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本文引用的文献

1
Single and hypofractionated stereotactic radiotherapy with CyberKnife for craniopharyngioma.使用 CyberKnife 行单次或低分割立体定向放射治疗颅咽管瘤。
J Neurooncol. 2012 Feb;106(3):571-7. doi: 10.1007/s11060-011-0693-3. Epub 2011 Aug 23.
2
Expression of carbonic anhydrase IX in craniopharyngiomas.颅咽管瘤中碳酸酐酶 IX 的表达。
J Neurosurg. 2011 Oct;115(4):796-801. doi: 10.3171/2011.6.JNS1168. Epub 2011 Jul 15.
3
CTNNB1 gene mutations, pituitary transcription factors, and MicroRNA expression involvement in the pathogenesis of adamantinomatous craniopharyngiomas.CTNNB1 基因突变、垂体转录因子和 MicroRNA 表达参与成釉细胞瘤发病机制。
Horm Cancer. 2010 Aug;1(4):187-96. doi: 10.1007/s12672-010-0041-7.
4
Increased Wingless (Wnt) signaling in pituitary progenitor/stem cells gives rise to pituitary tumors in mice and humans.Wingless(Wnt)信号在垂体祖细胞/干细胞中的增加导致了小鼠和人类的垂体肿瘤。
Proc Natl Acad Sci U S A. 2011 Jul 12;108(28):11482-7. doi: 10.1073/pnas.1101553108. Epub 2011 Jun 2.
5
Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults.丹麦颅咽管瘤发病率(n=189)和儿童及成人颅咽管瘤全球估计发病率。
J Neurooncol. 2011 Sep;104(3):755-63. doi: 10.1007/s11060-011-0540-6. Epub 2011 Feb 19.
6
Phosphorus-32 therapy for cystic craniopharyngiomas.磷-32 治疗囊性颅咽管瘤。
Radiother Oncol. 2011 Feb;98(2):207-12. doi: 10.1016/j.radonc.2010.12.001. Epub 2011 Jan 25.
7
Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series.成人和儿童颅咽管瘤的神经外科治疗:大型病例系列的早期和长期结果。
J Neurosurg. 2011 May;114(5):1350-9. doi: 10.3171/2010.11.JNS10670. Epub 2011 Jan 7.
8
Intracystic treatments for craniopharyngioma.颅咽管瘤囊内治疗。
Neurosurg Focus. 2010 Apr;28(4):E13. doi: 10.3171/2010.1.FOCUS09315.
9
Mortality in patients with pituitary disease.垂体疾病患者的死亡率。
Endocr Rev. 2010 Jun;31(3):301-42. doi: 10.1210/er.2009-0033. Epub 2010 Jan 19.
10
Radiosurgery for craniopharyngioma.颅咽管瘤的放射外科治疗。
Int J Radiat Oncol Biol Phys. 2010 Sep 1;78(1):64-71. doi: 10.1016/j.ijrobp.2009.07.1693. Epub 2009 Dec 16.

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.

DOI:10.3389/fendo.2012.00046
PMID:22654868
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3356097/
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。