Abstract
BackgroundGastrointestinal stromal tumours are rare (GIST). However, the incidence of GIST among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%. GIST can present clinically in different ways such as abdominal pain, gastrointestinal bleeding and obstruction.Case reportWe present 51 year female patient admitted with Background of neurofibromatosis type 1 admitted with melena. OGD has been done and showed duodenitis with large volume fresh blood in distal duodenum but no obvious bleeding point. Exploratory laparotomy revealed smooth nodular masses on the serosal surface of jejunum. Small bowel resection and side-to-side anastomosis were performed. Histopathoogical examination revealed small bowel gasrointestinal stromal tumour with low risk malignant potential.ConclusionThe incidence of GIST among neurofibromatosis type 1 (NF-1) patients is not uncommon and we should pay attention to gastrointestinal manifestation in such patients.
Highlights
Neurofibromatosis is an autosomal dominant disorder that affects all 3 germinal layers; thereby affecting any organ system
Gastrointestinal stromal tumours are rare (GIST) are usually located in the stomach and small intestine and can present in a variety of different ways ranging from vague symptoms to major G.I. bleeding
The first line treatment is surgical resection for operable GIST and 5year survival ranges from 21% to 88% depending on risk grading and completeness of surgical resection [3,4]
Summary
Neurofibromatosis is an autosomal dominant disorder that affects all 3 germinal layers; thereby affecting any organ system. Gastrointestinal stromal tumours (GIST) are generally rare ; the incidence of GIST among NF-1 patients is approximately 5-25% [2]. GISTs are usually located in the stomach and small intestine and can present in a variety of different ways ranging from vague symptoms to major G.I. bleeding. The first line treatment is surgical resection for operable GIST and 5year survival ranges from 21% to 88% depending on risk grading and completeness of surgical resection [3,4].
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