Primary synovial sarcoma of the kidney. A case report with pathologic appraisal investigation and literature review

A. Pitino, S. Squilaci, C. Spairani, M. F. Cosimi, E. Feyles, D. Rici, F. Bardari, M. Graziano, F. Morabito, F. Cesarani, M. Garuso, M. Beleti, K. Beierl, K. M. Murphy

Research output: Contribution to journalArticle

Abstract

Synovial sarcoma (SS) is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features that usually arises in the extremities of young adults. The occurrence of these tumors in the kidney is extremely rare and have been prevalently described in case reports. The objectives of this work were to evaluate the frequency of primary renal synovial sarcomas and the pathologic progression in recognition of this possibly under-diagnosed entity. A comprehensive review of the literature has also been performed with a focus on survival. We report the clinico-pathological features of an intrarenal SS occurring in a 67-year-old man. The tumour, measuring 4 cm in its greatest diameter, completely replaced the cortex and the medulla of the inferior region of the left kidney compressing the iliopsoas muscle. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumour was composed of atypical monotonous vimentin+, CD99+, bcl-2+ spindle cells exhibiting a haphazard fascicular growth pattern and a high mitotic rate (3 to 5 mitoses per HPF). The diagnosis was supported by reverse transcription-polymerase chain reaction which demonstrated SYT-SSX2 gene fusion. The patient was alive with local recurrence of disease 24 months after surgery. Synovial sarcomas occurring in the kidney, in analogy to other sites, tend to have an aggressive biologic behaviour. Despite being extremely uncommon, with only 44 cases reported to date, they should be included in the differential diagnosis of benign and malignant spindle cell tumours of the kidney. This study also emphasizes the importance of a correct pathologic diagnosis for prognostic and therapeutic implications.

Original languageEnglish (US)
Pages (from-to)271-278
Number of pages8
JournalPathologica
Volume103
Issue number5
StatePublished - Oct 2011
Externally publishedYes

Keywords

  • Differential diagnosis
  • Renal neoplasms
  • Synovial sarcoma
  • SYT/SSX gene fusion

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Pitino, A., Squilaci, S., Spairani, C., Cosimi, M. F., Feyles, E., Rici, D., Bardari, F., Graziano, M., Morabito, F., Cesarani, F., Garuso, M., Beleti, M., Beierl, K., & Murphy, K. M. (2011). Primary synovial sarcoma of the kidney. A case report with pathologic appraisal investigation and literature review. Pathologica, 103(5), 271-278.