Cutaneous epithelioid angiomatous nodule: a report of a series including a case with moderate cytologic atypia and immunosuppression

Abstract

BackgroundCutaneous epithelioid angiomatous nodule (CEAN) is a very rare and relatively recently recognized vascular proliferation characterized usually by minimal cytological atypia and accompanying mitotic activity. As such, CEAN represents an important diagnostic pitfall, which could lead to significant misdiagnosis and unnecessary treatment.MethodsThe clinicopathologic findings of 5 cases of CEAN were reviewed including a unique case with typical findings but also moderate cytologic atypia and brisk mitotic activity in a patient on immunosuppression.ResultsThe cases were in 3 women and 2 men ranging in age from 18 to 61 years with lesions in the neck (2 cases), upper arm, back and shoulder. In 4 of the cases, the patients did not have any relevant potentially contributory clinical history, and in 1 case the patient was on immunosuppressive treatment. All 5 cases were superficially located within the dermis, well-circumscribed and similarly composed of epithelioid cells displaying minimal (in 4 cases) and moderate (1 case) atypia. The mitotic count ranged from 1 to 3 per 10 high power fields (HPF) in 4 cases and up to 9 per 10 HPF in the immunosuppressed patient. Atypical mitoses were not encountered in any of the cases. Two lesions that were incompletely excised recurred, but none of the patients showed distant metastases.ConclusionWhile cytologically alarming, CEAN has a characteristic microscopic appearance and if completely excised follows an indolent course.

Granular cell cutaneous epithelioid angiomatous nodule

“…Low mitotic activity was reported in both series. One case was identified with moderate cytologic atypia . No lesions were described with cytoplasmic granular cell morphology.…”

“…Cutaneous epithelioid angiomatous nodule is an uncommon lesion with three case series reporting lesions with a wide distribution and age range . Most report a single papule or nodule with rare reports of multiple localized lesions.…”

Categorization of cutaneous epithelioid angiomatous nodule as epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia: Clinicopathologic, immunohistochemical, and molecular analyses of seven lesions

“…1,7 Instead, most of the CEAN cases in the literature seem to be similar to conventional subtype epithelioid hemangioma or classic cutaneous ALHE, as seen in Cases 1 and 2 in this study. 5,10,13,15,18,24 Moreover, CEAN cases combined with multiple pyogenic granulomas 19 or capillary malformations 11,20 have been reported. Therefore, the CEAN cases in literature form a heterogeneous group, and most of them may actually be conventional subtype epithelioid hemangioma or classic cutaneous ALHE.…”

“…We performed a thorough search regarding CEAN using PubMed search engines and found 66 CEAN cases in 28 existing studies in English between 2004 and 2021 1–28 . These CEAN tumors were observed in patients (38 males and 28 females) aged 14–84 years (average, 42.0 years).…”

“…A cutaneous epithelioid angiomatous nodule (CEAN) is a rare cutaneous vascular entity first described in 2004 by Brenn and Fletcher 1 . Since then, 66 cases have been reported in 28 papers 1–28 . Owing to the histopathological features of the epithelioid endothelial cells, this benign tumor can be easily confused with other benign, borderline, or malignant vascular tumors, including bacillary angiomatosis, Kaposi sarcoma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma.…”