Clin Pediatr Hematol Oncol.
2006 Oct;13(2):233-237.
A Case of Malignant Extrarenal Rhabdoid Tumor of the Neck
- Affiliations
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- 1Department of Pediatrics, College of Medicine, Chungnam National University, Daejeon, Korea. sunyoung@cnuh.co.kr
- 2Department of Pathology, College of Medicine, Chungnam National University, Daejeon, Korea.
- 3Department of Pediatrics, Korea Cancer Center Hospital, Seoul, Korea.
Abstract
- Malignant rhabdoid tumor is a rare but distinctive neoplasm of unknown histogenesis, occurring primarily in children. Extrarenal rhabdoid tumors have since been reported in multiple anatomic sites in the pediatric and adult populations. These tumors are characterized by an aggressive clinical course, resistance to treatment, and a rapidly fatal outcome. We experienced a case of malignant extrarenal rhabdoid tumor located in the posterior cervical area in a 16 month-old girl. The tumor was composed of round or polygonal monotonous cells with vesicular, prominent nucleoli and abundant cytoplasm containing inclusions. On immunohistochemistry, the atypical cells were vimentin and epithelial membrane antigen (EMA) positive. After having obtained consent, the patient was treated with systemic multiagent chemotherapy and will be receiving myeloablative chemotherapy and stem cell transplantation.
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