Department of Neurosurgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Head Neck. 2012 Jul;34(7):1036-44. doi: 10.1002/hed.21771. Epub 2011 May 16.
Craniopharyngioma is a rare and mostly benign epithelial tumor of the sellar and suprasellar region. Two principal patterns of craniopharyngioma are recognized: papillary and adamantinomatous. Papillary craniopharyngiomas are encountered in adults and may lack the cystic spaces filled with "motor oil" as well as the palisading peripheral rows of epithelial cells, keratinization, or calcification typical of pediatric adamantinomatous craniopharyngioma. Secondary to their anatomic location, craniopharyngiomas may present with endocrinologic dysfunction and visual disturbances. Differential diagnosis includes Rathke's cleft cyst, pituitary adenoma, dermoid/epidermoid cysts, and other rare sellar/suprasellar lesions as pituicytomas. Many controversies exist concerning the preferred surgical approach for these tumors. Endoscopic endonasal surgery is no longer reserved only for sellar or small cystic suprasellar lesions. Prechiasmatic/preinfundibular lesions are effectively removed using an endonasal transtuberculum/transplanum approach; subchiasmatic/transinfundibular tumors require the addition of a transellar approach with inferior pituitary transposition; and retrochiasmatic/retroinfundibular lesions are better accessed performing an endonasal superior pituitary transposition. Compared with well-established trancranial approaches (pterional, subfrontal, presigmoid), endoscopic endonasal surgery combines the virtues of the caudocranial and midline approaches, allowing for appropriate infrachiasmatic exposure without the need for manipulation of surrounding neurovascular structures to access the tumor. This anatomic advantage, combined with high-definition wide-angle visualization, exquisite endonasal microsurgical techniques, and devoted instrumentation facilitates a high rate of endocrine function preservation and visual improvement, while concurrently achieving comparable resections. Endoscopic skull base reconstruction with the vascularized nasoseptal flap has dramatically reduced the incidence of cerebrospinal fluid leak, consolidating endoscopic endonasal surgery as an effective and safe alternative for the treatment of these challenging tumors.
颅咽管瘤是一种罕见的，主要发生于鞍区和鞍上区的良性上皮性肿瘤。颅咽管瘤主要有两种类型：乳头型和造釉细胞瘤型。成人中多见乳头型颅咽管瘤，其特征为缺乏充满“机油样液体”的囊腔、无栅栏状排列的周边上皮细胞、角化或钙化，而这些特征在儿童型造釉细胞瘤型颅咽管瘤中较常见。颅咽管瘤由于其解剖位置的原因，可能导致内分泌功能障碍和视力障碍。鉴别诊断包括 Rathke 裂囊肿、垂体腺瘤、皮样/表皮样囊肿和其他少见的鞍区/鞍上病变如垂体细胞瘤。对于这些肿瘤，手术入路的选择存在许多争议。神经内镜经鼻入路已不再仅限于鞍区或小的囊性鞍上病变。通过经鼻蝶鞍内/视交叉前入路可有效切除视交叉前/视交叉上病变；视交叉下/视交叉内肿瘤需联合经蝶鞍下垂体移位的方法；视交叉后/视交叉后病变可通过经鼻蝶鞍上垂体移位的方法更好地显露。与成熟的经颅入路（翼点、额下入路、乙状窦前入路）相比，神经内镜经鼻入路结合了颅尾和中线入路的优点，可在无需操作周围神经血管结构的情况下进行适当的视交叉下显露，以暴露肿瘤。这种解剖优势，结合高清广角可视化、精细的经鼻内镜显微外科技术和专用的手术器械，有助于提高内分泌功能保留和视力改善率，同时实现类似的肿瘤切除率。带血管鼻中隔-鼻甲瓣的内镜颅底重建显著降低了脑脊液漏的发生率，巩固了神经内镜经鼻入路作为这些具有挑战性肿瘤的有效和安全替代治疗方法的地位。
