title: 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.
authors.affiliation: Department of Surgery (Gastrointestinal Surgery Unit), Hospital Universitario de Canarias, La Laguna, Tenerife, Spain. dretomassetti@gmail.com
authors.forename: Eudaldo M López-Tomassetti
authors.initials: EM
authors.lastname: Fernández
authors.forename: Norberto Hernández
authors.initials: NH
authors.lastname: Siverio
authors.forename: Ricardo López
authors.initials: RL
authors.lastname: Almaraz
authors.forename: Lucía Martín
authors.initials: LM
authors.lastname: Viota
authors.forename: José Rodriguez
authors.initials: JR
authors.lastname: Luis
authors.forename: Lucio Díaz
authors.initials: LD
authors.lastname: Flores
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D018233:Rhabdomyosarcoma, Embryonal / Q000188:drug therapy* / Q000601:surgery*; D014497:Urachus; D014750:Vincristine / Q000627:therapeutic use