TESTICULAR EMBRYONAL RHABDOMYOSARCOMA IN ADOLESCENCE: FROM RADICAL ORCHIECTOMY TO CATASTROPHIC LOCAL RECURRENCE – AN UROLOGICAL PERSPECTIVE ON THERAPEUTIC CHALLENGES AND FATAL OUTCOME
*M. El Idrissi El Jouhari, M. Bennani, M. Bouchareb, Y. Daghdagh, A. Kbirou, A. Moataz, M. Dakir, A. Debbagh, R. Aboutaieb
ABSTRACT
Background: Testicular rhabdomyosarcoma (RMS) is an aggressive soft tissue sarcoma, exceptionally rare in late adolescence, presenting significant diagnostic and therapeutic challenges distinct from common germ cell tumors. This report discusses the urological management of a metastatic embryonal RMS with a focus on surgical dilemmas and local control. Case Presentation: A 17-year-old male presented with a 16 cm painful left scrotal mass. Initial tumor markers were negative. CT imaging revealed pulmonary metastases and renal cystic lesions (Bosniak IV). He underwent left radical inguinal orchiectomy. Pathology confirmed embryonal RMS with deep dermal infiltration. Adjuvant VAC chemotherapy (Vincristine, Actinomycin-D, Cyclophosphamide) was initiated but complicated by severe septic shock and scrotal necrosis requiring multiple debridements. Despite initial systemic response, the patient developed extensive local recurrence infiltrating the perineum and contralateral scrotum, alongside progression of bone and pulmonary metastases. Surgical excision of the recurrence was performed for palliation, but the patient rapidly deteriorated due to pancytopenia and extensive metastatic burden, succumbing to the disease 11 months after diagnosis. Conclusion: This case illustrates the "therapeutic collision" between the need for aggressive local control and the limitations imposed by systemic metastatic disease. Radical orchiectomy remains the cornerstone of diagnosis and local control, yet scrotal skin involvement portends a dismal prognosis. Early recognition, multidisciplinary management, and balancing surgical morbidity with quality of life are critical in these catastrophic presentations.
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