Immunohistochemical Expression of Lymphatic Endothelial Markers in Blue Rubber Bleb Nevus Syndrome.
Pediatr Dev Pathol
; 27(3): 228-234, 2024.
Article
in En
| MEDLINE
| ID: mdl-38512910
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Skin Neoplasms
/
Immunohistochemistry
/
Biomarkers, Tumor
/
Nevus, Blue
/
Gastrointestinal Neoplasms
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
En
Journal:
Pediatr Dev Pathol
Journal subject:
PATOLOGIA
/
PEDIATRIA
Year:
2024
Document type:
Article
Affiliation country:
United States
Country of publication:
United States