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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 57-59

Epithelioid sarcoma: A diagnostic challenge of a rare presentation


1 Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Plastic Surgery, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Date of Web Publication28-Jul-2016

Correspondence Address:
Dr. Kafil Akhtar
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.187205

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  Abstract 

Epithelioid sarcoma, first described by Enzinger in 1970, is a rare, slow-growing, malignant, soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. It is associated with a high incidence of local recurrence and metastasis. Due to the tumor's infrequent occurrence and histological similarities with other disease processes, diagnosing epithelioid sarcoma has become extremely difficult. We report a rare case of epithelioid sarcoma occurring as a cauliflower-like growth between the index finger and middle finger of the left hand in a 30-year-old female with pathological challenges in the diagnosis.

Keywords: Epithelioid sarcoma, local recurrence immunohistochemistry, soft-tissue sarcoma


How to cite this article:
Akhtar K, Piyush AR, Haiyat S, Khan AH. Epithelioid sarcoma: A diagnostic challenge of a rare presentation. Arch Int Surg 2016;6:57-9

How to cite this URL:
Akhtar K, Piyush AR, Haiyat S, Khan AH. Epithelioid sarcoma: A diagnostic challenge of a rare presentation. Arch Int Surg [serial online] 2016 [cited 2021 Sep 16];6:57-9. Available from: https://www.archintsurg.org/text.asp?2016/6/1/57/187205


  Introduction Top


Epithelioid sarcoma is a rare, malignant, soft-tissue sarcoma of young adult men involving the extremities and account for less than 1% of all soft-tissue sarcomas.[1],[2] It is a slow-growing tumor with a paucity of symptoms and shows recurrence and metastasis.[3] The tumor is remarkable for the diagnostic difficulties it poses, both clinically and histologically, resulting in a high frequency of initial misdiagnosis.


  Case Report Top


A 30-year-old female presented with a painless, slow-growing, ulcerated swelling over her left palm between the index finger and middle finger for the last 2 years. On clinical examination, she was moderately built and had good general health. There was a tender, ulcerated, irregular erythematous growth with raised edges on the left palm measuring 7.0 × 6.0 cm with scarring, and it seemed to involve the underlying deeper tissues and bone [Figure 1].
Figure 1: Grossly a tender, ulcerated, irregular erythematous growth with raised edges on the left palm measuring 7.0 × 6.0 cm, with scarring and involving the underlying deeper tissues with no bony involvement seen

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A wide excision with below-wrist amputation was performed. Histopathological sections of the tumor showed an ulcerated stratified squamous epithelium with a nodular arrangement of tumor cells, polygonal in shape with large vesicular nuclei and abundant clear to eosinophilic cytoplasm. Abundant necrosis and desmoplastic reaction were seen in the background. All the resection margins were involved but there was no bony involvement [Figure 2].
Figure 2: Histopathological sections of the tumor showed ulcerated, stratified squamous epithelium with nodular arrangement of tumor cells, polygonal in shape with large vesicular nuclei and abundant clear to eosinophilic cytoplasm. Hematoxylin and eosin × 10X

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A differential diagnosis of epithelioid sarcoma, alveolar rhabdomyosarcoma clear cell sarcoma, and melanoma was made and immunohistochemical staining was done to confirm the definitive diagnosis. The tumor showed strong, diffuse positivity for cytokeratin [Figure 3] while S-100 and myogenin were negative, thus confirming a diagnosis of epithelioid sarcoma. Adjuvant six cycles of cisplatin-based chemotherapy was administered to our patient and she is doing well after 3 months of the follow-up period.
Figure 3: Immunohistochemical staining with cytokeratin 7 showed strong diffuse positivity of tumor cells. IHC cytokeratin × 40X

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  Discussion Top


Epithelioid sarcoma usually presents in 10-39 years of age with the average age of presentation being 27 years.[4] Males are affected twice more commonly than females.[5] The principal sites of affection are the fingers, hands, and forearms, with a lower frequency in the lower limbs.[4] It most commonly presents as a nodular lesion and involves the dermis and subcutaneous tissue. Early lesions are often diagnosed as indurated ulcer or infected wart.[6] Deep tumors may mimic ganglion cysts or be attached to tendons and be mistaken for giant cell tumors of tendon sheath.[5],[6]

The tumor may be confused histopathologically with alveolar rhabdomyosarcoma, clear cell sarcoma, melanoma, synovial sarcoma, squamous cell carcinoma, and infectious or palisading granuloma such as granuloma annulare or rheumatoid nodule. Its typical nodule formation, characteristic site, and pattern of involvement, larger cells with more eosinophilic cytoplasm and less mature appearance, with positivity to cytokeratin and CD 34 confirmed the diagnosis of epithelioid sarcoma. Also, the absence of striations, clear cells, keratinization, biphasic pattern, or glandular appearance with negativity to S-100 and myogenin helped in ruling out other differentials.

Although wide, total surgical excision with clear margins (amputation or wide en bloc excision) and high-dose chemoradiotherapy represent optimum treatment,[4],[5] it recurs and metastasizes even after wide excision with a metastatic rate of 40%.[2] It metastasizes through lymphatics and vascular invasion to regional lymph nodes, skin, lungs, and soft tissue of other parts of the body.[2],[6] Adverse prognostic factors included proximal location, amount of necrosis, vascular invasion, and inadequate excision.[2] Tumor grading has no prognostic value for predicting the overall survival or distant metastasis outcome.[7] Adequate treatment requires radical en bloc excision or amputation as early as possible, along with extensive lymph node dissection. The roles of radiotherapy and chemotherapy are uncertain. Being a slow-growing tumor, the patient often survives for 10 years or longer irrespective of the treatment given.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Chbani L, Guillou L, Terrier P, Decouvelaere AV, Grégoire F, Terrier-Lacombe MJ, et al. Epithelioid sarcoma: A clinicopathologic and immunohistochemical analysis of 106 cases from the French Sarcoma Group. Am J Clin Pathol 2009;131:222-7.  Back to cited text no. 1
    
2.
Alderman AK, Kim HM, Kotsis SV, Chung KC. Upper-extremity sarcomas in the United States: Analysis of the surveillance, epidemiology, and end results database, 1973-1998. J Hand Surg Am 2003;28:511-8.   Back to cited text no. 2
    
3.
Enzinger FM, Weiss SW. Soft Tissue Tumours. New York: The C.V. Mosby Company; 1983. p. 781-95.  Back to cited text no. 3
    
4.
McPhee M, McGrath BE, Zhang P, Driscoll D, Gibbs J, Peimer C. Soft tissue sarcoma of the hand. J Hand Surg Am 1999;24:1001-7.  Back to cited text no. 4
    
5.
Armah HB, Parwani AV. Epithelioid sarcoma. Arch Pathol Lab Med 2009;133:814-9.  Back to cited text no. 5
    
6.
Yoo JH, Sung SH. Epithelioid sarcoma of the spine: A case report and review. Open J Clin Diagn 2011;1:1-4.  Back to cited text no. 6
    
7.
Callister MD. Epithelioid Sarcoma: Results of conservative surgery and radiotherapy. Int J Rad Oncol Biol Phy 2001;51:384-91.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 Epithelioid Sarcoma of Upper Extremity: Diagnostic Dilemma With Therapeutic Challenges
Leon Alexander
Cureus. 2021;
[Pubmed] | [DOI]



 

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