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成人颅咽管瘤。

Craniopharyngioma in adults.

机构信息

Division of Endocrinology and Diabetes, Department of Medicine 1, Friedrich-Alexander University Erlangen-Nuremberg Erlangen, Germany.

出版信息

Front Endocrinol (Lausanne). 2012 Mar 29;3:46. doi: 10.3389/fendo.2012.00046. eCollection 2012.

Abstract

Craniopharyngiomas are slow growing benign tumors of the sellar and parasellar region with an overall incidence rate of approximately 1.3 per million. During adulthood there is a peak incidence between 40 and 44 years. There are two histopathological types, the adamantinomatous and the papillary type. The later type occurs almost exclusively in adult patients. The presenting symptoms develop over years and display a wide spectrum comprising visual, endocrine, hypothalamic, neurological, and neuropsychological manifestations. Currently, the main treatment option consists in surgical excision followed by radiation therapy in case of residual tumor. Whether gross total or partial resection should be preferred has to be balanced on an individual basis considering the extent of the tumor (e.g., hypothalamic invasion). Although the overall long-term survival is good it is often associated with substantial morbidity. Preexisting disorders are often permanent or even exacerbated by treatment. Endocrine disturbances need careful replacement and metabolic sequelae should be effectively treated. Regular follow-up by a multidisciplinary team is a prerequisite for optimal outcome of these patients.

摘要

颅咽管瘤是鞍区和鞍旁区生长缓慢的良性肿瘤,总体发病率约为每百万人口 1.3 例。在成年期,发病率峰值在 40 至 44 岁之间。有两种组织病理学类型,即造釉细胞瘤型和乳头型。后者几乎仅发生于成年患者。临床表现为多年逐渐出现,表现为广泛的包括视觉、内分泌、下丘脑、神经和神经心理学等方面的症状。目前,主要的治疗方法是手术切除,如果有肿瘤残留则进行放疗。是否应该选择全切除或部分切除,需要根据肿瘤的范围(如下丘脑侵犯程度)进行个体化平衡。尽管总体长期生存率较好,但往往与较高的发病率相关。治疗前已有的疾病通常是永久性的,甚至会因治疗而加重。内分泌紊乱需要仔细替代,代谢后遗症应有效治疗。多学科团队的定期随访是这些患者获得最佳结果的前提。

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