Fahlbusch R, Hofmann B M
Department of Neurosurgery, University of Erlangen-Nuremberg, Erlangen, Germany.
Acta Neurochir (Wien). 2008 Dec;150(12):1213-26. doi: 10.1007/s00701-008-0137-9. Epub 2008 Nov 11.
Multimodal treatment in the management of giant craniopharyngiomas (>4 cm in diameter) is necessary to obtain optimal results, and includes conservative or palliative treatment and "aggressive" removal. The significance of a new treatment algorithm including direct surgical resection with the intent to avoid radiation therapy and regrowth will be discussed here.
Between January 1996 and January 2005 16 patients were diagnosed with giant craniopharyngiomas. Two of them underwent only cyst aspiration because of their advanced age and/or lack of improvement of neuropsychological deficits. One patient underwent transsphenoidal operation and in the remaining 13 transcranial surgery was performed. Four additional patients underwent surgery for recurrence. The prospective protocol included pre- and post-operative dynamic endocrine tests, high field 1.5 T MRI and ophthalmological as well as neuropsychological examinations.
In resectable tumours, the rate of total removal was ten out of 12 with two recurrences. In the remaining two patients with recurrences this intention was abandoned because of a firm tumour or a deteriorating neuropsychological status prior to the scheduled additional operation. There was no mortality and the morbidity rate was 6.3%. Visual function improved in 11, was unchanged in one and deteriorated in two patients. Secretion of different adenohypophyseal hormones deteriorated after tumour resection in one to three patients, and new diabetes insipidus occurred in six patients. There was no permanent deterioration of neuropsychological function.
Special reference is given to direct resection of tumours at an optimal timing within this management. If hypothalamic disturbances are absent or improving due to pre-treatment (medical therapy, symptomatic surgery), giant craniopharyngiomas can be surgically removed in more than two of three patients with low morbidity and only moderate deterioration of endocrine function. The latter has to be accepted when curative surgery is intended, but even then, recurrences cannot be prevented. Contraindication for curative surgery is persisting hypothalamic damage necessitating conservative treatment modalities.
对于直径大于4厘米的巨大颅咽管瘤，多模式治疗对于取得最佳治疗效果是必要的，包括保守或姑息治疗以及“积极”切除。本文将讨论一种新的治疗方案的意义，该方案包括直接手术切除，旨在避免放疗和肿瘤复发。
1996年1月至2005年1月期间，16例患者被诊断为巨大颅咽管瘤。其中2例因年龄较大和/或神经心理缺陷无改善而仅接受囊肿抽吸。1例患者接受经蝶手术，其余13例接受开颅手术。另外4例患者因复发接受手术。前瞻性方案包括术前和术后动态内分泌检查、1.5T高场强MRI以及眼科和神经心理检查。
在可切除的肿瘤中，12例中有10例实现全切，2例复发。其余2例复发患者因肿瘤质地硬或在计划的二次手术前神经心理状态恶化而放弃全切意图。无死亡病例，发病率为6.3%。11例患者视力功能改善，1例无变化，2例恶化。1至3例患者肿瘤切除后不同腺垂体激素分泌恶化，6例患者出现新的尿崩症。神经心理功能无永久性恶化。
特别强调在此治疗方案中在最佳时机直接切除肿瘤。如果由于预处理（药物治疗、对症手术）下丘脑功能障碍不存在或正在改善，那么三分之二以上的巨大颅咽管瘤患者可以通过手术切除，发病率低，内分泌功能仅中度恶化。当旨在进行根治性手术时，必须接受后者，但即便如此，也无法预防复发。根治性手术的禁忌症是持续存在下丘脑损伤，需要采取保守治疗方式。
