Pathology of Glomeruloid Hemangioma

Dr Sampurna Roy MD              

Glomeruloid hemangioma is a term that was coined J.K.C. Chan and colleagues in 1990 because the condition is histopathologically similar to renal glomeruli.

This vascular tumour is associated with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes). POEMS syndrome: definitions and long-term outcome .Blood, 1 April 2003, Vol. 101, No. 7, pp. 2496-2506

Glomeruloid hemangiomas are more common among Japanese and clinically presents as multiple red or purple papules.

These lesions present most commonly on the trunk and extremities, but lesions on the head and neck and in extracutaneous tissues such as the uterus have been described.

Microscopic features:

Microscopically, a typical case of glomeruloid hemangioma is characterized by dilated vascular spaces (sinusoidal) in the superficial dermis containing aggregates of capillaries, resembling renal glomeruli. 

Around the capillaries there are large cells which stain positively with endothelial markers.

Some PAS positive hyaline globules may be present.

These globules stain positively for immunoglobulins.

Immunohistochemistry:  

                                                                       CD31       CD34        UAE- 1       CD68
             Capillary type endothelium:            +             +          +           -
             Sinusoidal endothelium:                   +              -          -            +

Dermatopathology Quiz Case 33

Glomeruloid haemangioma with cerebriform morphology in a patient with POEMS syndrome.

Glomeruloid hemangioma leading to a diagnosis of POEMS syndrome.

Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma.

Solitary glomeruloid haemangioma without POEMS syndrome.

Glomeruloid haemangioma and POEMS syndrome.

Glomeruloid hemangioma in POEMS syndrome shows two different immunophenotypic endothelial cells.

A case of glomeruloid hemangioma associated with multicentric Castleman's disease.

Glomeruloid hemangioma. A cutaneous marker of POEMS syndrome.

Glomeruloid hemangioma. A distinctive cutaneous lesion of multicentric Castleman's disease associated with POEMS syndrome.