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WHO Classification of Tumours
Lymphangioma, NOS
Liver and intrahepatic bile ducts


Definition

Lymphangioma is a rare benign tumour that is usually observed in children and adolescents. It may occur as a solitary mass or more commonly as multiple masses composed of multiple endothelial- lined spaces that vary in size from capillary channels to large, cystic spaces containing clear pink-staining lymph. The vascular spaces are lined by a single layer of endothelial cells. The supporting stroma is usually scanty and may contain lymphoid tissue. Painless swelling is the usual clinical manifestation.

Hepatic lymphangiomas extremely rarely occur in isolation; they are often accompanied by lymphangiomatosis of the spleen, skeleton, and other tissues, and may represent a malformation syndrome. Diffuse lymphangiomatosis involving the liver and multiple organs is associated with a poor prognosis

Click to access Pubmed
Datz C, Graziadei IW, Dietze O, Jaschke W, Königsrainer A, Sandhofer F, Margreiter R (2001)
Massive progression of diffuse hepatic lymphangiomatosis after liver resection and rapid deterioration after liver transplantation.
Am J Gastroenterol 96: 1278-81



. Single lesions have been successfully resected
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Shahi KS, Geeta B, Rajput P (2009)
Solitary hepatic lymphangioma in a 22-day-old infant.
J Pediatr Surg 44: E9-11




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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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