Hemangioendothelioma

Epidemiology and Etiology:

· Epithelioid hemangioendothelioma:

· Any age

· Kaposiform hemangioendothelioma:

· Infants and children usually

Common sites:

· Epithelioid hemangioendothelioma:

· Soft tissue

· extremities

· Lungs

· Liver

· Bones

· Retiform hemangioendothelioma:

· Skin

· Kaposiform hemangioendothelioma:

· Retroperitoneal mass

Gross features:

· epithelioid hemangioendothelioma:

· well-circumscribed

· firm, rubbery

· cartilaginous consistency

· calcifications occasionally

·

Histologic features:

· Epithelioid hemangioendothelioma:

· Short cords and nests of epithelioid endothelial cells

· Angiocentric

· Myxohyaline matrix

· Eosinophilic cytoplasm

· Intracellular vacuole / lumen occasionally

· May contain RBCs

· Features associated with malignancy:

· Mitoses > 1 / 10HPF

· Necrosis

· Spindled morphology

· Cytologic atypia

· Retiform hemangioendothelioma:

· Kaposiform hemangioendothelioma:

· Infiltrative, lobular growth

· Well-formed capillaries

· Short fascicles of spindle cells with slit-like spaces

Immunophenotype:

Marker:	Sensitivity:	Specificity:
Keratin (focal in epithelioid)	Maybe
CD31
CD34

Molecular features:

·

Other features:

· Borderline clinical behavior between hemangioma and Kaposi’s sarcoma or angiosarcoma

· Locally aggressive

· Epithelioid hemangioendothelioma has a 30% risk of lymph node metastasis

· Retiform hemangioendothelioma metastasizes only very rarely (1 case)

· Kaposiform hemangioendothelioma metastasis has not been reported

· Kaposiform hemangioendothelioma:

· May be associated with consumptive coagulopathy (Kasabach-Merritt syndrome)

· No association with HIV

References:

· Silverberg SG, DeLellis RA, Frable WJ, LiVolsi VA, Wick MR. Silverberg's Principles and Practice of Surgical Pathology and Cytopathology: 2-Volume Set. 4th ed. Churchill Livingstone; 2005:2656.