Losty P, Quinn F, Breatnach F, O'Meara A, Fitzgerald R J
Department of Paediatric Surgery, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Eur J Surg Oncol. 1993 Feb;19(1):33-6.
The role of an aggressive surgical policy in the management of neuroblastoma (NBL) was examined in a retrospective study from a total of 57 patients presenting to a single institution between 1979 and 1989. Surgery consisted of either primary excision of tumour or elective resection following intensive chemotherapy. Two year disease-free survival (DFS) for the entire group was 100% for Stage I patients (n = 2), 86% for Stage II (n = 7), 55% for Stage III (n = 11), 12% for stage IV (n = 33) and 50% for stage IVs (n = 4). In all long-term survivors, surgical excision of primary tumour had been achieved. Elective surgery of primary tumour was not performed in six patients with Stage IV disease; median survival for these patients was eight months compared with 19 months for those other patients with Stage IV disease who did have surgery +/- high dose melphalan and autologous bone marrow rescue. Postoperative complications were documented in 13 of 48 operated patients (27%), emphasising the technical challenges encountered in resection of NBL. Age at presentation and site of primary tumour were major factors affecting prognosis: patients who presented < 1 years of age (n = 15) achieved 80% DFS, > 1 year and < 2 years (n = 12), 33%, and > 2 years (n = 30) 13%; 10 of 11 patients (91%) with primary supradiaphragmatic disease are alive and well compared with 10 out of 46 (22%) with infradiaphragmatic disease (P = 0.01). Based on the experience from this centre, it would appear that surgery can be curative for patients with Stage I, II and III disease but can only, at best, prolong DFS for Stage IV patients. Alternative therapeutic strategies are indicated for this latter group of patients.
在一项回顾性研究中，对1979年至1989年间在某单一机构就诊的57例神经母细胞瘤（NBL）患者进行了分析，以探讨积极手术策略在NBL治疗中的作用。手术方式包括肿瘤原发灶切除或强化化疗后选择性切除。整个队列中，I期患者（n = 2）的两年无病生存率（DFS）为100%，II期患者（n = 7）为86%，III期患者（n = 11）为55%，IV期患者（n = 33）为12%，IVs期患者（n = 4）为50%。所有长期存活者均实现了原发肿瘤的手术切除。6例IV期患者未进行原发肿瘤的选择性手术；这些患者的中位生存期为8个月，而接受手术±大剂量美法仑及自体骨髓挽救的其他IV期患者的中位生存期为19个月。48例接受手术的患者中有13例（27%）记录了术后并发症，这凸显了NBL切除术中遇到的技术挑战。就诊时年龄和原发肿瘤部位是影响预后的主要因素：就诊年龄<1岁的患者（n = 15）DFS为80%，>1岁且<2岁的患者（n = 12）为33%，>2岁的患者（n = 30）为13%；11例原发于膈上疾病的患者中有10例（91%）存活且状况良好，而46例原发于膈下疾病的患者中有10例（22%）存活（P = 0.01）。基于该中心的经验，对于I期、II期和III期疾病患者，手术似乎可以治愈，但对于IV期患者，充其量只能延长DFS。对于后一组患者，需要采取其他治疗策略。
