Primary renal synovial sarcoma presenting with a retroperitoneal bleed

• Published in: BMJ case reports Vol. 14; no. 3; p. e237099
• Main Authors: Huned, Daanesh, Kam, Juinn Huar, Lee, Lui Shiong, Tiwari, Raj Vikesh
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 18-03-2021; BMJ Publishing Group
• Subjects: Abdomen; Angiomyolipoma - surgery; Case Report; Case reports; Chemotherapy; Histology; Humans; Kidney cancer; Kidney Neoplasms - complications; Kidney Neoplasms - diagnosis; Kidney Neoplasms - surgery; Metastasis; Nephrectomy; Retroperitoneal Space - diagnostic imaging; Sarcoma; Sarcoma, Synovial - complications; Sarcoma, Synovial - diagnosis; Sarcoma, Synovial - surgery; Tumors; Urological cancer; Young adults; urological cancer; urological surgery
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2020-237099

Synovial sarcomas are most commonly localised in extremities, especially in the lower thigh and knee areas. Comprising less than 1% of all malignancies, retroperitoneal synovial sarcoma is very rare with primary synovial sarcoma of the kidney being even more infrequent and difficult to diagnose. We describe a case report of a renal synovial sarcoma in a young adult who was initially managed as a case of Wunderlich’s syndrome secondary to what was believed to be a ruptured renal angiomyolipoma. After biopsy confirmation, the patient was eventually managed with neo-adjuvant chemotherapy followed by a right radical nephrectomy and right hepatectomy. Despite its rarity, synovial sarcoma should be considered as differential diagnosis of a bleeding retroperitoneal soft tissue mass detected in young adults.