Retiform Hemangioendothelioma

 More common in children



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

• Kaposi sarcoma

• Angiosarcoma



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Retiform Hemangioendothelioma at Low Magnification
Low-magnification examination of a retiform hemangioendothelioma (RHE) shows superficial dermal involvement by irregular, elongated, branching vascular spaces image.


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Retiform Hemangioendothelioma at Higher Magnification
Higher magnification view shows a proliferation of elongated vessels lined by hyperchromatic-staining hobnailed endothelial cells image. The surrounding stroma shows fibrosis and scattered lymphocytes.

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Retiform Hemangioendothelioma With Dense Lymphoid Aggregates
A deep area of an RHE shows elongated vascular spaces surrounded by prominent lymphoid aggregates image.

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Retiform Hemangioendothelioma: Cytologic Features
High-magnification view shows hyperchromatic endothelial cells with hobnail features, nuclear crowding image, and focally enlarged nucleoli image.


TERMINOLOGY


Abbreviations




• Retiform hemangioendothelioma (RHE)


Synonyms




• Hobnail hemangioendothelioma (HE)


Definitions




• Low-grade malignant vascular tumor composed of elongated vessels resembling rete testis


ETIOLOGY/PATHOGENESIS


Unknown




• Rare cases associated with lymphedema or preceding radiation

• 1 case reported to be positive for HHV8


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare vascular tumor

• Age
image Typically middle-aged adults but may occur in children

• Sex
image More common in females


Site




• Trunk or extremities

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Retiform Hemangioendothelioma
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