Rajan B, Ashley S, Gorman C, Jose C C, Horwich A, Bloom H J, Marsh H, Brada M
Neuro-oncology Unit, Royal Marsden Hospital, Sutton, Surrey, UK.
Radiother Oncol. 1993 Jan;26(1):1-10. doi: 10.1016/0167-8140(93)90019-5.
Between 1950 and 1986 173 patients with craniopharyngioma were treated at the Royal Marsden Hospital with external beam radiotherapy either alone or following surgery. Four patients had complete tumour excision, 21 subtotal and 78 partial resection, 14 had biopsy alone, 34 aspiration alone and 22 had no surgery directed at tumour eradication. Seventy-seven (45%) were children (aged < 16 years). The 10 and 20 year progression-free survival (PFS) rates were 83% and 79%. There were no independent prognostic factors for PFS. The 10 and 20 year survival rates were 77% and 66% at a median follow-up of 12 years. After adjustment for mortality in the normal population, age and technique of radiotherapy (which corresponded with era of treatment) were significant independent prognostic factors for survival. The risk of death (corrected for mortality from natural causes and controlling for radiotherapy technique) for age groups 16-39 and > or = 40 was 0.58 and 0.40 respectively, relative to a risk of 1.0 for the age group < 16 years. Survival and PFS were not influenced by the extent of surgical excision. Visual field defect improved after radiotherapy in 36% of patients (38/106) and visual acuity in 30% (27/91). No patient developed radiation optic neuropathy. We conclude that limited surgery and radiotherapy achieve excellent long-term tumour control and survival with low morbidity.
1950年至1986年间，皇家马斯登医院对173例颅咽管瘤患者进行了单纯外照射放疗或术后外照射放疗。4例患者肿瘤完全切除，21例次全切除，78例部分切除，14例仅行活检，34例仅行抽吸，22例未进行旨在根除肿瘤的手术。77例（45%）为儿童（年龄<16岁）。10年和20年无进展生存率（PFS）分别为83%和79%。无PFS的独立预后因素。中位随访12年时，10年和20年生存率分别为77%和66%。校正正常人群死亡率后，年龄和放疗技术（与治疗时代相对应）是生存的显著独立预后因素。相对于<16岁年龄组1.0的风险，16 - 39岁和≥40岁年龄组的死亡风险（校正自然原因死亡率并控制放疗技术）分别为0.58和0.40。生存和PFS不受手术切除范围的影响。36%（38/106）的患者放疗后视野缺损改善，30%（27/91）的患者视力改善。无患者发生放射性视神经病变。我们得出结论，有限的手术和放疗可实现出色的长期肿瘤控制和生存，且发病率低。
