Guglielmi M, De Bernardi B, Rizzo A, Federici S, Boglino C, Siracusa F, Leggio A, Cozzi F, Cecchetto G, Musi L, Bardini T, Fagnani A M, Bartoli G C, Pampaloni A, Rogers D, Conte M, Milanaccio C, Bruzzi P
Department of Hematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
J Clin Oncol. 1996 May;14(5):1537-44. doi: 10.1200/JCO.1996.14.5.1537.
To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma.
Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin.
The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected.
Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.
确定切除原发性肿瘤对IV - S期神经母细胞瘤婴儿（年龄0至11个月）的预后是否有有利影响。
1976年3月至1993年12月期间，在21家意大利机构诊断出的97例先前未经治疗的神经母细胞瘤婴儿被归类为IV - S期疾病。70%的婴儿年龄小于4个月。85例有可识别原发性肿瘤的患者中，64例的原发性肿瘤位于肾上腺。肝脏是最常被肿瘤浸润的器官（82例患者），其次是骨髓和皮肤。
5年总生存率（OS）为80%，无事件生存率（EFS）为68%。在24例婴儿中，由于疾病进展迅速导致死亡（n = 8）、无原发性肿瘤（n = 12）或部分切除（n = 4），无法评估原发性肿瘤切除的效果。在73例可评估的患者中，26例在诊断时接受了原发性肿瘤切除：1例死于手术并发症，1例局部复发死亡，另外2例复发（其中1例局部复发）并存活，5年OS率为92%，EFS率为84%。其余47例在诊断时未接受原发性肿瘤切除的患者中有11例发生不良事件，其中5例死亡，OS率为89%，EFS率为75%（与前一组无显著差异）。仅1例死亡患者的原发性肿瘤部位复发，进展或复发的6例幸存者中仅1例复发；在这些患者中，位于纵隔的原发性肿瘤被成功切除。
诊断时接受原发性肿瘤切除的婴儿的预后并不比决定不进行手术的婴儿更好。
