title: [Spontaneous malignant transformation of extra-abdominal fibromatosis to fibrosarcoma].
abstract: The desmoid tumour with infiltrative and destructive growth tendencies, is a rare benign alteration of the connective tissue from musculo-aponeurotic structures and fascial sheaths. The exact pathogenesis is unknown, however trauma (surgery), genetic (Gardener's Syndrome) or hormonal factors (pregnancy) have been implicated. A spontaneous malignant transformation of a desmoid (extra-abdominal fibromatosis) to a fibrosarcoma is a rarity. Only one case is recorded in the literature. We report on the second case in international literature and describe the coherence to an endocrine factor (pregnancy), the high recurrence rate and the difficulty of therapy with regard to the risk of late malignant transformation.
authors.affiliation: Frauenklinik des Evangelischen Jung-Stilling-Krankenhauses Siegen.
authors.forename: J
authors.initials: J
authors.lastname: Schwickerath
authors.forename: H J
authors.initials: HJ
authors.lastname: Künzig
mesh_terms: D000328:Adult; D001940:Breast / Q000473:pathology; D001943:Breast Neoplasms / Q000473:pathology* / Q000601:surgery; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D005260:Female; D018222:Desmoid Tumors / Q000473:pathology* / Q000601:surgery; D005354:Fibrosarcoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000473:pathology / Q000601:surgery; D012086:Reoperation; D013629:Tamoxifen / Q000008:administration & dosage
chemical_list: D013629:Tamoxifen
vernacular_title: Die spontane maligne Transformation einer extraabdominalen Fibromatose in ein Fibrosarkom.