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

Author:

Bakker Andrea C.1,Fishman Steven J.2,Liang Marilyn G.3,Al-Ibraheemi Alyaa1ORCID,Kozakewich Harry P.1,Mulliken John B.4,Slack Jonathan C.1

Affiliation:

1. Departments of Pathology, Boston Children’s Hospital, Boston, MA, USA

2. Department of Surgery, Boston Children’s Hospital, Boston, MA, USA

3. Department of Dermatology, Boston Children’s Hospital, Boston, MA, USA

4. Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, MA, USA

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.

Publisher

SAGE Publications

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