Pancucci G, Massimi L, Caldarelli M, D'Angelo L, Sturiale C, Tamburrini G, Tufo T, Di Rocco C
Unità di Neurochirurgia Infantile, Università Cattolica del Sacro Cuore, Roma, Italy.
Minerva Pediatr. 2007 Jun;59(3):219-31.
The aim of this study was to analyze the long-term results of the surgical management of craniopharyngioma in children by reviewing a series of patients consecutively treated in a single institution, and to assess the efficacy of intratumoral chemotherapy with interferon-alpha.
Sixty-one paediatric patients (38 males and 23 females; mean age: 8 years) have been surgically treated in the last 20 years. The goal of surgery was to remove the tumour as much as possible. Postoperative radiotherapy was administered only to children aged more than 4 year old and with postsurgical evolutive remnant. Three patients were treated with intratumoral administration of interferon-alpha. The mean follow-up is 8.4 years (median: 7 years).
All the 55 surviving patients enjoy a normal social life, except for 3 of them (visual and/or neurological deficits); endocrine function, compromised in 3/5 of cases, is managed by chronic hormone replacement; neuropsychological assessment is satisfactory in almost all the cases. Although obesity does not seem to be an important social limit, it represents a real management problem. Interferon-a was useful in transitorily arresting the growing cystic craniopharyngiomas.
The current experience confirms the still remarkable challenges in the treatment of craniopharyngioma in childhood. Surgery should aim not only at removing the tumour, but also at preserving the late neuro-endocrinological functions. Intracystic chemotherapy with interferon-alpha might represent an effective option to postpone the surgical operation until the maturation of the hypothalamic-hypophyseal pathway is completed. However, it can not replace the traditional surgical management.
本研究旨在通过回顾在单一机构连续治疗的一系列患者，分析儿童颅咽管瘤手术治疗的长期结果，并评估瘤内注射α-干扰素化疗的疗效。
在过去20年中，61例儿科患者（38例男性，23例女性；平均年龄：8岁）接受了手术治疗。手术目标是尽可能切除肿瘤。仅对4岁以上且术后有肿瘤残留进展的儿童进行术后放疗。3例患者接受了瘤内注射α-干扰素治疗。平均随访时间为8.4年（中位数：7年）。
55例存活患者中，除3例（有视力和/或神经功能缺损）外，其余均能正常社交；3/5的患者内分泌功能受损，通过长期激素替代治疗；几乎所有病例的神经心理学评估结果均令人满意。虽然肥胖似乎并非重要的社交限制因素，但却是一个实际的管理问题。α-干扰素有助于暂时阻止囊性颅咽管瘤的生长。
目前的经验证实，儿童颅咽管瘤的治疗仍面临巨大挑战。手术不仅应旨在切除肿瘤，还应保留晚期神经内分泌功能。瘤内注射α-干扰素化疗可能是一种有效的选择，可将手术推迟至下丘脑-垂体轴成熟后进行。然而，它不能替代传统的手术治疗。
