Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock

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.

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients

“…Tumors reported in the literature ranged in age from 4 to 76 years with a median of 36.2 years. [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] In published cases, 3% of patients were younger than 18 years of age which is less than in our cohort. There is a slight male predominance both in published cases (M: F = 1.14:1) and in our cohort (1.8:1).…”

“…The most commonly reported symptoms were hematuria (47%) or pain (43%) or palpable mass (38%). [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] In our cohort, flank pain was the most common symptom (100%), followed by hematuria (35%) and hypertension (35%). Of all published cases, three occurred during pregnancy.…”

“…The most common histopathologic type in the published tumors was monophasic synovial sarcoma (75%) followed by biphasic (14%) and poorly differentiated (11%) synovial sarcoma. [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] In our cohort, all 14 tumors were monophasic with 72% being monophasic spindle cell type and 22% being monophasic round cell type and 6% being monophasic round to epithelioid cell type. In the published patients, surgery was the most common treatment modality (96%) with chemotherapy as adjuvant treatment in 32% of patients.…”

“…In the published patients, the median follow-up was 12 months, mortality was 29%, and the recurrence rate was 40%. [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]30 In our cohort, the median follow-up was 11 months, mortality was 28% and no recurrence was observed.…”

“…5,6 Primary renal synovial sarcoma is one of the rarest types of sarcomas of kidney and comprises 2% of all malignant renal tumors and 1% of all synovial sarcomas across the anatomic sites. 7 The first case of primary renal synovial sarcoma was reported by Argani et al in 2000 and to date, 88 case reports and 23 case series, [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] with only two case series consisting of more than 14 tumors, have been reported in the literature. 8,9 The most common presenting symptoms include hematuria, pain, and palpable mass.…”