Abstract

S. Razine, S. Najem, S. Harrak, S. Lemsanes, K. Benchekroun, S. Sninate, S. Lkhoyaali, S. Boutayeb, B.Ghissassi, H. Errihani

ABSTRACT

Paratesticular rhabdomyosarcoma (RMS) accounts for only 7% of all the RMS cases arising from the mesenchymal tissues of the spermatic cord, epididymis, testis and testicular tunics. We report an 18?year?old man presenting with painless and rapidly growing mass in the scrotum. Radical inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an embryonic rhabdomyosarcoma. In addition, the CT scans showed Intra-abdominal lymph node metastasis and pulmonary metastases. The patient had three sessions of chemotherapy with vincristine, actinomycin C and cyclophosphamide with failure and disease progression. Paratesticular rhabdomyosarcoma (RMS) is a rare nongerm cell intrascrotal malignant tumor in children and young adult, Localized forms have a good prognosis whereas metastatic tumors show very poor results. The treatment of paratesticular rhabdomyosarcoma has evolved over several decades; the current standard of care is multimodal treatment including surgery, chemotherapy, and radiation. Whereas the data showed no upregulation of tumor markers such as b-human chorionic gonadotropin (b-HCG), alpha-fetoprotein (AFP), and lactate dehydrogenase (LDH), scrotal ultrasonography indicated the existence of paratesticular lesion. There was local recurrence in one patient who underwent radical orchiectomy for the sarcoma one year ago The two patients underwent radical inguinal orchiectomy or resection of the recurrent tumors with nerve-sparing retroperitoneal lymph node dissection. Histologic examination revealed embryonal RMS (eRMS) without lymph node metastasis. We highlight the importance of multi-disciplinary participation for paratesticular RMS detection and preoperative ultrasound-guided needle biopsy (UNB) for rapid confirmatory diagnosis.