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CONCISE COMMUNICATION
Year : 2019  |  Volume : 10  |  Issue : 4  |  Page : 463-466  

Cutaneous epithelioid angiomatous nodule of breast


1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubneswar, Odisha, India
2 Department of General Surgery, All India Institute of Medical Sciences, Bhubneswar, Odisha, India

Date of Web Publication28-Jun-2019

Correspondence Address:
Suvradeep Mitra
Department of Pathology and Lab Medicine, Academic Block, 2nd Floor, AIIMS, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_397_18

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How to cite this article:
Samal S, Monohar DB, Adhya AK, Sinha M, Mitra S. Cutaneous epithelioid angiomatous nodule of breast. Indian Dermatol Online J 2019;10:463-6

How to cite this URL:
Samal S, Monohar DB, Adhya AK, Sinha M, Mitra S. Cutaneous epithelioid angiomatous nodule of breast. Indian Dermatol Online J [serial online] 2019 [cited 2019 Jul 21];10:463-6. Available from: http://www.idoj.in/text.asp?2019/10/4/463/261801



Sir,

Cutaneous epithelioid angiomatous nodule (CEAN) is a recently described benign entity in the morphological spectrum of epithelioid vascular tumors with about 60 cases reported so far. It was initially described by Brenn and Fletcher in 2004.[1] It has been commonly described in head and neck, trunk, and extremities.[2] CEAN usually presents as a single lesion in young adults or adults without any significant sex predilection. Rarely, multiple metachronous lesions can occur. These lesions are papulonodular and are red to blue in color. However, CEAN can rarely involve breast,[3] vulva,[4] penis,[5] and mucosal surfaces.[1],[2],[3] The present case is the second case in literature involving breast. We describe a case of CEAN in breast and discuss the histomorphological differentials in details.

A 28-year-old lady presented with a painless nodule in the right breast that was present for the past 2 years, which was slowly increasing in size. Initially, it was a painless swelling. But the patient sought medical attention due to mild pain. On clinical examination, a nodule was identified in the upper inner quadrant of the right breast measuring approximately 1.0 × 0.8 cm. The skin over the nodule showed bluish black discoloration with a small punctum at the center. The nodule was fixed to the skin but was free from the underlying structure and was firm to feel and noncompressible. Mild tenderness could be elicited around the nodule along with mild erythema. Clinically, a possibility of a ruptured and inflamed epidermal inclusion cyst was suspected. The nodule was completely excised under local anesthesia and was sent for histopathological examination. Unfortunately, no clinical photograph was obtained as there was no clinical suspicion of a vascular tumor and the lesion clinically resembled an epidermal inclusion cyst.

Grossly, a well-circumscribed dermal nodule was identified measuring 0.8 × 0.5 cm. On cutting open, the cut surface of the nodule was solid and hemorrhagic, and no cystic component could be identified. No pultaceous material was found. Histopathological examination revealed a relatively circumscribed, unencapsulated, focally tissue permeating vascular lesion in the reticular dermis with overlying hyperplastic and acanthotic epidermis [Figure 1]a. The papillary dermis and the perilesional dermis showed moderate degree of perivascular and interstitial lymphoplasmacytic inflammation. The center of the lesion showed an epidermal inclusion cyst [Figure 1]b, lined by stratified squamous lining having granular cell layer and containing keratin flakes. No pilosebaceous unit was seen in the lining of this cyst to entertain a possibility of dermoid cyst. This epidermal inclusion cyst in other area showed accumulation of sheets of neutrophils within the cyst suggesting an inflamed cyst. Special stains [Gram stain for bacteria, Periodic acid–Schiff (PAS) stain for fungus, and Ziehl–Neelsen stain for tubercle bacilli] performed did not reveal any organisms within the suppurative area. A vascular lesion was seen surrounding the epidermal inclusion cyst, predominantly having solid areas with few interspersed cystically dilated vascular channels. The solid area was composed of relatively monomorphic epithelioid cells having a polygonal shape, central to eccentrically placed round to oval nuclei, vesicular chromatin, one to two prominent nucleoli, and abundant amount of glassy eosinophilic to clear vacuolated cytoplasm [Figure 1]c and [Figure 1]d. Intracytoplasmic abortive lumina was identified containing erythrocytes at places, whereas they were empty in other areas, giving rise to the appearance of blister cells. Nuclear atypia, hyperchromasia, mitosis, or necrosis was absent. The cystically dilated vascular channels were lined by a single layer of flattened endothelium and contained erythrocytes. There were scattered extravasated red blood cells, siderophages, plasma cells, and acellular hyaline globules in the intervening stroma, the latter being PAS-positive but diastase-resistant. Lymphoid follicles or excess of eosinophils were not seen within the tumor stroma. The mass appeared to be completely excised, and all the resection limits were free of any tumor infiltration.
Figures 1: (a and b) Photomicrograph showing an unencapsulated vascular lesion in the reticular dermis with overlying hyperplastic epidermis surrounding an epidermal inclusion cyst (hematoxylin and eosin) (a: 20×; b: 40×). (c and d) The vascular lesion showing relatively monomorphic epithelioid cells having a polygonal shape, central to eccentric nuclei, vesicular chromatin, one to two prominent nucleoli, and abundant amount of glassy eosinophilic to clear vacuolated cytoplasm (hematoxylin and eosin) (c: 100×; d: 400×)

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Immunohistochemistry was performed with appropriate (internal and external) controls. The epithelioid cells showed diffuse and strong membranocytoplasmic immunopositivity for CD34 [Figure 2]a and CD31 [Figure 2]b. Staining for Pancytokeratin, CD163, and estrogen receptor was negative in the epithelioid cells. The epidermal inclusion cyst lining was strongly highlighted by the Pancytokeratin stain [Figure 2]c, and all other stains were negative in the cyst. The macrophages scattered in the lesional stroma and in the perilesional distribution were highlighted by CD163 immunostain (membranocytoplasmic). The Ki67 index was 6%–10%. S100, HMB45, and p63 immunostains were negative (performed to rule out a possibility of malignant melanoma and poorly differentiated squamous cell carcinoma, respectively). A diagnosis of CEAN coexisting with an epidermal inclusion cyst was rendered, the latter also showing suppurative change.
Figure 2: Vascular lesion shows strong CD34 (a: 40×) and CD31 (b: 100×) positivity and Pancytokeratin (c: 200×) negativity, whereas cyst lining shows strong Pancytokeratin positivity

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The patient had been followed up and is currently doing well without any recurrence, till 3 months following surgery.

CEAN is a rare vascular tumor, and about 60 cases have been described in the English literature till date.[6] Clinically, the lesions are papulonodular and are red to blue in color. It usually involves adults or young adults and there is no significant gender predilection. There is no known predisposing factor. CEAN predominantly involves the head and neck region and involves both the appendicular and axial skeleton. The occurrence of CEAN involving multiple sites and eruptive features had also been documented. Breast is an extremely rare site of CEAN and only one case had previously been published in the literature.[3] Light microscopy shows a unilobular lesion with dominantly solid areas. The cells in the solid areas show an epithelioid morphology with scattered blister cells (cells containing intracellular lumina). Overlying epidermis can be thinned out due to stretching or is hyperplastic and acanthotic.[1] The periphery of the lesion usually contains scattered inflammatory cells especially siderophages. The vascular nature of the lesion can be documented by vascular markers such as CD31, CD34, and Factor VIII. It comes under the morphological spectrum of cutaneous epithelioid vascular lesion with diverse biological behavior ranging from reactive to neoplastic.[2]

The pathogenesis of CEAN is not well elucidated. The most widely accepted pathogenetic view is that it is a reactive process similar to pyogenic granuloma.[1] The other views include hamartomatous, vasoformative, and a neoplastic origin. Indeed, dermatopathologists often have highlighted the similarities between pyogenic granuloma, CEAN, and epithelioid hemangioma and have also proposed CEAN to be an intermediate entity between the pyogenic granuloma and epithelioid hemangioma. Although pyogenic granuloma dominantly occurs in the trauma-predisposed sites, it is known to occur in other locations as well. However, it usually shows a multilobular and a vascular growth pattern with relative paucity of the solid areas and epithelioid cells. In contrast, CEAN shows a dominantly solid and epithelioid morphology. Undoubtedly, morphologic overlaps between the CEAN and pyogenic granuloma and the CEAN and epithelioid hemangioma do exist. In fact, vague lobulation and patches of dilated and well-formed capillaries bear the resemblance with pyogenic granuloma in the index case.

Histomorphologically, different vascular lesions show epithelioid morphology including bacillary angiomatosis, epithelioid hemangioma [angiolymphoid hyperplasia and eosinophilia (ALHE)], CEAN, epithelioid hemangioendothelioma, and epithelioid angiosarcoma. Epithelioid morphology can also be seen in melanoma, glassy cell nevus, epithelioid fibrosarcoma, epithelioid malignant peripheral nerve sheath tumor (MPNST), and epithelioid rhabdomyosarcoma (RMS). The distinction between epithelioid hemangioma and CEAN is subtle with clinically overlapping features. Epithelioid hemangioma usually shows multilobular vascular proliferations without a solid component, and it usually extends into the deep dermis and subcutis with intralesional fibrosis.[1],[2],[3] In contrast, CEAN is usually a unilobular lesion confined to the dermis and is predominantly composed of solid component with a minor vascular component and perilesional fibrosis. Moreover, epithelioid hemangioma, synonymous to ALHE, shows a significant lymphoid component in the form of lymphoid aggregate or follicle along with numerous interspersed eosinophils.[1],[3] Bacillary angiomatosis can be distinguished from CEAN by its usual occurrence in the immunosuppressed individuals and by the presence of neutrophils, basophilic material, and occasional demonstration of the causative organism (Bartonella henselae) in the Warthin-Starry stain [Table 1].[7]
Table 1: Compare and contrast between the different epithelioid vascular lesions

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Epithelioid hemangioendothelioma is a low-grade malignant vascular tumor occurring in young females and shows angiocentric lesion in 30%–50% of cases.[8] The occurrence of epithelioid hemangioendothelioma in breast is extremely rare. However, other cases of epithelioid hemangioendothelioma lack in angiocentricity and instead show tumor cells invading the native tissue in infiltrating cords and trabeculae. The individual cells are relatively monomorphic and show characteristic blister cell morphology due to the presence of intracellular vacuoles/abortive lumina similar to the index case. Nevertheless, it can be distinguished from CEAN by its tissue infiltrating nature, trabecular pattern, angiocentricity when present, presence of cellular atypia, and brisk mitotic activity [Table 1].

Epithelioid angiosarcoma is the malignant vascular tumor and is much more common than CEAN in breast. The tumor cells usually are arranged in diffuse sheets and irregular vascular pattern. In contrast to epithelioid angiosarcoma, CEAN lacks the infiltrative growth pattern, deep soft tissue involvement, and leaky vascular channels lined by atypical endothelial cells with multilayering, frequent mitoses and atypical mitotic figures and occasional areas of necrosis [Table 1].

Nevus and melanoma can show epithelioid morphology. Whereas nevus shows a benign morphology, melanoma is its malignant counterpart typified by prominent nucleoli, atypia, and mitoses. Both nevus and melanoma cells show positivity for S100, Melan-A, and HMB45 on immunohistochemistry. Other epithelioid malignancies such as epithelioid fibrosarcoma, epithelioid MPNST, and epithelioid RMS are extremely rare in breast and show both mitosis and atypia. Usually, these lesions show loss of INI-1 expression, and the lineage determination can only be done after strong suspicion. Fibrosarcoma is often a diagnosis of exclusion, whereas MPNST in some cases shows expression of S100 without any expression of other melanosomal antigens. Epithelioid RMS can be identified by the expression of markers of skeletal muscle differentiation, namely, desmin, myogenin, and myoD1 [Table 1].

Our case had a few unique features. First, this case adds another example to the repertoire of CEAN, a recently described and rare histomorphological entity. It also adds to the clinical and histomorphological spectrum of the disease. Second, breast is a rarely described site for the lesion and there is only one previous report of CEAN occurring in breast. Third, the coexistence of this lesion with an epidermal inclusion cyst, a purely coincidental phenomenon, made the case clinically challenging. Finally, the differential list of epithelioid vascular lesions includes reactive, benign, and malignant entities. Variable other tumors can also show epithelioid cytomorphology. This often makes the job of histopathologist more challenging.

Most of the reported cases were single lesions, although cases with multiple lesions have also been reported.[1],[3],[6] Complete excision of the lesion is the most common and established mode of treatment for solitary and multiple lesions. Staged excision and cryotherapy followed by excision have also been described for multiple lesions.[5],[6]

CEAN is a rare benign tumor of breast encountered in clinical practice. Histopathological examination is the mainstay of diagnosis. Solitary lesions are successfully treated by excision. This case adds to the literature of CEAN and expands the list of benign epithelioid lesions involving breast.

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.
Pavlidakey PG, Burroughs C, Karrs T, Somach SC. Cutaneous epithelioid angiomatous nodule: A case with metachronous lesions. Am J Dermatopathol 2011;33:831-834.  Back to cited text no. 2
    
3.
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. 3
    
4.
Al-Daraji WI, Prescott RJ, Abdellaoui A, Khan MM, Kulkarni K, Youssef MM, et al. Cutaneous epithelioid angiomatous nodule: Different views or interpretations in the analysis of ten new cases. Dermatol Online J 2009;15:2.  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.
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. 6
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7.
Plettenberg A, Lorenzen T, Burtsche BT, Rasokat H, Kaliebe T, Albrecht H, et al. Bacillary angiomatosis in HIV infected patients – An epidemiological & clinical study. Dermatology 2000;201:326-31.  Back to cited text no. 7
    
8.
Weiss S W, Ishak K G, Dail DH, Sweet DE, Enzinger FM. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol 1986;3:259-87.  Back to cited text no. 8
    


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