title: [Desmoid tumors in children: current strategy].
abstract: Desmoid tumor (DT) or aggressive fibromatosis is a histologically benign-appearing neoplasms of the soft tissues, arising from connective tissues, the fascial sheaths and musculoaponevrotic structures of muscles. DT is a non-metastasizing tumor but has a potential for local invasion and local recurrence. DT can be seen in all age groups including young children. The overall incidence is reported as 2-4 cases per million per year. The etiology of DT is unknown. However, a genetic predisposition, familial adenomatous polyposis and Gardner's syndrome, has been implicated in 2% of the cases. The histological diagnosis has to be confirmed by biopsy in order to eliminate other sarcomas. The clinical behavior of DT seems unpredictable with a high rate of local recurrence. Treatment depends on the aggressiveness of the disease. A "wait-and-see" strategy is, at the present time, preferred in case of asymptomatic or non-progressive disease. Complete resection after surgery is rare, that is why first-line surgery should be seriously questioned and only considered when a complete resection can be feasible without functional or cosmetic damage. If not, medical treatments (cytotoxic or not) are rather discussed. Radiation therapy is rarely an option in children in this benign tumor. Authors present accurate knowledge of this disease in children.
authors.affiliation: CHU de Limoges, hôpital de la Mère et de l'Enfant, service d'oncologie pédiatrique, 8, avenue Dominique-Larrey, 87042 Limoges, France. oudot_caroline@yahoo.fr
authors.forename: Caroline
authors.initials: C
authors.lastname: Oudot
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Defachelles
authors.forename: Véronique
authors.initials: V
authors.lastname: Minard-Colin
authors.forename: Sylviane
authors.initials: S
authors.lastname: Olschwang
authors.forename: Laurent
authors.initials: L
authors.lastname: Fourcade
authors.forename: Sylvie
authors.initials: S
authors.lastname: Helfre
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D018222:Desmoid Tumors / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D005736:Gardner Syndrome / Q000150:complications; D017491:Genes, APC; D020022:Genetic Predisposition to Disease; D006801:Humans; D009154:Mutation / Q000235:genetics; D011183:Postoperative Complications; D035583:Rare Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D015996:Survival Rate; D057832:Watchful Waiting
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D000970:Antineoplastic Agents
keywords: APC gene; Gardner's syndrome; aggressive fibromatosis; children; desmoid tumor; familial adenomatous polyposis