title: [The role of radiotherapy in the treatment of desmoid tumors].
abstract: Aggressive fibromatoses are benign tumors which derive from connective tissues in muscles, tendons, joints and scars. They are characterized by a low mitotic index and present without major cellular changes. The incidence is low and reaches 0.4 per 100,000. The classification in extra-abdominal, intra-abdominal and abdominal wall tumors has prognostic significance for the therapeutic decision. Extra-abdominal tumors have the highest relapse rate with about two-thirds recurring even after R0 surgical resection. In this meta-analysis, 698 published cases are reviewed and analyzed with respect to the previous surgery and the therapeutic impact of postoperative radiotherapy. After an R1 surgical resection radiotherapy significantly decreases the risk for loco-regional relapse in about 40%. Radiotherapy is also recommended after an R0 surgical resection with safety margins less than 2 cm. A total radiotherapy dose of 50 Gy seems to be sufficient, whereas radiotherapy doses beyond 60 Gy provide no additional control. Following an R2 resection up to 28% of these tumors may not progress or even show a complete remission. A wait-and-see policy is justified for R1-2 resected lesions in the abdominal wall, especially if severe treatment sequelae have to be expected. The role of chemotherapy still remains questionable.
authors.affiliation: Strahlentherapeutische Klinik und Poliklinik, Universität Erlangen-Nürnberg.
authors.forename: M J
authors.initials: MJ
authors.lastname: Kirschner
authors.forename: R
authors.initials: R
authors.lastname: Sauer
mesh_terms: D000009:Abdominal Muscles; D000008:Abdominal Neoplasms / Q000453:epidemiology / Q000532:radiotherapy*; D000328:Adult; D000367:Age Factors; D003131:Combined Modality Therapy; D005260:Female; D005350:Fibroma / Q000453:epidemiology / Q000532:radiotherapy*; D006801:Humans; D015994:Incidence; D008297:Male; D009135:Muscular Diseases / Q000453:epidemiology / Q000532:radiotherapy; D011379:Prognosis; D012737:Sex Factors