TESTICULAR EMBRYONAL RHABDOMYOSARCOMA IN ADOLESCENCE: FROM RADICAL ORCHIECTOMY TO CATASTROPHIC LOCAL RECURRENCE - AN UROLOGICAL PERSPECTIVE ON THERAPEUTIC CHALLENGES AND FATAL OUTCOME

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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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2026-05-01
DOI
https://doi.org/10.5281/zenodo.19907229
Primary Topic
Sarcoma Diagnosis and Treatment
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article
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article

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
Zenodo (CERN European Organization for Nuclear Research)
Sarcoma Diagnosis and Treatment
article

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
article en

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.

Zenodo (CERN European Organization for Nuclear Research)
Good health and well-being
Openalex Percentile: Top 5%
Sarcoma Diagnosis and Treatment
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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 · Zenodo (CERN European Organization for Nuclear Research) (2026) | TGRS Research Map | TGRS