Soft tissue
Lymphangioma
Lymphangiomatosis

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

Revised: 29 September 2016, last major update November 2012

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

PubMed search: lymphangiomatosis [title] soft tissue

Related topics: Lung tumor chapter
Cite this page: Lymphangiomatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuelymphangiomatosis.html. Accessed December 8th, 2016.
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
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Colon

Omentum

Spleen

Micro 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
Micro Images
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Colon: dilated lymphatic channels

Colon

Colon

Lung: H&E and CD31

Mesenteric

Spleen

Positive Stains