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巨大侵袭性肠系膜纤维瘤病——病例报告

Giant aggressive mesenteric fibromatosis- a case report.

作者信息

Bn Anandaravi, Cd Jagadish Kumar, Ps Sreejith, M Mayur, Urs Roopa

机构信息

Associate Professor, Department of Surgery, Mysore Medical College and Research Institute , Karnataka, India .

Postgraduate Trainee, Department of Surgery, Mysore Medical College and Research Institute , Karnataka, India .

出版信息

J Clin Diagn Res. 2015 Feb;9(2):PD07-8. doi: 10.7860/JCDR/2015/11061.5594. Epub 2015 Feb 1.

Abstract

Fibromatosis are rare, accounting for 0.03% of all tumours. Mesenteric fibromatosis is a very rare (8% of all desmoid neoplasm). Aggressive fibromatosis of mesentery is a rare surgical problem affecting 2-4 per million people. Females are more commonly affected than males (Estrogen acts as a growth factor). It is locally invasive and tends to recur but never metastasize. Here, we are discussing about 24-year-old male presented with progressive abdomen distension associated with pain since one month. Abdominal examination showed a firm non-tender intra-abdominal mass, measuring around 15x14 cm size, with intrinsic mobility, which was perpendicular to mesenteric line, all borders were well-made out. CECT abdomen showed features suggestive of GIST . Elective Laparotomy was done and a giant mass arising from mesentery without any infiltration to the surrounding structures was noted. The entire mass was excised and mesentery repaired. Histopathology showed uniform band of spindle shaped cells arranged in fascicles admixed with blood vessels in a collagenous stroma. Immunohistochemistry showed Beta Catenin +ve, CD 117-ve, CD 34 -ve and SMA-ve, which is confirmative of Fibromatosis. Postoperative period was uneventful.

摘要

纤维瘤病很罕见,占所有肿瘤的0.03%。肠系膜纤维瘤病极为罕见(占所有硬纤维瘤性肿瘤的8%)。肠系膜侵袭性纤维瘤病是一个罕见的外科问题,每百万人中有2 - 4人受其影响。女性比男性更易受影响(雌激素起生长因子的作用)。它具有局部侵袭性且易于复发,但从不转移。在此,我们讨论的是一名24岁男性,自一个月前起出现进行性腹胀并伴有疼痛。腹部检查发现一个质地硬、无压痛的腹腔内肿块,大小约为15×14厘米,有内在活动度,与肠系膜线垂直,所有边界清晰可辨。腹部增强CT显示有提示胃肠道间质瘤的特征。进行了择期剖腹手术,发现一个源自肠系膜的巨大肿块,未对周围结构有任何浸润。整个肿块被切除,肠系膜得以修复。组织病理学显示梭形细胞呈均匀束状排列,混有血管,位于胶原性基质中。免疫组织化学显示β连环蛋白阳性、CD117阴性、CD34阴性和肌动蛋白阴性,这证实为纤维瘤病。术后过程顺利。

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