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Pathology of Angiolymphoid Hyperplasia with Eosinophilia [Epithelioid Hemangioma]

Dr Sampurna Roy MD


Path Quiz Case 8 - Pathology case history and images

Diagnosis: Angiolymphoid Hyperplasia with Eosinophilia [Epithelioid Hemangioma]

November 2015

Dr Sampurna Roy  MD

Consultant   Histopathologist (Kolkata - India)


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Vascular tumours


Lobular capillary hemangioma

  - Bacillary angiomatosis

  - Verruga Peruana

Masson's Tumour

Acro-angiodermatitis / pseudo-Kaposi's sarcoma

Reactive angioendotheliomatosis

Infantile Hemangioma

Glomeruloid hemangioma

Acquired tufted angioma

Verrucous hemangioma

Cherry angioma/senile angioma

Arteriovenous hemangioma 

Microvenular hemangioma

Targetoid hemosiderotic hemangioma

Spindle cell  hemangioma

Kaposiform hemangioendothelioma 

Retiform hemangioendothelioma 

Papillary intralymphatic angioendothelioma

Composite hemangioendothelioma 

Polymorphous  hemangioendothelioma

Kaposi's sarcoma

Epithelioid hemangioendothelioma


Glomus tumour


Angiolymphoid  hyperplasia with eosinophilia also known as epithelioid hemangioma is a distinctive vascular lesion which usually occurs during early and mid adult life (20 - 40 years).

Clinically, this lesion presents as single or multiple pink to red-brown papules and nodules on the face, scalp and ear.

It is still uncertain whether this lesion is a  true neoplasm or a reactive process.
Some cases  appear to be associated with trauma, pregnancy, HIV infection, pregnancy and oral contraceptives.

Microscopically, these are well circumscribed lesions usually located in the dermis and subcutis. Angiolymphoid hyperplasia with eosinophilia consists of vascular and inflammatory cellular components.


The vascular component  displays thick and thin walled vessels lined by plump endothelial cells.

The epithelioid endothelial cells have rounded nuclei abundant eosinophilic cytoplasm containing occasional vacuoles that represent primitive vascular lumen formation.

These endothelial cell protrude into the vessels in a 'tombstone'  pattern. Within some thicker vessels there is intravascular proliferations of the endothelial cells.


The inflammatory component consists of lymphocytes scattered eosinophils and mast cells within the stroma. Lymphoid follicle may be present.
In areas the stroma may  be myxoid or fibrous in character.

Differential diagnosis:  Kimura's  disease ; epithelioid hemangioendothelioma; epithelioid angiosarcoma ; injection site granuloma (epithelioid cells are not present).            

Kimura's  disease is a separate entity which usually affects young Asian men and is commonly associated with lymphadenopathy.

Kimura's disease is a deeper lesion and reactive lymphoid follicles and are present together with a dense infiltration of eosinophils sometimes forming eosinophilic abscesses.
Blood vessels are also increased in number but these are lined by flat endothelial cells.

Epithelioid hemangioendothelioma
the tumour cells are arranged in nests and cords surrounded by hyaline and myxoid stroma. Well formed blood vessels are usualy not present.  Path Quiz Image 1  ; Image2  ;  Image3  ; Image4

Visit: Cutaneous Epithelioid Angiomatous Nodule


Further reading:

Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.

Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the lung: a clinicopathologic and immunohistochemical study of two cases.

Cutaneous epithelioid angiomatous nodule: a distinct lesion in the morphologic spectrum of epithelioid vascular tumors.

Human herpesvirus-8 is not associated with angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia: evidence for a T-cell lympho-proliferative origin.

Angiolymphoid hyperplasia with eosinophilia: a classic clinical presentation with histologic features of angiosarcoma.

Angiolymphoid hyperplasia with eosinophilia with bilateral involvement of the lacrimal glands.

Polymerase chain reaction (PCR) for human herpesvirus 8 and heteroduplex PCR for  clonality assessment in angiolymphoid hyperplasia with eosinophilia and Kimura's disease.  

Traumatic pseudoaneurysm: a possible early lesion in the spectrum of epithelioid hemangioma/angiolymphoid hyperplasia with eosinophilia.

Differential diagnosis of tumorous space-occupying lesions of the parotid gland: angiolymphoid hyperplasia with eosinophilia and Kimura disease.

Angiolymphoid hyperplasia with eosinophilia and vascular tumors of the head and neck. 

Multiple angiolymphoid hyperplasia with eosinophilia of the hand: report of a case and review of the literature.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: new observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins.

Microcirculatory segments identified with monoclonal antibody against alpha-smooth muscle actin: comparison between Kimura's disease and angiolymphoid hyperplasia with eosinophilia.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: clinical and histopathologic differences.

Estrogen receptors and the response to sex hormones in angiolymphoid hyperplasia with eosinophilia.

The histiocytoid hemangiomas and other controversies.

Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia).

Orbital angiolymphoid hyperplasia with eosinophilia. Presentation as chalazion.

Angiolymphoid hyperplasia with eosinophilia of the hand. A case report.

[Angiolymphoid hyperplasia with eosinophilia--report of a case with reference to the vascular changes and cell infiltrates in this disease].

A clinicopathological study of Kimura's disease and epithelioid hemangioma .

Histiocytoid hemangioma: a disease spectrum. Report of a case with simultaneous  cutaneous and bone involvement limited to one extremity.

Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: a case report with electron microscopy and immunohistochemistry.

Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura's disease in Chinese.

Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia.

Epithelioid hemangioma versus Kimura's disease. A comparative clinicopathologic study.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities.

Oral angiolymphoid hyperplasia with eosinophilia.

Renin in angiolymphoid hyperplasia with eosinophilia. Its possible effect on vascular proliferation.

Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients.

Epithelioid hemangioma of the penis: a clinicopathologic and immunohistochemical analysis of 19 cases, with special reference to exuberant examples often confused with epithelioid hemangioendothelioma and epithelioid angiosarcoma. Am J Surg Pathol. 2004;28(4):523-33.

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