Papillary Intralymphatic Angioendothelioma (Dabska Tumor)
David Cassarino, MD, PhD
Julie C. Fanburg-Smith, MD
Key Facts
Terminology
Dabska tumor
Endovascular papillary angioendothelioma
Clinical Issues
Typically occur in children (minority in adults)
Distal extremities most common, but may occur in other sites
High rate of local recurrence, rare metastasis
Microscopic Pathology
Dermal proliferation of vessels with papillary projections lined by enlarged, cuboidal endothelial cells
Endothelial cells show prominent hobnail features with plump, rounded profiles protruding into lumina
Typically associated with surrounding lymphoid infiltrate and sclerotic collagen
TERMINOLOGY
Abbreviations
Papillary intralymphatic angioendothelioma (PILA)
Synonyms
Dabska tumor
Endovascular papillary angioendothelioma
Definitions
Low-grade malignant vascular tumor composed of hobnailed endothelial cells
ETIOLOGY/PATHOGENESIS
Unknown
May be associated with vascular or lymphatic tumor/malformation
CLINICAL ISSUES
Epidemiology
Incidence