Most cases occur in adults but some occur in children
May show significant overlap with Dabska tumor, with prominent papillary structures
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Composite hemangioendothelioma
Composed of at least 2 distinct HE types
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Kaposi sarcoma
Proliferation of small, slit-like vascular spaces lined by HHV8(+) spindle cells
Lacks papillary pattern of Dabska
TERMINOLOGY
Synonyms
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Endovascular papillary angioendothelioma
Definitions
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Low-grade malignant vascular tumor composed of hobnailed endothelial cells
ETIOLOGY/PATHOGENESIS
Unknown
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May be associated with vascular or lymphatic tumor/malformation
CLINICAL ISSUES
Epidemiology
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Incidence
Rare tumors
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Age
Typically occur in children (minority in adults)
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Sex
Slight female predominance