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Year : 2012  |  Volume : 55  |  Issue : 4  |  Page : 600-602
Diaphyseal giant cell-rich osteosarcoma: Unusual histological variant in an unusual site


Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

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Date of Web Publication4-Mar-2013
 

How to cite this article:
Kinra P, Valdamani S, Singh V, Dutta V. Diaphyseal giant cell-rich osteosarcoma: Unusual histological variant in an unusual site. Indian J Pathol Microbiol 2012;55:600-2

How to cite this URL:
Kinra P, Valdamani S, Singh V, Dutta V. Diaphyseal giant cell-rich osteosarcoma: Unusual histological variant in an unusual site. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Oct 21];55:600-2. Available from: https://www.ijpmonline.org/text.asp?2012/55/4/600/107848


Sir,

Purely diaphyseal (10%) or epiphyseal (<1%) location of osteogenic sarcoma is rare, but occasionally can be encountered as in the case discussed. [1] Giant cell-rich osteosarcoma, first described by Bathurst et al., [2] is a rare variant of osteosarcoma, accounting for 1-3% of conventional osteosarcomas. We present a rare case of diaphyseal osteogenic sarcoma in a young male, which on trucut biopsy showed giant cell-rich morphology.

A 21-year-old male presented with 3 months history of gradually progressive painful swelling over shaft of the left thigh. X-ray showed a diaphyseal growth, with medullary sclerosis and sun burst appearance suggestive of osteosarcoma [Figure 1]a. Magnetic resonance imaging (MRI) of the thigh showed a hypointense (sclerosed lesion) epicenter in the left distal femoral diaphysis with no obvious cortical breech. Tumor measured 2.8 × 3.7 × 14.5 cm in size. The surrounding muscles were displaced with complete obliteration of myofascial plane, suggestive of primary malignant bone tumor arising from the diaphysis, possibly diaphyseal osteosarcoma [Figure 1]b. Bone scan showed increased tracer uptake in the lower half of the left femoral shaft, suggesting a solitary lesion with no evidence of any distant skeletal foci or metastasis and consistent with primary bone tumor. A preoperative trucut biopsy from the lesion showed fragmented tissue bits composed of numerous scattered giant cells containing large pleomorphic nuclei with irregular nuclear membrane and few showing prominent nucleoli. Malignant osteoid was seen being layered by bizarre atypical cells with scanty foamy cytoplasm and atypical nuclear morphology. Few foci of necrosis were seen along with atypical mitosis. Based on these findings, diagnosis of giant cell-rich osteosarcoma was offered [Figure 1]c and d. The patient was given 3 cycles of neoadjuvant chemotherapy using ifosfamide, cisplatin, and adriamycin. Wide local resection and reconstruction was then carried out. Resected specimen showed a diaphyseal growth measuring 6 × 4 × 5 cm in size, obliterating the entire medullary cavity with erosion of the overlying cortical bone and involving the surrounding soft tissue. Hematoxylin and eosin sections from the growth showed large areas of necrosis (100%) along with dead bone. No evidence of residual tumor was seen. Sections from the surrounding soft tissue showed no evidence of residual tumor.
Figure 1: (a) X-ray thigh: Diaphyseal growth, with medullary sclerosis and sun burst appearance. (b) Magnetic resonance imaging (MRI) thigh: hypointense (sclerosed lesion) epicenter in the left distal femoral diaphysis with no obvious cortical breech with soft tissue extension. (c: 100×; d: 400×) Hematoxylin and eosin stained slides of tumor showing numerous scattered giant cells containing large pleomorphic nuclei with irregular nuclear membrane and few showing prominent nucleoli. Malignant osteoid was seen being layered by bizarre atypical cells having scanty foamy cytoplasm, atypical nuclear morphology. (e) Gross examination revealed diaphyseal bony hard growth with variegated cut surface, areas of necrosis, and few bluish areas in between (e)

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About 25% of osteosarcomas contain benign multinucleated giant cells resembling osteoclasts. [3] The term osteoclast-rich osteosarcoma is reserved for those osteosarcomas that contain abundant osteoclast-like giant cells distributed throughout the tumor. [4] At low-power view, these lesions show multinucleated giant cells simulating a giant cell tumor (GCT); but on high-power view, cytologic anaplasia of the stromal cells and malignant osteoid production can usually be identified. [1] The problem of differentiation further arises when true GCTs become malignant. Here, the pathologist is left with only one tool of malignant osteoid for differentiation as the degree of anaplasia is similar between malignant GCT and giant cell-rich osteogenic sarcoma.

Malignant fibrous histiocytoma of bone too contains small foci of osteoid in half of the cases, and also contains multinucleate tumor cells (malignant giant cells). However, the basic proliferating component is a fibrohistiocytic cell exhibiting a storiform or cartwheel pattern; this pattern helps to differentiate malignant fibrous histiocytoma from giant cell-rich osteosarcoma. [5] Multinucleated giant cells are also numerous in telangiectatic osteosarcomas, but here the giant cells are not uniformly distributed and are present around the blood-filled spaces. In addition, there usually is a great deal of nuclear pleomorphism evident in telangiectatic osteosarcomas.

The age and site of bone tumors also helps in differential diagnosis. Usually GCT tends to arise in skeletally mature people and extends from the area that corresponds to the metaphyseal side of the growth plate to the ends of the long bones. A tumor that appears to be a GCT histologically but arises in a skeletally immature person, therefore, should be regarded with great suspicion and sampled widely to rule out osteosarcoma. Fortunately, in our case, the site of the tumor (diaphysis) clearly ruled out the GCT. Over half of osteosarcomas in the long bones occur in the metaphyseal region, a further third in both epiphyseal and metaphyseal sites, with only a small percentage (less than 10%) being purely diaphyseal. [1] This was another unusual feature in the case discussed though the radiological findings hinted toward an osteosarcoma.

From a treatment standpoint, it is important to report osteosarcomas as high or low grade in a two-tiered system. High-grade osteosarcomas require high-dose chemotherapy. Giant cell-rich osteosarcoma is considered high-grade conventional osteosarcoma, hence its importance of reporting this variant as it has therapeutic and prognostic significance. This explains 3 cycles of neoadjuvant chemotherapy in our patient before surgical resection of the tumor.

 
   References Top

1.Mirra JM. Osseous tumours of intramedullary region. In: Mirra JM, editor. Bone tumors: Clinical, radiologic and pathologic correlation, 2 nd ed. Philadelphia: Lea and Febiger publishers; 1989. p. 248-438.  Back to cited text no. 1
    
2.Bathurst N, Sanerkin N. Osteoclast-rich osteosarcoma. Br J Radiol 1986;59:667-73.  Back to cited text no. 2
    
3.Klein MJ, Siegal GP. Osteosarcoma: Anatomic and histologic variants. Am J Clin Pathol 2006;125:555-81.  Back to cited text no. 3
[PUBMED]    
4.Shuhaibar H, Friedman L. Dedifferentiated parostal osteosarcoma with high-grade osteoclast-rich osteogenic sarcoma at presentation. Skeletal Radiol 1998;27:574-7.  Back to cited text no. 4
[PUBMED]    
5.Sato K, Yamamura KS, Iwata H, Sugiura H, Nakashima N, Nagasaka T. Giant cell-rich osteosarcoma: A case report. Nagoya J Med Sci 1996;59:151-7.  Back to cited text no. 5
    

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Correspondence Address:
Prateek Kinra
Department of Pathology,Armed Forces Medical College, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.107848

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