Gastrointestinal Neurofibromas: Three Cases And Review Of The Literature

Background and Purpose:Neurofibromatosis is an autosomal dominant genetic disease with multi-system damage that derived from abnormal differentiation of nervous crest cells, divided into four types. Gastrointestinal neurofibromas belong to visceral type, which is extremely rare. This article was designed to discuss the clinical symptoms, imaging evidence, pathology, treatment and prognosis of it.Methods:We collected clinical data of 3 patient with Gastrointestinal neurofibromas, in the same period there were 403 cases of neurofibromas, all the patients were treated in the Second Affiliated Hospital, Zhejiang University School of Medicine from January 2000 to February 2011, we discuss the clinical symptoms, imaging evidence pathology, treatment and prognosis of Gastrointestinal neurofibromas through retrospective analysis and literature review.Results:The isolated Gastrointestinal neurofibromas (M:F 3:0)account for 7.4‰in all neurofibromas cases. and the average age was 48.7 years. The main symptoms were abdominal pain and distension. Through the US and CT examination, we found the size of lumps are variable, with the maximum 10 x 5 x 4.5 cm, which is low-grade malignant, all patients were confirmed by pathology and Immunohistochemistry accompanied by S-100+.Conclusion:Gastrointestinal neurofibromas generally associated with NF, the isolated Gastrointestinal neurofibromas is very rare. To make a early diagnosis with he clinical symptoms, imaging evidence, and pathology performance is crucial, because of the probability of malignant change. Contrast to other therapy, surgery is the most important, but there still have the probability of postoperative recurrence and malignant change.