Sacrococcygeal mass in a 3-year-old unfolding as Dabska tumor-A very rare entity

Papillary intralymphatic angioendothelioma, previously known as Dabska's tumor, is a very rare entity in children involving the skin and soft tissue. It is a low grade tumor characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels in the dermis. We present a 3-year-old male child, with a history of swelling and mild pain in the right upper gluteal region, progressive in nature for 1 month duration. On examination, the swelling was soft to firm and mildly tender. Radiology was suggestive of Sacro-coccygeal teratoma. Grossly, it was an ill-defined grey brown lesion measuring 3.5 × 3 × 2 cm. Microscopy showed lesion arranged in papillary pattern with slit like vascular channels and intra luminal proliferations of hobnail like endothelial cells. Morphology and immunohistochemical studies revealed a diagnosis of Papillary intralymphatic angioendothelioma.