Soft tissue
Vascular
Benign
Lymphangiomatosis


Topic Completed: 1 November 2012

Minor changes: 16 September 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed search: Lymphangiomatosis [title] soft tissue

Related topics: Lung tumor chapter

Vijay Shankar, M.D.
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Cite this page: Shankar V. Lymphangiomatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuelymphangiomatosis.html. Accessed September 23rd, 2020.
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


Lung: H&E and CD31

Mesenteric

Spleen

Positive stains
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