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一名患有Ⅰ型神经纤维瘤病的成年患者食管胃交界处存在少年型息肉与神经纤维瘤并存的情况。

Coexistence of Juvenile-Like Polyp with Neurofibroma at the Gastroesophageal Junction in an Adult with Neurofibromatosis Type I.

作者信息

Aijaz Namrah, Draganov Peter, Iqbal Atif, Liu Xiuli

机构信息

Department of Pathology, University of Florida, Gainesville, FL, USA.

Department of Gastroenterology, University of Florida, Gainesville, FL, USA.

出版信息

Case Rep Pathol. 2017;2017:9836759. doi: 10.1155/2017/9836759. Epub 2017 Jan 12.

DOI:10.1155/2017/9836759
PMID:28168076
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5266816/
Abstract

A 23-year-old Caucasian male with Neurofibromatosis Type I (NF-I) was found to have a submucosal nodule at the gastroesophageal junction (GEJ) and underwent endoscopic submucosal dissection. Histological examination revealed two different lesions within the nodule. The dominant lesion was inflammatory/hyperplastic (juvenile-like) polyp with obliterative vasculopathy while the smaller lesion was a neurofibroma. Gastric/GEJ lesions in NF-I are very rare with only seven cases reported in the literature. Three cases of juvenile-like gastric polyps (located in the antrum, greater curvature, and fundus) have been reported in adult NF-I patients. An inflammatory polyp associated with a neurofibroma has only been described once in the pediatric literature but never in an adult. Our case is unique from those previously described in the literature due to the age of the patient, the presence of 2 histologically separate lesions in one endoscopically evident lesion, and the presence of obliterative vasculopathy in the juvenile-like polyp.

摘要

一名患有I型神经纤维瘤病(NF-I)的23岁白种男性在胃食管交界处(GEJ)发现一个黏膜下结节,并接受了内镜下黏膜下剥离术。组织学检查显示结节内有两种不同病变。主要病变为伴有闭塞性血管病的炎症性/增生性(幼年样)息肉,而较小的病变是神经纤维瘤。NF-I患者的胃/GEJ病变非常罕见,文献中仅报道过7例。成人NF-I患者中曾报道过3例幼年样胃息肉(分别位于胃窦、大弯和胃底)。炎症性息肉合并神经纤维瘤仅在儿科文献中有过一次描述,在成人中从未有过报道。我们的病例与文献中先前描述的病例不同,原因在于患者的年龄、在一个内镜可见病变中存在两种组织学上不同的病变以及幼年样息肉中存在闭塞性血管病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/2c186ee09cc0/CRIPA2017-9836759.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/db043dec5151/CRIPA2017-9836759.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/9e272cbaa277/CRIPA2017-9836759.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/09e4a668cf91/CRIPA2017-9836759.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/20e003417c31/CRIPA2017-9836759.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/c691133b9fe4/CRIPA2017-9836759.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/2c186ee09cc0/CRIPA2017-9836759.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/db043dec5151/CRIPA2017-9836759.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/9e272cbaa277/CRIPA2017-9836759.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/09e4a668cf91/CRIPA2017-9836759.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/20e003417c31/CRIPA2017-9836759.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/c691133b9fe4/CRIPA2017-9836759.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f4b2/5266816/2c186ee09cc0/CRIPA2017-9836759.006.jpg

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2
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Gastrointestinal manifestations of neurofibromatosis type 1 (Recklinghausen's disease): clinicopathological spectrum with pathogenetic considerations.
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