Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A.
Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies. Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreported association of MEN-2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options. The high penetrance of malignancy in patients with MEN warrants a heightened suspicion for the development of nonendocrine malignancies. The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients.
Department of Pediatrics, University of Colorado Denver School of Medicine, The Children's Hospital, Aurora, Colorado, USA. jones.ashley@tchden.org
Ashley E
AE
Jones
Edythe A
EA
Albano
Mark A
MA
Lovell
Stephen P
SP
Hunger
D006801:Humans; D007223:Infant; D008297:Male; D018813:Multiple Endocrine Neoplasia Type 2a / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D018232:Rhabdomyosarcoma, Alveolar / Q000556:secondary* / Q000628:therapy