[IPSILATERAL SYNCHRONOUS FUMARATE HYDRATASE-DEFICIENT RENAL CELL CARCINOMA AND MULTILOCULAR CYSTIC RENAL NEOPLASM OF LOW MALIGNANT POTENTIAL: A CASE REPORT AND LITERATURE REVIEW].

Q4 Medicine Japanese Journal of Urology Pub Date : 2022-01-01 DOI:10.5980/jpnjurol.113.42
Kohki Doi, Hironobu Okugi, Hiroshi Okazaki, Hayato Ikota, Toshiyuki Nakamura
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Abstract

A 75-year-old man was being followed up at a nearby clinic for hypertension and chronic renal failure. The patient was referred to our department as abdominal ultrasound revealed a left renal tumor. Plain computed tomography (CT) showed a 50 mm complex renal cyst in the upper pole of the left kidney. Plain magnetic resonance imaging showed a cystic mass with numerous septa. Partial thickening of the septa was suspected, and the lesion was classified as Bosniak IIF or III. As the patient had renal dysfunction, regular imaging study of the tumor lesion was performed to determine the timing of surgery. In the following year, plain CT revealed a new renal tumor 20 mm in diameter located lateral to the known tumor, with the mass having a tendency to increase. The patient underwent a laparoscopic radical left nephrectomy after the introduction of hemodialysis. Histopathological examination revealed that the tumor located in the medial upper pole of the left kidney was a multilocular cystic renal neoplasm of low malignant potential and that the new tumor located lateral to the known tumor was fumarate hydratase-deficient renal cell carcinoma. Simultaneous occurrence of fumarate hydratase-deficient renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential in the ipsilateral kidney is extremely rare. We report our case with a review of the literature.

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【同侧同步富马酸水合酶缺乏型肾细胞癌和低恶性潜能多房囊性肾肿瘤1例报告并文献复习】。
一名75岁男子因高血压和慢性肾衰竭在附近的一家诊所接受随访。病人因腹部超声显示左肾肿瘤而被转介到我科。CT平扫显示左肾上极一50毫米复杂肾囊肿。磁共振平扫显示囊性肿块伴大量间隔。怀疑鼻中隔部分增厚,病变分类为Bosniak IIF或III级。由于患者有肾功能不全,定期对肿瘤病变进行影像学检查以确定手术时机。次年CT平扫发现肾新发肿瘤,直径20mm,位于已知肿瘤外侧,肿块有增大趋势。患者在引入血液透析后接受了腹腔镜根治性左肾切除术。组织病理学检查显示,位于左肾内上极的肿瘤为低恶性潜能的多室囊性肾肿瘤,位于已知肿瘤外侧的新肿瘤为富马酸水合酶缺陷型肾细胞癌。同侧肾脏同时发生富马酸水合酶缺乏的肾细胞癌和低恶性潜能的多室囊性肾肿瘤是极为罕见的。我们在回顾文献的同时报告我们的病例。
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Japanese Journal of Urology
Japanese Journal of Urology Medicine-Urology
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