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成人全身性淋巴异常:一种明显的放射学诊断。

Generalized lymphatic anomaly in adult patients: An eminently radiological diagnosis.

机构信息

Servicio de Radiodiagnóstico, Hospital Universitario Miguel Servet, Zaragoza, Spain.

Servicio de Radiodiagnóstico, Hospital Universitario Miguel Servet, Zaragoza, Spain.

出版信息

Radiologia (Engl Ed). 2023 Sep-Oct;65(5):481-485. doi: 10.1016/j.rxeng.2022.08.004. Epub 2023 Sep 9.

DOI:10.1016/j.rxeng.2022.08.004
PMID:37758338
Abstract

Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis.

摘要

全身性淋巴管异常(GLA)是一种罕见的先天性疾病,继发于除中枢神经系统以外的任何器官中淋巴管的增殖。GLA 具有广泛的临床和影像学表现,其中溶骨性病变最为广泛,肋骨是最常受累的骨骼。GLA 主要在儿童和青年中诊断;然而,在罕见情况下,它也可以无症状存在,并在老年患者中偶然发现。我们报告了一例 54 岁无症状男性的 GLA 不典型病例,其 CT 显示分布不均、单纯囊性骨病变;测量这些病变的 Hounsfield(HU)值有助于我们怀疑 GLA。该怀疑通过 MRI、PET/CT、CT 引导下细针抽吸活检和透视引导下经皮椎体活检得到证实。在切除其中一个病变后,组织学研究提供了明确的诊断。

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