title: Congenital extrarenal non-central nervous system malignant rhabdoid tumor.
abstract: Malignant rhabdoid tumor (MRT) is a rare tumor occurring mostly in kidneys and central nervous system (CNS). Its prognosis is not good. Occasionally, MRTs are diagnosed at or immediately after birth. A female neonate presented with MRT in the chest wall, axilla, right elbow, and bone marrow. Chest wall lesion was resected completely. Although the masses in axilla and bone marrow responded rapidly to chemotherapy, the elbow lesion increased in size. Despite intense treatment, the tumor relapsed in lungs and the patient died 12 months after diagnosis. Review of the literature showed twenty additional congenital MRTs arising from sites outside of the kidney and central nervous system were published in the literature. Eighteen patients had disseminated disease at diagnosis. The median overall survival time for all (n = 21) patients was 2.0 months (0-24 months). The only patient who survived had a localized tumor at initial diagnosis. Congenital, extrarenal, non-CNS MRTs are aggressive tumors with poor outcome.
authors.affiliation: Southern Alberta Children's Cancer Program, Alberta Children's Hospital and Tom Baker Cancer Center, Calgary, Alberta, Canada.
authors.forename: Mitra
authors.initials: M
authors.lastname: Sajedi
authors.forename: Johannes E A
authors.initials: JE
authors.lastname: Wolff
authors.forename: R Maarten
authors.initials: RM
authors.lastname: Egeler
authors.forename: Alfredo
authors.initials: A
authors.lastname: Pinto
authors.forename: Rhiannon
authors.initials: R
authors.lastname: Hughes
authors.forename: Ron A
authors.initials: RA
authors.lastname: Anderson
authors.forename: Max J
authors.initials: MJ
authors.lastname: Coppes
mesh_terms: D001365:Axilla / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D001932:Brain Neoplasms / Q000473:pathology* / Q000628:therapy; D004550:Elbow / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008854:Microscopy, Electron; D018335:Rhabdoid Tumor / Q000151:congenital / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D013909:Thorax / Q000473:pathology