RADICAL SURGERY AND IVA-CHEMOTHERAPEUTIC REGIMEN TO TREAT EMBRYONAL RHABDOMYOSARCOMA OF THE URACHUS: Case Report

Abstract

Although rhabdomyosarcoma is the most frequent soft tissue tumor in children, there are extremely few reports of this tumor arising from the urachus. The authors describe another case in a 6-year-old female associated with constipation and a painless suprapubic mass. The specimen had the pathological criteria used to define urachal sarcoma (cytological, histological, and immunohistochemical findings) and urachal remnants were not observed. After complete resection of the primary tumor the patient was treated with chemotherapy (ifosfamide, vincristine, and dactinomycin) and remains alive at 4 years' follow-up with no signs of recurrence. Nowadays the management of children with urachal rhabdomyosarcoma usually includes surgery and chemotherapy. Multimodal therapy and surgery are constantly evolving and have significantly improved overall survival of these patients especially in R0 resected patients.

Costello Syndrome and Umbilical Ligament Rhabdomyosarcoma in Two Pediatric Patients: Case Reports and Review of the Literature

“…They may arise from any portion of the urachus and are most commonly found in adults and males (60–70%). Although sarcomas represent only 8%, they are the most frequent urachal neoplasms reported in patients younger than 20 years of age (67%) [ 27 ]. Poor prognosis is a common feature in adults and pediatric urachal malignancies with 50% to 60% of 5-year survival.…”

Non‑syndromic first case of pediatric rhabdomyosarcoma originating from the umbilical left medial ligament: A case report

“…In the general population, umbilical ligament malignant tumors are extremely rare, usually observed in adult males, accounting for 0.01% of all tumors (11). On the other hand, umbilical ligament RMS is mostly reported in paediatric patients with CS.…”