Benign Lymphangioendothelioma

Updated: Oct 06, 2015
  • Author: Donald Shenenberger, MD, FAAD, FAAFP; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Benign lymphangioendothelioma (BLAE) (also known as acquired progressive lymphangioma) is an uncommon vascular tumor that is of importance primarily because it can be confused histologically with Kaposi sarcoma (KS) or angiosarcoma. [1, 2] Jones et al first described the tumor as acquired progressive lymphangioma and later as benign lymphangioendothelioma. [3]

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Pathophysiology

Benign lymphangioendothelioma is a proliferation of lymphatic endothelial cells that stain positively for CD31, CD34, podoplanin (D2-40, a lymphatic marker), LYVE-1, and PORX-1. [4] Benign lymphangioendothelioma is not associated with preexisting vascular malformations or lymphedema. Although the lesion rarely is identified during infancy, some suggest it is a hamartoma that first becomes apparent during adolescence or young adult life; the development of benign lymphangioendothelioma is possibly triggered by hormonal changes.

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Epidemiology

US frequency

Benign lymphangioendothelioma is rare; fewer than 30 cases have been reported.

Race

No racial predisposition is reported.

Sex

Males and females are affected equally.

Age

Benign lymphangioendothelioma can affect patients ranging from age 17-90 years (median age 54 y). [1]

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