Soft tissue

Topic Completed: 1 November 2012

Minor changes: 9 September 2019

Copyright: 2002-2019,, Inc.

PubMed search: Lymphangioendothelioma [title]

Vijay Shankar, M.D.
Page views in 2019: 962
Page views in 2020 to date: 573
Cite this page: Shankar V. Lymphangioendothelioma. website. Accessed August 6th, 2020.
Definition / general
  • Proliferation of D2-40+ endothelial cells
  • Uncommon benign vascular lesion that may mimic well differentiated angiosarcoma or patch stage Kaposi's sarcoma (Am J Surg Pathol 2000;24:1047)
  • Also called acquired progressive lymphangioma
  • Rare
  • No gender preference, median age 54 years, range 17 - 90 years
  • Usually not associated with other vascular anomalies or HIV infection
Clinical features
  • Solitary red or bruise-like slow growing plaque present for median 5.5 years
  • Often in head and neck, but variable sites
  • May resemble actinic keratosis (Cutis 2001;67:29)
Prognostic factors
  • Benign
  • Occasional local recurrence
Case reports
Clinical images
Images hosted on PathOut server:

Progressive aquired lymphangioma, courtesy of Dr. Mark R. Wick

Images hosted on other servers:


Erythematous nodule

Hypogastrium (lower abdomen)


Gross description
  • Median 1.5 cm, range 0.3 cm to 10 cm
Microscopic (histologic) description
  • Delicate, thin walled, endothelium lined dilated vascular spaces involving the superficial dermis
  • Intravascular papillary stromal projections resembles papillary endothelial hyperplasia
  • Deeper portion of lesions have vascular space collapse and dissect collagen bundles, mimicking patch stage Kaposi's sarcoma
  • Preexisting vessels and adnexal structures of the dermis also appear dissected by newly formed vascular channels
  • Smooth muscle often focally present around vascular spaces
  • Endothelial cells may hobnail, may form morula resembling giant cells
  • Crowding of endothelial cells present, but no endothelial atypia
  • Vascular spaces lack erythrocytes and hemosiderin deposits
  • No mitotic figures
Microscopic (histologic) images
Images hosted on PathOut server:

Breast, courtesy of Dr. Mark R. Wick

Podoplanin, breast, courtesy of Dr. Mark R. Wick

Progressive aquired lymphangioma, courtesy of Dr. Mark R. Wick

Images hosted on other servers:



Lymphatic endothelium is podoplanin+

Site unknown

Negative stains
Differential diagnosis
  • Atypical or benign vascular proliferations of breast: history of radiation therapy
  • Kaposi sarcoma: patch stage usually has 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, often other forms of Kaposi‚Äôs sarcoma present
  • Lymphangioma circumscriptum
  • Angiosarcoma - well differentiated: elderly patients, reddish blue plaques or nodules, more endothelial atypia, multilayering and micropapillary tufting, often epithelioid or spindle cell component, inflammatory response common (Am J Dermatopathol 2000;22:151)
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