Affordable Access

Publisher Website

Morphologic Changes and Altered Gene Expression in an Epithelioid Hemangioendothelioma during a Ten-Year Course of Disease

Authors
Journal
Pathology - Research and Practice
0344-0338
Publisher
Elsevier
Publication Date
Volume
199
Issue
3
Identifiers
DOI: 10.1078/0344-0338-00370
Keywords
  • Epithelioid Hemangioendothelioma
  • Tp53/Murine Double Minute-2
  • Vascular Endothelial Growth Factor
  • Caveolin-1
Disciplines
  • Biology
  • Medicine

Abstract

Summary Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular neoplasm with unpredictable prognosis. We report on a patient suffering from a vascular neoplasm with primary manifestation in the skeleton and subsequent development of lesions of EHE in the spleen, liver and lung. Based on the assumption that involvement of visceral organs was due to metastatic spread, changes in clinical behavior, morphology, and proliferation index suggest malignant progression of the tumor. Analyzing the expression of genes known to be involved in the pathogenesis of vascular tumors, we found that the appearance of less differentiated tumor was paralleled by an accumulation of TP53 and murine double minute-2 (MDM-2) protein, decreased caveolin-1 ( CAV-1) expression, and increased vascular endothelial growth factor (VEGF) expression. Mutations of the von-Hippel-Lindau-tumor-suppressor-gene ( VHL) were excluded as mechanism of VEGF upregulation. Therefore, we propose that the expression of TP53, MDM-2, CAV-1 and VEGF as a marker of biologic behavior be verified in a larger case study of EHE.

There are no comments yet on this publication. Be the first to share your thoughts.