Skin tumor - Nonmelanocytic - Hemangioendothelioma and subtypes

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Skin - Nonmelanocytic tumors

Vascular tumors

Hemangioendothelioma and subtypes

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 14 August 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Subtypes: epithelioid, kaposiform, retiform, spindle cell

Epithelioid hemangioendothelioma

General
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● Superficial and soft tissues in adults; also lung, liver, GI, other sites
● Arise from endothelial cells
● Intermediate biology between hemangioma and angiosarcoma

Case reports
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● 41 year old woman with tumor of temporal artery (Rare Tumors 2009;1:e20)

Micro description
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● Well differentiated nests and cords of cells with abundant eosinophilic cytoplasm and prominent intracytoplasmic lumina

Micro images
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Various images

Positive stains
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● CD31, CD34

Differential diagnosis
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● Angiosarcoma: infiltrating, freely anastomosing channels lined by spindled to epithelioid endothelial cells with marked atypia, surrounding adnexae and dissecting dermal collagen
● Hemangioma: usually smaller, heavy infiltrate of eosinophils and lymphocytes with germinal centers; usually less atypia
● Masson tumor: dilated vessel with papillary proliferation of plump endothelial cells without atypia overlying fibrous tissue; fibrin deposition and thrombi present

Kaposiform hemangioendothelioma

General
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● Rare tumor of childhood (mean age 4 years, range 2 weeks to 20 years)
● Some cases may previously have been called acquired tufted angioma
● Usually on extremities or head and neck; may affect skin or deep soft tissue
● Intermediate malignancy: does not regress; metastases limited to regional perinodal soft tissue
● 50% are associated with Kasabach-Merritt phenomenon (profound thrombocytopenia and life-threatening hemorrhage); occasionally associated with lymphangiomatosis; deaths are due to phenomenon, not tumor (Am J Surg Pathol 2004;28:559, Mod Pathol 2001;14;1087)

Case reports
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● 24 days old male with ill-defined arm mass (J Res Med Sci 2009;14;6:389)

Clinical images
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Bulky red mass on arm of neonate

Gross description
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● Skin lesions are slightly raised, blue-red

Micro description
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● Biphasic with vascular and lymphatic component
● Irregular, infiltrating nodules of compressed vessels, evoking a dense hyaline stromal response
● Vessels are tightly coiled and highly convoluted, and budded off larger vessels, resembling either capillary hemangioma or Kaposi’s sarcoma
● Scattered epithelioid or glomeruloid islands are associated with pericytes, hemosiderin and fibrin thrombi
● Also has lymphatic component with thin-walled vessels

Micro images
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Various images

Positive stains
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● CD31, CD34, FLI1

Negative stains
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● GLUT1 (glucose transporter protein isoform 1), Lewis Y antigen, HHV8

Differential diagnosis
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● Juvenile hemangioma: spontaneously involutes, not associated with Kasabach-Merritt phenomenon; GLUT1+, LewisY+

Retiform hemangioendothelioma

General
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● Low-grade variant of angiosarcoma
● Usually distal extremities of young individuals
● Weiss and Goldblum use term “hobnail hemangioendothelioma” for retiform and Dabska-type tumors, which they believe to be closely related
● Rarely multiple (Am J Dermatopathol 1996;18:606)
● 2/3 recur, particularly without wide local excision
● Low rate of metastases, no tumor related deaths (Am J Surg Pathol 1994 18;2:115)

Case reports
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● 37 year old man with lesion on back (Case of the Week #107)

Treatment
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● Wide local excision

Gross description
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● Lesion of reticular dermis and subcutaneous tissue

Micro description
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● Retiform (net-like, similar to rete testis) pattern of blood vessels that disperse through reticular dermis and subcutis
● Vessels lined by monomorphic hobnail endothelial cells with scant cytoplasm and rounded, naked-type nuclei
● Often prominent lymphocytic infiltrate
● No epithelioid areas or cytoplasmic vacuoles (Am J Surg Pathol 1994;18:115)

Micro images
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Various images

CD31

Positive stains
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● Endothelial cells - CD34 (strong), CD31, vWF
● Also VEGFR3 (Mod Pathol 2000;13;180)

Negative stains
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● Endothelial cells - keratin

Differential diagnosis
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● Angiosarcoma: may focally have low grade features, but also exhibits areas of marked atypia and pleomorphism; also dissects between individual collagen bundles and has mitotic activity)
● Hobnail hemangioma: smaller, more superficial and more localized, with papillary dermal vessels that disappear into reticular dermis

Spindle cell hemangioendothelioma

General
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● Rare tumor of dermis and subcutis
● Males = females, any age
● Frequent local recurrences likely to actually represent multifocal involvement of vessel, only rare metastases (Am J Surg Pathol 1996;20:1196)
● Possible association with Mafucci syndrome

Micro description
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● Thin-walled, large cavernous blood vessels and spindle cells, thrombi, phleboliths (Cutis 1998;62:23)

End of Skin - Nonmelanocytic tumors > Vascular tumors > Hemangioendothelioma and subtypes

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