Skin - nonmelanocytic tumors
Vascular tumors
Benign lymphangioendothelioma

Author: Joel Tjarks, M.D. (see Authors page)
Editor: Sara C. Shalin, M.D., Ph.D.

Revised: 13 August 2018, last major update August 2018

Copyright: (c) 2001-2018, PathologyOutlines.com, Inc.

PubMed Search: Benign lymphangioendothelioma skin free full text[sb]

Cite this page: Tjarks, J. Benign lymphangioendothelioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticbenignlymphangioendothelioma.html. Accessed September 22nd, 2018.
Definition / general
Essential features
  • Delicate, thin walled, endothelium lined dilated vascular spaces involving the superficial dermis
  • Intravascular papillary stromal projections resemble papillary endothelial hyperplasia
  • Deeper in dermis, vascular spaces collapse and dissect dermal collagen in angiosarcoma-like pattern
Terminology
  • Also called acquired progressive lymphangioma
ICD-10 coding
Epidemiology
  • Uncommon; around 50 reported cases in English literature
  • Wide age distribution
  • No sex predilection
Sites
  • Trunk / limbs usually; other sites have been described
Pathophysiology
  • Favored to be a benign vascular malformation rather than a true neoplasm
Etiology
  • Some cases possibly related to trauma
Clinical features
Case reports
Treatment
Microscopic (histologic) description
  • Delicate, thin walled, endothelium lined dilated vascular spaces involving the superficial dermis
  • Intravascular papillary stromal projections resemble papillary endothelial hyperplasia
  • Deeper portion of lesions have vascular space collapse and dissect collagen bundles, mimicking patch stage Kaposi 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:

Contributed by Joel Tjarks, M.D.

6.8x

20x

CD31

D2-40



Contributed by Sara C. Shalin, M.D., Ph.D.

2x

10x

20x

Positive stains
Negative stains
  • HHV8
  • Most cases are WT1-; however, rare WT1 positivity has been reported
Differential diagnosis
  • Kaposi sarcoma: HHV8+; usually demonstrates slit-like vasculature without marked dilation of vessels and plasma cell infiltrate
  • Atypical vascular lesion: demonstrates more endothelial prominence and atypia; associated with radiation
  • Angiosarcoma: dissects through dermal collagen with significant atypia, atypical intraluminal cells, endothelial stacking (multilayering) and mitoses
  • Superficial lymphangioma (lymphangioma circumscriptum): congenital hamartoma often associated with cytogenetic abnormalities; typically does not dissect dermal collagen but may show dilated superficial dermal vessels
  • Targetoid hemosiderotic hemangioma (hobnail hemangioma): may have similar infiltration pattern to benign lymphangioendothelioma; endothelial cells protruding into vascular lumen (hobnail cells); deep stromal hemosiderin deposition