3. Hemangiomas
Hemangiomas are the most common tumors of
the head and neck in infancy and childhood,
comprising approximately 7% of all benign soft
tissue tumors .
4. Development
The hemangioma is a true vascular tumor that
results from a overgrowth of normal vascular tissue
.
It exhibits relatively rapid early growth until
approximately 6 to 8 months of age (proliferative
phase), followed by regression by 5 to 9 years of
age (involutory phase).
It grows by “endothelial proliferation”. During the
rapid growth phase, an increased number of
mast cells is seen within the endothelial wall.
5.
The majority of the hemangiomas in infants are
noted by the parent within the first month of life.
Hemangiomas are initially noticed as an
erythematous, macular patch, which progresses
through a rapid proliferative phase whereby it
changes its color and grows faster than the
commensurate growth of the child.
By the time the patient is 12 months of age most
hemangiomas have shown signs of involution. The
process of involution is normally slow and will not be
completed until the age of 5 to 9 years.
6. Clinical presentation
Hemangiomas are found in the superficial tissue,
the deep tissue, or both and may affect organ
systems such as the liver, lung, spleen, and
gastrointestinal tract.
7.
Deep hemangiomas involve muscle or visceral
organs and, are more difficult to diagnose.
Therefore, further diagnostic studies are required.
Intra-osseous hemangiomas are extremely rare.
However the soft tissue lesion may deform the
underlying skeleton.
The predilection for females is approximately a 3
:1 ratio.
8. Clinical presentation
On examination, the
superficial hemangioma
usually consists of a raised,
reddish to purple tumor with a
distinct margin.
In contrast, deep
subcutaneous hemangiomas
often have a deep bluish hue
with normal overlying skin,
making diagnosis more
difficult.
Both the lesions are firm to
palpation and do not pulsate
or exhibit any thrills or bruits.
9. Investigations
Computed tomography (C. T .Scan) and
Magnetic resonance imaging ( M. R. I) imaging
techniques are used as diagnostic aids to
document the extent of the deep hemangiomas.
Arteriography is rarely indicated for the diagnosis
of a hemangiomas.
10. A proliferation of vascular channels filled with
red blood cells, shown here beneath skin.
11. Management
Observation and parental support are the initial
approaches in the management of maxillofacial
hemangiomas.
If functional compromise such as visual change,
airway or masticatory compromise, bleeding,
ulceration, or infection occurs intervention is
necessary.
12.
This may initially involve cortico-steroids for rapidly
proliferating lesions or therapy with interferon alfa2a.
Surgery is generally reserved for small lesions and
as a secondary procedure after initial therapy
and involution.
Treatment modalities include routine excision,
injection of sclerosing agents, cryotherapy, and
ablation using an argon laser.
13. Lymphangiomas
Lymphangiomas are malformations of the
lymphatic system, which is the network of vessels
responsible for returning to the venous system
excess fluid from tissues.
These malformations can occur at any age and
may involve any part of the body, but 90% occur
in children less than 2 years of age and involve
the head and neck.
14. -Capillary lymphangiomas
Capillary lymphangiomas are composed of small, capillary-sized lymphatic
vessels and are characteristically located in the epidermis.
-Cavernous lymphangiomas
Composed of dilated lymphatic channels, cavernous lymphangiomas
characteristically invade surrounding tissues.
-Cystic hygromasCystic hygromas are large, macrocystic lymphangiomas
filled with straw-colored, protein-rich fluid.
-Hemangiolymphangioma
hemangiolymphangiomas are lymphangiomas with a vascular component.
-Lymphangiomas may also be classified into microcystic, macrocystic, and
mixed subtypes, according to the size of their cysts.
Microcystic lymphangiomas
Microcystic lymphangiomas are composed of cysts, each of which
measures less than 2 cm3 in volume.
Macrocystic lymphangiomasMacrocystic lymphangiomas contain cysts
measuring more than 2 cm3 in volume.Mixed
lymphangiomasLymphangiomas of the mixed type contain both microcystic
and macrocystic components.
15.
16. Glomus tumor
A glomus tumor (also known as a "solitary glomus
tumor,"solid glomus tumor or glomangioma) is a rare
benign neoplasm arising from the glomus body and
mainly found under the nail, on the fingertip or in the
foot.
They account for less than 2% of all soft tissue
tumors. Glomus tumors were first described by Hoyer
in 1877, while the first complete clinical description
was given by Masson in 1924.
Histologically, glomus tumors are made up of an
afferent arteriole, anastomotic vessel, and collecting
venule. These lesions should not be confused with
paragangliomas, which were formerly also called
glomus tumors in now-antiquated clinical usage.
17.
18. Vascular Malformations
Vascular malformations are present at birth and
unlike hemangiomas, do not go through a a
“rapid proliferative phase” and they do not
“involute”.
They grow commensurately with the patient.
19. Vascular Malformations Types
Vascular malformations may be capillary, venous,
arterial, or combinations of these.
Approximately 31% of these malformations are
found in the head and neck region.
20.
Vascular Malformations are divided into two
categories: Low-flow and High-flow lesions.
Capillary, venous, and lymphatic malformations
exhibit “low flow lesions”.
Arterial and arterio-venous malformations exhibit
“high flow” and are capable of severe
hemorrhage with significant morbidity.
21.
Vascular malformations are thought to “result
when there is interruption at a particular stage of
development of a vessel”.
The type of vascular malformation that results
depends on the stage at which normal
morphogenesis is interrupted.
22.
Thus, vascular malformations are sub-classified by
tissue type into capillary, venous, arterial,
lymphatic, and combinations thereof, with the
type of malformation that develops depending
on the type of tissue affected during abnormal
development.
23.
This results in recruitment of “collateral vessels”
and dilatation of involved vessels.
Unlike the hemangioma, the vascular
malformation exhibits a steady increase in growth,
without signs of involution.
24. Clinical presentation
In many cases, the diagnosis of a vascular
malformation can be made from the patient’s
history.
Although present at birth, these lesions are often
not identified immediately, but only later on when
the lesion enlarges enough to be clinically
identifiable. This is particularly true for intra-bony
lesions.
25.
Venous malformations are bluish, soft
and easily compressible, and
auscultation reveals no bruits.
These malformations can vary from
superficial, localized, mucosal
spongy ectasis to complex invasive
lesions that permeate tissue planes
and alter the regional anatomy.
26.
Any maneuver that increases venous pressure
(e.g.Valsalva maneuver or supine positioning) can
temporarily enlarge a venous malformation.
The clinical absence of “pulsations or a thrill”
generally indicates a low flow Venous vascular
malformation.
Phleboliths that may be noted on radiographic
examination are found only in low flow lesions.
27. Bacillary angiomatosis
is a form of angiomatosis associated with
bacteria of the Bartonella genus
BA is characterised by the proliferation of blood
vessels, resulting in them forming tumour-like
masses in the skin and other organs
28.
29. Kaposi Sarcoma
Kaposi's sarcoma is a tumor caused by human
herpesvirus 8 (HHV8, also known as Kaposi's
sarcoma-associated herpesvirus, KSHV).
It became more widely known as one of the AIDSdefining illnesses in the 1980s.
The viral cause for this cancer was discovered in
1994.
Although KS is now well-established to be caused
by a viral infection, there is widespread lack of
awareness of this even among persons at risk for
KSHV/HHV-8 infection.
31. Hemangioendothelioma
Hemangioendothelioma is used to describe a group of vascular
neoplasms that may be considered benign as well as malignant,
depending on the specific group member's activity.
Epithelioid sarcoma-like hemangioendothelioma
Spindle-cell hemangioendothelioma
Kaposiform hemangioendothelioma
Endovascular papillary angioendothelioma
Infantile hemangioendothelioma
33. Angiosarcoma
Angiosarcoma is a malignant neoplasm of
endothelial-type cells that line vessel walls. This may
be in reference to blood (hemangiosarcoma) or
lymphatic vessels (lymphangiosarcoma).
34. The images show abundant
small blood vessels lined by
large atypical endothelial cells
35. Hemangiopericytoma
A hemangeopericytoma (HPC) is a type of soft
tissue sarcoma that originates in the pericytes in
the walls of capillaries. When inside the nervous
system, although not strictly a meningioma tumor,
it is a meningeal tumor with a special aggressive
behavior. It was first characterized in 1942
36. Tumor cells can be fibroblastic, myxoid, or
pericytic. These tumors, in contrast to
meningiomas, do not stain with epithelial
membrane antigen.
