Synovial sarcoma of the kidney: a clinicopathologic, immunohistochemical, and molecular genetic study of 16 cases.
We report the clinicopathologic and immunohistochemical features of 16 cases of genetically confirmed primary synovial sarcoma of the kidney. The cases occurred in 9 men and 7 women ranging in age from 17 to 78 years (mean, 46 y). The tumors were grossly large, solid, and variably cystic (2.2 to 19.0 cm; mean 8.6 cm). Microscopically, all tumors were of the monophasic type and diffusely immunoreactive for TLE1 and BCL-2. Focal pankeratin positivity was found in just under half. Ten cases carried an SS18-SSX2 fusion transcript, and 5 cases showed an SS18-SSX1 transcript by reverse transcription polymerase chain reaction. The remaining case demonstrated SS18 rearrangement by fluorescence in situ hybridization. Clinical follow-up information was available for 12 patients (range, 1 to 77 mo; mean, 32.5 mo). Fourteen patients underwent radical nephrectomy, and 3 patients had lung metastases at presentation. Six patients died of disease within 1 to 58 months (mean, 31 mo) of their diagnosis. Five patients were alive without evidence of disease 12 to 77 months (mean, 39 mos) after surgery. A single patient was alive with metastases to the spine 11 months after surgery. We conclude that renal synovial sarcoma is an aggressive tumor, with adverse patient outcome in >50% of cases. Synovial sarcoma must be distinguished from morphologically similar lesions of the kidney.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
John Kenneth
JK
Schoolmeester
John C
JC
Cheville
Andrew L
AL
Folpe
D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D056970:Co-Repressor Proteins; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007680:Kidney Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D025202:Molecular Diagnostic Techniques; D009363:Neoplasm Proteins / Q000235:genetics; D009392:Nephrectomy; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011237:Predictive Value of Tests; D011518:Proto-Oncogene Proteins / Q000235:genetics; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000032:analysis; D012097:Repressor Proteins / Q000032:analysis / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013584:Sarcoma, Synovial / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000556:secondary / Q000601:surgery; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D047368:Tumor Burden; D055815:Young Adult
D014408:Biomarkers, Tumor; D056970:Co-Repressor Proteins; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D011518:Proto-Oncogene Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D012097:Repressor Proteins; C093961:SS18 protein, human; C116512:SYT-SSX fusion protein; C499043:TLE1 protein, human; C093962:synovial sarcoma X breakpoint proteins