INTRAABDOMINAL FIBROMATOSIS - A PATHOLOGICAL ANALYSIS OF 130 TUMORS WITH COMPARISON OF CLINICAL SUBGROUPS
INTRAABDOMINAL FIBROMATOSIS - A PATHOLOGICAL ANALYSIS OF 130 TUMORS WITH COMPARISON OF CLINICAL SUBGROUPS
复制标题
DOI：
10.1097/00000478-199004000-00004
复制
发表时间：
1990-04-01
影响因子：
5.6
通讯作者：
HELWIG, EB
中科院分区：
文献类型：
--
作者：
BURKE, AP;SOBIN, LH;HELWIG, EB
我们研究了130例肠系膜和其他腹膜部位纤维瘤病的临床、大体和组织学表现。17例患者患有加德纳综合征，12例既往腹部手术，6例明显雌激素升高，包括5例孕妇或产后妇女和1例患有男性乳房发育症的酗酒男性。肿瘤通常很大，范围很广。大多数情况下，它们位于小肠肠系膜。其中18例为多例。典型的组织学特征包括致密的胶原间质；突出，扩张，薄壁血管；小动脉肌肉增生；keloidal变化;黏液状的变化;纤维组织渗透到肠固有肌层。虽然在许多肿瘤中都发现有丝分裂，但它们的数量通常很少。临床亚组大体和组织学特征相似；然而，瘢痕样变在女性患者中较少见。不到一半的病例最初得到了正确的诊断。大多数没有Gardner综合征的患者在随访时没有复发，即使病变未完全切除。
We studied the clinical, gross, and histologic findings of 130 fibromatoses of the mesentery and other peritoneal sites. Seventeen patients had Gardner syndrome, 12 had prior abdominal surgery, and six had apparent estrogen elevation, including five pregnant or postpartum women and an alcoholic male with gynecomastia. The tumors were usually large and grossly circumscribed. Most often, they were located in the mesentery of the small bowel. They were multiple in 18 cases. Typical histologic features included a dense, collagenous stroma; prominent, dilated, thin-walled vessels; muscular hyperplasia of small arteries; keloidal change; myxoid change; and fibrous tissue insinuation into the muscularis propria of the bowel. Although mitoses were noted in many tumors, they were usually few in number. The gross and histologic features were similar in the clinical subgroups; however, keloidal change was seen less often in female patients. Less than half of the cases were initially correctly diagnosed. Most patients without Gardner syndrome were without recurrence at follow-up, even when the lesions had been incompletely excised.