Acute GI bleeding by multiple jejunal gastrointestinal autonomic nerve tumour associated with neurofibromatosis type I

We describe a surgical emergency due to GI-bleeding caused by gastrointestinal autonomic nerve tumours (GANT’s) in a pa tient with von Recklinghausen’s disease. A 72 year old female patient with von Recklinghausen’s dis ease was admitted with maelena. Endoscopy showed no active bleeding in the stomach and the colon. Therefore an angio-CTscan was performed which revealed masses of the proximal je junum as source of bleeding. Laparotomy was indicated and a 20 cm segment of jejunum which carried multiple extraluminal tu mours was resected. The source of the bleeding was a 2 cm tumour which had eroded the mucosal surface. Immunohistologically, evidence of neuronal differentiation could be shown in the spindle-formed cells with positive staining for C-Kit (CD 117), CD 34, and a lo cally positive staining for synaptophysine and S100. This case report illustrates the association between neurofibro matosis and stromal tumours and should alert surgeons and gas troenterologist about gastrointestinal manifestations in patients with von Recklinghausen’s disease.