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Congenital Extrarenal Non-Central Nervous System Malignant Rhabdoid Tumor

Sajedi, Mitra M.D; Wolff, Johannes E.A. M.D.; Egeler, R. Maarten M.D., Ph.D.; Pinto, Alfredo M.D.; Hughes, Rhiannon M.D.; Anderson, Ron A. M.D.; Coppes, Max J. M.D., Ph.D.

Journal of Pediatric Hematology/Oncology: May 2002 - Volume 24 - Issue 4 - p 316-320
Clinical And Laboratory Observations

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.

From the Southern Alberta Children's Cancer Program at Alberta Children's Hospital and Tom Baker Cancer Center (M.S., J.E.A.W., R.M.E., R.A.A., M.J.C.), Calgary, Alberta, Canada; and the Departments of Oncology (J.E.A.W., R.M.E., R.A.A., M.J.C.), Pediatrics (J.E.A.W., R.M.E., R.H., R.A.A., M.J.C.), Pathology (A.P.), Genetics (R.H.), and the Cancer Biology Research Group (J.E.A.W., R.M.E., R.H., M.J.C.), University of Calgary, Calgary, Alberta, Canada.

Submitted for publication January 31, 2001; accepted March 9, 2001.

Address correspondence and reprint requests to Dr. Max J. Coppes, Southern Alberta Pediatric Oncology Program, Alberta Children's Hospital, 1820 Richmond Road SW, Calgary, Alberta, Canada T2T 5C7. E-mail:

© 2002 Lippincott Williams & Wilkins, Inc.