Papillary intralymphatic angioendotelioma: case report

Abstract

Papillary intra-lymphatic angioendothelioma (PILA) is a rare vascular tumor which was first characterized in 1969 by María Dabska, who described in her series of 6 cases a low-grade angiosarcoma, usually developed in childhood and that had distinctive histological characteristics. Since then about 30 cases have been reported, mostly in the skin and subcutis, but also in deep organs such as spleen, bone and testis.

The objective of our work was to communicate a case of this very rare vascular neoplasm, review its clinical aspects, its gross and microscopic appearance and establish its biological behavior.

 Our case corresponded to a 9 year old girl with a lower lip injury. A wedge-shaped fragment partially covered by 5 x 2.5 cm semi-mucosa was received. In one sector, a lesion of warty, papillary appearance of approximately 3 x 1 cm was observed. Microscopy showed a skin proliferation of vessels covered by cuboid cells, enlarged in size. The endothelial cells had a "hobnail" appearance with nuclei protruding towards the light. They were arranged constituting papillae and in other sectors forming glomeruloid structures. These findings were compatible with a papillary intra-lymphatic angioendothelioma. In immunohistochemistry it was positive for CD34.

PILA is a rare vascular neoplasm with a high rate of local recurrences but with infrequent metastases. Differential diagnoses must be taken into account for which immunohistochemistry techniques can be used