Case Report: The Paratesticular Rhabdomyosarcoma in Children

Abstract

Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the prerogative of the child and the young adult. On physical examination, a painless scrotal tumefaction is observed. His diagnosis is essentially provided by testicular ultrasound. His therapeutic strategy is codified and based on the combination of chemotherapy and surgical excision or radiotherapy. The precocity of the management and the accuracy of the extension and radiology tests are important for the vital and functional prognosis. We report a case of paratesticular rhabdomysarcoma of alveolar type, diagnosed in front of a large left painless scrotal mass, which has been evolving for less than one month in a 7-year-old child. An inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an epididymal RMS of the alveolar type. Our patient had 9 chemotherapy sessions with vincristine, actinomycin C and cyclophosphamide. Each chemotherapy session took place over four days, with a 21-day cycle. He was evaluated for 4 years and demonstrated good clinical improvement. The experience gained from the index case is crucial for the management of patients with intratesticular rhabdomyosarcoma who must always ensure that the radical inguinal orchiectomy with lymph node dissection is covered by chemotherapy and/or radiotherapy. This will potentially reduce the chances of tumor recurrence and/or metastasis, thus improving patient prognosis.

Abordaje clínico, diagnóstico y terapéutico de los tumores testiculares y paratesticulares en pacientes pediátricos del Hospital Civil de Guadalajara Fray Antonio Alcalde: Estudio retrospectivo de 10 años

“…There is a bimodal age distribution, with the first peak occurring during the first 2 years of life and the second around 15 years of age [1][2][3] . The increased incidence of tumors after 9 years of age could be associated with the high hormonal levels at puberty 9,10 . These tumors usually have a good prognosis, with an approximate benignity of 50% and an overall survival of 99% at five years 1,5 .…”

“…Among prepubertal intratesticular tumors, 90% are germ cell tumors and only 10-15% are epidermoid cysts 10,11 . Benign germ cell tumors are frequent, approximately 40-50% are teratomas 9,13,14 . Epidermoid cysts are always benign.…”

“…Epidermoid cysts are always benign. Malignant germ cell tumors are mainly represented by yolk sac tumors 8-30% 9,14,15 . On the other hand, among non-germ cell derived tumors, Leydig cell tumors are usually the most frequent, followed by juvenile stromal granulosa cell tumors 9,14,16 .…”

“…The traditional treatment of choice for testicular masses is radical orchiectomy; however, due to the benignity in children, in recent years the medical-surgical approach has been re-evaluated, where lumpectomy (conservative surgery) is considered depending on tumor markers (alpha-fetoproteins or beta subunit of human chorionic gonadotropin), tumor size and histological findings 9 .…”

“…in those where malignancy is not suspected. In some cases intraoperative frozen section examination can be used to confirm a pathological tumor, as well as to justify the choice of conservative surgery 9,19,23 . Cases of malignant testicular tumors are rare and usually occur in older children, so radical orchiectomy with postoperative follow-up is recommended in patients 5 years of age or older 9 .…”