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Папілярна інтралімфатична ангіоендотеліома

ORPHA:458768· ICD-10 D48.1· Papillary intralymphatic angioendothelioma

Визначення(English summary)

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.

Поширеність
<1 / 1 000 000
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Childhood, Elderly, Infancy