Journal article
Gastrointestinal neurofibromatosis: an unusual cause of gastric outlet obstruction
The American surgeon, v 71(2)
Feb 2005
PMID: 16022006
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Neurofibromatosis type-1 (NF-1), also known as von Recklinghausen disease, is a common autosomal dominant condition occurring in approximately 1/3000 births. NF-1 is known to be associated with gastrointestinal neoplasms in 2-25 per cent of patients. We report the first case of gastric outlet obstruction with perforation caused by neurofibroma in a patient with NF-1. The literature is reviewed, examining 61 previously reported cases of noncarcinoid gastrointestinal (GI) neoplasms in patients with NF-1 for symptoms, location, and types of neoplasms. Neoplasms were located most often in the small intestine (72%). Neurofibromas, found in 52 per cent of patients, were the most frequently diagnosed benign neoplasms followed by leiomyomas (13%), ganglioneurofibromas (9.8%), and gastrointestinal stomal tumor (GIST) (6.5%). Adenocarcinoma was present in 23 per cent of patients. Patients with NF-1 and GI symptoms are at risk for gastrointestinal neoplasms from which symptomatic patients are likely to experience significant morbidity.
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Details
- Title
- Gastrointestinal neurofibromatosis: an unusual cause of gastric outlet obstruction
- Creators
- Jennifer R Bakker - Graduate HospitalMarian M Haber - Drexel UniversityFernando U Garcia - Drexel University
- Publication Details
- The American surgeon, v 71(2)
- Publisher
- Sage
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pathology (and Laboratory Medicine)
- Web of Science ID
- WOS:000227621700002
- Scopus ID
- 2-s2.0-22844434867
- Other Identifier
- 991019168989904721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Surgery