NDP012: Fumarate hydratase-deficient renal cell carcinoma with inferior vena cava invasion-case report
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  • 2017-12-25,
  • 上傳者: TUA秘書處,
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延胡索酸水合酶缺乏腎細胞癌併下腔靜脈侵犯-案例報告
劉家翔1、林子平1, 3、林登龍1, 3
台北榮民總醫院泌尿部1國立陽明大學醫學系泌尿學科2書田泌尿科學研究中心3
Fumarate hydratase-deficient renal cell carcinoma with inferior vena cava invasion-case report
Chia-Hsiang Liu1, Tzu-Ping Lin1,3, Alex T. L. Lin1,3
1 Department of Urology; 2Department of Radiology,
Taipei Veterans General Hospital, Taiwan
3 Department of Urology, School of Medicine, and Shu-Tien Urological Institute,
National Yang-Ming University, Taipei, Taiwan
Introduction:
Papillary renal cell carcinomas (pRCCs) are a diverse family of tumors with mysteries awaiting to be unraveled. PRCCs account for up to 15% of renal cell carcinomas and share a common origin with the more frequent clear-cell subtype, arising from the proximal nephron . PRCCs can be divided into two established histological subtypes, type 1 pRCCs, which bear recurrent oncogenic MET alterations, and type 2 pRCCs, which represent a wide range of diseases including hereditary cancer syndromes such as germline mutations of Fumarate hydratase (FH). Fumarate hydratase-deficient renal cell carcinoma (FH-RCC) is a rare and aggressive tumor affecting mostly younger patients. We shared a case of older patient with Fumarate hydratase-deficient renal cell carcinoma and inferior vena cava invasion.
Case presentation:
This 92-year-old man with arrhythmia, hypertension and aortic stenosis was found renal function deterioration during OPD follow-up for benign prostatic hyperplasia. Besides, persistent stinging sensation of left flank area without knocking tenderness was noted. Urine routine only showed microscopic hematuira. Abdominal ultrasonography revealed a 5.8cm echogenic lesion at the right suprarenal region. MRI disclosed left infiltrating renal tumor with left renal vein, left adrenal vein and infrahepatic inferior vena cava invasion. Ultrasound-guided biopsy pathology showed Fumarate hydratase-deficient renal cell carcinoma. He received left radical nephrectomy with inferior vena cava thrombectomy. Pathology report revealed Fumarate hydratase-deficient renal cell carcinoma, pT3c without lymphadenopathy.
Conclusion:
Fumarate hydratase-deficient renal cell carcinoma (FH-RCC) is a rare tumor. We believed that this case was the first case in older patient of Fumarate hydratase-deficient renal cell carcinoma and inferior vena cava invasion who received radical nephrectomy with inferior vena cava thrombectomy. However, prognosis needed time to prove and follow-up.
 
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    2017-12-25 17:03:21
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    324
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    TUA秘書處
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    台灣泌尿科醫學會
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