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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 36-38

Early-onset epithelioid angiosarcoma: Diagnostic enigma, a rare case report


Department of Dermatology, BMCRI, Bengaluru, Karnataka, India

Date of Web Publication13-Jun-2019

Correspondence Address:
Dr. Kanathur Shilpa
Department of Dermatology, BMCRI, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_58_18

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  Abstract 


Epithelioid angiosarcoma (AS) is a rare vascular tumor affecting more commonly men in their seventh decade. We are reporting a case of epithelioid AS affecting a male patient in his third decade. He presented with a well-defined ulcerated plaque with an elevated border on the scalp. Histopathology showed sheets of epithelioid cells infiltrating the dermis, formation of slit-like vascular spaces, and cellular atypia. Immunohistochemistry showed positive CD31, D2-40, and FLI-1 and negative cytokeratin and epithelial membrane antigen. With these findings, a diagnosis of epithelioid AS was made. This case highlights the rare occurrence of this condition in younger age group.

Keywords: Epithelioid angiosarcoma, immunohistochemistry, squamoid differentiation


How to cite this article:
Shilpa K, Leelavathy B, Gorur D, Biligi D. Early-onset epithelioid angiosarcoma: Diagnostic enigma, a rare case report. Indian J Dermatopathol Diagn Dermatol 2019;6:36-8

How to cite this URL:
Shilpa K, Leelavathy B, Gorur D, Biligi D. Early-onset epithelioid angiosarcoma: Diagnostic enigma, a rare case report. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2019 Sep 22];6:36-8. Available from: http://www.ijdpdd.com/text.asp?2019/6/1/36/260193




  Introduction Top


Epithelioid angiosarcoma (AS) is one of the rare highly aggressive vascular tumors which can arise from different primary sites like skin, bone, thyroid, and adrenals. It poses a great diagnostic challenge both clinically and histopathologically. Histopathologically, it can mimic various malignancies such as metastatic carcinomas, malignant mesothelioma, melanoma, anaplastic lymphoma, epithelioid peripheral nerve sheath malignancies, and epithelioid sarcomas when obvious vasoformative foci are not found. Here, we report a case of epithelioid AS which not only posed a diagnostic challenge but also an unusual presentation in very young individuals.


  Case Report Top


A male patient aged 24 years presented to the dermatology department with a history of a skin lesion on the right side of the scalp. The lesion started as a small papule 3 months back, gradually increased in size to about 3 cm × 5 cm. It was associated with pain and discharge for which he had consulted a local practitioner where he was prescribed antibiotics. After taking antibiotics, discharge stopped, but the lesion continued to grow and was associated with pain.

On examination, a well-defined plaque with central ulceration and irregular, elevated border measuring 3 cm × 5 cm was present on the right side of the occipital area [Figure 1]. There was tenderness present but no bleeding, discharge, or lymphadenopathy at the time of examination. Histopathological examination of the punch biopsy specimen showed vascular spaces lined by plump endothelial cells and dense interstitial inflammation, suggesting a probable diagnosis of Kaposi's sarcoma. His routine investigations, i.e. complete hemogram, liver function, renal function test, and chest X–ray, were normal; VDRL for treponemal infection and enzyme-linked immunosorbent assay for HIV were nonreactive. However, to reconfirm the diagnosis and also to devise therapeutic mode of management, excision biopsy was advised. Histopathological examination of the excised specimen showed significant acantholysis with islands of tumor cells showing squamoid differentiation infiltrating the dermis [Figure 2]. Dermis showed vascular channels forming slit-like spaces, few of which were filled with red blood cells [Figure 3]. Features of cellular atypia were evident [Figure 4]. With the above findings, a diagnosis of moderately differentiated squamous cell carcinoma was suggested. However, in view of young age, significant acantholysis, and slit-like spaces, immunohistochemistry (IHC) examination to rule out vascular tumor was advised. On IHC, the neoplastic cells expressed positive for CD31, D2-40, and FLI-1 and negative for cytokeratin (CK) and epithelial membrane antigen (EMA) [Figure 5]. Ki-67 proliferation was around 30%, and the neoplastic cells retained INI1. With this immunomorphological features, a final diagnosis of epitheloid AS was made. The patient was further evaluated to rule out metastasis and referred for radiotherapy.
Figure 1: Well-defined solitary plaque on the left side of the scalp

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Figure 2: Histopathology showing tumor cells with squamoid differentiation infiltrating the dermis (H and E, ×4)

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Figure 3: Dermis showed vascular channels forming slit-like spaces (H and E, ×10)

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Figure 4: Higher magnification showing cellular atypia (H and E, ×40)

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Figure 5: Immunohistochemistry stains showing CD31, D2-40, and FLI-1 positivity and cytokeratin and epithelial membrane antigen negativity (×4)

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  Discussion Top


ASs are rare, highly malignant tumors of endothelial origin. Based on the site of origin, these are classified into cutaneous, visceral, and soft-tissue subtypes. Most of the ASs are of soft-tissue type, with very few cases falling into cutaneous and visceral subtypes.[1] Histopathologically, these lesions may show variable presentation which range from endothelial atypia to high-grade spindle cell malignancies. A subset in which the malignant endothelial cells have a predominantly epithelioid appearance is called as epithelioid angoisarcoma.[2] Rosai et al. first described the epithelioid phenotype of AS in 1976, which was later documented by Fletcher et al. in 1991.[3]

Epithelioid AS predominantly affects males in their seventh decade though a few pediatric cases have been reported.[1] This rare subset involves mainly deep soft tissues and has male preponderance, with predilection sites being trunk, legs, and head-and-neck regions. It has a poor prognosis and can histologically mimic nonvascular neoplasms and impose serious diagnostic challenge.[4]

Cutaneous AS is again subclassified into following four groups: first and most common being idiopathic occurring in elderly individuals with poor prognosis; the second one being associated with chronic lymphedematous limb;[5] third, rare variety associated with radiotherapy;[6] and the fourth variant is miscellaneous which includes noncutaneous AS and AS arising on benign lesions such as arteriovenous malformations.

On hematoxylin and eosin-stained sections, focal areas of irregularly anastomosing vessel formation are usually present; purely epithelioid lesions are uncommon, but a completely epithelioid focus may be encountered with scanty biopsy material. Throughout the tumor, mitotic figures are numerous, and varying degrees of necrosis and hemorrhage are present.

Epithelioid AS is characterized by epithelioid morphology with scarce or even entirely absent vascular channel formation mimicking squamous cell carcinoma. In such a situation, IHC markers aid in the confirmation of diagnosis. However, even epithelioid ASs express CKs in around 50% of cases (CK was negative in our case) and they make diagnosis more challenging.[7] Vascular IHC markers have a significant role in such challenging cases. CD31 is a sensitive marker, being at least weakly positive in almost all cases. CD34 positivity ranges from 40% to 100%, and it tends to stain areas with readily apparent vessel formation. Consequently, its utility in sheeted, poorly differentiated lesions is limited.[1] Another new marker of lymphatic endothelium monoclonal antibody D2-40 is found to be positive in vascular tumors such as Kaposi's sarcoma and a subset of ASs.[8] Recently, FLI-1 has been reported as the first nuclear marker of endothelial differentiation.[9]

Al-Abbadi et al. in their study to evaluate the positivity of epithelial cell markers showed that CK was positive in only one and EMA was positive in two out of 33 cases of AS.[10]

In the present case, neoplastic cells expressed CD31, D2-40, and FLI-1 positivity and were negative for CK and EMA.


  Conclusion Top


Epitheloid AS with its diversity of clinical presentations, histopathological resemblance to epitheloid tumors, and overlapping IHC markers positivity poses a diagnostic challenge to both dermatologists and pathologists. This case is being reported for its rarity, early onset and diagnostic dilemma associated with it.

Acknowledgement

I would like to thank Dr. Premalatha, Kidwai Memorial Institute of Oncology, Bengaluru.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hart J, Mandavilli S. Epithelioid angiosarcoma: A brief diagnostic review and differential diagnosis. Arch Pathol Lab Med 2011;135:268-72.  Back to cited text no. 1
    
2.
Fletcher CD, editors. Diagnostic Histopathology of Tumors. 3rd ed. Philadelphia: Elsevier Limited.; 2007. p. 66-7.  Back to cited text no. 2
    
3.
Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ. Epithelioid angiosarcoma of deep soft tissue: A distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol 1991;15:915-24.  Back to cited text no. 3
    
4.
Mobini N. Cutaneous epithelioid angiosarcoma: A neoplasm with potential pitfalls in diagnosis. J Cutan Pathol 2009;36:362-9.  Back to cited text no. 4
    
5.
Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema; a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64-81.  Back to cited text no. 5
    
6.
Goette DK, Detlefs RL. Postirradiation angiosarcoma. J Am Acad Dermatol 1985;12:922-6.  Back to cited text no. 6
    
7.
Agaimy A, Kirsche H, Semrau S, Iro H, Hartmann A. Cytokeratin-positive epithelioid angiosarcoma presenting in the tonsil: A diagnostic challenge. Hum Pathol 2012;43:1142-7.  Back to cited text no. 7
    
8.
Kahn HJ, Bailey D, Marks A. Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas. Mod Pathol 2002;15:434-40.  Back to cited text no. 8
    
9.
Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos AP, et al. Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004;17:547-52.  Back to cited text no. 9
    
10.
Al-Abbadi MA, Almasri NM, Al-Quran S, Wilkinson EJ. Cytokeratin and epithelial membrane antigen expression in angiosarcomas: An immunohistochemical study of 33 cases. Arch Pathol Lab Med 2007;131:288-92.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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