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

Abstract

Background The status of cutaneous epithelioid angiomatous nodule (CEAN) as a distinct entity remains controversial. This study investigated the relationship between CEAN and epithelioid hemangioma/angiolymphoid hyperplasia with eosinophilia (ALHE). Methods Data of seven lesions with CEAN features from four cases (Cases 1–4: 61‐year‐old, 76‐year‐old, 53‐year‐old, and 21‐year‐old men, respectively) were investigated. Results Cases 1 and 2 showed multiple lesions in the head and neck region, but Cases 3 and 4 showed solitary lesions on the back and scalp, respectively. Moreover, the histopathologic findings of the lesions of Cases 1 and 2 were consistent with those of conventional epithelioid hemangioma or classic cutaneous ALHE. Diffuse immunoexpression of FOSB was observed in Cases 1 and 2, but FOSB split signals were absent in break‐apart fluorescence in situ hybridization (FISH). In contrast, the histopathologic findings of the lesions of Cases 3 and 4 were consistent with those of cellular‐type epithelioid hemangiomas. Diffuse immunoreactivity for c‐FOS was observed in Cases 3 and 4, and split signals of FOS were present in break‐apart FISH in Case 3. Conclusions This study showed that the seven tumors with CEAN features could be reclassified under the epithelioid hemangioma/ALHE group, although the small sample size is a limitation.

Cellular Cutaneous Epithelioid Hemangioma Harboring the Rare GATA6::FOXO1 Gene Fusion

“…Some argue that CEAN and EH may lie within the spectrum of the same entity. 26,27 In a series of 7 vascular lesions initially diagnosed as CEAN, 5 exhibited similar morphology to conventional EH and all showed FOSB immunohistochemical expression. 27 The remaining 2 lesions resembled cellular EH; 1 demonstrated FOS rearrangement by FISH, whereas the other exhibited c-FOS immunohistochemical expression.…”

“…26,27 In a series of 7 vascular lesions initially diagnosed as CEAN, 5 exhibited similar morphology to conventional EH and all showed FOSB immunohistochemical expression. 27 The remaining 2 lesions resembled cellular EH; 1 demonstrated FOS rearrangement by FISH, whereas the other exhibited c-FOS immunohistochemical expression. Given the mid to deep dermal localization and the unique GATA6::FOXO1 fusion, our case is best classified as cellular EH.…”

Cutaneous Epithelioid Angiomatous Nodule: Report of a New Case and Literature Review

“…CEAN is a rare vascular proliferative lesion, of which 66 cases have so far been reported after the initial description of this entity in 2004 [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ], including the case presented here. Clinically, CEAN presents as a bluish-red papule or nodule measuring 0.2–2.5 cm.…”

“…The lesion was single in the majority of cases (53/66, 80%); multiple lesions were present in 20% of cases. Four patients had multiple lesions on several body sites [ 3 , 11 , 21 , 22 ]. CEAN develops on the head and neck (41%—including the face, nasal cavity, scalp, oral cavity, external ear), the trunk (38%) and the extremities (26%).…”

“…Cutaneous epithelioid angiomatous nodule (CEAN) is a rare benign vascular tumour of the skin first described by Brenn and Fletcher in 2004 [ 1 ], of which 65 cases have so far been reported [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ]. It belongs to the spectrum of epithelioid vascular proliferative lesions, including other neoplasms, either benign (such as epithelioid haemangioma and pyogenic granuloma) or malignant (such as epithelioid haemangioendothelioma and epithelioid angiosarcoma).…”