Papillary Intralymphatic Angioendothelioma (Dabska Tumor)

 Most cases occur in adults but some occur in children


image May show significant overlap with Dabska tumor, with prominent papillary structures


• Composite hemangioendothelioma
image Composed of at least 2 distinct HE types

• Kaposi sarcoma
image Proliferation of small, slit-like vascular spaces lined by HHV8(+) spindle cells

image Lacks papillary pattern of Dabska

• Angiosarcoma



image
Scanning Magnification of Dabska Tumor
Scanning magnification view of a papillary intralymphatic angioendothelioma (Dabska tumor) shows a polypoid lesion in the dermis with irregularly dilated vascular spaces image.


image
Low Magnification of Dabska Tumor
Low magnification shows a background of lymphangioma-like areas with scattered lymphoid aggregates image. Note the focal intraluminal papillary projections image.

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High Magnification Showing Cytologic Details
High power shows papillae lined by mildly enlarged hobnailed cells projecting into the vascular lumina. The cells show vesicular chromatin, small nucleoli, and occasional grooves image.

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Papillae Lined by Plump Endothelial Cells
Multiple prominent intralymphatic projections of papillary structures with hyaline cores are lined by plump, hyperchromatic-staining endothelial cells, some of which contain cytoplasmic hemosiderin pigment image.


TERMINOLOGY


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
image Rare tumors

• Age
image Typically occur in children (minority in adults)

• Sex
image Slight female predominance

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Papillary Intralymphatic Angioendothelioma (Dabska Tumor)
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