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.
Jones
Ashley E
AE
Department of Pediatrics, University of Colorado Denver School of Medicine, The Children's Hospital, Aurora, Colorado, USA. jones.ashley@tchden.org
Albano
Edythe A
EA
Lovell
Mark A
MA
Hunger
Stephen P
SP
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