Soft Tissue

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 29 September 2016, last major update November 2012

Copyright: (c) 2003-2016,, Inc.

PubMed search: lymphangioendothelioma [title]

Cite this page: Lymphangioendothelioma. website. Accessed October 23rd, 2016.
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
Micro 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
Micro 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)