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Vascular Tumours

Pathology of Kaposiform  Hemangioendothelioma         

Dr Sampurna Roy MD          


Dermatopathology Quiz Case 99

Pathology of Kaposiform Hemangioendothelioma





Kaposiform  hemangioendothelioma is a locally aggressive, non-metastasizing  vascular tumour.

This lesion is commonly associated with Kasabach-Merritt syndrome and lymphangiomatosis.  

Site: Retroperitoneum, subcutaneous or deep soft tissue of upper extremities, head and neck area. 

Age: Occurs as solitary tumour in infants (first decade of life) .

Microscopic features:


Microscopic images from the Path Quiz Case

Tumour consists of infiltrating lobulated nodules of spindle shaped endothelial cells

Nodules are separated by thick fibrous bands ; 

Spindle cells form short fascicles and line slit-like and round vessels ; 

Minimal cytological  atypia ; 

Mitotic activity is low  ; 

Epithelioid cells are sometimes  present ;

Cells  may contain hemosiderin pigment, hyaline globules and cytoplasmic vacuoles;

Ectatic vessels are present in the periphery ; 

Scattered microthrombi may be present within vascular spaces.


The spindle cells stain positively for CD34 and focally for CD31.

The pericytes around the capillaries stain positively for smooth muscle actin.  

Vascular Endothelial Growth Factor Receptor-3 (VEGFR-3): A Marker of Vascular Tumors with Presumed Lymphatic Differentiation, Including Kaposi's Sarcoma, Kaposiform and Dabska-Type Hemangioendotheliomas, and a Subset of Angiosarcomas.

Differential diagnosis :   

Kaposi's sarcoma (rare in children; multicentric; prominent chronic inflammatory infiltrate; lobular architecture is absent; dense fibrous bands are not present); infantile hemangioma acquired tufted hemangioma ; spindle cell hemangioendothelioma ;  

Angiosarcoma (endothelial atypia, increased mitotic activity and dermal collagen dissection by infiltrating tumour)


Further reading:

Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up.

Kaposiform hemangioendothelioma complicated by kasabach-merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components 

Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals.

Mediastinal Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon in a Patient with no Skin Changes and a Normal Chest CT.

Primary kaposiform hemangioendothelioma of a long bone: two cases in unusual locations with long-term follow up.

Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl.

Microscopic Kaposiform hemangioendothelioma with extensive lymphangiomatosis: an extraordinary example of an unusual entity.

Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report.

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon.

D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma.

Thoracic kaposiform hemangioendothelioma. Four consecutive cases with distinct outcome.

Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis.

Kaposi-like infantile hemangioendothelioma. A distinctive vascular neoplasm of the retroperitoneum.







Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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