Primary intralymphatic angioendothelioma

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Primary intralymphatic angioendothelioma

Synonyms: Dabska tumor

A rare vascular tumor characterized by an ill-defined slowly growing asymptomatic cutaneous plaque or nodule mostly involving the limbs in fewer cases the trunk. The tumor is composed of lymphatic-like channels with prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. It is locally aggressive while metastasis is rare. Infants and children are much more often affected than adults.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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