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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 653-654
Primary rhabdomyosarcoma of proximal tibia in an adult: A rare entity

1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Orthopedics, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication20-Sep-2011

How to cite this article:
Kumar N, Das P, Ahuja A, Gupta C, Rastogi S, Ray R. Primary rhabdomyosarcoma of proximal tibia in an adult: A rare entity. Indian J Pathol Microbiol 2011;54:653-4

How to cite this URL:
Kumar N, Das P, Ahuja A, Gupta C, Rastogi S, Ray R. Primary rhabdomyosarcoma of proximal tibia in an adult: A rare entity. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Nov 30];54:653-4. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/653/85138


A 72 year female presented with swelling and pathological fracture of right proximal tibia following minor trauma. The swelling was progressive, expansile and tender to palpate. Overlying skin of the lesion was warm and rubor was not seen. On radiological investigation, a mixed osteosclerotic-osteolytic lesion was noted in the right proximal tibia [Figure 1]. Based on the radiological findings a primary bone neoplasm, possibly chondrosarcoma, was suspected. Radiological examinations performed all over the body did not reveal any other tumor. Biochemical examination showed normal calcium, phosphorus along with normal thyroid, and parathyroid hormone levels.
Figure 1: Roentgenogram, lateral and posterior-anterior view of right knee joint show a mixed osteolytic and osteosclerotic lesion in the upper end of tibia

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Initially, a wide-bore needle biopsy was performed. The histopathological examination revealed a spindle cell sarcoma with considerable cellular pleomorphism and frequent atypical mitoses. Focally some strap cells with large vesicular nucleus and abundant elongated eosinophilic cytoplasm were identified. These cells showed cross striations on PTAH (Phosphotungstic acid hematoxylin) staining. Destruction of the adjacent bone was noted. Immunohistochemical stains for vimentin, cytokeratin, smooth muscle actin (SMA), myogenin, desmin, CD34 and S-100 proteins were performed. The tumor cells were immune-positive for vimentin, SMA, myogenin and desmin, characterizing the lesion as rhabdomyosarcoma (RMS) [Figure 2]a-d. Subsequently a limb salvage therapy was undertaken and an amputated below right knee specimen was received. Histological examination of the amputated specimen also showed features of a pleomorphic RMS. Post- operatively the patient was treated with adjuvant chemotherapy.
Figure 2: (a) Photomicrographs of the lesion showed an infiltrating spindle cell lesion (H and E, ×40), with (b) cellular pleomorphism, atypical mitotic fi gures (H and E, ×400) and (c) a few strap cells (H and E, ×400). (d) Strong immune-positivity for desmin and smooth muscle actin was seen (IHC, ×100)

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Primary RMS of long bone in an adult is rare, while metastatic lesions are common. [1] Apart from a metastasis, RMS of bone may represent a heterogeneously differentiated component in other sarcomas. To the best of our knowledge, there are only eight reported cases of primary RMS of long bone in the English literature with histopathological proof. Out of these eight reported cases five were identified in femur, two in tibia, and one case in humerus. Our case represents the third well-established case of a pleomorphic RMS. Lamovec et al. [2] described three cases of primary bone sarcomas with rhabdomyosarcomatous differentiation. A mixed pleomorphic and embryonal RMS was reported by Rashid et al. [3] in diaphysis of femur in a 68-year-old woman. Primary embryonal RMS of long bone was reported by Lucas et al. [4] presenting as a lytic destructive lesion at diaphysis of the right femur. Wang et al. [5] also reported a primary pleomorphic RMS of femur in 45-year-old male, who was treated successfully with limb salvage surgery after intra-arterial chemotherapy.

As primary long bone RMS is rare in adults, it is important for pathologists and physicians to be aware of its occurrence. Although rare, it would be rewarding if this lesion is also included in the differential diagnosis of primary malignant bone tumors of long bones and investigated accordingly.

Financial Disclosure: The authors declare no source of financial support from any organizations

   References Top

1.Hollowood K, Fletcher CD. Rhabdomyosarcoma in adults. Semin Diag Pathol 1994;11:47-57.  Back to cited text no. 1
2.Lamovec J, Zidar A, Bracko M, Golouh R. Primary bone sarcoma with rhabdomyosarcomatous component. Pathol Res Pract 1994;190:51-60.  Back to cited text no. 2
3.Rashid A, Dickersin. RG, Rosenthal ID, Mankin H, Rosenberg AE. Rhabdomyosarcoma of the Long Bone in an Adult. A Case Report and Literature Review. Int J Surg Pathol 1994;1:253-9.  Back to cited text no. 3
4.Lucas DR, Ryan JR, Zalupski MM, Gross ML, Ravindranath Y, Ortman B. Primary embryonal rhabdomyosarcoma of long bone. Case report and review of the literature. Am J Surg Pathol 1996;20:239-44.  Back to cited text no. 4
5.Thomas F, Lipton JF, Barbera C, Vigorita VJ, Bryk E. Primary rhabdomyosarcoma of the humerus: A case report and review of the literature. J Bone Joint Surg Am 2002;84:813-7.  Back to cited text no. 5

Correspondence Address:
Ruma Ray
Department of Pathology, All India Institute of Medical sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85138

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