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髓质血管母细胞瘤:单一机构的34例患者。

Medullary hemangioblastoma: 34 patients at a single institution.

作者信息

Yin Luxin, Zhang Liwei, Hao Shuyu, Zhang Junting, Wu Zhen

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili 6, Chongwen District, Beijing 100050, People's Republic of China; Department of Neurosurgery, The Affiliated Hospital of Xu Zhou Medical College, Huaihai Xilu 99, Quanshan District, Xuzhou 221000, People's Republic of China.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili 6, Chongwen District, Beijing 100050, People's Republic of China.

出版信息

J Clin Neurosci. 2014 Feb;21(2):250-5. doi: 10.1016/j.jocn.2013.03.037. Epub 2013 Nov 8.

DOI:10.1016/j.jocn.2013.03.037
PMID:24210696
Abstract

This study aimed to elucidate the surgical experience of medullary hemangioblastoma (MH) at a single institution. We reviewed 34 consecutive patients with MH operated on between January 2005 and June 2012 in the neurosurgery department of the Beijing Tiantan Hospital. There were 14 men and 20 women. The patients were aged from 17 to 60 years with an average age of 38 years. Tumors were cystic in 12 patients (Type A), and solid in 22 patients. The solid tumors were of a small size in six patients (<3 cm, Type B), large in 12 (3.1-5 cm, Type C), and giant in four (>5 cm, Type D). Radical tumor removal was achieved in all patients. Tracheotomy was performed in 10 patients (one Type B patient, seven Type C, two Type D) postoperatively. Pneumonia secondary to lower cranial nerve palsy occurred in six patients (all Type C). Complications including intracranial infection (n=5), gastrointestinal bleeding (n=2), and intracranial hematoma (n=1) also occurred in this group. Follow-up (range, 2-82 months; mean, 30 months) was available in all patients. At follow-up, 29 patients (85.3%) had a good outcome. Twenty-eight of these (82.4%) had an excellent outcome postoperatively (Karnofsky Performance Status ⩾ 80). Although transient surgical complications are possible especially for large solid tumors, total surgical resection can be performed with favorable long-term outcomes with meticulous microsurgical technique and understanding of the vascular pattern of the tumor. Postoperative management of MH is as important as the operation.

摘要

本研究旨在阐明单机构中髓内血管母细胞瘤(MH)的手术经验。我们回顾了2005年1月至2012年6月在北京天坛医院神经外科接受手术的34例连续性MH患者。其中男性14例,女性20例。患者年龄在17至60岁之间,平均年龄为38岁。12例患者的肿瘤为囊性(A型),22例为实性。6例实性肿瘤体积较小(<3 cm,B型),12例体积较大(3.1 - 5 cm,C型),4例为巨大型(>5 cm,D型)。所有患者均实现了肿瘤的根治性切除。10例患者(1例B型患者、7例C型、2例D型)术后进行了气管切开术。6例患者(均为C型)发生了继发于下颅神经麻痹的肺炎。该组还发生了包括颅内感染(n = 5)、消化道出血(n = 2)和颅内血肿(n = 1)在内的并发症。所有患者均进行了随访(范围2 - 82个月;平均30个月)。随访时,29例患者(85.3%)预后良好。其中28例(82.4%)术后预后极佳(卡氏功能状态评分⩾80)。尽管尤其是对于大型实性肿瘤可能会出现短暂的手术并发症,但通过精细的显微外科技术和对肿瘤血管模式的了解,可以实现全切除并获得良好的长期预后。MH的术后管理与手术同样重要。

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