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原发性肾滑膜肉瘤:1例罕见病例报告

Primary Renal Synovial Sarcoma: A Rare Case Presentation.

作者信息

Abbas Mansoor, Majeed Muhammad Awais, Liaqat Maryyam, Bano Shehar, Khalid Tuba, Chaudhry Nahel, Qayum Faisal, Ahmed Shahzeb, Karim Amna, Javaid Farzouq

机构信息

Medical Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, PAK.

Internal Medicine, King Edward Medical University, Lahore, PAK.

出版信息

Cureus. 2024 Oct 24;16(10):e72256. doi: 10.7759/cureus.72256. eCollection 2024 Oct.

DOI:10.7759/cureus.72256
PMID:39583501
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11584758/
Abstract

Synovial sarcoma is a rare mesenchymal tumor, and its occurrence as a primary renal tumor is exceedingly rare. We are presenting a case of renal synovial sarcoma with lung involvement in a 47-year-old female patient who initially presented with typical renal symptoms, including blood in urine and left flank pain. Imaging revealed a large renal mass with extension into the renal vein and metastatic nodules in the lungs. Histopathological examination and genetic analysis confirmed monophasic synovial sarcoma with SYT-SSX2 translocation. Treatment included radical nephrectomy followed by systemic chemotherapy with doxorubicin and ifosfamide. Despite the initial response, the disease progressed, leading to fatal complications. This case highlights the diagnostic challenges, limited treatment options, and poor prognosis associated with primary renal synovial sarcoma.

摘要

滑膜肉瘤是一种罕见的间叶组织肿瘤,而其作为原发性肾肿瘤的情况极为罕见。我们报告一例47岁女性肾滑膜肉瘤伴肺转移的病例,该患者最初表现为典型的肾脏症状,包括血尿和左侧腰痛。影像学检查显示肾脏有一个大肿块,延伸至肾静脉,并伴有肺部转移结节。组织病理学检查和基因分析证实为单相性滑膜肉瘤伴SYT-SSX2易位。治疗包括根治性肾切除术,随后使用阿霉素和异环磷酰胺进行全身化疗。尽管最初有反应,但疾病仍进展,导致致命并发症。该病例凸显了原发性肾滑膜肉瘤在诊断方面的挑战、有限的治疗选择以及预后不良。

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