Skin - nonmelanocytic tumors
Vascular tumors
Benign lymphangioendothelioma

Author: Joel Tjarks, M.D.
Editor: Sara C. Shalin, M.D., Ph.D.

Revised: 6 November 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 December 12th, 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 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
Board review question #1
Which of the following immunohistochemical stains is negative in benign lymphangioendothelioma?

  1. CD31
  2. CD34
  3. D2-40
  4. ERG
  5. HHV8
Board review answer #1
E. HHV8
Board review question #2
Which of the following histologic features argues against the diagnosis of benign lymphangioendothelioma?

  1. Delicate thin walled vessels
  2. Frequent mitotic figures
  3. Hobnail endothelial cells
  4. Infiltration into the deep dermis
  5. Intravascular papillary stromal projections
Board review answer #2
B. Frequent mitotic figures

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