DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20212253

Rare case of primary esophageal synovial sarcoma with (x;18) translocation presenting as dysphagia

Anuradha Sekaran, Purva Shinde, Veena Vanere, Mohan Ramchandani, Duvvur N. Reddy

Abstract


Synovial sarcoma (SS) in young adult mainly involves periarticular region of the extremities. Synovial sarcomas are exceedingly rare neoplasms of the digestive tract. In this report, we describe a very rare occurrence of primary SS of the esophagus in a 30-year-old female. Patient presented with dysphagia. Endoscopy showed submucosal esophageal polyp. Piecemeal polypectomy was done. Histologically, the tumor demonstrated biphasic morphology with epithelial and mesnchymal component. Tumor cells expressed pancytokeratin, bcl-2 and CD99 antigens. Differential diagnosis of synovial sarcoma and epithelial mesenchymal biphasic tumor was made. Cytogenetics was done to confirm the diagnosis of SS. It showed translocation (x;18). Synovial sarcomas are very rare tumor entities, particularly in the gastrointestinal tract and are likely to be mistaken with other more common tumors such as gastrointestinal stromal tumors.


Keywords


Synovial sarcoma, Cytogenetics, Translocation (x;18), Biphasic tumor

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References


Golioto M, McGrath K. Primary lymphoma of the esophagus in a chronically immunosuppressed patient with hepatitis C infection: case report and review of the literature. Am J Med Sci. 2001;321(3):203-5.

Patricia S, Saikat D, Rajesh B, Rajesh I, Selvamani B, Subhashini J. Rare Cause of Stricture Esophagus-Sarcoma: A Case Report and Review of the literature. Case Rep Gastrointest Med. 2011;192423.

Butori C, Hofman V, Attias R, Mouroux J, Pedeutour F, Hofman P. Diagnosis of primary esophageal synovial sarcoma by demonstration of t(X;18) translocation: a case report. Virchows Arch. 2006;449(2):262-7.

Sasaki. K, Noda. M, Tsuruda, Y. Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review. Surg Case Rep. 2020;6:176.

Lokesh V, Naveen T, Pawar YS. Spindle cell sarcoma of esophagus: a rare case presentation. J Cancer Res Ther. 2010;6(1):100-1.

Ioannidis JP, Lau J. 18F-FDG PET for the diagnosis and grading of soft-tissue sarcoma: a meta-analysis. J Nucl Med. 2003;44(5):717-24.

Billings SD, Meisner LF, Cummings OW, Tejada E. Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization. Mod Pathol. 2000;13(1):68-76.

Adad SJ, Etchebehere RM, Hayashi EM. Leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review. Am J Trop Med Hyg. 1999;60(5):879-81.

Raza MA, Mazzara PF. Sarcomatoid carcinoma of esophagus. Arch Pathol Lab Med. 2011;135(7):945-8.

Maggiani F, Debiec-Rychter M, Ectors N, Lerut A, Sciot R. Primary epithelioid sarcoma of the oesophagus. Virchows Arch. 2007;451(4):835-8.

Nakagawa, S., Yabusaki, H. & Tanaka, O. Rapid-growth carcinosarcoma of the esophagus arising from 0-IIc squamous cell carcinoma after definitive chemoradiotherapy: a case report. Esophagus. 2009;6:123-6.

Maesawa C, Iijima S, Sato N, et al. Esophageal extraskeletal Ewing's sarcoma. Hum Pathol. 2002;33(1):130-2.

Baranov E, McBride MJ, Bellizzi AM. A Novel SS18-SSX Fusion-specific Antibody for the Diagnosis of Synovial Sarcoma. Am J Surg Pathol. 2020;44(7):922-33.

Lee SH, Chang MH, Baek KK. High-dose ifosfamide as second- or third-line chemotherapy in refractory bone and soft tissue sarcoma patients. Oncology. 2011;80(3-4):257-61.

Demetri GD, von Mehren M, Jones RL. Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. J Clin Oncol. 2016;34(8):786-93.