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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 204-206

Giant cutaneous epithelioid angiomatous nodule: A rare vascular tumor


Department of Dermatology, Seth G.S. Medical College and K.E.M Hospital, Mumbai, Maharashtra, India

Date of Submission10-Feb-2020
Date of Decision31-May-2020
Date of Acceptance03-Jun-2020
Date of Web Publication26-Aug-2021

Correspondence Address:
Jayati Shailesh Dave
OPD 17/18, Seth G.S. Medical college and K.E.M. Hospital, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_48_20

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  Abstract 


Cutaneous epithelioid angiomatous nodule (CEAN) is an uncommon benign vascular proliferation that presents as a reddish solitary nodule. Less than 60 cases have been reported in the literature. As it is benign, the prognosis is good, but it is sometimes difficult to differentiate it from other vascular neoplasms. A 16-year-old male presented with a single, large, brownish multinodular swelling over the left pinna. Biopsy showed conglomeration of epithelioid endothelial cells with eosinophilic cytoplasm in the dermis. These cells stained positive for CD31 and CD34. Diagnosis of CEAN was made

Keywords: Cutaneous epithelioid angiomatous nodule, pinna, vascular tumor


How to cite this article:
Dave JS, Dongre AM, Mahajan SA, Khopkar US. Giant cutaneous epithelioid angiomatous nodule: A rare vascular tumor. Clin Dermatol Rev 2021;5:204-6

How to cite this URL:
Dave JS, Dongre AM, Mahajan SA, Khopkar US. Giant cutaneous epithelioid angiomatous nodule: A rare vascular tumor. Clin Dermatol Rev [serial online] 2021 [cited 2021 Nov 28];5:204-6. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/2/204/324563




  Introduction Top


Cutaneous epithelioid angiomatous nodule (CEAN) is a benign vascular lesion commonly found on the trunk and the head and neck region. It presents as a reddish blue papule. The mode of origin of CEAN is unclear. Patients generally present with a history of 4–6 months. Prognosis is favorable, but it needs to be differentiated from other similar lesions that have a malignant course.


  Case Report Top


A 16-year-old male presented with reddish swelling over the left pinna for 2½ years. It appeared as a small, red, raised, occasionally painful lesion over the helix, which progressed slowly to involve more than 2nd/3rd of the pinna. There was no history of bleeding, pus discharge, hypoesthetic patches over the body, trauma, or hearing impairment. Examination showed ill-defined lobulated reddish-brown warm, tender swelling, which was soft to firm in consistency and measuring approximately 7 cm × 4.5 cm × 2 cm [Figure 1]. External auditory canal and tympanic membrane were normal. General and systemic examination was normal.
Figure 1: Ill-defined lobulated reddish brown swelling measuring 7 cm × 4.5 cm × 2 cm involving 2nd/3rd of helix and medial aspect of pinna

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Biopsy was performed from the lateral surface of the pinna. Hematoxylin and eosin-stained sections showed nonepithelial, well-circumscribed neoplasm occupying the dermis. Solid proliferation of large epithelioid endothelial cells with vesicular nuclei, abundant eosinophilic cytoplasm, prominent nucleoli, and intracytoplasmic vacuolation were present. There was scant inflammatory infiltrate of eosinophils and lymphocytes with no evidence of thick muscular vessels or vasoformative structures. Nuclear atypia, hyperchromasia, and mitotic figures were absent [Figure 2] and [Figure 3]. Immunohistochemical staining was positive for CD31 on endothelial cells [Figure 4]. The diagnosis of giant CEAN was made.
Figure 2: H and E staining of biopsy on ×25 showing nonepithelial well circumscribed neoplasm occupying the dermis

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Figure 3: H and E staining of section on ×200 showing proliferation of large epithelioid endothelial cells with vesicular nuclei, abundant eosinophilic cytoplasm, prominent nucleoli, and intracytoplasmic vacuolation

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Figure 4: CD31-stained positive endothelial cells (×200)

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The patient was referred to the plastic surgeon for excision.


  Discussion Top


Cutaneous epithelioid vascular lesions are a group of diverse conditions that may be reactive, infective, or neoplastic in origin. CEAN is one such entity. CEAN was recognized for the first time by Brenn and Fletcher in 2004.[1] Less than 60 cases have been reported since then.[2] Most of the cases present during adulthood (15–79 years). There is no sex predilection.[3] The most common sites of presentation are trunk, extremities, and the head–neck.[3],[4] Other uncommon sites of presentation are penis, vulva, mucosa, and back.[3],[5] CEAN has been reported in the pre and postaural region, but not on the pinna.[3],[6] It generally presents as a single, bluish papule or nodule measuring on an average 1–1.5 cm. However, cases with multiple metachronous lesions have also been reported.[3],[7],[8],[9] In contrast to the general presentation, our patient had large (7 cm), multinodular lesion on the pinna. Histopathology shows a well-circumscribed, unilobular growth extending up to the dermis.[2] Solid sheets of enlarged endothelial cells with abundant eosinophilic cytoplasm, prominent nucleoli, and intracytoplasmic vacuolation are present. Atypia is rare. In immunosuppressed patients, however, there may be cytologic atypia with brisk mitotic activity.[10] Intralesional vascular channels are almost always present. CEAN stains positive for either CD31, CD34, or Factor VIII-related antigen.[1] Other condition closely mimicking CEAN are Epithelioid hemanigioma (EH), Epithelioid hemangioendothelioma (EHE), Epithelioid angiosarcoma (EA), Kaposi's sarcoma (KS), Bacillary angiomatosis (BA) and Pyogenic granuloma (PyG).[1],[3],[4] Epithelioid hemanigioma (EH) also known as angiolymphoid hyperplasia with eosinophilia is a multilobular papulonodular lesion presenting mostly in the head and neck area. It is usually located in the deep dermis/subcutis. It is usually located in the deep dermis/subcutis, while CEAN presents in the superficial dermis. Inflammatory infiltrate of lymphocytes and eosinophils are present in both of these lesions; however, the infiltrates are less dense and located at the periphery of the lesion in CEAN.[8] EH has multilobular vascular proliferation with thick vasoformative structures and muscular vessels that lack solid proliferation of epithelioid endothelial cells, fibrosis, and hemosiderin deposition. Fibrosis (when encountered) is located within the lesion, rather than perilesionally (as seen in CEAN).[2] Epithelioid angiosarcoma (EA) and CEAN can be morphologically indistinguishable. EA involves deeper structures and viscera with a high rate of metastasis to lymph nodes and distant organs, hence carries poorer prognosis.[1] EA shows solid sheet-like growth pattern of epithelioid endothelial cells with abundant eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. It has infiltrative margins with areas of necrosis.[1],[3] There is nuclear pleomorphism and atypia with a tendency to dissect collagen by vascular channels known as endothelial multilayering.[1],[3] Epithelioid hemangioendothelioma is a malignant vascular neoplasm with a predilection for solid organs, bone, and deep soft tissue.[1] It is composed of epithelioid endothelial cells arranged in cords embedded in a fibromyxoid stroma.[1],[3] Vascular structures are rarely seen. Other vascular lesions such as Kaposi's sarcoma (KS), bacillary angiomatosis (BA), and pyogenic granuloma[11] may be confused with CEAN. The presence of previous infections or trauma helps differentiate them from CEAN. KS and BA are known to occur in immunocompromised state.

Simple excision is the treatment of choice.[1] Intralesional corticosteroids and cryotherapy have also been tried.[6] This case is being reported for rarity of this tumor and its occurrence on an uncommon site like pinna.


  Conclusion Top


CEAN is a rare vascular tumor which can be easily confused with malignant vascular tumor; thus, it is important to have knowledge of the same. Histopathology and immunohistochemistry play an important role in diagnosing CEAN and excision is the treatment of choice.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Brenn T, Fletcher CD. Cutaneous epithelioid angiomatous nodule: A distinct lesion in the morphologic spectrum of epithelioid vascular tumors. Am J Dermatopathol 2004;26:14-21.  Back to cited text no. 1
    
2.
Gupta D, Kumari R, Jacob SE, Rajesh NG, Thappa DM. Cutaneous epithelioid angiomatous nodule versus epithelioid hemangioma: A dilemma. Indian J Dermatol Venereol Leprol 2017;83:99-101.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Pavlidakey PG, Burroughs C, Karrs T, Somach SC. Cutaneous epithelioid angiomatous nodule: A case with metachronous lesions. Am J Dermatopathol 2011;33:831-4.  Back to cited text no. 3
    
4.
Sangüeza OP, Walsh SN, Sheehan DJ, Orland AF, Llombart B, Requena L. Cutaneous epithelioid angiomatous nodule: A case series and proposed classification. Am J Dermatopathol 2008;30:16-20.  Back to cited text no. 4
    
5.
Kaushal S, Sharma MC, Ramam M, Kumar U. Multifocal cutaneous epithelioid angiomatous nodules of the penis. J Cutan Pathol 2011;38:369-71.  Back to cited text no. 5
    
6.
Fernandez-Flores A, Montero MG, Renedo G. Cutaneous epithelioid angiomatous nodule of the external ear. Am J Dermatopathol 2005;27:175-6.  Back to cited text no. 6
    
7.
Dastgheib L, Aslani FS, Sepaskhah M, Saki N, Motevalli D. A young woman with multiple cutaneous epithelioid angiomatous nodules (CEAN) on her forearm: A case report and follow-up of therapeutic intervention. Dermatol Online J 2013;19:1.  Back to cited text no. 7
    
8.
Blackwood L, Chapman I, Lyon M, Hernandez C. Multifocal eruptive cutaneous epithelioid angiomatous nodules. JAAD Case Rep 2016;2:454-6.  Back to cited text no. 8
    
9.
Misago N, Inoue T, Narisawa Y. Subcutaneous epithelioid angiomatous nodule: A variant of epithelioid hemangioma. J Dermatol 2006;33:73-4.  Back to cited text no. 9
    
10.
Chetty R, Kamil ZS, Wang A, Al Habeeb A, Ghazarian D. Cutaneous epithelioid angiomatous nodule: A report of a series including a case with moderate cytologic atypia and immunosuppression. Diagn Pathol 2018;13:50.  Back to cited text no. 10
    
11.
Okano S, Takahara M, Tanaka M, Nakahara T, Suzuki H, Fujii H, et al. Cutaneous epithelioid angiomatous nodule in a patient with a history of multiple pyogenic granulomas. Eur J Dermatol 2015;25:268-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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