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肠道弹力纤维瘤样改变:3例患者4处病变的报告并文献复习

Elastofibromatous change of the intestine: report of four lesions from three patients with review of the literature.

作者信息

Ishida Mitsuaki, Iwai Muneo, Kagotani Akiko, Iwamoto Nozomi, Okabe Hidetoshi

机构信息

Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.

出版信息

Int J Clin Exp Pathol. 2014 Apr 15;7(5):2291-7. eCollection 2014.

Abstract

Elastofibromatous change, also referred to as elastofibromatous polyp or elastofibroma, has been extremely rarely described in the gastrointestinal tract. This lesion is characterized histopathologically by an excessive accumulation of elastic fibers occasionally with a fibrous component involving the submucosa and/or muscularis mucosae of the gastrointestinal tract. Herein, we report four additional lesions of the intestine and review the clinicopathological features of this rare lesion. Three patients (76-, 72-, and 52-year-old males) were detected with polypoid lesions in the jejunum, transverse and sigmoid colons, and sigmoid colon, respectively. All four lesions showed fundamentally the same histopathological and immunohistochemical features. The polypoid lesions were covered by non-neoplastic epithelium, and degenerated and truncated elastic fibers occasionally with a fibrous component had accumulated in the submucosa and/or muscularis mucosae. The characteristic feature was the elastofibromatous change centered around collections of elastotic submucosal vessels. Desmin-positive degenerative ruptured smooth muscle fibers were scattered within the elastic fibers in the submucosa. Our analyses of the clinicopathological features of the previously reported 32 cases of elastofibromatous change of the gastrointestinal tract as well as the present cases demonstrated that this type of lesion is most commonly found in the colon or rectum (29 cases), males, and middle-aged to elderly persons. Although the pathogenesis remains unclear, the convincing hypothesis that this lesion represents elastic degeneration of submucosal vessels by previous persistent vascular injury has been proposed. The collections of degenerative elastotic vascular walls may have an important role in the development of this lesion.

摘要

弹性纤维瘤样改变,也被称为弹性纤维瘤样息肉或弹性纤维瘤,在胃肠道中极为罕见。该病变的组织病理学特征是弹性纤维过度积聚,偶尔伴有纤维成分,累及胃肠道黏膜下层和/或黏膜肌层。在此,我们报告另外4例肠道病变,并回顾这种罕见病变的临床病理特征。3例患者(分别为76岁、72岁和52岁男性)在空肠、横结肠和乙状结肠以及乙状结肠中检测到息肉样病变。所有4个病变在组织病理学和免疫组织化学特征上基本相同。息肉样病变被非肿瘤性上皮覆盖,黏膜下层和/或黏膜肌层中积聚了退化和截断的弹性纤维,偶尔伴有纤维成分。其特征性表现是以弹性变性的黏膜下血管聚集为中心的弹性纤维瘤样改变。结蛋白阳性的退化性破裂平滑肌纤维散在于黏膜下层的弹性纤维内。我们对先前报道的32例胃肠道弹性纤维瘤样改变病例以及本病例的临床病理特征分析表明,这种类型的病变最常见于结肠或直肠(29例)、男性以及中老年人。尽管发病机制尚不清楚,但有人提出了一个有说服力的假说,即这种病变代表先前持续性血管损伤导致的黏膜下血管弹性变性。退化的弹性血管壁聚集可能在该病变的发生发展中起重要作用。

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