Giant primary paratesticular rhabdomyosarcoma: a case report and review of surgical and oncologic management

Abstract Background Paratesticular rhabdomyosarcoma is a sporadic tumor arising from the para-testicular structures, and is often diagnosed in children and adolescents. Objective To highlight the aggressive nature of rhabdomyosarcoma and the feasibility of inguinal orchidectomy for a giant locally advanced paratesticular rhabdomyosarcoma. This case is unique because it is one of the few reported cases of adult paratesticular rhabdomyosarcoma. It also discusses the surgical and oncological management of paratesticular rhabdomyosarcoma. Case presentation An 18-year-old young adult presented to the emergency department of Federal Teaching Hospital Gombe with a 6-month history of right hemi-scrotal mass. He was chronically ill-looking and pale with mobile and non-tender bilateral superficial inguinal lymphadenopathy. There was a giant and tender right hemiscrotal swelling extending to the groin which was firm to hard in consistency with an area of skin ulceration. A malignant right testicular tumor was diagnosed. Serum alpha-feto protein, beta-human chorionic gonadotropin, and lactate dehydrogenase were within normal range. The abdominopelvic ultrasound scan and chest x-ray were normal. Scrotal ultrasound scan showed a huge lobulated heterogenous hypoechoic right intratesticular mass extending to the inguinal region with focal areas of increased echogenicity with no colour flow. He underwent a right inguinal orchidectomy. A huge right testicular tumor with solid and cystic components measuring 26 cm × 10 cm × 10 cm with the overlying ulcerated skin was resected en-bloc. The histology was consistent with an embryonal rhabdomyosarcoma. The patient recovered uneventfully and was referred for adjuvant therapy. Conclusion Embryonal paratesticular rhabdomyosarcoma is rare, especially in adults and its diagnosis is mostly established following histology. This is because ultrasound findings are often non-specific and testicular tumor markers are not elevated. Inguinal orchidectomy is feasible even in the presence of a giant testicular tumor. This provides local tumor control, reduces the need for repeated blood transfusions, and provides specimens for definitive diagnosis. Adult patients have a worse prognosis with less than 30% overall 5-year survival and therefore, long-term follow-up is needed.