Abstract

M. El Aamraoui*, N. Chebli, S. Naciri, S. Lkhoyaali, S. Boutayeb, B. Ghissassi, H. Mrabti and H. Errihani

ABSTRACT

Paratesticular rhabdomyosarcoma (RMS) is a rare type of RMS that represents only 7% of all RMS cases originating from the mesenchymal tissues of the spermatic cord, epididymis, testis, and testicular tunics. We report an 17?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 an uncommon malignant tumor in children and young adults that arises in the scrotal area and is not of germ cell origin. While localized forms of the disease have a favorable prognosis, metastatic tumors exhibit a very poor outcome. The current standard of care for treating paratesticular RMS involves a combination of surgery, chemotherapy, and radiation therapy. Even though tumor markers such as b-human chorionic gonadotropin (b-HCG), alpha-fetoprotein (AFP), and lactate dehydrogenase (LDH) were not elevated, scrotal ultrasonography indicated the presence of a paratesticular lesion. In one case, a patient who underwent radical orchiectomy for paratesticular sarcoma experienced local recurrence one year later. Both patients were treated with either radical inguinal orchiectomy or resection of recurrent tumors along with nerve-sparing retroperitoneal lymph node dissection. Histologic examination showed that both patients had embryonal RMS (eRMS) without lymph node metastasis. We emphasize the importance of involving a multidisciplinary team in the detection of paratesticular RMS and the use of preoperative ultrasound-guided needle biopsy (UNB) for rapid and accurate diagnosis.