Abstract:FIGURE 1 Coronal section of a computed-tomographic scan of the abdomen and pelvis, showing large right retroperitoneal hematoma with indwelling punctate calcifications, raising concern for underlying retroperitoneal or renal neoplasia and mass. Right kidney is displaced antero-inferiorly. FIGURE 2 Cross-section of the abdomen and pelvis with contrast, showing the liver displaced to the left (1) and the inferior vena cava displaced anteriorly and to the left (2).
“…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).…”
Section: Discussionmentioning
confidence: 76%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Primary renal synovial sarcoma is a rare aggressive mesenchymal neoplasm of the kidney that accounts for less than 1% of renal sarcomas. Herein, we describe the clinicopathologic and molecular findings of 14 renal synovial sarcoma patients in one of the largest case series to date and to our knowledge, the only renal synovial sarcoma series to use novel SS18-SSX IHC. Clinicopathologic, IHC, molecular, management, and follow-up data were reviewed and analyzed. Macroscopically, the tumors had either homogeneous, tan-white, and solid ( n = 10), variegated and solid ( n = 3), or variegated and solid-cystic ( n = 1) cut surfaces. Spindle cell ( n = 10), round cell ( n = 3), and round to epithelioid morphologies ( n = 1) were observed. SS18-SSX IHC was positive in all 14 tumors (diffuse, n = 10; multifocal, n = 2; focal, n = 2). All the tumors harbored SS18::SSX1/2 gene rearrangement. Metastases to the liver, brain, and lung ( n = 1); liver and bone ( n = 1); liver and diaphragm ( n = 1) were identified. Adjuvant chemotherapy was administered in 11/12 patients. Follow-up was available for 10 patients (time period range: 5 to 24 months). Four patients died of disease, and six patients are alive with no recurrence or metastasis. As SS18-SSX IHC showed an excellent concordance with the FISH results, this may reliably be used in the IHC panel of spindle/round cell sarcomas of the kidney and as a molecular surrogate for renal synovial sarcoma, particularly in a resource-limited setting. Also, the tumors with focal SS18-SSX expression had lower break apart signals in the FISH assay (19% and 23% in two tumors with focal SS18-SSX IHC positivity).
“…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).…”
Section: Discussionmentioning
confidence: 76%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Primary renal synovial sarcoma is a rare aggressive mesenchymal neoplasm of the kidney that accounts for less than 1% of renal sarcomas. Herein, we describe the clinicopathologic and molecular findings of 14 renal synovial sarcoma patients in one of the largest case series to date and to our knowledge, the only renal synovial sarcoma series to use novel SS18-SSX IHC. Clinicopathologic, IHC, molecular, management, and follow-up data were reviewed and analyzed. Macroscopically, the tumors had either homogeneous, tan-white, and solid ( n = 10), variegated and solid ( n = 3), or variegated and solid-cystic ( n = 1) cut surfaces. Spindle cell ( n = 10), round cell ( n = 3), and round to epithelioid morphologies ( n = 1) were observed. SS18-SSX IHC was positive in all 14 tumors (diffuse, n = 10; multifocal, n = 2; focal, n = 2). All the tumors harbored SS18::SSX1/2 gene rearrangement. Metastases to the liver, brain, and lung ( n = 1); liver and bone ( n = 1); liver and diaphragm ( n = 1) were identified. Adjuvant chemotherapy was administered in 11/12 patients. Follow-up was available for 10 patients (time period range: 5 to 24 months). Four patients died of disease, and six patients are alive with no recurrence or metastasis. As SS18-SSX IHC showed an excellent concordance with the FISH results, this may reliably be used in the IHC panel of spindle/round cell sarcomas of the kidney and as a molecular surrogate for renal synovial sarcoma, particularly in a resource-limited setting. Also, the tumors with focal SS18-SSX expression had lower break apart signals in the FISH assay (19% and 23% in two tumors with focal SS18-SSX IHC positivity).
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