Huned Daanesh, Kam Juinn Huar, Lee Lui Shiong, Tiwari Raj Vikesh
Urology, Sengkang General Hospital, Singapore
General Surgery, Sengkang General Hospital, Singapore.
BMJ Case Rep. 2021 Mar 18;14(3):e237099. 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.
滑膜肉瘤最常发生于四肢，尤其是大腿下部和膝部区域。腹膜后滑膜肉瘤占所有恶性肿瘤的比例不到1%，非常罕见，肾原发性滑膜肉瘤更为少见且诊断困难。我们报告一例年轻成人肾滑膜肉瘤病例，该患者最初被诊断为继发于肾血管平滑肌脂肪瘤破裂的温德利希综合征。活检确诊后，患者最终接受了新辅助化疗，随后进行了右肾根治性切除术和右肝切除术。尽管滑膜肉瘤罕见，但对于年轻成人发现的腹膜后出血性软组织肿块，应考虑将其作为鉴别诊断之一。
