|Year : 2014 | Volume
| Issue : 1 | Page : 57-59
Pleomorphic Rhabdomyosarcoma in upper limb in an adult
Pulla G Rao1, Durgaprasad S Hegde1, Rama Mohan B Rao2
1 Department of Orthopaedics, Konaseema Institute of Medical Sciences, Amalapuram, Andhra Pradesh, India
2 Department of Pathology, Konaseema Institute of Medical Sciences, Amalapuram, Andhra Pradesh, India
|Date of Web Publication||10-Mar-2014|
Durgaprasad S Hegde
Department of Orthopaedics, Konaseema Institute of Medical Sciences, Chaitanya Nagar, Amalapuram - 533 201, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in the first 2 decades of life, with a peak age incidence in very young children. Due to the rarity of adult RMS, information regarding its clinical and biologic characteristics is very limited. We report a case of pleomorphic RMS of upper extremity in a 50-year-old man. There was no metastasis of the tumor. The patient underwent surgery with enbloc excision of the tumor. Histopathological examination showed typical features of pleomorphic RMS composed of large polygonal pleomorphic cells with eosinophilic cytoplasm and plenty of large multinucleated tumor giant cells having bizarre nuclei. Postoperatively patient took radiotherapy. There was no recurrence of tumor with follow-up of 2 years.
Keywords: Pleomorphic cells, pleomorphic rhabdomyosarcoma, metastasis
|How to cite this article:|
Rao PG, Hegde DS, Rao RB. Pleomorphic Rhabdomyosarcoma in upper limb in an adult. J NTR Univ Health Sci 2014;3:57-9
| Introduction|| |
Rhabdomyosarcoma (RMS) is a highly malignant mesenchymal tumor thought to originate from immature striated muscle. It is characterized by the presence of cells having an identifiable striated muscular differentiation with rhabdomyoblasts cells. The pleomorphic form is typically more frequent in adults and has a poor prognosis. 
RMSs are solid tumors that are common in children, representing 5% of all childhood cancers.  In contrast, RMSs are rare in adults, with soft-tissue sarcomas making up fewer than 1% of malignancies in adults and RMSs accounting for 3% of all soft tissue sarcomas. 
| Case Report|| |
A male patient aged fifty years presented with history of swelling and pain in right upper arm since 6 months that had been exacerbated by recent fall. He took treatment in the form of analgesics, pain decreased but swelling gradually increased in size. Physical examination revealed swelling confined to upper part of arm on medial side [Figure 1]. Swelling was firm in consistency, freely mobile with no lymphadenopathy, and any distal neurovascular deficit. Radiographs of right upper limb showed only soft tissue swelling without bony involvement. Blood parameters were within normal range. Chest and spine radiographs revealed no metastatic lesions.
Patient was operated under general anesthesia with medial incision. Intraoperatively tumor was confined to medial head of triceps. Enbloc excision of tumor was done. Grossly tumor appeared grey white to grey brown of size 10 × 7.5 × 5 cm and irregular in appearance [Figure 2]. Cross-section showed well-circumscribed and partially encapsulated mass with grey brown, grey white area with focal nodular, and whorled pattern. Histopathological examination showed typical features of pleomorphic RMS composed of large polygonal pleomorphic cells with eosinophilic cytoplasm and plenty of large multinucleated tumor giant cells having bizarre nuclei [Figure 3]. Patient took radiotherapy for 6 weeks and there was no recurrence of symptoms with a follow-up of 2 years.
|Figure 2: Specimen of pleomorphic rhabdomyosarcoma after enbloc excision|
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|Figure 3: Photomicrograph showing very large pleomorphic polygonal cells with hyperchromatic nuclei, tumor giant cells with bizarre nuclei, and eosinophilic cytoplasm (hematoxylin and eosin stain (H and E), 400×)|
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| Discussion|| |
The different subtypes of RMS are embryonal, alveolar, and pleomorphic. Overall, the embryonal subtypes the most common subtype, accounting for up to 49% of all RMSs.  The alveolar subtype accounts for approximately 30% of all RMSs and most commonly affects adolescents. It is most commonly an intramuscular tumor in the soft tissues of the extremities. 
The pleomorphic subtype is the least common subtype and almost exclusively affects patients older than 45 years. It arises most commonly in the skeletal muscles of the thigh. It is histologically similar to a malignant fibrous histiocytoma; in fact, many pleomorphic RMSs have been reclassified as fibrous histiocytomas, making the diagnosis of pleomorphic RMS an even rarer entity. 
In adults with RMS, lymphadenopathy appears to be more common. Specifically, 46% of patients had lymph node involvement at diagnosis in one series.  Lymphadenopathy was most prominent in alveolar tumor subtypes and was least common in extremity tumors and pleomorphic tumor subtypes. Pulmonary metastases have been reported to be the most frequent site of distant disease. 
The presence of metastases at presentation along with tumor size, tumor resectability, and patient age at presentation are also prognostic factors. Histologic subtype also has a bearing on prognosis. Embryonal tumors are usually associated with the most favorable outcome and pleomorphic, the least favorable. In our case there was no metastasis.
Treatment of patients with RMS is usually primarily surgery if excision is attainable, followed by radiation therapy and chemotherapy. Survival rates for patients with nonmetastatic disease have improved substantially throughout the last 30 years. , Although surgical resection, either as a primary procedure or a secondary strategy (after initial chemotherapy), is important for local control, complete resection is not feasible at some sites of disease. Radiotherapy has therefore assumed a major role in the management of many patients, though late sequelae can be significant.
The prognosis for patients with RMS has been typically very poor, with a 5-year survival rate of less than 50% even in the most recent series. , However, this survival rate is improved compared with the rates reported in older series, which were as low as 21%. 
| Conclusion|| |
Though the treatment for adult RMS is surgery, radiotherapy and chemotherapy or in combination, careful long-term follow-up is necessary due to high risk of recurrence.
| References|| |
|1.||Ahmed HU, Arya M, Muneer A, Mushtaq I, Sebire NJ. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol 2010;11:476-83. |
|2.||McCarville MB, Spunt SL, Pappo AS. Rhabdomyosarcoma in pediatric patients: The good, the bad, and the unusual. AJR Am J Roentgenol 2001;176:1563-9. |
|3.||Weiss S, Goldblum JR. Rhabdomyosarcoma. In: Weiss S, Goldblum JR, editors. Enzinger and Weiss's Soft Tissue Tumors. 4 th ed. St. Louis, MO: Mosby; 2001. p. 785-835. |
|4.||Newton WA Jr, Gehan EA, Webber BL, Marsden HB, van Unnik AJ, Hamoudi AB, et al. Classification of rhabdomyosarcomas and related sarcomas: Pathologic aspects and proposal for a new classification - an Intergroup Rhabdomyosarcoma Study. Cancer 1995;76:1073-85. |
|5.||Esnaola NF, Rubin BP, Baldini EH, Vasudevan N, Demetri GD, Fletcher CD, et al. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg 2001;234:215-23. |
|6.||Raney RB Jr, Tefft M, Maurer HM, Ragab AH, Hays DM, Soule EH, et al. Disease patterns and survival rate in children with metastatic soft-tissue sarcoma: A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I. Cancer 1988;62:1257-66. |
|7.||Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: A selective review of Intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001;23:215-20. |
|8.||Stiller CA, Stevens MC, Magnani C, Corazziari I; EUROCARE Working Group. Survival of children with soft tissue sarcoma in Europe since 1978: Results from the EUROCARE study. Eur J Cancer 2001;37:767-74. |
|9.||Little DJ, Ballo MT, Zagars GK, Pisters PW, Patel SR, El-Naggar AK, et al. Adult rhabdomyosarcoma: Outcome following multimodality treatment. Cancer 2002;95:377-88. |
|10.||Lloyd RV, Hajdu SI, Knapper WH. Embryonal rhabdomyosarcoma in adults. Cancer 1983;51:557-65. |
[Figure 1], [Figure 2], [Figure 3]