PRIMARY RENAL RHABDOMYOSARCOMA IN AN ADULT PATIENT PRESENTING WITH MYASTHENIA GRAVIS: UNUSUAL PRESENTATION OF A RARE MALIGNANCY

Prakasa RB; Rambabu B; Sridhar P

Volume : IV, Issue : X, October - 2015

PRIMARY RENAL RHABDOMYOSARCOMA IN AN ADULT PATIENT PRESENTING WITH MYASTHENIA GRAVIS: UNUSUAL PRESENTATION OF A RARE MALIGNANCY

Prakasa Rb, Rambabu B, Sridhar P

Abstract :

Genitourinary rhabdomyosarcoma is commonly seen in children and is rare in kidneys. Renal sarcomas account for less than 1% of cases of renal cell carcinomas. Myasthenia gravis is rarely seen as a paraneoplastic syndrome in renal cell carcinomas. Renal rhabdomyosarcoma in adult is hence a rare entity with only three or four cases reported in the literature. Usually the diagnosis is made postoperatively after histopathologic examination and immunohistochemistry of the radical nephrectomy specimen. Here we present a case of primary renal rhabdomyosarcoma in an adult who presented with myasthenia gravis as a paraneoplastic syndrome. A radical nephrectomy was done with careful perioperative use of acetylcholine esterase inhibitors. Histopathologic features were suggestive of mesenchymal tumour with skeletal muscle differentiation and positive staining for desmin and myogenin indicating a rhabdomyosarcoma.