Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock: A Rare Presentation
Main Article Content
Keywords
primary renal synovial sarcoma, rare renal tumours, renal tumour histology, soft-tissue sarcomas
Abstract
Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising.
References
2. Öztürk H. Prognostic features of renal sarcomas. Oncol Lett. 2015;9(3):1034–1038. 10.3892/ol.2014.2838
3. Faria P. Primary synovial sarcoma of the kidney: A molecular subset of so-called embryonal renal sarcoma. Mod Pathol. 1999;12:94A.
4. Kim DH, Sohn JH, Lee MC, Lee G, Yoon GS, Hashimoto H, et al. Primary synovial sarcoma of the kidney. Am J Surg Pathol. 2000;24(8):1097–1104. 10.1097/00000478-200008000-00007
5. Krishnappa P, Tabrez S, Harinatha S, Aswathaiya MB. Primary renal synovial sarcoma–A rare histology. Urol Case Rep. 2020;33:101402. 10.1016/j.eucr.2020.101402
6. Clark MA, Fisher C, Judson I, Thomas JM. Soft-tissue sarcomas in adults. N Engl J Med. 2005;353(7):701–711. 10.1056/NEJMra041866
7. Moorthy HK, Pillai BS, Varghese J. Primary renal synovial sarcoma: An oncologic surprise. Urol Case Rep. 2014;2(5):152–153. 10.1016/j.eucr.2014.07.001
8. Park SJ, Kim HK, Kim CK, Park SK, Go ES, Kim ME, et al. A case of renal synovial sarcoma: Complete remission was induced by chemotherapy with doxorubicin and ifosfamide. Korean J Intern Med. 2004;19(1):62. 10.3904/kjim.2004.19.1.62
9. Blas L, Roberti J. Primary renal synovial sarcoma and clinical and pathological findings: A systematic review. Curr Urol Rep. 2021;22(4):1–3. 10.1007/s11934-021-01038-w
10. Shuch B, Amin A, Armstrong AJ, Eble JN, Ficarra V, Lopez-Beltran A, et al. Understanding pathologic variants of renal cell carcinoma: Distilling therapeutic opportunities from biologic complexity. Eur Urol Suppl. 2015;67(1):85–97. 10.1016/j.eururo.2014.04.029