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成人罕见无症状巨大脑海绵状血管畸形:两例报告及文献综述

Rare asymptomatic giant cerebral cavernous malformation in adults: two case reports and a literature review.

作者信息

Wang Zhen, Hu Junwen, Wang Chun

机构信息

Department of Neurosurgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China.

出版信息

J Int Med Res. 2020 Dec;48(12):300060520926371. doi: 10.1177/0300060520926371.

DOI:10.1177/0300060520926371
PMID:33307903
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7739106/
Abstract

Cavernous malformations are benign vascular malformations. Giant cavernous malformations are very rare. All reported cases have been symptomatic because of the large size and compression of the surrounding brain tissue. We report two asymptomatic cases of giant cavernous malformation that were both misdiagnosed as neoplasms because of their atypical presentations. The first case was a 54-year-old man whose computed tomography and magnetic resonance imaging scans revealed an inhomogeneous lesion of 6 cm diameter and mild enhancement in the left frontal lobe. A left lateral supraorbital and transcortical approach was applied and the lesion was completely removed. The second case was a 36-year-old man with an irregular large mass in the parasellar region. Craniopharyngioma was suspected and gross total resection was performed. Post-surgical pathological analyses confirmed the diagnoses as cavernous malformations. Both patients recovered uneventfully. The rare asymptomatic giant cavernous malformations reported here in adults had benign behavior for this specific disease entity. The different clinical characteristics of ordinary cavernous malformation and adult and pediatric giant cavernous malformation imply complex and distinct genetic backgrounds. Concerns should be raised when considering giant cavernous malformation as a differential diagnosis for atypical large lesions. Surgical resection is recommended as the primary treatment option.

摘要

海绵状血管畸形是良性血管畸形。巨大海绵状血管畸形非常罕见。所有已报道的病例均因病灶体积大及对周围脑组织的压迫而出现症状。我们报告两例无症状的巨大海绵状血管畸形病例,因其非典型表现均被误诊为肿瘤。第一例为一名54岁男性,其计算机断层扫描和磁共振成像扫描显示左侧额叶有一个直径6 cm的不均匀病灶,并有轻度强化。采用左侧眶上外侧经皮质入路,病灶被完全切除。第二例为一名36岁男性,鞍旁区域有一个不规则的大肿块。怀疑为颅咽管瘤并进行了全切除。术后病理分析确诊为海绵状血管畸形。两名患者均顺利康复。本文报道的成人罕见无症状巨大海绵状血管畸形在该特定疾病实体中表现为良性。普通海绵状血管畸形与成人及儿童巨大海绵状血管畸形的不同临床特征提示其遗传背景复杂且不同。在将巨大海绵状血管畸形作为非典型大病灶的鉴别诊断时应予以关注。建议手术切除作为主要治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f5a/7739106/12066d03131e/10.1177_0300060520926371-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f5a/7739106/fa46eaef4394/10.1177_0300060520926371-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f5a/7739106/12066d03131e/10.1177_0300060520926371-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f5a/7739106/fa46eaef4394/10.1177_0300060520926371-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f5a/7739106/12066d03131e/10.1177_0300060520926371-fig2.jpg

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