Gorham-Stout disease and generalized lymphatic anomaly-clinical, radiologic, and histologic differentiation
Gorham-Stout disease and generalized lymphatic anomaly-clinical, radiologic, and histologic differentiation
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DOI：
10.1007/s00256-012-1565-4
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发表时间：
2013-07-01
影响因子：
2.1
通讯作者：
Chaudry, Gulraiz
中科院分区：
文献类型：
--
作者：
Lala, Shailee;Mulliken, John B.;Chaudry, Gulraiz
Gorham-Stout病（GSD）是一种罕见的以进行性骨溶解为特征的淋巴源性血管疾病。广泛性淋巴异常（GLA）是一种多系统疾病，通常也会影响骨骼。我们假设Gorham-Stout病不同于其他骨淋巴异常。我们建议通过分析骨骼成像结果来区分这些实体。我们回顾性回顾了到血管异常中心就诊的伴有骨淋巴异常的患者的临床资料、影像学研究和组织病理学结果。在一组51例淋巴疾病患者中，放射学证据显示骨骼受累，出现了两种不同的类别。19例患者符合GSD的影像学标准：进行性骨溶解伴骨吸收和皮质丢失。32例被归类为GLA：随着时间的推移，放射率离散，受影响的骨数量增加，但没有进行性骨溶解的证据。肋骨是两组中最常见的部位，其次是GSD中的头盖骨、锁骨和颈椎，GLA中的胸椎、肱骨和股骨。GSD受累的骨骼较少，附肢骨骼相对较少。18例GSD伴浸润性软组织异常，6例GLA伴浸润性软组织异常。14例GLA患者有大囊性淋巴畸形，GSD患者无。GSD和GLA在放射学上有明显的差异，尽管有一些重叠的特征。GSD的主要特征是进行性骨溶解。提示GLA的发现更广泛地累及，特别是阑尾骨骼，存在离散的巨囊性淋巴畸形和内脏器官病变。
Gorham-Stout disease (GSD) is a rare vascular disorder of lymphatic origin characterized by progressive osteolysis. Generalized lymphatic anomaly (GLA) is a multisystem disorder that also commonly affects bone. We hypothesized that Gorham-Stout disease is different from other osseous lymphatic anomalies. We proposed to discriminate these entities by analyzing findings on skeletal imaging.Clinical data, imaging studies, and histopathologic findings were retrospectively reviewed in patients presenting to our Vascular Anomalies Center with lymphatic anomalies of bone.Within a cohort of 51 patients with lymphatic disorder and radiological evidence of bony involvement, two distinct categories emerged. Nineteen patients met the imaging criteria for GSD: progressive osteolysis with resorption and cortical loss. Thirty-two were categorized as GLA: Discrete radiolucencies and increasing numbers of bone affected over time, but without evidence of progressive osteolysis. The ribs were the most common site in both groups, followed by the cranium, clavicle, and cervical spine in GSD, and thoracic spine, humerus, and femur in GLA. Fewer bones were involved in GSD, with relative sparing of the appendicular skeleton. Associated infiltrative soft tissue abnormality was seen in 18 in GSD, but only six with GLA. Macrocystic lymphatic malformations were identified in 14 with GLA, but none with GSD.There are significant radiological differences between GSD and GLA, although there are some overlapping features. The major distinguishing characteristic is the progressive osteolysis seen in GSD. Findings suggestive of GLA are more extensive involvement, particularly of the appendicular skeleton, presence of discretemacrocystic lymphatic malformations and visceral organ lesions.