作者: Leila Ghaharamani , Ali Bananzadeh , Waheeb Radman Al-Kubati , Seyed Hosseini
DOI:
关键词: Peripheral Nerve Sheath 、 Medicine 、 Neurofibroma 、 Surgery 、 Neurofibromatosis 、 Pelvic pain 、 Stomach 、 Connective tissue 、 Enteropathy 、 Intussusception (medical disorder)
摘要: Neurofibromatosis type-1 (NF1), also known as Von Recklinghausen’s disease, is an autosomal dominant disorder with incidence of one in 4,000. Neurofibromas are benign, heterogeneous, peripheral nerve sheath tumours arising from the connective tissue sheaths, especially endometrium. Visceral involvement disseminated neurofibromatosis considered rare. Neurofibroma occurs most frequently stomach and jejunum, but colon anorectal canal may be involved. Gastrointestinal neurofibromas lead to bleeding, obstruction, intussusception, protein-losing enteropathy bowel perforation. We encountered a difficult case huge neurofibroma involving area. The patient presented pelvic pain, watery diarrhoea urgency.