Kaposiform hemangioendothelioma

July 14, 2008 at 10:50 pm | Posted in hiv | Leave a comment
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Kaposiform hemangioendothelioma

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Rare, locally aggressive; tumor of infants and children; affects skin (75%), retroperitoneum (18%), bone

Death due to extensive disease and severe coagulopathy (Kasabach-Merritt syndrome), although no metastatic potential

Usually initial tumor is cutaneous

Micro: infiltrating nodules and sheets of compact spindle cells with formation of slit-like lumen

Micro images: figure 4C, 4D

Micro images: image1, image2, image3, image4

Positive stains: vascular endothelial growth factor receptor-3

DD: Kaposi’s sarcoma

References: Mod Path 2001;14:1087, Mod Path 2000;13:180

AIDS associated (epidemic)

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Historically, 40% of homosexual men with AIDS got Kaposi vs. 5% of others with AIDS

Incidence of Kaposi has been decreasing over time, Archives 2002;126:182

Early involvement of lymph nodes and gut and wide dissemination

Usually not a direct cause of death, although 1/3 develop lymphoma or another second malignancy

Gross: indolent disease has 3 stages: early – macule/patch, intermediate – plaque, late – nodule/tumor

Macule/patch: pink-purple macules of lower extremity or feet

Micro: dilated irregular blood vessels in background of lymphocytes, plasma cells, macrophages; resembles granulation tissue; disease spreads proximally, converts to raised

Micro images: image1

Macule/patch: superficial or mid-dermal proliferation of collagen-dissecting jagged capillary vessels with inconspicuous spindle cell component; may be confluence of vessels

Plaque: dermal, dilated, jagged vascular channels that dissect collagen fibers and contain isolated or small groups of spindle cells; red blood cell extravasation prominent; also hemosiderin laden macrophages, pink hyaline globules

Nodule/tumor: more distinctly neoplastic, most of lesion composed of spindle cells with intersecting fascicle like pattern in a background of inflammatory cells and red blood cells; small vessels and slitlike spaces with hyaline droplets and rows of red blood cells; mitotic figures common; may involve lymph nodes and viscera (African and AIDS variants)

Positive stains: smooth muscle actin

Negative stains: Factor 8

Molecular: detect HHV8 by PCR or in-situ hybridization

References: Mod Path 2002;15:434

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