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Pathology of Cutaneous Epithelioid Angiomatous Nodule

Dr Sampurna Roy MD              

 

Dermatopathology Quiz Case 157

Diagnosis: Cutaneous Epithelioid Angiomatous Nodule

 

                                                                                                                      

 

 

Cutaneous Epithelioid Angiomatous Nodule (CEAN) was first described in 2004 by Brenn and Fletcher after a study of 15 cases.

In 2008, Sanguenza et al. described an additional 10 cases of CEAN and proposed that they should be classified as epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia. Some authors consider "CEAN"  to be a type of pyogenic granuloma.

Cutaneous Epithelioid Angiomatous Nodule is a rare benign vascular proliferation and a distinct entity.

The lesion usually occurs in adults. There is a wide age range of presentation (15-80 years).

Clinical presentation: Clinically, Cutaneous Epithelioid Angiomatous Nodule (CEAN) presents as a solitary rapidly growing reddish-blue nodule. The size ranges between 0.5 to 1.5 cm in maximum diameter.

Site: It is usually located on the trunk and extremities. Cases have been reported on the face and nasal mucosa.

Microscopic features:

The lesion is usually located superficially in the dermis.

Some rare cases are located in the submucosa.

It consists of well-circumscribed, solid proliferation of large polygonal epithelioid like endothelial cells.

The cells have vesicular nuclei and conspicuous nucleoli and abundant eosinophilic cytoplasm. Prominent intra-cytoplasmic vacuoles may be present.

Endothelial lined channels are present focally.

Nuclear atypia is absent. Normal mitotic figures are present (5 to 10 per high power field). Atypical mitoses are absent.

Presence of dilated blood vessels, deposition of  hemosiderin pigment and fibrosis have been reported in some cases.

There is a moderate chronic inflammatory infiltrate composed of mainly lymphocytes together with some plasma cells.

The inflammatory cells are prominent at the periphery of the lesion. Eosinophils are scattered throughout the lesion. 

Immunohistochemistry:  Immunohistochemically, the epithelioid cells are positive for endothelial markers such as CD31, CD34 and von Willebrand factor.

The lymph vessels are positive to D2-40.

Staining for αlpha-smooth muscle actin highlight pericytes with epithelioid features. Cytokeratin is negative.

 

Differential Diagnosis:

Differential diagnosis include all vascular lesions composed of epithelioid cells.

Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) ; Epithelioid hemangioendothelioma ; Epithelioid Angiosarcoma ; Bacillary angiomatosisand pyogenic granuloma.

1) Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia - ALHE) -  ALHE is usually located on the head and neck region. These are multiple lesions. CEAN usually presents as a solitary lesion on the trunk and extremities. ALHE is a multilobular lesion located in the deep dermis and has a very prominent inflammatory infiltrate and higher number of eosinophils. No lymphoid follicles or germinal centers are present in CEAN.

2) Epithelioid Angiosarcoma - It is a poorly circumscribed aggressive tumour. The cells show nuclear pleomorphism, atypia, numerous atypical mitotic figures and patchy areas of necrosis. More than 50% cases are cytokeratin positive

3) Epithelioid hemangioendothelioma - This is a malignant tumour which is larger, deeper and less well-defined compared to CEAN. The tumour has a hyaline, myxoid, chondroid or collagenous stroma. Cytokeratin is positive in many cases.

4) Bacillary angiomatosis - Bacillary angiomatosis is an infectious disease causing proliferation of small blood vessels in the skin and visceral organs of patients with human immunodeficiency virus infection and other immunocompromised hosts. This vasoproliferative, pseudoneoplastic lesion is caused by small , gram-negative bacilli Bartonella henselae.

5) Pyogenic granuloma - Lobular proliferation of capillaries consisting of cytologically bland endothelial cells, surrounded by pericytes. These are usually post traumatic lesions and located on the face and mouth. It may extend into deep dermis, subcutis, or intravascular space.

 

Further reading:

Case of a cutaneous epithelioidangiomatous nodule on the foot.

A young woman with multiple cutaneousepithelioid angiomatous nodules (CEAN) on her forearm: a case report and follow-up of therapeutic intervention.

A case of multiple epithelioidangiomatous nodules.

Cutaneous epithelioidangiomatous nodule: a case with metachronous lesions.

Cutaneous epithelioid angiomatousnodule of the chest wall with expression of estrogen receptor: a mimic of carcinoma and a potential diagnostic pitfall.

Multifocal cutaneous epithelioidangiomatous nodules of the penis.

D2-40 and cutaneous epithelioidangiomatous nodule.

Cutaneous epithelioid angiomatous nodule: a recently described vascular tumor].

Cutaneous epithelioidangiomatous nodule arising in capillary malformation.

Proliferative cutaneous epithelioid angiomatous nodule.

Cutaneous epithelioidangiomatousnodule: different views or interpretations in the analysis of ten new cases.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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