# Primary Renal Synovial Sarcoma: A Report of a Rare Case and Management Approach

## TL;DR

This paper reports a rare case of primary renal synovial sarcoma and outlines the challenges in diagnosing and managing this uncommon kidney cancer.

## Contribution

The paper contributes a detailed case report and management approach for a rare form of synovial sarcoma originating in the kidney.

## Key findings

- A 35-year-old male was diagnosed with primary renal synovial sarcoma through histopathology and SS18 gene translocation testing.
- The patient underwent radical nephrectomy followed by adjuvant chemotherapy with the AIM regimen.
- The case highlights the diagnostic and therapeutic challenges associated with this rare malignancy.

## Abstract

Primary renal synovial sarcoma (PRSS) is an exceedingly rare malignancy. Due to its rarity, the diagnosis and management of PRSS remain challenging, as there are no standardized treatment guidelines.

We present a case of a 35-year-old male who presented with right flank pain for two months. Contrast-enhanced CT (CECT) revealed a 10 × 11 × 12 cm mass at the upper pole of the right kidney. The patient underwent radical nephrectomy, and histopathological examination suggested synovial sarcoma. To confirm the diagnosis, we performed a translocation study, which identified the SS18 gene translocation at 18q11, a hallmark of synovial sarcoma. Postoperatively, the patient received adjuvant chemotherapy with the AIM (doxorubicin, ifosfamide, and mesna) regimen.

Given the extreme rarity of PRSS, we discuss the diagnostic challenges, molecular characteristics, and treatment approach adopted at our institution, contributing to the limited but growing body of knowledge on this rare entity.