Fisher P G, Jenab J, Gopldthwaite P T, Tihan T, Wharam M D, Foer D R, Burger P C
Department of Neurology, Stanford University School of Medicine, Palo Alto, CA 94305-5235, USA.
Childs Nerv Syst. 1998 Oct;14(10):558-63. doi: 10.1007/s003810050272.
Past studies of craniopharyngiomas in children have shown overall survival (OS) up to 95% at 5 years and 80% progression-free survival (PFS) at 5 years, although many of these series predate modern neuroimaging and current therapeutic management. Moreover, little mention has been made of failure patterns for craniopharyngioma in children. To obtain a contemporary assessment of outcome among pediatric craniopharyngioma patients, and also to determine the failure patterns for this tumor, we completed a retrospective study of a consecutive cohort of all children with craniopharyngioma diagnosed at the Johns Hopkins Hospital from 1980 to 1996. Resection was performed in 30 children, in 8 of whom gross total resection (GTR) was achieved. Initial treatment took the form of GTR followed by observation for 8, subtotal resection (STR) plus observation in 11, and STR followed immediately by radiotherapy in 8. The timing of radiotherapy following STR was unclear for 3. OS was 95.2% (SE= 4.7%) at 5 years, with only 2 children dying after 4 years from diagnosis. Five-year PFS was 59.4% (SE=10.2%). Before surgery, 19 children had visual loss and 15, endocrine deficits; after surgery, 21 children had visual loss and 29, endocrine deficits. Median time to relapse was 0.98 years (SD=2.5 years). Radiographic (n=4) and clinical (n=7) relapses did not differ in time to progression (P=0.32), but radiographic relapses were significantly associated with age at diagnosis less than 5 years (P=0.02). Degree of resection was not significantly associated with PFS (P=0.32) or with postoperative visual or endocrine deficits. Absence of calcification on diagnostic neuroimaging (n=8) was significantly associated with improved PFS [5-year PFS 100% vs. 42.9% (SE=14.7%), P=0.02], even when adjusted for extent of resection (P=0.03). Preoperative visual loss was predictive of postoperative visual loss (P=0.03). Survival for children diagnosed with craniopharyngioma in the current era is outstanding, even with relapse, although postoperative visual and endocrinological morbidities are high. Failures occurred both radiographically and clinically, typically in the first 3-4 years after surgery, suggesting a need for close surveillance initially with neuroimaging, particularly in younger children, and also clinical examination. The short times to relapse observed here may stem from a tendency to delay radiotherapy until recurrence. Lack of calcification at diagnosis is associated with a tendency to remain free of relapse.
过去关于儿童颅咽管瘤的研究显示，5年总生存率（OS）高达95%，5年无进展生存率（PFS）为80%，尽管其中许多系列研究早于现代神经影像学和当前的治疗管理方法。此外，很少有人提及儿童颅咽管瘤的失败模式。为了对儿科颅咽管瘤患者的预后进行当代评估，并确定该肿瘤的失败模式，我们对1980年至1996年在约翰霍普金斯医院确诊的所有儿童颅咽管瘤连续队列进行了回顾性研究。30名儿童接受了手术，其中8名实现了全切除（GTR）。初始治疗方式为GTR后观察8例，次全切除（STR）加观察11例，STR后立即放疗8例。3例STR后放疗的时间不明确。5年OS为95.2%（SE = 4.7%），仅有2名儿童在诊断后4年死亡。5年PFS为59.4%（SE = 10.2%）。术前，19名儿童有视力丧失，15名有内分泌缺陷；术后，21名儿童有视力丧失，29名有内分泌缺陷。复发的中位时间为0.98年（SD = 2.5年）。影像学复发（n = 4）和临床复发（n = 7）在进展时间上无差异（P = 0.32），但影像学复发与诊断时年龄小于5岁显著相关（P = 0.02）。切除程度与PFS（P = 0.32）或术后视力或内分泌缺陷无显著相关性。诊断性神经影像学检查无钙化（n = 8）与PFS改善显著相关[5年PFS 100% vs. 42.9%（SE = 14.7%），P = 0.02]，即使在调整切除范围后（P = 0.03）。术前视力丧失可预测术后视力丧失（P = 0.03）。尽管术后视力和内分泌疾病发生率较高，但当代诊断为颅咽管瘤的儿童即使复发，生存率也很高。失败发生在影像学和临床上，通常在术后前3 - 4年，这表明最初需要通过神经影像学密切监测，特别是在年幼儿童中，以及进行临床检查。此处观察到的短复发时间可能源于倾向于将放疗推迟到复发时。诊断时无钙化与无复发倾向相关。
