Soft tissue
Lymphangioma
Lymphangiomatosis



Topic Completed: 1 November 2012

Revised: 26 March 2019

Copyright: 2002-2018, PathologyOutlines.com, Inc.

PubMed search: Lymphangiomatosis [title] soft tissue

Related topics: Lung tumor chapter

Vijay Shankar, M.D.
Page views in 2018: 761
Page views in 2019 to date: 509
Cite this page: Shankar V. Lymphangiomatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuelymphangiomatosis.html. Accessed July 20th, 2019.
Definition / general
  • Diffuse / multicentric lymphangiomas, often in thoracic cavity, also bone, extremities, soft tissue, viscera
Clinical features


  • Diffuse retroperitoneal cystic abdominal lymphangiomatosis: attributed to congenital malformation of lymphatic ducts; distribution often corresponds to primitive fetal lymphatic sacs; either capillary, cavernous or cystic; may have multisystem involvement involving bone, brain, extremities, liver, pancreas, soft tissue, spleen (Acta Cytol 2009;53:191)
  • Gorham’s disease: diffuse lymphangiomatosis also involving bony tissue
Case reports
Treatment
  • Excision if symptomatic
Clinical images
Images hosted on other servers:

Colonic

Scrotal

Gross images
Images hosted on other servers:

Colon

Omentum

Spleen

Microscopic (histologic) description
  • Increase in size and number of thin walled channels
  • Lymphatic spaces are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis and occasionally underlying fascia and skeletal muscle
Microscopic (histologic) images
Images hosted on other servers:

Colon: dilated lymphatic channels

Colon

Colon

Lung: H&E and CD31

Mesenteric

Spleen

Positive stains
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