Epithelioid sarcoma: A case series of three different presentations

Abstract

Background: Epithelioid sarcoma is a rare malignancy that presents diagnostic difficulties.

Cases: We report three cases of epithelioid sarcoma with different presentations and clinical course. One patient had a classicaltype epithelioid sarcoma while rest two suffered from the rarer proximal-type. All three underwent surgical excision while two of them also had postoperative adjuvant therapy. The diagnostic challenges and the final outcome has been discussed in detail.

Conclusion: The diagnosis of epithelioid sarcoma needs a high index of clinical suspicion and immunohistochemistry is extremely valuable. Adequacy of index surgery affects the ultimate prognosis.

Keywords

Epithelioid sarcoma / Distal type / Proximal type / Classical type / Immunohistochemistry

References

[1] Enzinger FM. Epithelioid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer. 1970; 26(5): 1029-1041. https://doi.org/10.1002/1097-0142(197011)26:5＜1029::AID-CNCR2820260510＞3.0.CO;2-R

[2] Armah HB, Parwani AV. Epithelioid sarcoma. Arch Pathol Lab Med. 2009 May; 133(5): 814-9. PMid: 19415960.

[3] Karagkounis G, Argyrakos T, Charkiolakis G, et al. A case of distal epithelioid sarcoma of the thumb expressing podoplanin, TLE1 and Ca 125. Case Rep Pathol [Internet]. 2013 [cited 2017 Mar 3]; 2013. Available from: https://www.hindawi.com/journals/cripa/2013/312786/abs/

[4] Echchaoui A, Sadrati Y, Elbir Y, et al. Proximal-type epithelioid sarcoma: a new case report and literature review. Pan Afr Med J [Internet]. 2016 [cited 2017 Mar 3]; 24(1). Available from: http://www.ajol.info/index.php/pamj/article/view/144247

[5] Guillou L, Wadden C, Coindre JM, et al. " Proximal-type" Epithelioid Sarcoma, a Distinctive Aggressive Neoplasm Showing Rhabdoid Features: Clinicopathologic, Immunohistochemical, and Ultrastructural Study of a Series. Am J Surg Pathol. 1997; 21(2): 130-146. PMid: 9042279. https://doi.org/10.1097/00000478-199702000-00002

[6] Bhama BA, Dhir RS, Pandit AA. Epithelioid sarcoma of the upper extremity (a case report). J Postgrad Med. 1987; 33(3): 140. PMid: 3430402.

[7] Jawad MU, Extein J, Min ES, et al. Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database. Clin Orthop. 2009 Nov; 467(11): 2939-48. PMid: 19224301. https://doi.org/10.1007/s11999-009-0749-2

[8] Asano N, Yoshida A, Ogura K, et al. Prognostic Value of Relevant Clinicopathologic Variables in Epithelioid Sarcoma: A Multi-Institutional Retrospective Study of 44 Patients. Ann Surg Oncol. 2015 Aug 1; 22(8): 2624-32. PMid: 25663591. https://doi.org/10.1245/s10434-014-4294-1

[9] Suárez-Zamora DA, Barrera-Herrera LE, Rodríguez-Urrego PA, et al. Proximal-Type Epithelioid Sarcoma: Report of an Unusual Case in the Uterine Cervix. Int J Surg Pathol. 2017 Feb 1. https://doi.org/10.1177/1066896917696744

[10] Santos LM dos, Nogueira L, Matsuo CY, et al. Proximal-type epithe-lioid sarcoma-Case report. An Bras Dermatol. 2013; 88(3): 444-447. PMid:23793215. https://doi.org/10.1590/abd1806-4841.20131922

[11] Hasegawa T, Matsuno Y, Shimoda T, et al. Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases. Mod Pathol. 2001; 14(7): 655-663. PMid: 11454997. https://doi.org/10.1038/modpathol.3880368

[12] Yang GZ, Li J, Jin H, et al. Is mammary not otherwise specified-type sarcoma with CD10 expression a distinct entity? A rare case report with immunohistochemical and ultrastructural study. Diagn Pathol. 2013; 8(1): 14. PMid:23356903. https://doi.org/10.1186/1746-1596-8-14

[13] Iwata J, Fletcher CD. Immunohistochemical detection of cytokeratin and epithelial membrane antigen in leiomyosarcoma: a systematic study of 100 cases. Pathol Int. 2000; 50(1): 7-14. PMid: 10692172. https://doi.org/10.1046/j.1440-1827.2000.01001.x