Bees N R, Ng C S, Dicks-Mireaux C, Kiely E M
Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, London, UK.
Br J Radiol. 1997 Sep;70(837):952-5. doi: 10.1259/bjr.70.837.9486074.
Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a high grade sarcoma with a potential for local recurrence and distant metastasis that may occur at any site in the body where there is neural tissue. MPNST is rare in children and is unreported in the stomach in the paediatric age group. MPNST presents either as an expanding mass, with or without pain, or in the gastrointestinal tract with haemorrhage or obstruction. Many cases occur without evidence of neurofibromatosis but thers is a reported association between MPNST and neurofibromatosis of up to 50%. MPNST has a higher incidence at sites of previous irradiation. Treatment is by complete surgical excision. Radiology is of value in initial diagnosis and staging prior to surgery. The definitive diagnosis is made on the histopathological appearance and immunohistochemical profile. The findings on barium meal, abdominal ultrasound and CT are presented.
恶性神经鞘瘤（恶性周围神经鞘膜瘤，MPNST）是一种高级别肉瘤，具有局部复发和远处转移的可能性，可发生于体内任何存在神经组织的部位。MPNST在儿童中罕见，在儿科年龄组的胃中未见报道。MPNST表现为逐渐增大的肿块，伴有或不伴有疼痛，或在胃肠道表现为出血或梗阻。许多病例发生时无神经纤维瘤病证据，但据报道MPNST与神经纤维瘤病之间的关联高达50%。MPNST在既往接受过放疗的部位发病率较高。治疗方法是完整手术切除。放射学在术前的初步诊断和分期中具有重要价值。最终诊断依据组织病理学表现和免疫组化特征做出。本文展示了钡餐、腹部超声和CT的检查结果。
