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[巨大肠系膜纤维瘤病:一例报告]

[Giant mesenteric fibromatosis: a case report].

作者信息

Cianchi F, Perigli G, Pucciani F, Nesi G, Amorosi A

机构信息

Università degli Studi di Firenze, Clinica Chirurgica Generale e Terapia Chirurgica.

出版信息

Ann Ital Chir. 1995 Jul-Aug;66(4):531-5.

PMID:8687006
Abstract

The fibromatoses represent a broad group of fibroblastic proliferations characterized by infiltrative growth and a tendency toward recurrence but, unlike malignant fibrous tumors, they do not metastasize. Mesenteric fibromatosis arising from the mesentery of the small bowel is rare. It may be sporadic or may occur in association with polyposis coli and other soft tissue tumors as a component of Gardner's syndrome. We report a case of mesenteric fibromatosis in a 52-year-old man with no evidence of Gardner's syndrome. The neoplasm occupied the whole abdominal cavity, weighing 12 kg, with the greatest diameter being 50 cm. Histological findings (i.e moderate degree of cellularity, lack of nuclear pleomorphism and mitotic figures) allowed to rule out malignancy. Surgical removal is actually the only effective treatment for mesenteric fibromatosis. Excision must be as wide as possible in order to prevent local recurrence. Until now, no satisfactory results have been obtained with external radiotherapy. More recently, anti-inflammatory drugs have been used in the management of this tumor.

摘要

纤维瘤病是一组广泛的成纤维细胞增殖性疾病,其特征为浸润性生长且有复发倾向,但与恶性纤维性肿瘤不同的是,它们不会发生转移。起源于小肠系膜的肠系膜纤维瘤病较为罕见。它可能是散发性的,也可能作为加德纳综合征的一部分,与结肠息肉病及其他软组织肿瘤相关联发生。我们报告一例52岁男性的肠系膜纤维瘤病病例,该患者无加德纳综合征证据。肿瘤占据整个腹腔,重达12千克,最大直径为50厘米。组织学检查结果(即细胞密度中等、无核异型性及有丝分裂象)可排除恶性肿瘤。手术切除实际上是肠系膜纤维瘤病唯一有效的治疗方法。切除范围必须尽可能广泛,以防止局部复发。到目前为止,外照射放疗尚未取得满意效果。最近,抗炎药物已被用于该肿瘤的治疗。

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