Papillary intralymphatic angioendothelioma: An extremely rare tumor

Papillary intralymphatic angioendothelioma (PILA), an intralymphatic lesion of vascular ducts, is an extremely rare tumor. It is generally encountered as an unexpected pathology following excision of a vascular skin lesion. Re-excision is the commonly preferred treatment option once the pathological diagnosis is established. In this article, we present a 12-year-old male patient with a PILA treated with re-excision, skin grafting, and sentinel lymph node biopsy. The patient was symptom-free at annual follow-up. A sentinel lymph node biopsy during re-excision might be a good option in the surgical management of PILA. Frequent examination of lymph nodes and skin lesion also might be beneficial during the follow-ups.