Giant cell tumor

1. Dr. Brajesh Nandan
Consultant orthopaedic oncology
Sir Ganga Ram Hospital- New Delhi

2.  GCT is a locally aggressive neoplasm .
 GCT represents approximately 5% (94) to 8.6%
(82) of all primary bone tumors and about 22.7% of
benign bone tumors .

3.  In cytogenetic studies, telomeric associations (end-
to-end fusions of apparently intact chromosomes)
involving chromosomes 11p, 13p, 14p, 15p, 19q,
20q, and 21p have been identified as the most
commonly occurring chromosomal aberration .

4.  GCT occurs almost exclusively after skeletal
maturity,
 between the ages of 20 and 40 years, with a
female preponderance (2:1).
 At least 60% of cases in long bones and almost all
extend to the articular end .
 . Those unusual cases with presentation in the
metaphysis are seen in skeletally immature patients

5.  Most common sites are the distal femur, the proximal tibia,
the distal radius, the proximal humerus, and sacrum.
 Rarely, bones of hands and feet, the vertebral bodies, the
ribs the scapula , the ischium , and the skull .
 Very uncommonly, GCT may be multifocal with a reported
frequency of 0.04% to 1% . The majority of multifocal GCTs
are associated with Paget disease of bone , and most have a
predilection for the skull, face , and ilium .

6.  The symptoms include pain of increasing intensity,
with local swelling and tenderness of the affected
area. Limitation of motion of the adjacent joint is
also present in many patients .
 Only in exceptional cases is a pathologic fracture
the first sign of a GCT.
 Recurrences after surgical curettage and bone
grafting are common [reported recurrence rates
range from 12% to 50%

7.  Purely osteolytic lesion with a geographic type of
bone destruction.
 The lesion ; radiolucent, frequently expansive,
eccentrically located, without marginal sclerosis but
usually with well-defined borders
 Internal trabeculations or pseudotrabeculations are
occasionally present , most likely representing a
nonuniform growth of the tumor . There is usually
no periosteal reaction

8. .
 According to some investigators , nonaggressive
tumors exhibit a prominent trabeculation and no
cortical expansion or soft tissue mass. Conversely,
aggressive tumors exhibit a lack of trabeculation,
and expansion or destruction of the cortex and soft
tissue mass are present

10. Giant cell tumor. A: Anteroposterior radiograph of the right knee of a 30-year-old
woman shows an osteolytic lesion eccentrically located in the proximal tibia and
extending into the articular end of the bone. B: Radiolucent lesion in a 27-year-old
woman affects almost the entire proximal end of the humerus. Note a pathologic
fracture at the distal extent of the tumor

11. Giant cell tumor. Anteroposterior (A) and lateral (B) radiographs of the right knee of a 32-
year-old man show a purely osteolytic lesion in the distal end of the femur. Note its
eccentric location, the absence of reactive sclerosis, and the extension of the tumor into the
articular end of the bone. C: In another patient, an eccentric osteolytic lesion extends into
the articular end of the distal radius.

12. . A: Anteroposterior radiograph of the right hip of a 27-year-old woman shows a
radiolucent lesion with internal trabeculations in the femoral head. B: Anteroposterior
radiograph of the left wrist of a 36-year-old woman shows a trabeculated lesion in the
distal radius

13. Giant cell tumor. Dorsovolar radiograph of the left wrist of a 56-year-old woman shows
an osteolytic lesion in the distal radius that has destroyed the cortex and that extends
into the soft tissues.

14.  When a soft tissue mass is present, (CT) or m
(MRI) examination may be particularly helpful.
 CT may outline the tumor extent and better
delineate areas of cortical destruction.
 On MRI, GCT exhibits a low to intermediate signal
intensity on T1-weighted images and a high signal
on T2-weighted sequences

15. (CT). A: Anteroposterior radiograph of the left knee of a 33-year-old woman shows an
osteolytic lesion in the medial femoral condyle (arrows). There is no definite evidence of
cortical break-through. B: Axial CT section through the tumor demonstrates destruction
of the cortex and the presence of a soft tissue mass

16.  Radionuclide bone scan rarely provides additional
information
 However, scintigraphy may help in detection of
multiple foci if a multicentric GCT is clinically
suspected.

17. : (MRI). A: Dorsovolar radiograph of the
right wrist of a 36-year-old woman shows
an osteolytic lesion in the distal radius.
B: Coronal T1-weighted (SE, TR 500, TE
20) MR image shows the tumor to be of
low signal intensity. C: On coronal T2-
weighted (SE, TR 2000, TE 80) MRI the
lesion becomes bright, displaying low-
signal septations.

19. (MRI). Coronal (A) and sagittal (B) T1-
weighted MR images show a low-signal-
intensit Axial T1-weighted fat-suppressed
MRI obtained after intravenous
administration of gadolinium shows
significant enhancement of the lesion

20.  Although 5% to 10% of GCTs undergo malignant
degeneration , malignant GCT is unaccompanied by
additional or specific radiologic characteristics and therefore
cannot be diagnosed radiographically.
 The radiographic presentation of GCT complicating Paget
disease is usually that of an expansive lytic lesion, frequently
accompanied by a soft tissue mass.

21.  A dual population of fibrocytic or monocytic mononuclear
stromal cells and of giant cells that are usually uniformly
distributed throughout the tumor .
 Morphologically, the giant cells somewhat resemble
osteoclasts and, like all resorptive giant cells, exhibit marked
acid phosphatase activity . However, they are much larger
than normal osteoclasts and are not apposed to bone
surfaces and therefore do not possess a ruffled border

22.  In general, they exhibit large nuclei with little chromatin and
very few inconspicuous nucleoli . The number of nuclei may
vary widely but is usually 20 or more.
 Giant cells never exhibit mitotic activity. Areas of
hemorrhage, and occasional groups of foam cells and of
hemosiderin-laden macrophages, are frequently present

23.  Histologic classification of GCT into three grades,
corresponding to the number and size of giant cells and the
degree of pleomorphism of the mononuclear cells (grade III
being frankly malignant histologically), has been attempted
in the past .

24.  grade I tumor is characterized by a dense layer of huge
giant cells with up to 100 or more nuclei, almost covering the
tumor tissue proper of mononuclear cells.
 Grade II tumor exhibits a considerable reduction in the size
and number of giant cells, and the mononuclear cells may be
pleomorphic to some degree.
 In grade III tumors, the giant cells are further reduced in
number and there is an increase in pleomorphic mononuclear
cells

25.  Although GCT is usually benign, malignant tumors have been
reported . one must be very cautious in making the diagnosis
of malignant GCT.
 Several reported cases of “primary” malignant GCT later
proved to be sarcomas rich in multinucleated giant cells of
the osteoclast type, such as telangiectatic osteosarcomas,
giant cell–rich osteosarcomas, fibrosarcomas, or malignant
fibrohistiocytomas

26.  Dahlin and Unni believe that primary malignant
GCT is extremely unusual and that most of these
cases actually represent another type of
malignancy arising within GCT .
 Nevertheless, malignant transformation may occur
spontaneously, after surgery , or after radiation
therapy

27.  McGrath divided malignant GCT into three types:
 (a) primary, in which the tumor was malignant from the
onset (de novo);
 (b) evolutionary, in which there was malignant
transformation of originally benign GCT spontaneously or
after multiple surgical resections for recurrent lesions, or
after a long latency period; and
 (c) secondary, developing as a result of malignant
transformation of an initially benign tumor after radiation
therapy

28.  Under the heading “Malignancy in giant cell tumor” the
World Health Organization (WHO) only discriminates
between primary malignancy, i.e., sarcomas arising in a
GCT, or secondary malignancy, i.e., sarcomas arising at
the site of a previously documented GCT .
 The diagnosis of malignant GCT requires either a frankly
sarcomatous change in the lesion or a sarcoma arising in
the previous site of a treated GCT

29.  Giant cell tumors of tendon sheaths are the
second most common solid cellular tumors of
the hand.
 Age distribution is 8 to 80 years.
 occur in the hand more frequently than in
any other part.
 occur more often on the palmar side
 Multiple xanthomas may be associated with
hypercholesterolemia.

30.  Grow slowly and can remain thesame size for many
years. Pain and tenderness are rare. If occur at a
joint, often the proximal interphalangeal joint.
 rarely erode bone. Grossly, the appear as a yellow or
tan lobulated mass.
 Histological sections reveal spindle cells, fibrous
tissue, cholesterol-laden histiocytes, multinucleated
giant cells, and hemosiderin.
 Excision often is difficult because the tumors may
wind in and around the flexor tendons and their
sheaths, the digital nerves, and even the extensor
tendons and may involve three fourths of the
circumference of involved digits

31.  The lesions are benign, but 27% recur even
after meticulous excision of the friable
fragments.
 Risk factors for recurrence include adjacent
degenerative joint disease, location at the
distal interphalangeal joint of the fingers or
interphalangeal joint of the thumb, and
radiographic evidence of osseous pressure
erosion

32.  ABC ;fluid-fluid level is demonstrated either on CT or MRI
examination, this feature is more consistent with ABC
 However, it should be noted that ABC may sometimes
coexist with other lesions, among them the GCT. The so-
called solid ABC, also referred to as a giant cell reparative
granuloma (GCRG) when affecting the articular end of
bone, may have the same radiologic characteristics as a
conventional GCT

33.  Benign fibrous histiocytoma, because of its frequent
location at the end of a long bone, may appear identical to
a GCT. However, some investigators consider benign
fibrous histiocytoma to be an end stage of a “burned-out”
GCT
 Brown tumor of hyperparathyroidism ; usually accompanied
by other skeletal manifestations of hyperparathyroidism,
such as osteopenia, cortical or subperiosteal resorption,
resorptive changes at the distal phalangeal tufts, or loss of
the lamina dura of the teeth .

34.  Occasionally, an unusually large intraosseous ganglion
may be mistaken for GCT, although the former lesion
invariably exhibits a sclerotic border .
 Multicentric GCT should be differentiated from osteolytic
metastases, myeloma, brown tumor of
hyperparathyroidism, and multicentric GCRG

35. Intraosseous ganglion. Large lytic eccentric lesion extending into the articular end of
the distal tibia of a 40-year-old man was originally thought to represent a giant cell
tumor. The biopsy revealed an intraosseous ganglion. Note a distinct sclerotic border,
rarely present in giant cell tumors.

36. Metastasis. A, B: Osteolytic metastasis from a renal cell
carcinoma into the articular end of the tibia in this 42-year-old
woman has an appearance of a giant cell tumor.

37.  Extended curettage.
 EC and bone grafting
 EC and bone cement
 Excision and whole bone graft/ prosthesis.

38.  25 year male.
 Painful progressive
fusiform swelling x
8 m
 Restriction of
movement x 3m
 No other significant
local or systemic
complaints.

39.  No local temp. rise.
 Diffuse tenderness.
 Swelling- hard, immobile.
 Terminal ROM restricted and painful.

40.  X-ray
 MRI
 CT scan

43. Plan of management ?

44.  FNAC
 Core biopsy
 Open biopsy
 Site of biopsy.

53. ????

54. What do we lean from
this case.