© Angel Fernandez Flores, MD, PhD Benign lymphangioendothelioma

 

Benign lymphangioendothelioma

Aka: acquired progressive lymphangioma.

 

Cause

Some postulate that it would be a reactive, rather than tumoral, condition.

Some others consider it an hamartomatous condition.

It has been described after surgery, radiation, inflammation or tick bites.

 

Clinical presentation

An erythematous to brownish plaque.

An enlarging hematoma-like patch.

 

Morphology

Proliferation of irregular, thin-walled vascular spaces, with endothelial linning, dissecting the collagen bundles.

Promontory sign can be evidenced.

Papillary projections into the lumen are not uncommon.

Bundles of smooth muscle in the walls of the vessels can be found.

Mild perivascular inflammatory infiltrate, made of lymphocytes.

No extravasated eythrocytes or hemosiderin deposits.

 

Immunohistochemsitry

CD31+, Cd34+, Factor VIII+/-, podoplanin+, WT1+.

 

 

Variants

Revelles et al. have described a giant variant (J Cutan Pathol. 2012 Oct;39(10):950-6).

 

Main differentials

Kaposi sarcoma: Plasma cells. Extravasated eythrocytes. Hemosiderin deposits. HHV8+.

Angiosarcoma: cellular atypia in the endothelia. Mitoses. Multilayering.

Post-radiation angiosarcoma: cMYC+.

 

© Angel Fernandez Flores, MD, PhD