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颅内轴内脑簇状血管瘤:病例报告。

Intracranial intraaxial cerebral tufted angioma: case report.

机构信息

Departments of1Neurological Surgery.

2Pathology and Cell Biology.

出版信息

J Neurosurg. 2018 Feb;128(2):524-529. doi: 10.3171/2016.10.JNS162207. Epub 2017 Feb 24.

Abstract

Tufted angioma (TA) is a rare, slow-growing, vascular lesion that commonly presents as a solitary macule, papule, or nodule arising in the soft tissues of the torso, extremities, and head and neck in children and young adults. Adult-onset cases have been infrequently reported. While typically benign, TAs may be locally aggressive. Complete physical examination and hematological workup are recommended in patients with TA to exclude the presence of Kasabach-Merritt phenomenon (KMP). The authors describe the case of a 69-year-old man with a contrast-enhancing frontal lobe lesion, with surrounding vasogenic edema, which was treated by gross-total resection. Characteristic histological features of a TA were demonstrated, with multiple cannonball-like tufts of densely packed capillaries emanating from intraparenchymal vessels in cerebral cortex and adjacent white matter. Tumor recurrence was detected after 4 months and treated with adjuvant Gamma Knife radiosurgery. To the extent of the authors' knowledge, this case illustrates the first report of TA presenting in an adult as an intracranial intraaxial tumor without associated KMP. The fairly rapid regrowth of this tumor, requiring adjuvant treatment after resection, is consistent with a potential for locally aggressive growth in a TA occurring in the brain.

摘要

丛状血管瘤(TA)是一种罕见的、生长缓慢的血管病变,常见于儿童和年轻成人的躯干、四肢和头颈部的软组织中,表现为单个斑疹、丘疹或结节。成人发病的病例少见。虽然通常为良性,但 TA 可能具有局部侵袭性。建议对 TA 患者进行全面的体格检查和血液学检查,以排除卡-梅现象(KMP)的存在。作者描述了一名 69 岁男性的病例,该患者有一个增强的额叶病变,伴有周围的血管源性水肿,病变通过大体全切除进行治疗。显示出 TA 的特征性组织学特征,多个炮弹状的密集毛细血管丛从脑皮质和相邻的白质内的脑实质内血管发出。4 个月后发现肿瘤复发,并接受辅助伽玛刀放射外科治疗。据作者所知,该病例首次报道了成人颅内轴内肿瘤,无相关 KMP。这种肿瘤在切除后需要辅助治疗,且相当快速地复发,这与发生在大脑中的 TA 具有局部侵袭性生长的潜在风险一致。

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