Oral cavity
Other benign tumors / conditions

Topic Completed: 1 November 2013

Revised: 31 January 2019

Copyright: (c) 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Lymphangioendothelioma[TI] OR acquired progressive lymphangioma[TI]

Nat Pernick, M.D.
Page views in 2018: 470
Page views in 2019 to date: 351
Cite this page: Pernick N Lymphangioendothelioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/oralcavitylymphangioendothelioma.html. Accessed September 19th, 2019.
Definition / general
  • Also called acquired progressive lymphangioma
  • Very rare, particularly in mouth / lip
  • Slow growing, solitary patch lesion
  • Usually not associated with other vascular anomalies or HIV infection
Case reports
  • Complete excision; may recur locally
Clinical images

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Diffuse, marked macroglossia

Microscopic (histologic) description
  • Anastomosing, often widely dilated vascular structures in superficial mucosa
  • At deeper levels, vascular spaces collapse and dissect collagen in angiosarcoma-like pattern
  • Lining epithelium is flat, single layered, with stromal papillary projections resembling papillary endothelial hyperplasia and occasional intravascular red blood cells
  • Smooth muscle often focally present around vascular spaces
  • Endothelial cells may hobnail, may form morula resembling giant cells
  • No / rare mitotic figures; no / mild atypia
Microscopic (histologic) images

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H&E and vascular markers

Differential diagnosis
  • Kaposi sarcoma (patch stage): usually widespread multiple lesions in HIV+ patients or extensive lesion of lower extremities in elderly patients of Jewish or Mediterranean origin; usually lymphoplasmacytic infiltrate, with inflammatory cells aggregating around vessels, commonly extravasated red blood cells and often other forms of Kaposi sarcoma present
  • Well differentiated angiosarcoma: elderly patients, red-blue plaques or nodules, more endothelial atypia, multilayering and micropapillary tufting, often epithelioid or spindle cell component and inflammatory response common
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