I型神经纤维瘤病与具有多消化系统定位的间质瘤

Neurofibromatosis Type I and Stromal Tumor with a Multiple Digestive Localization.

作者信息

Chaka Amina, Ennaceur Farouk, Tormen Mohamed Amine, Korbi Ibtissem, Noomen Faouzi, Zouari Khadija

机构信息

Department of General and Digestive Surgery at the University Hospital Fattouma Bourguiba of Monastir, University of Monastir, Tunisia.

出版信息

Case Rep Surg. 2021 Oct 21;2021:2868966. doi: 10.1155/2021/2868966. eCollection 2021.

DOI:10.1155/2021/2868966

PMID:34721919

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8553436/

Abstract

Neurofibromatosis type I (NF1) is also known as von Recklinghausen disease. It is a genetic disorder that affects the growth and development of nerve cell tissue, which is characterized by a multisystem disorder and an increased risk for cancer. The incidence of gastroduodenal stromal tumor during Recklinghausen disease can reach 35% in autopsies and 5% in clinical cases. In our case, the diagnosis of neurofibromatosis type I was made in a middle-aged women initially diagnosed with a pancreaticoduodenal tumor.

摘要

I型神经纤维瘤病（NF1）也被称为冯雷克林霍增氏病。它是一种影响神经细胞组织生长和发育的遗传性疾病，其特征为多系统紊乱和患癌风险增加。在冯雷克林霍增氏病患者中，胃十二指肠间质瘤的尸检发病率可达35%，临床病例中为5%。在我们的病例中，一名最初被诊断患有胰十二指肠肿瘤的中年女性被诊断为I型神经纤维瘤病。