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目的探讨腹内纤维瘤病(IAF)的临床病理特征、诊断与鉴别诊断、治疗及其预后。方法对10例腹内纤维瘤病的临床表现、病理学形态,免疫表型进行分析,并复习相关文献。结果本组男性6例,女性4例,年龄23~77岁,平均49.3岁。以腹部肿块、腹胀及腹痛为主要症状,2例伴有家族性腺瘤性息肉病(FAP),其中1例并发肠腺癌。肿瘤位于肠系膜6例,腹膜后、脾门、膀胱和输尿管下段、胃壁各1例。巨检:灰白色实性肿瘤,质硬。镜检:增生性病变由相对一致的、伸长的梭形细胞排列成编织状及束状,细胞核呈卵圆形、空泡状至尖梭形,1~2个小核仁,缺乏恶性特征,其周有不等量的胶原基质。肿瘤浸润周围脂肪组织和肌层,灶性淋巴细胞浸润伴淋巴滤泡形成。术后3例复发。免疫组化:肿瘤细胞vimentin(+),部分病例SMA、actin和CD117(+),4例中3例β-catenin核(+)。结论 IAF是一种少见的肿瘤,部分病例伴发FAP,细胞核β-catenin阳性具有一定的特异性和敏感性,富于细胞伴有浸润肠壁者应与胃肠间质瘤相鉴别。
Objective To investigate the clinical and pathological features, diagnosis and differential diagnosis, treatment and prognosis of intra-abdominal fibromatosis (IAF). Methods 10 cases of intra-abdominal fibromatosis clinical manifestations, pathological morphology, immunophenotype analysis, and review the relevant literature. Results The group of 6 males and 4 females, aged 23 to 77 years, mean 49.3 years. Abdominal mass, abdominal distension and abdominal pain were the main symptoms. Two cases were associated with familial adenomatous polyposis (FAP), of which 1 was complicated by adenocarcinoma. The tumor was located in 6 cases of mesentery, retroperitoneum, spleen door, lower urinary bladder and ureter, gastric wall in 1 case. Giant check: gray solid tumors, hard. Microscopic examination: Hyperplastic lesions by a relatively consistent, elongated spindle cells arranged in a woven and bundles, the nucleus was oval, vacuolar to spindle fusiform, 1 to 2 small nucleoli, the lack of malignant features There are different amounts of collagen matrix in the weeks. Tumor infiltration around the fatty tissue and muscle, focal lymphocytic infiltration with lymphoid follicles. Three patients relapsed after surgery. Immunohistochemistry: vimentin (+) tumor cells, SMA, actin and CD117 (+) in some cases, and β - catenin nucleus (+) in 4 cases. Conclusions IAF is a rare tumor. Some cases are associated with FAP. The nucleus β-catenin possesses certain specificity and sensitivity. Differentiation of gastrointestinal stromal tumor with rich-cell-associated intestinal wall should be performed.