title: Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature.
abstract: Desmoid tumors are fibroblastic/myofibroblastic neoplasms, which originate from musculo-aponeurotic structures and are classified as deep fibromatoses. Despite their benign histologic appearance and lack of metastatic potential, desmoid tumors may cause aggres?sive local infiltrations and compression of surrounding structures. They are often associated with female gender, familial adenomatous polyposis (FAP) and sporadically may occur at sites of previous trauma, scars or irradiation. Molecular studies have demonstrated that these patients are associated with a bi-allelic APC mutation in the affected tissue. Radical tumor resection with free margins remains the first therapy of choice. In cases with anatomical or technical limitations for a wide excision, radiation therapy represents a proven and effective alternative or supplementary treatment.
authors.affiliation: Department of Radiation Oncology, University Hospital Düsseldorf, Germany.
authors.forename: E
authors.initials: E
authors.lastname: Bölke
authors.forename: H
authors.initials: H
authors.lastname: Krasniqi
authors.forename: G
authors.initials: G
authors.lastname: Lammering
authors.forename: R
authors.initials: R
authors.lastname: Engers
authors.forename: C
authors.initials: C
authors.lastname: Matuschek
authors.forename: S
authors.initials: S
authors.lastname: Gripp
authors.forename: P A
authors.initials: PA
authors.lastname: Gerber
authors.forename: G
authors.initials: G
authors.lastname: Fischer
authors.forename: M
authors.initials: M
authors.lastname: Peiper
authors.forename: S
authors.initials: S
authors.lastname: Shaikh
authors.forename: Wilfried
authors.initials: W
authors.lastname: Budach
authors.forename: K
authors.initials: K
authors.lastname: Orth
mesh_terms: D000293:Adolescent; D005260:Female; D018221:Fibromatosis, Abdominal / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D013902:Radiography, Thoracic / Q000379:methods; D013899:Thoracic Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D035441:Thoracic Wall / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed