Venugopal S, Char G, Duncan N D, Carpenter R
Department of Surgery, U.W.I., Jamaica.
West Indian Med J. 1994 Sep;43(3):89-92.
A retrospective analysis of 26 children with neuroblastic tumours treated at the University Hospital of the West Indies (UHWI) between 1970 and 1991 was undertaken to evaluate factors affecting prognosis. The peak incidence was between 48 months and 60 months of age, and 75% of the deaths occurred in children older than 3 years. The abdomen was the most common site of the tumours 79% of which were from the adrenal gland and carried a dismal outcome. Other sites were thoracic, pelvic and cervical. Extra-adrenal tumours have a good outcome even when the histology is unfavourable; 92% of the tumours were in Stages III or IV at the time of initial presentation. Stage IV disease accounted for all but one of the deaths. Of the 8 children with favourable histology (ganglioneuroma or well-differentiated ganglioneuroblastoma), only one (12.5%) died, wheras of the remaining 18 cases with unfavourable histology (neuroblastoma and undifferentiated ganglioneuroblastoma), eleven (61.1%) died. When managed by surgery alone or with adjuvant chemo- and/or radio-therapy, only 36.4% succumbed while all 4 children with chemotherapy only died. Ten children are alive without disease for more than three years post-therapy. A scoring system was designed which takes into account the factors influencing the outcome in neuroblastic tumours, namely, age, location, stage and histological types of the tumours and therapy. All the children with a score of 21 or less survived, whereas all those with scores of 22 or above succumbed. This underlines the multifactorial influences on the final outcome of neuroblastic tumours.
对1970年至1991年间在西印度群岛大学医院（UHWI）接受治疗的26例神经母细胞瘤患儿进行了回顾性分析，以评估影响预后的因素。发病高峰年龄在48个月至60个月之间，75%的死亡病例发生在3岁以上的儿童中。腹部是肿瘤最常见的部位，其中79%来自肾上腺，预后不佳。其他部位包括胸部、骨盆和颈部。肾上腺外肿瘤即使组织学表现不佳，预后也较好；92%的肿瘤在初次就诊时处于III期或IV期。除1例死亡外，所有死亡病例均为IV期疾病。在8例组织学表现良好（神经节瘤或高分化神经节母细胞瘤）的患儿中，仅1例（12.5%）死亡，而其余18例组织学表现不佳（神经母细胞瘤和未分化神经节母细胞瘤）的患儿中，11例（61.1%）死亡。仅接受手术治疗或联合辅助化疗和/或放疗的患儿中，只有36.4%死亡，而仅接受化疗的4例患儿全部死亡。10例患儿在治疗后无病存活超过三年。设计了一种评分系统，该系统考虑了影响神经母细胞瘤预后的因素，即肿瘤的年龄、位置、分期、组织学类型和治疗方法。所有评分为21分或以下的患儿均存活，而所有评分为22分或以上的患儿均死亡。这突出了神经母细胞瘤最终预后的多因素影响。
