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患者多发血管球瘤中出现大斑块状血管球瘤:影像学和组织病理学复习。

Large plaque-like glomangioma in a patient with multiple glomus tumours: review of imaging and histology.

机构信息

Imaging Department, University Hospital, North Staffordshire NHS Trust, Stoke-on-Trent, UK.

出版信息

Clin Exp Dermatol. 2013 Oct;38(7):693-700. doi: 10.1111/ced.12122.

Abstract

Glomus tumours are benign tumours of the temperature-sensitive neuromyoarterial glomus body, present within the dermis, which are most commonly seen as solitary subungual lesions on the arms. Multiple glomus tumours or glomangiomas are a less common subtype of extradigital glomus tumours, which very rarely present as large plaque-like dermal lesions. Glomangiomas do not often show the classic triad of symptoms associated with glomus tumours, namely: pain, point tenderness on compression, and temperature sensitivity. As a result of this and of their atypical morphology, they can often be misdiagnosed as vascular malformations (VMs), resulting in delayed diagnosis and inappropriate treatment. We report a 29-year-old man with multiple extradigital glomus tumours that had been present since childhood, with the lesion on the patient's leg being the largest plaque-like glomangioma yet reported, to our knowledge. Spectral greyscale and Doppler shift ultrasonography showed multiple, tubulonodular, ectatic, noncompressible, vascular structures with aberrant flow within the thickened dermis. Using magnetic resonance imaging, low to intermediate signal was seen on T1-weighted images and high signal on T2-weighted images, and there was florid enhancement with gadolinium, with no evidence of extension into muscle or bone. Histology showed abnormal, dilated, thin-walled, vascular channels lined with multiple layers of glomus cells, confirming the diagnosis of a glomangioma. We discuss imaging techniques for plaque-like glomangiomas, and review the clinical, radiological and histological characteristics that help differentiate them from other superficial VMs.

摘要

血管球瘤是一种位于真皮内、温度敏感性神经肌动脉血管球的良性肿瘤,最常见于手臂的单一甲下病变。多发性血管球瘤或血管球瘤是一种罕见的甲下血管球瘤的亚型,很少表现为大斑块状皮肤病变。血管球瘤并不经常表现出与血管球瘤相关的典型三联征,即疼痛、压迫时压痛和温度敏感性。由于这一点和它们的非典型形态,它们经常被误诊为血管畸形 (VMs),导致诊断延迟和治疗不当。我们报告了一名 29 岁男性的多发性甲下血管球瘤,自童年起就存在,患者腿部的病变是我们所知的最大的斑块状血管球瘤。频谱灰阶和多普勒移位超声显示在增厚的真皮内有多发性管状结节状、扩张性、不可压缩、血管结构,血流异常。磁共振成像显示 T1 加权图像上的低到中等信号和 T2 加权图像上的高信号,钆增强后有明显强化,无延伸至肌肉或骨骼的证据。组织学显示异常扩张的薄壁血管通道,内衬多层血管球细胞,证实了血管球瘤的诊断。我们讨论了斑块状血管球瘤的影像学技术,并回顾了有助于将其与其他表浅 VM 区分开来的临床、放射学和组织学特征。

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