Pathology of Retiform Hemangioendothelioma

Dr Sampurna Roy MD           2022

This is  a slow growing, exophytic or plaque like tumour.

Rarely these lesions may be associated with radiotherapy or chronic lymphedema.

Local recurrences are common. Metastatic spread to regional lymphnode is extremely rare.

Site: Distal extremities. 

Age: Young adults.

Microscopic features: 

Located in the dermis or subcutis ; characterized by  'long arborizing vessels'  with features reminiscent of rete-testis, focal solid areas composed of spindle and epithelioid cells may be present.

Other features:  Vessels lined by hobnail endothelial cells ;  prominent stromal lymphocytic infiltrate; occasional intraluminal papillae with hyaline collagenous cores and lymphocytes within vascular lumen.

Immunohistochemistry:  Spindle and epithelioid cells in the focal solid area express endothelial markers.

Differential diagnosis: Angiosarcoma  (endothelial atypia, increased mitotic activity and dermal collagen dissection by infiltrating tumour) and Dabska's tumour (lacks arborizing vessels and shows prominent papillary tufts).

Retiform hemangioendothelioma: another tumor associated with human herpesvirus type 8 .

Retiform hemangioendothelioma. A new case in a child with diffuse endovascular papillary endothelial proliferation.

Retiform hemangioendothelioma. Clinico-pathologic case report and discussion of the group of low malignancy vascular tumors.

Multiple retiform hemangioendotheliomas. A low-grade angiosarcoma.

Retiform haemangioendothelioma.

Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. 

Clinical, dermoscopic and histopathologic findings of retiform hemangioendotheliom.

A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features.