Rollán Villamarín V, García Aroca J, Costa Borrás E, Cuadros García J, Jiménez Alvarez C, Martínez-Caro A, Martínez Ibáñez V, Mulet Ferragut F, Obiols Arderius P, Pérez La Fuente G
Servicio de Cirugía Pediátrica, Hospital Infantil del Niño Jesús, Madrid.
Cir Pediatr. 1994 Oct;7(4):167-70.
A retrospective multicentric study of 23 patients with stage IV-S neuroblastoma treated in 10 national Pediatric Surgery Departments between January 1988 and December 1992, is presented. All cases were classified as stage IV-S following staging criteria proposed by Evans et al. The age diagnosis was an average of 3 months. An acute clinical course was seen in 7 patients. Primary sites of the tumor were: adrenal gland in 6 patients (30.5%), abdominal paravertebral in 3 (13%) and unknown in 2 (9%). Distant tumor sites were: liver in 20 patients, bone marrow in 9, and skin in 5. There were not systematic therapeutic approach, in 16 cases (69%) the primary tumor was resected, 18 (78%) received chemotherapy, the liver was irradiated in 3 (13%), and 1 case (4%) received no treatment at all. One children died. The overall disease-free survival is 95.6% at 43 months post-diagnosis. A common treatment protocol is proposed, with systematic determination of N-myc oncogene which will indicate the appropriate therapy.
本文介绍了一项回顾性多中心研究,该研究对1988年1月至1992年12月期间在10个国家小儿外科科室接受治疗的23例IV-S期神经母细胞瘤患者进行了研究。所有病例均按照埃文斯等人提出的分期标准分类为IV-S期。诊断时的平均年龄为3个月。7例患者出现急性临床病程。肿瘤的原发部位为:6例(30.5%)位于肾上腺,3例(13%)位于腹部椎旁,2例(9%)原发部位不明。远处肿瘤部位为:20例位于肝脏,9例位于骨髓,5例位于皮肤。没有系统的治疗方法,16例(69%)患者切除了原发肿瘤,18例(78%)接受了化疗,3例(13%)对肝脏进行了放疗,1例(4%)根本未接受治疗。1名儿童死亡。诊断后43个月时的总体无病生存率为95.6%。提出了一种通用的治疗方案,即系统测定N-myc癌基因,这将指示适当的治疗方法。