Behari Sanjay, Banerji Deepu, Mishra Ajay, Sharma Sunil, Sharma Sidhiraj, Chhabra Devendra K, Jain Vijendra K
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Surg Neurol. 2003 Sep;60(3):245-52; discussion 252-3. doi: 10.1016/s0090-3019(03)00132-0.
Craniopharyngiomas constitute 2 to 4% of intracranial neoplasms. However, the purely intraventricular variety are rare. Their magnetic resonance imaging (MRI) characteristically shows an intact third ventricular floor, a patent suprasellar cistern, an intact pituitary stalk, and the absence of sellar abnormalities.
Between 1994 and June 2002, 6 patients with purely intraventricular craniopharyngioma were surgically managed. There were 4 cystic and 2 solid lesions. The surgical approaches utilized included a frontal, parasagittal, transcallosal approach with the third ventricle being accessed using either the transforaminal or subchoroidal approach (n = 3); pterional, transsylvian (n = 1), and bifrontal interhemispheric (n = 2) approaches in which the third ventricle was accessed via the lamina terminalis. A ventriculoperitoneal shunt was required for one of the 3 patients with hydrocephalus.
Total excision was performed in 3 patients with cystic craniopharyngioma, while a small residual lesion was left adherent to the third ventricular floor in the others. There was one perioperative mortality because of septicemia. Two patients required thyroxine supplementation. Two patients developed transient and one other patient a sustained diabetes insipidus. The 2 patients with solid tumors received radiotherapy for the residual lesions. At follow up ranging from 8 to 36 months, neither tumor recurrence nor regrowth was observed in any of the patients. The symptoms of raised intracranial pressure, hypothalamic dysfunction or visual field defects had resolved.
Intraventricular craniopharyngiomas occur in an older population and present mainly with raised intracranial pressure. Visual and endocrinologic imbalances are much less in these lesions compared to the suprasellar craniopharyngiomas. They mainly attach to the third ventricular floor. The surgical approaches to the third ventricle, along with radiotherapy and hormone supplementation, were successful in the management of these rare tumors.
颅咽管瘤占颅内肿瘤的2%至4%。然而，纯脑室内型颅咽管瘤较为罕见。其磁共振成像（MRI）特征性表现为第三脑室底部完整、鞍上池通畅、垂体柄完整且鞍区无异常。
1994年至2002年6月期间，对6例纯脑室内颅咽管瘤患者进行了手术治疗。其中有4例囊性病变和2例实性病变。采用的手术入路包括额部、矢状旁、经胼胝体入路，通过经室间孔或脉络丛下入路进入第三脑室（n = 3）；翼点、经侧裂入路（n = 1）和双额半球间入路（n = 2），通过终板进入第三脑室。3例脑积水患者中有1例需要行脑室腹腔分流术。
3例囊性颅咽管瘤患者实现了全切，其他患者在第三脑室底部留有一小部分残留病灶。有1例患者因败血症在围手术期死亡。2例患者需要补充甲状腺素。2例患者出现短暂性尿崩症，另1例患者出现持续性尿崩症。2例实性肿瘤患者对残留病灶进行了放疗。在8至36个月的随访中，未观察到任何患者出现肿瘤复发或再生长。颅内压升高、下丘脑功能障碍或视野缺损等症状均已缓解。
脑室内颅咽管瘤多见于老年人群，主要表现为颅内压升高。与鞍上颅咽管瘤相比，这些病变的视觉和内分泌失衡情况要少得多。它们主要附着于第三脑室底部。进入第三脑室的手术入路，联合放疗和激素补充，成功地治疗了这些罕见肿瘤。
