Rhabdomyosarcoma of the Urachus

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

“…Immunohistochemical methods currently are proving very valuable to demonstrate the myogenic components of RMS. Positive immunoreactivity for desmin and actin is considered the most useful method to establish the differential diagnosis from other small round-cell tumors in childhood [12,[16][17][18]20]. Moreover, RMS may also express neural markers such as NSE and neurofilaments [20], as occurs in our case.…”

“…Despite being the most frequent soft-part sarcomas in children and teenagers, those originating in the urachus are extremely rare. Only 3 detailed cases arising from the urachus have been properly reported in the English literature [9,12,15]. Thus, our case represents the fourth well-reported case of embryonal rhabdomyosarcoma of the urachus.…”

“…The second case was reported by Yokoyama et al [12] in a 2-year-old boy complaining of episodic lower abdominal pain. Laboratory investigations revealed slowly progressive anemia, elevation of serum lactic dehydrogenase and urine ketone bodies.…”

“…Urothelial carcinoma is the second most frequent tumor. Although sarcomas represent only 8%, they are the most frequent urachal neoplasms reported (67%) in patients younger than 20 years of age [7,[9][10][11][12].…”

“…They are usually diagnosed as a suprapubic palpable mass (64%) without hematuria, except in urachal adenocarcinomas [7], in which hematuria is a common finding. Doppler US, CT scan, and MRI may help to detect and delineate urachal tumors [1,6,15,16], but final diagnosis depends on the analysis of a biopsy of the primary tumor [7,12,[16][17][18][19]. Immunohistochemical methods currently are proving very valuable to demonstrate the myogenic components of RMS.…”