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巨大的颅鼻型及囊实性颅咽管瘤,伴有广泛骨质侵蚀和骨化。

Giant cranionasal and cystic-solid craniopharyngioma associated with extensive bone erosion and ossification.

作者信息

Chen Haifeng, Zhou Liangxue, Luo Linli, Jiang Su, Guo Gang, You Chao

机构信息

Department of Neurosurgery, West China Hospital, Chengdu, China.

出版信息

J Craniofac Surg. 2013 Jul;24(4):e398-401. doi: 10.1097/SCS.0b013e318280249f.

Abstract

Craniopharyngioma (CP), a rare benign and slow-growing epithelial tumor, is mainly located within the sellar/parasellar region. Primary CP involving the nasal cavity and the sellar region with extensive erosion of the skull base and ossification simultaneity has not been described previously. The authors report a 23-year-old man who presented to our institute with complaints of repeated nasal cavity bloodshed, liquid flow, and progressive visual loss. A neuroimaging examination showed a giant cranionasal and cystic-solid CP extending from the suprasellar region to the nasopharynx with inhomogeneous enhancement, which is associated with extensive erosion of the skull base and ossification. The patient underwent a transsphenoidal surgery to resect the nasopharyngeal component of CP and a subfrontal craniotomy with a total removal of intracranial component by grinding 3 months later. A histopathologic examination revealed characteristic features of adamantinomatous CP associated with ossification. The current study demonstrates that CP can exhibit cranionasal growth pattern and arise from residue of craniopharyngeal duct. Extensive erosion of the skull base, calcification, and ossification can present in tumor simultaneity. A 2-stage stratagem is important for its total removal because of the peculiar hardness. Postsurgical course is unevenly and should be dealt with carefully.

摘要

颅咽管瘤(CP)是一种罕见的良性、生长缓慢的上皮性肿瘤,主要位于鞍区/鞍旁区域。以前尚未描述过原发性CP累及鼻腔和鞍区并同时伴有广泛颅底侵蚀和骨化的情况。作者报告了一名23岁男性,他因反复鼻腔出血、流涕及进行性视力丧失前来我院就诊。神经影像学检查显示一个巨大的颅鼻型囊实性CP,从鞍上区域延伸至鼻咽部,强化不均匀,伴有广泛的颅底侵蚀和骨化。患者接受了经蝶窦手术切除CP的鼻咽部成分,3个月后行额下入路开颅手术,通过磨除完全切除颅内成分。组织病理学检查显示为伴有骨化的成釉细胞瘤型CP的特征性表现。当前研究表明,CP可呈现颅鼻生长模式,起源于颅咽管残余。肿瘤可同时出现广泛的颅底侵蚀、钙化和骨化。由于其硬度特殊,分两阶段的策略对其完全切除很重要。术后病程不一,应谨慎处理。

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