Evans A E, Baum E, Chard R
Arch Dis Child. 1981 Apr;56(4):271-4. doi: 10.1136/adc.56.4.271.
The study reports the results of treatment in 31 infants under age 12 months with stage IV-S neuroblastoma diagnosed between 1976 and 1979. All had small or undetected primary tumours associated with disease in one or more of the following sites: liver, skin, or marrow. The primary site was left adrenal (in 16), and other areas included paravertebral, mediastinum, and pelvis (in 5); in 3 the primary site could not be found. Distant disease was found in the liver (in 29), marrow (in 16), and skin (in 8). Other sites affected were pancreas, pleura, peritoneum, and regional nodes. Treatment varied according to the clinical course of the disease; most patients had very little. In 19 primary tumour was resected, in 21 the liver was irradiated with a median dose of 450 rad, and 15 received chemotherapy in courses varying between 1 month and 1 year. Nine patients had resection of the primary tumour as their only treatment and all survive; a total of 16 patients had sites of disease which regressed spontaneously. Four of 31 patients died in the first 2 months despite vigorous measures, all from some complication of the disease or its treatment. The projected 2-year survival rate is 87%. Children with this 'special' pattern of widespread neuroblastoma fare well with little or no treatment unless early complications develop. In this study none died of late progression of their disease.
该研究报告了1976年至1979年间诊断出的31例12个月以下IV-S期神经母细胞瘤婴儿的治疗结果。所有患儿均有小的或未被发现的原发性肿瘤,并伴有以下一个或多个部位的疾病:肝脏、皮肤或骨髓。原发部位为左肾上腺(16例),其他部位包括椎旁、纵隔和骨盆(5例);3例未发现原发部位。远处转移见于肝脏(29例)、骨髓(16例)和皮肤(8例)。其他受累部位为胰腺、胸膜、腹膜和区域淋巴结。治疗根据疾病的临床进程而异;大多数患者接受的治疗很少。19例患者切除了原发性肿瘤,21例患者接受了肝脏照射,中位剂量为450拉德,15例患者接受了1个月至1年不等疗程的化疗。9例患者仅接受了原发性肿瘤切除术,全部存活;共有16例患者的疾病部位自发消退。31例患者中有4例在最初2个月内死亡,尽管采取了积极措施,均死于疾病或其治疗的某种并发症。预计2年生存率为87%。患有这种“特殊”广泛型神经母细胞瘤模式的儿童,除非出现早期并发症,否则很少或无需治疗就能预后良好。在本研究中,没有患者死于疾病的晚期进展。