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Soft tissue tumors

Lymphangioma

Lymphangiomatosis

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 26 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Definition
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● Diffuse / multicentric lymphangiomas, often in thoracic cavity, also bone, extremities, soft tissue, viscera
● See also Lung tumor chapter

Clinical features
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● Rare, idiopathic, occurs mostly in children
● Cases with visceral involvement are often fatal (Am J Surg Pathol 1993;17:329), but cases involving limbs have good prognosis (Am J Surg Pathol 1995;19:125)
● Associated with kaposiform hemangioendothelioma (Am J Surg Pathol 1993;17:321), bony lytic lesions and chylothorax (J Pediatr Hematol Oncol 2008;30:66), total cavo-pulmonary connection for complex congenital heart disease (Pediatr Cardiol 2008;29:771)

● 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
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● 15 year old girl with perinephric lymphangiomatosis (Indian J Radiol Imaging 2010;20:224)
● 25 year old woman, with acute presentation of colonic lymphangiomatosis (Int J Surg Case Rep 2012;3:158)
● 25 year old woman with colonic lymphangiomatosis mimicking acute appendicitis (Indian J Cancer 2011;48:139)
● 33 year old woman with fatal case (Am J Med Sci 2008;336:445)
● 39 year old healthy man with recurrent, complicated scrotal lymphangiomatosis (Can Urol Assoc J 2012;6:E11)
● 71 year old man with mesenteric lymphangiomatosis (World J Gastroenterol 2009;15:3947)

Treatment
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● Excision if symptomatic

Clinical images
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Colonic

Scrotal

Gross images
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Colon

Omentum

Spleen

Micro description
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● 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
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● CD31, Factor VIII, D2-40

End of Soft tissue tumors > Lymphangioma > Lymphangiomatosis

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