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一位年轻孕妇的肾滑膜肉瘤:病例报告及临床病理特征

Renal Synovial Sarcoma in a Young Pregnant Lady: A Case Report and Clinico-Pathological Profile.

作者信息

Pathrose Gregory, John Nirmal Thampi, Hariharan Pradeep

机构信息

Assistant Professor, Department of Urology, Mar Baselious Medical Mission, Ernakulam, Kerala, India.

Professor, Department of Urology, Christian Medical College, Vellore, Tamil Nadu, India.

出版信息

J Clin Diagn Res. 2017 Jul;11(7):PD13-PD14. doi: 10.7860/JCDR/2017/25733.10245. Epub 2017 Jul 1.

Abstract

Synovial sarcoma is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features. These tumours usually arise in the extremities of young adults. Their occurrence in the kidney is extremely rare. A 25-year-old pregnant lady in her first trimester was incidentally found to have a left renal mass on perinatal ultrasonography. MRI showed a well encapsulated, heterointense mass replacing the left kidney. Following medical termination of her pregnancy, a radical nephrectomy was performed. Histopathology revealed a primary synovial cell sarcoma of the kidney. Postoperatively, she received ifosfamide based adjuvant chemotherapy. This report highlights the challenges involved in the diagnosis of this extremely rare neoplasm. A high index of clinical suspicion, complimented by the use of immunohistochemistry and cytogenetics during histopathological analysis aide in the diagnosis. Aggressive management with a combination of complete surgical extirpation and chemotherapy gives the best results.

摘要

滑膜肉瘤是一种具有明确组织学、免疫组化和分子特征的软组织肿瘤。这些肿瘤通常发生在年轻成年人的四肢。它们在肾脏中的发生极为罕见。一名25岁处于孕早期的孕妇在围产期超声检查时偶然发现左肾有一肿块。磁共振成像(MRI)显示一个边界清晰、信号不均的肿块取代了左肾。在她药物流产后,进行了根治性肾切除术。组织病理学显示为原发性肾滑膜细胞肉瘤。术后,她接受了以异环磷酰胺为基础的辅助化疗。本报告强调了诊断这种极其罕见肿瘤所涉及的挑战。高度的临床怀疑,辅以组织病理学分析过程中免疫组化和细胞遗传学的应用有助于诊断。采用完全手术切除和化疗相结合的积极治疗可取得最佳效果。

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