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International J. of Healthcare and Biomedical Research, Volume: 03, Issue: 02, January 2015, Pages 46-49
46
www.ijhbr.com ISSN: 2319-7072
Case Report
Primary malignant Giant cell tumour of femur
Sanjay D. Mulay 1, Smita S. Mulay 2, Dinesh R. Kulkarni 3
1Professor, Dept. of orthopedics, Rural Medical College, Loni, Maharashtra
2Professor and Head, Dept. of Pathology, M.G. M’s Medical College, Aurangabad Maharashtra
3Professor, Dept. of Pathology, Bidar Institute of Medical Sciences, Bidar Karnataka
Corresponding author: Dr. Sanjay D. Mulay
Abstract:
Primary malignant giant cell tumors are distinctively separate from benign giant cell tumors which metastasize. It
has a high mortality rate. Radiologically these tumors show total destruction of bone with soft tissue infiltration.
With the breakdown of cortex, articular cartilage with infiltration to surrounding tissue or joint space indicates giant
cell tumor is aggressive or malignant. The incidence is difficult to access as there are scattered reports without a
large series published.
Key words : Primary Malignant Giant Cell Tumour, Femur
Introduction:
Primary malignant giant cell tumors are distinctively
separate from benign giant cell tumours which
metastasize (1) and secondary malignant giant cell
tumors occurring in response to radiotherapy and
repeated curettage. (2) It has a high mortality rate.
Usually it affects lower end of femur and upper end
of tibia. (3) Primary malignant giant cell tumors show
histological evidence of conventional giant cell tumor
along with areas of sarcomatous stroma. This should
be correlated with radiological appearance. The
incidence is difficult to access as there are scattered
reports without a large series published. (4)
Case History:
A 21 year male presented with progressive swelling
of at the lower end of left thigh. There was a history
of trauma 8 weeks back, which appears tobe
coincidental. He narrated the history of manipulation
by a local quack, after which the swelling started
increasing at a faster pace. At the time of
presentation, there was antalgic limp with rise in
local temperature, skin was otherwise normal. Total
duration of illness was eight weeks.
Conventional x-ray of left knee joint revealed an
eccentric osteolytic lesion involving lower end of
femur with predominance in lateral condyle with
pathological fracture. Marrow involvement extending
upto 6 cm form the condyles and soft tissue
involvement was also seen (Fig. 1). Computerized
Tomography (CT) Scan revealed osteolysis with
bone destruction upto 12 cm and soft tissue
involvement on the posterolateral aspect of lateral
condyle (Fig. 2). With this presentation and
radiological features the provisional clinical
diagnosis was? Parosteal osteosarcoma ?? Giant cell
tumor.
Fine needle aspiration cytology (FNAC) from tumor
mass yieled cellular material consisting of plenty of
International J. of Healthcare and Biomedical Research, Volume: 03, Issue: 02, January 2015, Pages 46-49
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www.ijhbr.com ISSN: 2319-7072
spindle shaped cells containing pleomorphic
hyperchromatic nuclei. Plenty of mitotic figures and
tumor giant cells were seen in hemorrhagic
background. It was reported as pleomorphic sarcoma
(Fig. 3). With preoperative investigations being
within normal limits, above knee amputation was
done and the specimen was sent for histopathological
examination.
Morphology:
On gross received a specimen of above knee
amputation of left leg. Portion below the knee
appearance normal. The area above knee joint around
it showed a swelling of 12x12 cm with normal skin
above it. Cut surface had a variegated appearance
with infiltration into muscles. Thorough dissection
upto bone showed total destruction of lower end of
femur along with fleshy grayish soft mass arising
from lower end of femur, destroying periosteum and
infiltrating into surrounding skeletal muscles and
subcutaneous plane. Bone at the site of mass was soft
and friable, could be cut with ease. Proximal end of
amputed femur was unremarkable (Fig. 4).
Microscopic Features
Multiple serial sections taken after extensive
sampling from various areas showed tumor mass
consisting of highly cellular areas consisting of sheets
and storiform arrangement of spindle shaped cells
containing bizarre hyperchromatic nuclei with
prominent nucleoli (Fig. 5). Plenty of mitotic figures
were seen (more than 10 per ten hpf) with tumor
giant cells. There was abundance of osteoclastic giant
cells (Fig. 6). Large areas of haemorrhages and
necrosis were seen. No osteoid could be detected. As
there was no history of previous biopsy, surgery or
radiation and absence of osteoid, the diagnosis of
primary malignant giant cell tumor was conveyed.
Discussion:
Radiologically these tumors show total destruction of
bone with soft tissue infiltration.(5) A differential
diagnosis of malignant giant cell tumor should
include radiologically lytic sarcomas like osteogenic
sarcoma, fibrosarcoma and malignant fibrous
histiocytoma. However, expansile eccentric
osteolytic lesions involving epiphyseal region should
point towards giant cell tumor. (6) With the
breakdown of cortex, articular cartilage with
infiltration to surrounding tissue or joint space
indicates giant cell tumor is aggressive or malignant.
C.T. and M.R.I. scans are helpful to study nature,
vascularity, necrosis, extent and infiltration.(7) More
cases of primary malignant giant cell tumor with
proper treatment and follow up are essential to
project definite trends on early diagnosis, treatment
and to improve prognosis which is gloomy at present.
(8)
Conclusion:
Usually the Giant Cell tumor has been reported to be
the benign or the locally malignant condition.
Secondary malignancy is reported after inadequate
curettage. But this lesion was very aggressive and the
clinical & the radiological features were suggestive
of osteogenic sarcoma. It was only on histopathology
that revealed that lesion was Primary malignant giant
cell tumor of femur.
Fig. 1- X-ray left
knee showing
osteolytic lesion in
lower end of femur
at lateral condyle.
International J. of Healthcare and Biomedical Research, Volume: 03, Issue: 02, January 2015, Pages 46-49
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www.ijhbr.com ISSN: 2319-7072
Fig. 2 – C.T. scan showing destroyed lateral condyle
of femur.
Fig. 3– FNAC from tumor mass showing
pleomorphic spindle cells with hyperchromatic nuclei
(PAP 10x x 10x)
Fig. 4 – Above knee amputed left leg with variegated
appearance on cut surface.
Fig. 5 – Shows fibro-sarcomatous pattern of tumor
cells (H & E 10x X 10x)
Fig. 6– Shows sarcomatous pattern with osteoclastic
giant cells (H & E 10x X 10x)
Fig 4. Knee amputed leg
International J. of Healthcare and Biomedical Research, Volume: 03, Issue: 02, January 2015, Pages 46-49
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www.ijhbr.com ISSN: 2319-7072
References:
1. Siebenrock KA, Unni KK, Rock MG. Giant cell tumor of bone metastasing to lungs.
J Bone Joint Surg 1988 : 80 B ; 43-47. Wold LE, Swee RG : Giant cell tumor of small bones of hands and
feet. Semin Diagn Pathol 1994:1;173-84.
2. Rock M, Capanne R. Treatment of giant cell tumor. In : Advances in operative orthopaedics Ed. Stauffer
RN, Vol. 1, Mosby year book Inc, St. Louis. 1986 ; 367-390
3. Campannaci M. Giant cell tumor. In : Bone and soft tissue tumour Ed. Gaggi A. Springer-Valas, Bolonga
1990 ; 117-153.
4. Meis KM, Dorfman HD, Natanson SD, Haggar AM, Wu KK. Primary malignant giant cell tumor of bone.
Mod. Pathol 1989 : 2 ; 541-546.
5. Shutton D. Tumor and tumor like conditions of bone. In. Text book of radiology and medical imaging,
Chapter 6, Ed. Shutton, 4th edn, Churchill Livingstone, London 1987 : 170-196.
6. Sanerkin NG. Malignancy, aggressiveness and recurrence in giant cell tumor of bone. Cancer 1980 : 46 ;
1641-1649.
7. Hagga JR, Lanzeeri CF. Musculoskeletal tumors. In. Computed tomography and magnetic resonance
imaging of whole body. Ed. Sartoris DJ. Chapter 40, 3rd edn, Mosby year book Inc, New York 1994 ; 1413-
1453.
8. Mondal Asitava, Kundu Ramendranath, Chatterjee Jamunesh. Primary malignant giant cell tumor of bone.
Indian J Pathol Microbiol 2000 : 43(4) ; 403-407
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