Endovascular papillary angioendothelioma (Dabska tumor): underrecognized malignant tumor in childhood

Endovascular papillary angioendothelioma, or Dabska tumor, was first presented in 1969 by Maria Dabska where she presented cases occurring in 6 children. The tumor was described as being a low-grade angiosarcoma characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels within the dermis. Since its original description, only 30 case reports have been written describing the varying presentation, clinical course, and histopathology of this tumor. This case report documents the presentation and management of endovascular papillary angioendothelioma in a young male and reviews the current literature. Because more cases of EPAs are reported, perhaps a more standard approach to accurate diagnosis and treatment with surgical intervention and pathologic recognition via microscopy and immunohistochemistry can be determined.