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1型神经纤维瘤病患者出现胃肠道出血的小肠胃肠道间质瘤:治疗与腹腔镜手术。病例报告及文献复习

Small bowel gastrointestinal stromal tumor presenting with gastrointestinal bleeding in patient with type 1 Neurofibromatosis: Management and laparoscopic treatment. Case report and review of the literature.

作者信息

Mandalà S, Lupo M, Guccione M, La Barbera C, Iadicola D, Mirabella A

机构信息

Department of General and Emergency Surgery - Azienda Ospedaliera Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy.

Department of General and Emergency Surgery - Azienda Ospedaliera Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy.

出版信息

Int J Surg Case Rep. 2021 Feb;79:84-90. doi: 10.1016/j.ijscr.2020.12.095. Epub 2021 Jan 6.

DOI:10.1016/j.ijscr.2020.12.095
PMID:33444965
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7808908/
Abstract

INTRODUCTION AND IMPORTANCE

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. It may be asymptomatic; nevertheless, gastrointestinal bleeding is the most frequent symptom, due to mucosal erosion. Its poor lymph node metastatic spread makes GIST often suitable of minimally invasive surgical approach. The importance of this study is to increase the awareness among physicians about this condition in particular scenarios as in our case and to stress the role of laparoscopic surgery.

CASE PRESENTATION

A 74-year-old female patient presented to the emergency department with hematemesis, followed by haematochezia and melena. The patient had a medical history of type 1 Neurofibromatosis (NF1). She underwent, after CT scan, esophagogastroduodenoscopy, and endoscopic haemostasis. Finally, we performed a laparoscopic resection of a mass of the first jejunal loop. The postoperative period was predominantly uneventful. Pathological examination confirmed a low-risk GIST.

CLINICAL DISCUSSION

Proximal jejunal GIST may cause an upper and lower gastrointestinal bleeding. A multidisciplinary team approach is mandatory for the correct management of this disease and its complications (bleeding). GISTs are indicated as the most commonly gastrointestinal NF1 associated tumours. In case of localised and resectable GIST surgical treatment is the mainstay and laparoscopic surgery is a valid alternative.

CONCLUSION

In case of abdominal bleeding mass in a NF1 patient, it is important to keep in mind the well-known association between NF1 and GIST to facilitate the diagnosis and to quickly perform the appropriate treatment. Laparoscopic approach is safe and effective if the oncological radicality is respected.

摘要

引言与重要性

胃肠道间质瘤(GIST)是胃肠道最常见的间叶性肿瘤。它可能无症状;然而,由于黏膜糜烂,胃肠道出血是最常见的症状。其淋巴结转移率低使得GIST通常适合采用微创手术方法。本研究的重要性在于提高医生对我们病例这种特殊情况下该疾病的认识,并强调腹腔镜手术的作用。

病例介绍

一名74岁女性患者因呕血就诊于急诊科,随后出现便血和黑便。该患者有1型神经纤维瘤病(NF1)病史。在进行CT扫描、食管胃十二指肠镜检查和内镜止血后,我们最终对空肠第一段的一个肿块进行了腹腔镜切除。术后恢复过程基本顺利。病理检查证实为低风险GIST。

临床讨论

空肠近端GIST可能导致上、下消化道出血。对于该疾病及其并发症(出血)的正确管理,多学科团队方法是必不可少的。GIST被认为是最常见的与胃肠道NF1相关的肿瘤。对于局限性且可切除的GIST,手术治疗是主要方法,腹腔镜手术是一种有效的替代方法。

结论

对于NF1患者出现腹部出血性肿块的情况,重要的是要牢记NF1与GIST之间的已知关联,以促进诊断并迅速进行适当治疗。如果尊重肿瘤根治性,腹腔镜手术方法是安全有效的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/38cead74766d/gr8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/4113a0871f25/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/0bb399a5b552/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/8cac17d8de55/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/34b6796d0a43/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/ba67ef4415eb/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/eed42b974f0f/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/d3a84ab5f4d7/gr7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/38cead74766d/gr8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/4113a0871f25/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/0bb399a5b552/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/8cac17d8de55/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/34b6796d0a43/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/ba67ef4415eb/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/eed42b974f0f/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/d3a84ab5f4d7/gr7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9eb/7808908/38cead74766d/gr8.jpg

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