Franz T, Häfner T, Horn L C, Kassahun W, Stolzenburg J-U
Klinik und Poliklinik für Urologie, Universitätsklinikum Leipzig AöR, Leipzig.
Urologe A. 2013 Jan;52(1):74-8. doi: 10.1007/s00120-012-2990-7.
We report the case of a 57-year-old patient with a huge, retrovesical, aggressive desmoid fibromatosis detected incidentally during preoperative staging for radical prostatectomy. The tumor was locally invasive consisting of monoclonal and fibroblastic proliferations. The etiology of such tumors is not well known but they are mostly sporadic and aggressive fibromatosis is sometimes associated with familial adenomatous polyposis (FAP) or Gardner's syndrome. Such desmoid tumors are slow growing and locally invasive but lack metastatic potential. Complete resection with wide margins is the therapy of choice and the tendency to recur is high. Radiotherapy following surgery is controversial. We discuss the pathogenesis, clinical manifestations and therapy of such rare tumors.
我们报告了一例57岁患者，在根治性前列腺切除术的术前分期时偶然发现患有巨大的膀胱后侵袭性硬纤维瘤病。肿瘤呈局部浸润性，由单克隆和成纤维细胞增殖组成。此类肿瘤的病因尚不清楚，但大多为散发性，侵袭性纤维瘤病有时与家族性腺瘤性息肉病（FAP）或加德纳综合征相关。此类硬纤维瘤生长缓慢且局部浸润，但无转移潜能。广泛切缘完整切除是首选治疗方法，复发倾向较高。术后放疗存在争议。我们讨论了此类罕见肿瘤的发病机制、临床表现及治疗方法。
