Immunohistochemical Expression of Lymphatic Endothelial Markers in Blue Rubber Bleb Nevus Syndrome.

IF 1.3 4区医学 Q3 PATHOLOGY

Pediatric and Developmental Pathology Pub Date : 2024-05-01 Epub Date: 2024-03-21 DOI:10.1177/10935266241228930

Andrea C Bakker, Steven J Fishman, Marilyn G Liang, Alyaa Al-Ibraheemi, Harry P Kozakewich, John B Mulliken, Jonathan C Slack

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引用次数: 0

Abstract

Introduction: Blue rubber bleb nevus syndrome (BRBNS) is an uncommon vascular anomaly characterized by multifocal cutaneous, visceral, and other soft tissue or solid organ venous malformations. We observed that BRBNS lesions express immunohistochemical markers of lymphatic differentiation.

Methods: BRBNS histopathologic specimens assessed at our institution during the past 27 years were reviewed. Slides from 19 BRBNS lesions were selected from 14 patients (9 cutaneous, 9 gastrointestinal, and 1 hepatic). We recorded the involved anatomical compartments and presence/absence of thrombi or vascular smooth muscle. Immunohistochemical endothelial expression of PROX1 (nuclear) and D2-40 (membranous/cytoplasmic) was evaluated semi-quantitatively.

Results: Endothelial PROX1 immunopositivity was noted in all specimens; the majority (89.5%) demonstrated staining in more than 10% of cells. D2-40 immunopositivity was present in one-third (33%) of cutaneous lesions and only 1 gastrointestinal lesion.

Conclusion: Endothelial cells in BRBNS almost always express 1 or more immunohistochemical markers of lymphatic differentiation.

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蓝橡皮痣综合征淋巴内皮标志物的免疫组化表达

导言：蓝橡皮痣综合征（BRBNS）是一种不常见的血管畸形，以多灶性皮肤、内脏和其他软组织或实体器官静脉畸形为特征。我们观察到，BRBNS 病变表达淋巴分化的免疫组化标记：方法：对过去 27 年中我院评估的 BRBNS 组织病理标本进行回顾性研究。我们从 14 位患者（9 位皮肤癌患者、9 位胃肠癌患者和 1 位肝癌患者）的 19 例 BRBNS 病变中选取了切片。我们记录了受累的解剖区域以及血栓或血管平滑肌的存在/消失情况。对PROX1（核）和D2-40（膜/胞质）的免疫组化内皮表达进行了半定量评估：结果：所有标本均显示内皮 PROX1 免疫阳性；大多数标本（89.5%）10%以上的细胞染色。D2-40免疫阳性出现在三分之一（33%）的皮肤病变中，只有1例胃肠道病变：结论：BRBNS 中的内皮细胞几乎总是表达一种或多种淋巴分化的免疫组化标记。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric and Developmental Pathology 医学-病理学

CiteScore

3.70

自引率

5.30%

发文量

审稿时长

6-12 weeks

期刊介绍： The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.