Sanford R A, Muhlbauer M S
Le Bonheur Children's Medical Center, St. Jude Children's Research Hospital, Memphis, Tennessee.
Neurol Clin. 1991 May;9(2):453-65.
Craniopharyngioma of childhood is a different entity than the tumor found in adulthood. Microscopically adamantinomata tissue is found in children whereas a squamous epithelial origin is more predominant in adults. Partial resection is of little benefit in children because adamantinomous tumors grow at a fast rate. Confidence in a prebiopsy diagnosis of craniopharyngioma provided by modern diagnostic imaging allows the surgeon to choose either radical surgical resection or limited surgery combined with radiotherapy. As aggressive surgery followed by radiotherapy compounds the deleterious effects of the two treatments without any added benefit, this course of action should be avoided when possible. The neurosurgeon should be clear in making a recommendation and plan of action to patients and families. Radical surgery is recommended by the vast majority of pediatric neurosurgeons at academic centers. The long-term survival achieved following radical surgery performed by a highly experienced surgeon may approach the results achieved by limited resection and radiotherapy, but the quality of life issue is left unresolved. Unfortunately the children in series reporting the results of aggressive surgery have not had the benefit of detailed psychologic, IQ, and endocrinologic testing. The major question regarding quality of life with limited resection plus radiotherapy regards the delayed effects of irradiation. Are the complications cumulative with time? The only series with long-term follow-up reporting both treatments overwhelmingly favors limited resection followed by irradiation. This polarization of opinion by the experts needs to be resolved. A true multi-institutional, cooperative study is needed. Unfortunately the bias of potential participants prohibits a randomized study at this time. It is hoped that a prospective, natural history study with detailed psychologic and physiologic testing will give a clearer evaluation of the outcome and standard of care in this country.
儿童颅咽管瘤与成人所患的肿瘤是不同的实体。在显微镜下，儿童颅咽管瘤可见成釉细胞瘤组织，而成人颅咽管瘤则以鳞状上皮起源为主。由于成釉细胞瘤生长迅速，部分切除对儿童益处不大。现代诊断成像技术对颅咽管瘤活检前诊断的信心，使外科医生能够选择根治性手术切除或有限手术联合放疗。由于积极手术加放疗会使两种治疗的有害影响叠加且无额外益处，应尽可能避免这种治疗方案。神经外科医生应向患者及其家属明确提出建议和行动计划。学术中心的绝大多数儿科神经外科医生都推荐根治性手术。由经验丰富的外科医生进行根治性手术后实现的长期生存率可能接近有限切除加放疗的结果，但生活质量问题仍未得到解决。不幸的是，报告积极手术结果的系列研究中的儿童并未受益于详细的心理、智商和内分泌测试。关于有限切除加放疗后的生活质量的主要问题在于放疗的延迟效应。这些并发症会随着时间累积吗？唯一一项对两种治疗方法都进行长期随访报告的系列研究压倒性地支持有限切除后再进行放疗。专家们的这种意见两极分化需要得到解决。需要进行真正的多机构合作研究。不幸的是，潜在参与者的偏见目前阻碍了随机对照研究。希望一项进行详细心理和生理测试的前瞻性自然史研究会对该国的治疗结果和护理标准给出更清晰的评估。
