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成人中的儿科肿瘤:皇家马斯登医院1974 - 1990年的经验

Paediatric tumours in the adult population: the experience of the Royal Marsden Hospital 1974-1990.

作者信息

Moody A M, Norman A R, Tait D

机构信息

Department of Radiotherapy, Royal Marsden Hospital, Sutton, Surrey, United Kingdom.

出版信息

Med Pediatr Oncol. 1996 Mar;26(3):153-9. doi: 10.1002/(SICI)1096-911X(199603)26:3<153::AID-MPO2>3.0.CO;2-J.

Abstract

Adult patients (greater than 18 years), referred to the Royal Marsden Hospital between 1974 and 1990 with embryonal tumours, have been reviewed. The aim of the study was to document the presentation, management and outcome for this group of patients and to compare these parameters with those of tumours of the same histology arising in the paediatric population. The study population consisted of 15 patients with medulloblastoma, 15 with Ewing's sarcoma, three with neuroblastoma, seven with rhabdomyosarcoma and two with nephroblastoma. Actuarial survival, at 5 years, for adults with medulloblastoma was 80%, which compares very favourably with the outcome for children treated over the same time span. In addition, salvage therapy after relapse was in some cases successful. In the Ewing's sarcoma group the outcome was less favourable, with 5-year actuarial survival of 50%. This is disappointing in view of the lack of tumours with poor prognostic features and may be an area in which these tumours differ from those that arise in children. The number of patients with the diagnosis of neuroblastoma, rhabdomyosarcoma and Wilms' tumour was too small for statistical analysis and they are presented as case reports. Embryonal tumours arising in adults provide an opportunity to study clinical behaviour and biology from an extreme standpoint. This may provide useful information with regard to aetiology, natural history and treatment response. The establishment of registers facilitate the collection of relevant data and also offers the opportunity to improve the treatment received by patients with these rare tumours.

摘要

对1974年至1990年间转诊至皇家马斯登医院的成年患者(年龄大于18岁)中的胚胎性肿瘤患者进行了回顾性研究。本研究的目的是记录该组患者的临床表现、治疗及预后情况,并将这些参数与儿科人群中相同组织学类型肿瘤的参数进行比较。研究人群包括15例髓母细胞瘤患者、15例尤因肉瘤患者、3例神经母细胞瘤患者、7例横纹肌肉瘤患者和2例肾母细胞瘤患者。成年髓母细胞瘤患者的5年精算生存率为80%,与同期接受治疗的儿童患者的预后相比非常有利。此外,复发后的挽救治疗在某些情况下是成功的。在尤因肉瘤组中,预后较差,5年精算生存率为50%。鉴于缺乏预后不良特征的肿瘤,这一结果令人失望,这可能是这些肿瘤与儿童肿瘤不同的一个方面。神经母细胞瘤、横纹肌肉瘤和威尔姆斯瘤的诊断患者数量太少,无法进行统计分析,因此作为病例报告呈现。成人发生的胚胎性肿瘤为从极端角度研究临床行为和生物学提供了机会。这可能为病因学、自然史和治疗反应提供有用信息。建立登记册有助于收集相关数据,也为改善这些罕见肿瘤患者的治疗提供了机会。

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