desmoid tumors in the pediatric population硬纤维瘤在儿科人口.pdfVIP

Cancers 2012, 4, 295-306; doi:10.3390/cancers4010295 OPEN ACCESS cancers ISSN 2072-6694 /journal/cancers Review Desmoid Tumors in the Pediatric Population Joshua N. Honeyman and Michael P. La Quaglia * Department of Surgery, Pediatrics Service, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA; E-Mail: honeymaj@ * Author to whom correspondence should be addressed; E-Mail: laquaglm@; Tel.: +1-212-639-7003; Fax: +1-212-717-3214. Received: 10 February 2012; in revised form: 29 February 2012 / Accepted: 6 March 2012 / Published: 9 March 2012 Abstract: Desmoid tumors are benign soft tissue tumors associated with locally aggressive growth and high rates of morbidity, but they do not metastasize via lymphatic or hematogenous routes. While most of the data on desmoid tumors originates in the adult literature, many of the findings have been applied to the management of pediatric patients. This article discusses the epidemiology, etiology, clinical presentation, pathology, and treatment of this rare tumor in the pediatric population and includes a literature review of the most recent large series of pediatric patients with desmoid tumors. Keywords: desmoid tumor; aggressive fibromatosis; surgery; pediatric surgical oncology 1. Introduction Desmoid tumors are rare soft tissue neoplasms that do not metastasize, but exhibit aggressive growth and local invasion. Thus, they are designated as “intermediate” tumors, according t