Zanon C, Capozzi M P, Natta F, Alluminio P, Cirigliano W, Ballario F
Divisione di Chirurgia, Ospedale di Chivasso, Torino.
Minerva Chir. 1994 Mar;49(3):211-4.
The paper reports a rare case of malignant ileal schwannoma. A review of the most recent literature confirms the diagnostic and therapeutic procedures used by the authors. Minimal resection or necessary resection in relation to the site and local situation are the only real therapy apart from palliative surgery. It is vital to identify any possible polycentric manifestation since schwannoma is often associated with Recklinghausen's disease. Radiotherapy and chemotherapy are completely ineffective. Responses were obtained in around 30% of cases in the most common trials; the gastrointestinal localization also appears to be even less responsive. The most active drugs are decarbazine, doxorubicin and ifosfamide, associated with CIVADIC or MAID polychemotherapy protocols. It is often difficult to diagnose malignancy; the overall survival rate (taking grading into account) is 7-10% after 10 years and 50% after 5 years for those forms with the lowest degree of malignancy. Antoni classified schwannoma into: type A with a solid component, and type B with a microcystic component. Treatment and prognosis are identical.
本文报道了一例罕见的恶性回肠神经鞘瘤病例。对最新文献的回顾证实了作者所采用的诊断和治疗方法。除姑息性手术外，根据肿瘤部位和局部情况进行的最小切除或必要切除是唯一真正的治疗方法。由于神经鞘瘤常与神经纤维瘤病相关，识别任何可能的多中心表现至关重要。放疗和化疗完全无效。在最常见的试验中，约30%的病例有反应；胃肠道定位的反应似乎更低。最有效的药物是达卡巴嗪、多柔比星和异环磷酰胺，与CIVADIC或MAID联合化疗方案联合使用。恶性肿瘤的诊断往往很困难；考虑分级后，恶性程度最低的类型10年后的总生存率为7 - 10%，5年后为50%。Antoni将神经鞘瘤分为：具有实性成分的A型和具有微囊性成分的B型。治疗和预后相同。
