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蓝色橡皮疱痣综合征患者的颈部囊性淋巴管瘤:临床病例报告及文献复习

Cervical cystic lymphangioma in a patient with blue rubber bleb nevus syndrome: clinical case report and review of the literature.

作者信息

Marín-Manzano Elena, Utrilla López Agustin, Puras Magallay Enrique, Cuesta Gimeno Carmen, Marín-Aznar J L

机构信息

Angiology and Vascular Surgery Service, Ramón y Cajal Hospital, Madrid, Spain.

出版信息

Ann Vasc Surg. 2010 Nov;24(8):1136.e1-5. doi: 10.1016/j.avsg.2010.02.052.

Abstract

We present the case of a 9-year-old boy diagnosed with blue rubber bleb nevus syndrome, who showed a very large left cervical cystic lymphangioma. He was previously subjected to various treatments for lesions in the intestinal tract including blood transfusions for anemia, sclerosis, enterotomies or resections. The tumor was resected without any complications and the anatomopathologic report confirmed this diagnosis. The blue cavernous hemangioma syndrome (or blue rubber bleb nevus syndrome) is a rare disease characterized by cavernous angiomas involving the skin and gastrointestinal tract. Several cases of cystic lymphangiomas associated with this syndrome have been published recently and lymphomatous differentiation has been identified in the cells of cutaneous lesions. Given their common embryological origin, we underscore the importance of bearing in mind that it is possible for different types of vascular malformations to coexist in the same patient.

摘要

我们报告一例9岁男孩,诊断为蓝色橡皮疱痣综合征,其左颈部有一个非常大的囊性淋巴管瘤。他之前因肠道病变接受了各种治疗,包括因贫血输血、硬化治疗、肠切开术或切除术。肿瘤被切除,无任何并发症,解剖病理学报告证实了这一诊断。蓝色海绵状血管瘤综合征(或蓝色橡皮疱痣综合征)是一种罕见疾病,其特征为累及皮肤和胃肠道的海绵状血管瘤。最近已发表了几例与该综合征相关的囊性淋巴管瘤病例,并且在皮肤病变细胞中已鉴定出淋巴瘤样分化。鉴于它们共同的胚胎学起源,我们强调必须牢记同一患者可能并存不同类型的血管畸形。

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