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Year : 2010  |  Volume : 53  |  Issue : 1  |  Page : 170-171
Epithelioid sarcoma with angiomatous pattern: A diagnostic dilemma

LTM General Hospital & Municipal Medical College, India

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Date of Web Publication19-Jan-2010

How to cite this article:
Chaturvedi M, Khare MS. Epithelioid sarcoma with angiomatous pattern: A diagnostic dilemma. Indian J Pathol Microbiol 2010;53:170-1

How to cite this URL:
Chaturvedi M, Khare MS. Epithelioid sarcoma with angiomatous pattern: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2010 [cited 2023 Jun 7];53:170-1. Available from:


Epithelioid sarcoma (ES), a rare high-grade soft tissue sarcoma, has a remarkable capacity to mimic a variety of benign and malignant lesions, both clinically and on histology, resulting in frequent initial misdiagnosis. [1],[2] It can show a pseudo vascular pattern and hemorrhages which can look like angio sarcoma (AS). [1] We report an ES case with angiomatous pattern misinterpreted as AS on light microscopy.

A 40-year-old male presented with a gradually progressing painless swelling over the right upper arm since three months. His complaints started three years ago when he had trauma to a pea sized swelling present at the same site since childhood. The swelling was incised and the biopsy was reported as granuloma pyogenicum, elsewhere. The swelling persisted and gradually increased in size. On examination a firm, blackish, nodular swelling was found with a scar of previous surgery. It measured 6x5x4 cm. There was no bleeding or discharge from the swelling. A biopsy performed was reported as AS.

A wide local excision with axillary dissection was performed. The specimen consisted of skin-covered elliptical tissue measuring 9.5x 5x 4.5 cm. External surface showed scar. Cut surface showed hard nodular grey white mass with focus of blackish blood filled cystic spaces.

Microscopic examination of the mass showed a nodular tumor in the dermis with extension up to the subcutaneous tissue. Epidermis was unremarkable. Like an AS, the tumor was composed of a proliferation of cells with epithelioid features and spindle cells with moderate amount of nuclear pleomorphism and prominent nucleoli, lining anastomosing vascular spaces and dissecting collagen. In addition, there were tumor cells with intracytoplasmic vacuoles, as well as prominent foci of hemorrhage and blood filled spaces, features suggestive of angiomatous process. [Figure 1]a-c Therefore, on light microscopy, diagnosis of AS was considered and immunohistochemistry was advised for confirmation as this facility is not available in our institute. The tissue was sent outside for immunohistochemistry and tumor cells were found to be positive for epithelial markers -cytokeratins 1 and 2 and for mesenchymal marker CD 34 and negative for specific vascular marker CD 31. [Figure 2]

Thus diagnosis of epithelioid sarcoma was established . Both ES and AS may stain for CK and CD34 but absence of specific endothelial marker CD31 in ES allows their distinction. [1] Due to the presence of pseudo AS pattern and absence of distinct nodular arrangement of tumor cells with central necrosis (necrotizing pseudo granulomas) and densely hyalinized collagen, typical of ES, [1] misinterpretation occurred on light microscopy.

This prompted us to do a literature search and we came across a similar type of report of epithelioid AS mimicking ES by Von Hochstetter et al. [3] Thus, Immunohistochemistry played a significant role in resolving the dilemma.

   References Top

1.Weiss SW, Goldblum JR. Malignant soft tissue tumours of uncertain type. In: Enzinger FM, Weiss SW, editors. Soft tissue tumours 4th ed. St Louis: Mosby; 2001. p. 1521-38.  Back to cited text no. 1      
2.Pai KK, Pai SB, Sripathi H, Saha PK, Rao P. Epithelioid sarcoma: A diagnostic challenge. Indian J Dermatol Venereol Leprol 2006;72:446-8.  Back to cited text no. 2  [PUBMED]  Medknow Journal  
3.Von Hochstetter AR, Meyer VE, Grant JW, Honegger HP, Schreiber A. Epithelioid sarcoma mimicking angiosarcoma: value of immunohistochemistry in the differential diagnosis. Virchows Arch A Pathol Anat Histopathol 1991;418:271-8.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  

Correspondence Address:
Madhu Chaturvedi
B-406, Queens, Hiranandani estate, Thane (W), Maharashtra- 400 0607
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.59220

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  [Figure 1], [Figure 2]

This article has been cited by
1 Long period of relative quiescence in distal-type epithelioid sarcoma of the forearm with recurrence after surgery
Xiupeng Zhang,Biying Jiang,Xiuying Shi,Chuifeng Fan
Medicine. 2018; 97(36): e12276
[Pubmed] | [DOI]


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