Bailey S, Skinner R, Lucraft H H, Perry R H, Todd N, Pearson A D
Department of Child Health, University of Newcastle upon Tyne.
Arch Dis Child. 1996 Sep;75(3):181-5. doi: 10.1136/adc.75.3.181.
The records of 38 patients under 25 years of age presenting with pineal tumours between 1968 and 1993, identified from the Northern Region Children's and Young Adults' Malignant Disease Registry, were analysed retrospectively with regards to clinical presentation, diagnostic approach, treatment strategy, and outcome. The overall five year survival was 45%. Fifteen patients had a histological diagnosis: six with germinomas, three with teratomas, three with astrocytomas, and three with pinealoblastomas. One patient had a definitive diagnosis of teratoma made on the basis of raised tumour markers (alpha fetoprotein). Treatment consisted of surgery (87%) (ventriculoperitoneal or atrial shunt and/or biopsy), and/or radiotherapy (82%), and/or chemotherapy (26%). Those patients with a tissue diagnosis appeared to have a more favourable outcome, especially after 1976 when treatment was determined by tumour type (five year survival for those with a tissue diagnosis was 91% v 51% for those without, 95% confidence intervals 74 to 100% and 26 to 75%). This study suggests that tissue diagnosis allows more appropriate treatment to be delivered for children with pineal tumours resulting in improved survival. Referral to a centre with neurosurgery, radiotherapy, neuropathology, and paediatric oncology collaboration is essential.
从北部地区儿童及青年恶性疾病登记处识别出1968年至1993年间38例25岁以下患有松果体肿瘤的患者记录,对其临床表现、诊断方法、治疗策略及预后进行回顾性分析。总体五年生存率为45%。15例患者有组织学诊断结果:6例为生殖细胞瘤,3例为畸胎瘤,3例为星形细胞瘤,3例为松果体母细胞瘤。1例患者根据肿瘤标志物(甲胎蛋白)升高确诊为畸胎瘤。治疗包括手术(87%)(脑室腹腔或心房分流及活检),和/或放疗(82%),和/或化疗(26%)。那些有组织学诊断的患者似乎预后更佳,特别是在1976年之后,治疗根据肿瘤类型确定(有组织学诊断患者的五年生存率为91%,无组织学诊断患者为51%,95%置信区间为74%至100%和26%至75%)。本研究表明,组织学诊断能为松果体肿瘤患儿提供更恰当的治疗,从而提高生存率。转诊至有神经外科、放疗、神经病理学及儿科肿瘤协作的中心至关重要。