肠系膜纤维瘤病的临床及组织病理分析

Clinicopathological analysis of mesenteric fibromatosis

目的总结肠系膜纤维瘤病的临床及组织病理特征。方法回顾性分析9例肠系膜纤维瘤病患者的临床资料。结果4例表现为腹部疼痛,2例扪及腹部肿块,腹部不适、腹部和腰部不适各1例,1例无明显不适;实验室检测发现轻度贫血1例,低钾血症1例,余血常规、肝功能、肾功能、电解质及肿瘤标志物均无明显异常;CT检查显示病灶呈实性、单发,位于空肠系膜4例、回肠系膜3例、结肠系膜2例,肿瘤直径3~22(12.7±6.5)cm,形态不规则,2例与周围组织分界不清,肿瘤周围未见肿大淋巴结、肠系膜血管癌栓,平扫病灶密度较均匀,未见钙化、坏死及出血性改变,增强扫描1例呈均匀强化,8例呈不均匀强化,动脉期、静脉期、延迟期强化程度逐渐增强;9例均行手术治疗,术后组织病理显示肿瘤由大小不同的梭形或肌纤维母细胞组成,其间质内含有大量胶原纤维,免疫组织化学显示9例β-catenin表达为强阳性或阳性,CD117均为阴性、Ki-67阳性细胞数<5%,Desmin、S-100、SMA表达为弱阳性各1例,8例行DOG-1、CD34检测,DOG-1均为阴性,CD34血管阳性2例;随访6~101个月,6例存活,2例失访,1例死亡。结论肠系膜纤维瘤病临床表现及CT影像特征缺乏特异性,细胞核中β-catenin过表达可能对其诊断有重要作用。

Objective To investigate the clinicopathological features of mesenteric fibromatosis.Methods The clinical data of 9 patients with mesenteric fibromatosis were retrospectively analyzed.Results Four patients were presented with abdominal pain,and 2 patients had palpable abdominal mass.Abdominal discomfort,abdominal discomfort and lumbar discomfort were found in 1 patient respectively.Only 1 patient had no obvious clinical symptoms.The laboratory examinations,including routine blood test,liver function,renal function,electrolyte and serum tumor markers were normal in all patients except mild anemia in 1 and hypokalemia in 1,respectively.CT showed the tumors were solid and solitary,and were in mesojejunum in 4 cases,in mesoileum in 3 cases and in mesocolon in 2 cases.The tumors ranged from 3 to 22(12.7±6.5)cm in diameter and were irregular in shape.The tumors in 2 patients were not clearly demarcated from the surrounding tissues,and no enlarged lymph node or mesenteric vascular tumor thrombus was found around the tumors.On the unenhanced CT scan,all tumors were homogeneous in density,and no calcification,necrosis or hemorrhage was found.The enhanced CT scan showed homogeneous enhancement in 1 patient,heterogeneous enhancement in 8 patients,and the enhancement degree of 8 patients continued to increase in the arterial phase,portal venous and delayed phases.All these 9 patients received operation.Postoperative histopathology showed that the tumors were composed of variably spindle cells or myofibroblasts,which were separated by abundant collagen.Immunohistochemistry showed moderate to strong positiveβ-catenin in all patients,but negative CD117.The count of Ki-67 positive cells was less than 5%,while Desmin,S-100 and SMA showed weak positive in 1 patient,respectively.DOG-1 and CD34 were detected in 8 patients,of which DOG-1 was negative in all patients,and CD34 was positive in 2 patients.The follow-up survey for 6 to 101 months showed that 6 patients survived,2 were lost and 1 died.Conclusion Mesenteric fibromatosis lacks specific clinical and CT features.Overexpression ofβ-catenin in the nucleus plays a key role in the diagnosis.