,12,13,27,28,29 Diffuse low grade astrocytomas (grade II) diffusely expand the affected part of the brain with poor margin, while pilocytic astrocytomas are well circumscribed rounded or oval tumors. 11,12,13,27,28 2. Although central necrosis in highly malignant glioblastoma multiforme might, morphologically, create the appearance of a cyst with a projecting mural nodule, however this can easily be differentiated from pilocytic astrocytomas by the fact that the walls of the cyst in pilocytic astrocytomas, being composed of non-neoplastic compressed neural tissues, never enhance while the walls of the cyst in glioblastomas with central necrosis invariably enhance because it is composed of viable tumor tissues. 11,12,13 Wall enhancement is characteristic of glioblastomas with central necrosis and when it is observed radiologically should shift the tumor grade from
  • 9. the most benign pilocytic astrocytoma to the most malignant glioblastoma multiforme, see figure 8. 11,12,13 The presence of significant edema and short history before clinical presentation favor the diagnosis of glioblastoma multiforme. In general glioblastoma multiforme occurs at an older age compared with pilocytic astrocytoma. 11,12,13,27,28 The presence of blood products on CT scan or MRI is characteristic of the highly malignant glioblastoma multiforme. Figure 7. Two CT scan studies showing juvenile pilocytic astrocytomas in left cerebral hemisphere, A and the cerebellum, B. Notice the wall calcification in (B). Notice that the mural nodule has a hypodense core and a hyperdense rim in A (pattern I), and is diffusely hyperdense in B (pattern II). The cystic component of the tumors is diffusely hypodense. Figure 8. A,B Precontrast and postcontrast CT scan studies showing parietal pilocytic astrocytoma, notice that only the mural nodule was enhanced on postcontrast scan A, while the cyst wall remained unenhanced. For comparison a postcontrast CT scan study of a case with glioblastoma and with central necrosis is presented (C). Notice that in glioblastoma both the cyst wall and the mural nodule are enhanced.
  • 10. On imaging studies, the presence of a cystic Juvenile pilocytic astrocytomas are usually well defined component in these lesions is suggestive of on both CT and magnetic resonance imaging (MRI). In pilocytic astrocytomas. 30 The lesions may the cerebellum, they arise either within the vermis or cerebellar hemispheres. They tend to have a large single appear on both CT and MR as a classic cyst cyst with a "mural nodule". They can, however, have with nodule mass. Purely solid masses are multiple cysts or be completely solid. Tumors without not common. Complex shapes (e.g., cysts are more frequently the diffuse (nonpilocytic) multiloculated) are frequent and may create astrocytomas. Before contrast enhancement, the a misleading appearance. With contrast appearance of the tumor is similar to cerebellar tissue. infusion, both on CT and on MR, PA almost invariably demonstrate prominent enhancement. The pattern may be a classic cyst with nodule, (only the mural nodule enhances brightly while the cyst wall, being composed of nonneoplastic compressed neural tissues, usually does not enhance beyond the edge of nodule) although variable degrees of wall enhancement also occur. More complex patterns of enhancement may appear to suggest necrosis and a high-grade neoplasm. Although this is a low-grade (WHO grade I) tumor, in certain examples, especially those presenting in the cerebral hemispheres, the prominent surrounding vasogenic edema may create a disturbing appearance. Figure 9. A, Precontrast MRI T1, and MRI T2 (B,C) showing cerebellar pilocytic astrocytoma, Notice that the mural nodule is isointense, relative to the normal cerebellar tissues, on the T1 image hypointense, relative to the cyst, on the T2 MRI images, (B,C). The cyst is hypointense on the T1 image and hyperintense on the T2 images In neuroimaging studies of juvenile pilocytic astrocytomas, the acellular cystic part generally appears as a low signal intensity on T1-weighted sequences, as a high signal intensity on T2-weighted sequences and diffusely hypodense on CT scan studies. The mural
  • 11. nodule, which is the neoplastic part of the tumor, have four neuroimaging patterns of CT density and MRI T1, T2 signal intensities as follows: Table 3. Neuroimaging patterns of the mural nodule 30 (see Fig 16) Pattern Comment Pattern I The mural nodule has a large central core and a peripheral thin rim. The core is hypointense on precontrast T1 MRI studies, hypodense on precontrast CT scan studies and hyperintense on the MRI T2 images. The peripheral rim is hyperintense on the precontrast MRI T1 images, and hyperdense on precontrast CT scan studies (even though not calcified) and hypointense on the MRI T2 studies. Because the peripheral thin rim has CT density/MRI signal intensity different from the central core of the mural nodule and from the tumor cystic cavity, it separates the mural nodule from the cystic cavity on neuroimaging studies making it easier to differentiate between the cystic component and the mural nodule. CT Density measurement reveals no evidence of calcification in all cases with this pattern. In this pattern the peripheral thin rim is hypercellular/vascular while the central core of the mural nodule is relatively acellular/avascular and composed of microcysts. After contrast enhancement only the hypercellular peripheral rim of the mural nodule enhances, while the acellular central core does not enhance. Mural nodule with this pattern is larger in size. See Fig 7A pattern II The nodule appears diffusely hyperdense on precontrast CT scan and diffusely hypointense on MRI T2 images. CT Density measurement reveals no evidence of calcification in all cases with this pattern. In this pattern the mural nodule has a compact, dense appearance and is diffusely hypercellular/vascular and enhances diffusely and brightly after contrast injection. Mural nodule with this pattern is smaller in size. See Fig 7B and Fig 13 pattern III The nodule appears diffusely hypodense on precontrast CT scan, diffusely hypointense on precontrast MRI T1 images, and diffusely hyperintense on MRI T2 images. Differentiation between the mural nodule and the cystic part of the tumor might not be easy. The nodule in pattern III is relatively diffusely acellular and has a looser appearance. Contrast enhancement in this pattern is poor. See Fig 10b Pattern IV The nodule appears irregularly hyperdense on precontrast CT scan and hypointense on MRI T2 images. CT Density measurement reveals evidence of patchy calcification of the mural nodule in all cases with this pattern. See Fig. 14 In the author experience the prevailing radiological pattern of the mural nodule is pattern I. Microscopically, the mural nodule is composed of a biphasic pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes (with high nuclear to cytoplasmic ratio and
  • 12. with minimal extracellular fluid), these astrocytes are arranged peripherally in most tumor nodules, and alternating with looser regions that are rich in fluid filled microcysts, these regions are arranged centrally in most tumor nodules. The hypercellular peripheral rim (with cells that have a high nuclear to cytoplasmic ratio with minimal extracellular fluid) appear hyperintense on the precontrast T1 images, hypointense on the MRI T2 studies and hyperdense on precontrast the CT scan studies, while the relatively acellular fluid filled microcystic core appear hypointense on the precontrast T1 studies, hyperintense on the MRI T2 studies and hypodense on the precontrast CT scan studies. The topographic distribution of the hypercellular part, peripherally, and the microcystic acellular part, centrally, within the mural nodule is responsible for the production of the patten I. Should the hypercellular part predominate the histopathological architecture of the mural nodule, pattern II is the expected result. Should the relatively acellular fluid filled microcystic part predominate the histopathological architecture of the mural nodule, pattern III is the expected result. Should the mural nodule become calcified, pattern IV is the expected result.30 Figure 10. A, CT scan showing pattern I of the mural nodule, B, CT scan showing pattern II of the mural nodule, C, MRI T2 image showing pattern III of the mural nodule, the nodule is not showing because it is isointense to the cystic cavity. D,E MRI T1,T2 showing pattern I of the mural nodule. F, MRI T2 image showing pattern II of the mural nodule, the nodule is hypointense relative to the cystic cavity.
  • 13. Pilocytic tumors are sometime wholly solid (noncystic) and composed of elongated bipolar hairlike (pilocytic) astrocytes (with high nuclear to cytoplasmic ratio and with minimal extracellular fluid). The tumor in this case appears hyperintense on the precontrast T1 images, hypointense to isointense on the MRI T2 studies and hyperdense on precontrast CT scan studies, with dense postcontrast enhancement. In the author experience perilesional edema is common in solid tumors. 30 Figure 11. A solid pilocytic astrocytoma, The tumor is hyperdense on noncontrast CT scan (A), with dense postcontrast enhancement. (B). The tumor contains some cystic spaces and is surrounded by edema. This is probably the initial stage in the natural evolution of the tumor. After the infusion of an intravenous contrast agent, the solid hypercellular components of the mural nodule tend to enhance brightly and to appear as a distinct, well-defined mass. Contrast enhancement is prominent is mural nodule with pattern II tissue, while in mural nodule with pattern I tissues enhancement, though present in the hypercellular/vascular peripheral thin rim, might not be appreciated visually (enhancement can be appreciated if the CT density of the peripheral rim is taken before and after contrast injection). Enhancement is prominent in purely solid tumors. The cyst wall, being composed of nonneoplastic compressed neural tissues, usually does not enhance with contrast material.
  • 14. Figure 12. A,B, Precontrast MRI T1, and MRI T2 (C) showing frontal pilocytic astrocytoma. The cyst is hypointense on the T1 images and hyperintense on the T2 images. The mural nodule has pattern III and cannot be seen as it is isointense to the cystic cavity. In general pilocytic astrocytomas have heterogenous histopathological composition (biphasic pattern of dense areas with elongated bipolar hairlike (pilocytic) astrocytes alternating with looser regions that may have microcysts) and subsequently the tumor might be completely solid and occasionally the tumor might be cystic with a small dense hypercellular peripheral mural nodule (pattern II). Sometimes the mural nodule itself might have a large cystic core with a peripheral hypercellular thin cover (patten I). Although the share taken by the cystic (acellular/avascular) and the solid (hypercellular/vascular) in the histopathological composition of the pilocytic astrocytomas might vary (resulting in heterogenous appearance of the tumors in neuroimaging study both in precontrast and in postcontrast studies), however the neuroimaging appearance of pilocytic astrocytomas simply reflects the natural evolution of the tumors. 30 A pilocytic astrocytoma usually starts as hypercellular solid tumor with elongated bipolar hairlike (pilocytic) astrocytes with high nuclear to cytoplasmic ratio and with minimal extracellular fluid (purely solid tumors). Solid tumors are vascular and their capillaries may be abnormal and can be coiled (angiomatous) and thick-walled. Apparently the blood- brain barrier is not well formed in these tumors and proteinaceous fluid probably leaks from the abnormal vessels and accumulates in the tumors as microcysts, first, and macrocysts later on. With progressive enlargement of the macrocysts (microcysts enlarge and coalesce forming a single large cyst), the viable tumor tissues are progressively compressed into a smaller, dense and hypercellular peripheral mural nodule (pattern II). Progressive leakage of proteinaceous fluid within the core of the mural nodule will result in progressive enlargement of the mural nodule, the core of which will be cystic with a thin
  • 15. outer cover of viable tumor tissues (pattern I). Although the typical appearance of a pilocytic tumor is a large single cyst with a mural nodule, however the spatial distribution of the solid (hypercellular/vascular) and the cystic components within the tumors can vary, also the share taken by the solid the cystic parts in the histopathological make-up of the tumors might vary. These histopathological variations might result in tumors that have quite atypical appearance with irregular cystic and solid parts and with irregular or patchy contrast enhancement. 30 Color plate 1. A pilocytic astrocytoma commonly starts as a solid mas (1), however due to defective blood brain barrier in the newly formed blood vessels proteinaceous fluid probably leaks and accumulates inside the tumor as microcysts, first (2), and macrocysts later on (3). With progressive enlargement of the macrocysts (microcysts enlarge and coalesce forming a single large cyst), the viable tumor tissues are progressively compressed into a smaller, dense and hypercellular peripheral mural nodule (pattern II) against a large cyst (3). Progressive leakage of proteinaceous fluid within the core of the mural nodule will result in progressive enlargement of the mural nodule, the core of which will be cystic with a thin outer cover of viable tumor tissues (pattern I) (4,5,6). (Blue = cystic parts and brown = solid parts of the tumor) If the CT scan discloses that both the wall of the cyst and the solid component of the neoplasm enhance with intravenous injection of contrast material, MRI scan commonly disclose that the tumor is more extensive than the CT scan suggested and the surgical specimens disclose highly malignant gliomas (anaplastic astrocytoma or glioblastoma).
  • 16. In general calcification is commonly present in pilocytic astrocytomas (28% 30), Calcification might be present in the mural nodule (see figure 14) or in the cyst wall (see figure 7). Figure 13. Pilocytic astrocytoma. Axial and sagittal T1 -weighted gadolinium-enhanced MR images show a classic cyst with nodule morphology (with pattern II tissues). Notice that the wall of the cyst does not enhance beyond the edge of nodule. The appearance of cystic astrocytomas of the brain stem is very similar to that of cystic astrocytomas of the cerebellum. The CT scans/MRI disclose only the mural nodule enhanced with contrast. Cystic astrocytomas are often associated with a large cyst that excavates much of the brain stem. These tumors are commonly found in the cerebral peduncle or pons, and both CT scan and MRI give satisfactory imaging. Figure 14. A, Precontrast CT scan showing frontal pilocytic tumor with a calcified mural nodule (pattern IV), and (B) cystic cerebellar pilocytic tumor with a large, calcified mural nodule (pattern IV).
  • 17. After intravenous infusion of contrast material, the tumor will normally enhance brightly and thoroughly. Two patterns seem to exist in the primarily cystic varieties. Most commonly, the mural nodule enhances brightly and the cyst wall does not enhance. In this case, pathologic assessments show that the cyst wall is composed of compressed cerebellar tissue and that the tumor is confined to the mural nodule. In some tumors, the entire wall of the cyst enhances. This apparently means that there has been degeneration within the center of the tumor, and viable tumor completely surrounds the cyst. In this case, the pathological diagnosis is glioblastoma multiforme and the entire cyst wall must be resected. Figure 15. A, postcontrast CT scan study showing a large parietal pilocytic astrocytoma. Neither the mural nodule nor the cyst wall was enhanced. B. postcontrast CT scan study showing a large hypothalamic pilocytic astrocytoma with wall enhancement. Figure 16. The stages of the natural evolution of pilocytic astrocytoma. The initial solid stage with some microcysts is illustrated in (A). The second stage is illustrated in (B) where the tumor is composed of a large cyst with a single densely enhanced non-cystic small mural nodule (pattern II). The third stage is illustrated in (C) The mural nodule has enlarged and is composed or a cystic hypodense central core and a peripheral rim of viable tumor tissue (pattern I). The mural nodule ultimately becomes cystic. From the genetic point of view pilocytic astrocytoma is different from diffuse astrocytomas in the following points  The analyses of the genetic lesions of pilocytic astrocytoma have targeted the TP53 gene on chromosome 17. Investigations have not confirmed a critical role for
  • 18. alterations of this gene in the development of these tumors, however. In one series, the cytogenetic analysis of 14 pilocytic tumor cultures did not identify a specific pattern of chromosomal aberration. (15) Patients with tumors characterized by normal stem line karyotypes had the most favorable outcomes. The presence of clonal structural abnormalities and the presence of markers were associated with a high risk of early recurrence. (15)  On the other hand genetic lesions associated with the development and malignant transformation of diffuse astrocytomas have been well described in the cytogenetic literature. (16, 17, 18, 19) To date, three distinct clinical, histologic, and genetic patterns of glioblastoma multiforme have been characterized. In younger patients, most diffuse astrocytomas are believed to begin as low-grade astrocytoma, with progression to glioblastoma multiforme through a stepwise acquisition of genetic lesions. These secondary glioblastoma multiforme often contain areas of well- differentiated residual tumor. (20, 21) The most frequent chromosomal abnormality identified in diffuse astrocytomas is the abnormal gain of chromosome 7 with an associated loss of one of the sex chromosomes. Additionally, allelic loss or mutation of 17p, resulting in critical alterations of the TP53 gene, has been targeted as an essential step in the early development of glioma. (22, 23) Mutant TP53, identified in at least one third of all astrocytomas, may contribute to the formation of these tumors by inhibiting programmed cell death. glioblastoma multiforme in older patients are usually primary-that is, they occur as glioblastoma multiforme from their inception, without progression from a lower- grade tumor. 16, 17, 18, 19, In this group, the development of glioblastoma multiforme involves a parallel sequence of genetic alterations, including amplifications and deletions, that up-regulate growth factor receptors and drive cell proliferation. 16, 17, 18, 19, 24, 25
  • 19. Figure 17. MRI T1, T2 images showing cystic pontine pilocytic astrocytoma with a large -pattern I- cystic mural nodule  Management of juvenile pilocytic astrocytoma Because pilocytic astrocytomas grow by expansion rather by infiltration of the nearby neural structure (infiltration results in tumor cells being found histologically radiating diffusely from the mother tumor to the surrounding normal neural structures), they remain circumscribed and can be separated from normal neural tissues 11,12,13,27,28,29, thus allowing complete surgical removal without leaving behind any residual tumor cells and it is because of this that postoperative radiotherapy or chemotherapy are not indicated 11,12,13,27,28,29 and probably even contraindicated because Burger and Fuller 28 reported a pilocytic astrocytoma recurring after 28 years as a glioblastoma multiforme in a child who received postoperative radiotherapy and they attributed this rare occurrence to the probable teratogenic effect of radiotherapy. The prognosis in pilocytic astrocytoma is good with a five year survival rate reaching up to 95% to 100% in many studies after complete surgical removal. 27,28,29 Recurrence was attributed in most studies to incomplete surgical removal in technically difficult anatomical sites such as patients with hypothalamic neoplasm.11,12,13,27,28,29,30 Because of the very good prognosis of this neoplasm it is important to be familiar with its clinical and neuroimaging pictures. The presence of a cystic component in these lesions is suggestive. The lesions may appear on both CT and MR as a classic cyst with a nodular mass. Purely solid masses are not common. Complex shapes (e.g., multiloculated) can occur and may create a misleading appearance. 1,3,5,6,7,8,10,11,12,13,14,15 With contrast infusion, both on CT and on MR, the mural nodule might or might not enhance, however the cyst wall
  • 20. does not enhance. Histopathological confirmation is invariably needed for the ultimate diagnosis of this neoplasm. Although the word astrocytoma is typed in the name of this neoplasm, however it should not be equated with the more common diffuse astrocytoma. "Overgrading" occurs when the generic name "astrocytoma" is applied to pilocytic astrocytomas, so it is very important to distinguish between diffuse astrocytoma and pilocytic astrocytoma as there is a real chance that pilocytic astrocytomas are cured by surgery alone once the neoplasms are completely removed. 30 REFERENCES 1. Cottingham SL, Boesel CP, Yates AJ: Pilocytic astrocytoma in infants: A distinctive histologic pattern. J Neuropathol Exp Neurol 55:654, 1996 2. Ashby LS, Coons SW, Scheck AC, et al: Cytogenetic abnormalities in juvenile pilocytic astrocytomas (JPA) and patient prognosis. Neurooncology 1:S3, 1999 3. Lee YY, van Tassel P, Bruner JM, et al: Juvenile pilocytic astrocytomas: CT and MR characteristics. AJNR Am j Neuroradiol 10:363-370, 1989 4. Brown MT, Friedman HS, Oakes Wj, et al: Chemotherapy for pilocytic astrocytomas. Cancer 71:3165, 1993 5. Clark GB, Henry JM, McKeever PE: Cerebral pilocytic astrocytoma. Cancer 56:1128, 1985 6. Forsyth PA, Shaw EG, Scheithauer BW, et al: Supratentorial pilocytic astrocytomas. A clinicopathologic, prognostic, and flow cytometric study of 51 patients. Cancer 72:1335,1993 7. Mamelak AN, Prados MD, Obana WG, et al: Treatment options and prognosis for multicentric juvenile pilocytic astrocytoma. J Neurosurg 81:24, 1994 8. Palma L, Guidetti B: Cystic pilocytic astrocytomas of the cerebral hemispheres. Surgical experience with 51 cases and long-term results. J Neurosurg 62:811 - 815, 1985 9. Rollins-NK; Nisen-P; Shapiro-KN: The use of early postoperative MR in detecting residual juvenile cerebellar pilocytic astrocytoma. AJNR-Am-J-Neuroradiol. 19(1): 151-6, 1998 10. Garcia DM, Fulling KH: Juvenile pilocytic astrocytomas of the cerebrum in adults. A distinctive neoplasm with favorable prognosis. j Neurosurg 63:382 -386, 1985 11. Luh GY, Roger Bird C: Imaging of brain tumors in the pediatric population. Neuroimaging clinics of north America, 9, 4:691-716, 1999
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  • 22. 26. Ashby LS, Coons SW, Scheck AC, et al: Cytogenetic abnormalities in juvenile pilocytic astrocytomas (JPA) and patient prognosis. Neurooncology 1:S3, 1999 27. Afra D, Muller W, Solwik F :Supratentorial lober pilocytic astrocytoma: report of 45 operated cases including 9 recurrence. Acta neurochir (Wien) 81:90-93, 1986 28. Burger PC, Fuller GN : Pathology-trends and pitfalls in histological diagnosis, immunopathology and application of oncogene research. Neurologic clinics, Vol. 2, No. 2, pp 249-271, 1991 29. Rekate HL, Rakfal SM: Low grade astrocytoma in children. Neurologic clinics, Vol. 2, No. 2, pp 423-440, 1991 30. Metwally MYM: The pilocytic astrocytoma, a clinico-radiological study with histopathological correlation. The Egyptian J Neurol Psychiatr Neurosurg Vol 38 (1)279- 289, 2002 Professor Yasser Metwally Professor of neurology, Ain Shams University, Cairo, Egypt http://yassermetwally.com