Solitary intestinal neurofibroma with no associated systemic syndromes causing intussusception: Case report and literature review

孤立性肠神经纤维瘤伴无相关全身综合征引起肠套叠:病例报告及文献复习

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Abstract

INTRODUCTION: The isolated presence of neurofibromatous lesions in the gastrointestinal tract, with no associated systemic syndromes, is a rarely reported clinical entity. PRESENTATION OF CASE: A 48-year-old lady, with no history of neurofibromatosis or other systemic disease, presented with small bowel obstruction secondary to an ileo-ileal intussusception induced by an isolated ileal neurofibromatous mass. The patient underwent a segmental enterectomy and after a smooth recovery, she was put on a long-term follow-up schedule. DISCUSSION: This article presents a review of the literature of this area clinical entity. Very few reports of gastrointestinal isolated neurofibromas could be found. Similarly, extra-digestive isolated lesions have been rarely reported. CONCLUSION: Isolated ileal neurofibroma is a rare pathological entity. The clinical significance of such a diagnosis lies mainly in the need of further follow up of these patients as the bowel involvement could be the first manifestation of neurofibromatosis type 1 or multiple endocrine neoplasia type 2b.

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