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撒哈拉以南非洲国家的高危神经母细胞瘤:如实相告。

High-risk neuroblastoma in a sub-Saharan African country: telling it like it is.

作者信息

Hadley G P, van Heerden J

机构信息

1 Professor Emeritus, Department of Paediatric Surgery, University of KwaZulu-Natal, Durban, South Africa.

2 Paediatric Oncologist, Paediatric Haematology and Oncology Service, School of Paediatrics and Child Health, University of KwaZulu Natal, Pietermaritzburg, South Africa.

出版信息

Trop Doct. 2017 Oct;47(4):370-374. doi: 10.1177/0049475517704363. Epub 2017 Apr 12.

DOI:10.1177/0049475517704363
PMID:28403698
Abstract

Neuroblastoma is uncommon in Africa, but when seen usually presents as high-risk disease with a poor prognosis. This aggressive biology of the tumour is frequently augmented by delayed presentation. Current treatment depends upon technologies and skills that are scarce in developing countries and the cost involved is generally beyond the means of healthcare providers who are faced with a myriad more pressing healthcare issues. The presentation, treatment and outcome of 45 African children with neuroblastoma are described. Due to a lack of resources precise risk stratification was impossible but visceral or bone metastases were present in 73% of patients at diagnosis. In 91% the primary tumour was intra-abdominal. Three children (7%) were paraplegic on admission. A localised tumour was seen in one child (2%). Fifteen children (33%) underwent a surgical procedure, with intent to cure in five among whom resection was incomplete in three. For all other children, treatment was palliative using chemotherapy with judicious use of radiotherapy. Thirteen children (29%) survived longer than six months. Overall survival at three years was 4%.

摘要

神经母细胞瘤在非洲并不常见,但一旦出现,通常表现为高危疾病,预后较差。肿瘤这种侵袭性生物学行为常常因就诊延迟而加剧。目前的治疗依赖于发展中国家稀缺的技术和技能,而且所涉及的费用通常超出了面临众多更紧迫医疗问题的医疗服务提供者的承受能力。本文描述了45例非洲神经母细胞瘤患儿的临床表现、治疗及预后情况。由于缺乏资源,无法进行精确的风险分层,但73%的患者在诊断时存在内脏或骨转移。91%的原发肿瘤位于腹腔内。三名患儿(7%)入院时截瘫。一名患儿(2%)可见局限性肿瘤。15名患儿(33%)接受了手术,其中五例旨在治愈,三例切除不完全。对于所有其他患儿,采用化疗并酌情使用放疗进行姑息治疗。13名患儿(29%)存活超过6个月。三年总生存率为4%。

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