Primary Renal Synovial Sarcoma: An Oncologic Surprise

Abstract

Primary renal synovial sarcoma is a rare tumor having a specific chromosomal translocation t(X; 18) (p11.2; q11.2). The clinical features of this tumor and radiologic appearances are quite similar to those of renal cell carcinoma. Confirmatory diagnosis requires fluorescent in situ hybridization or reverse transcriptase polymerase chain reaction validation for differentiating the tumors from sarcomatoid renal cell carcinoma. We present a case of primary renal synovial sarcoma that was diagnosed in a middle-aged man.

Primary synovial sarcoma of kidney: a rare case report with review of literature

“…Synovial sarcomas usually stain positive for CD99, vimentin, bcl2, EMA and CD 56 while they stain negatively for S100, CK and desmin [7]. It is associated with a very specific chromosomal translocation [t(X; 18) (p11.2; q11.2)], which results in fusion of the SYT gene located on chromosome 18 to SSX gene on chromosome X [3].…”

“…It has three subtypes: monophasic, biphasic and poorly differentiated. This tumor is associated with a characteristic translocation [t(X;18)(p11.2;q11.2)] forming SYT-SSX gene fusion [3]. Owing to its rarity it poses a challenge in its diagnosis and management.…”

Doxorubicin and ifosfamide for recurrent renal synovial sarcoma: The first case report in Indonesia

“…This tumor often causes diagnostic dilemmas because of its similarity with adult Wilms Tumor, sarcomatoid renal cell carcinoma, and hemangiopericytoma based on its clinical and histological features [4] , [5] . Primary renal SS rarely occurs in the kidney, with only about 50 cases reported in the literature [6] . The tumor grows slowly and mimics a benign lesion, leading to a possible misdiagnosis and improper treatment [4] .…”

Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock

“…Moreover, there are no imaging characteristics that confirm the diagnosis of synovial sarcoma (1, 4, 5). Histologically, renal synovial sarcoma can be classified as a biphasic synovial sarcoma, monophasic spindle synovial sarcoma and monophasic epithelial synovial sarcoma (6,7). These tumours have a specific chromosomal translocation t (X; 18) (p11.2; q11.2), resulting in the fusion of the synovial sarcoma genes SYT-SSX1 and SYT-SSX2, and rarely, SYT-SSX4 (6, 7).…”