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同时发生在自体显性多囊肾病人的同时发生在自体显性多囊肾病人的 侧肾细胞癌—病 报告及文献回顾
张裕 钟孝仁 陈光国
台 荣民总医院外科部 外科 国 阳明大学医学院 学科
BILATERAL RENAL CELL CARCINOMA IN AUTOSOMAL DOMINANT
POLYCYSTIC KIDNEY DISEASE - A CASE REPORT AND LITERATURE
REVIEW
Yu-Lung Chang, Hsiao-Jen Chung, Kuang-Kuo Chen
Division of Urology, Department of Surgery, Taipei Veterans General Hospital, and Department of
Urology, School of Medicine, National Yang-Ming University, Taipei, Taiwan, Republic of China
Introduction: Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney disease (ADPKD) is
a very rare disease. Since 1994 ADPKD-associated RCCs were reported for a total of about 30 cases.
Herein, we will present a rare case of bilateral renal cell carcinoma with different cell variants in
autosomal dominant polycystic kidney disease.
Case present: This 58-year-old male was a case of end-stage renal disease under regular hemodialysis
since September 1988. Bilateral multiple renal tumors were noted incidentally by ultrasonography in
April 2004 during annual follow-up. Then he was referred to our Nephrology out-patient department
(OPD) for further survey and management. The computer tomography was performed and autosomal
dominant polycystic disease with bilateral renal malignancy was suspected. Under the impression of
ADPKD with bilateral renal tumor, he received laparoscopic bilateral radical nephrectomy in May 2004.
Two tumor masses, 8x6x6 cm and 2x1x1 cm, were noted over right kidney and the pathologic reports
showed papillary renal cell carcinoma, eosinophilic variant, pT2 and pT1. Two tumor masses, 5x4x4 cm
and 4x4x3.5 cm, were noted over left kidney and the pathologic reports showed clear cell conventional
renal cell carcinoma, pT1with cystic necrosis in one mass and pT1 without necrosis in the other. The
postoperative recovery was smooth and the patient was discharged and regularly followed at OPD.
Discussion: Since 1954 there were 11 cases with bilateral renal cell carcinoma in ADPKD. In our case
there were 2 kinds of different cell variant in bilateral polycystic kidneys. The current data revealed that
the incidence of ADPKD-associated RCC is similar to that of RCC in the general population. However,
ADPKD-associated RCCs were characterized by a younger patient age, no sex preference, frequent
simultaneous or sequential bilaterality, frequent multifocality, and a preference for sarcomatoid type. The
preoperative diagnosis of RCC in the context of ADPKD may be difficult, because tumor may be masked
by the complex cystic background superimposed by bleeding, degenerated blood clot, proteinaceous
debris, and infection. So the follow-up of ADPKD patient should be very careful due to above reasons.
When we visit a patient with ADPKD, the possibility of RCC in bilateral kidney simultaneously or
sequentially should keep in mind.
395519
·上一篇：!超诊断胎儿十二指肠闭锁合并多囊肾"例
·下一篇：!超诊断胎儿期婴儿型多囊肾并脑膜膨出畸形"例
张裕 钟孝仁 陈光国
台 荣民总医院外科部 外科 国 阳明大学医学院 学科
BILATERAL RENAL CELL CARCINOMA IN AUTOSOMAL DOMINANT
POLYCYSTIC KIDNEY DISEASE - A CASE REPORT AND LITERATURE
REVIEW
Yu-Lung Chang, Hsiao-Jen Chung, Kuang-Kuo Chen
Division of Urology, Department of Surgery, Taipei Veterans General Hospital, and Department of
Urology, School of Medicine, National Yang-Ming University, Taipei, Taiwan, Republic of China
Introduction: Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney disease (ADPKD) is
a very rare disease. Since 1994 ADPKD-associated RCCs were reported for a total of about 30 cases.
Herein, we will present a rare case of bilateral renal cell carcinoma with different cell variants in
autosomal dominant polycystic kidney disease.
Case present: This 58-year-old male was a case of end-stage renal disease under regular hemodialysis
since September 1988. Bilateral multiple renal tumors were noted incidentally by ultrasonography in
April 2004 during annual follow-up. Then he was referred to our Nephrology out-patient department
(OPD) for further survey and management. The computer tomography was performed and autosomal
dominant polycystic disease with bilateral renal malignancy was suspected. Under the impression of
ADPKD with bilateral renal tumor, he received laparoscopic bilateral radical nephrectomy in May 2004.
Two tumor masses, 8x6x6 cm and 2x1x1 cm, were noted over right kidney and the pathologic reports
showed papillary renal cell carcinoma, eosinophilic variant, pT2 and pT1. Two tumor masses, 5x4x4 cm
and 4x4x3.5 cm, were noted over left kidney and the pathologic reports showed clear cell conventional
renal cell carcinoma, pT1with cystic necrosis in one mass and pT1 without necrosis in the other. The
postoperative recovery was smooth and the patient was discharged and regularly followed at OPD.
Discussion: Since 1954 there were 11 cases with bilateral renal cell carcinoma in ADPKD. In our case
there were 2 kinds of different cell variant in bilateral polycystic kidneys. The current data revealed that
the incidence of ADPKD-associated RCC is similar to that of RCC in the general population. However,
ADPKD-associated RCCs were characterized by a younger patient age, no sex preference, frequent
simultaneous or sequential bilaterality, frequent multifocality, and a preference for sarcomatoid type. The
preoperative diagnosis of RCC in the context of ADPKD may be difficult, because tumor may be masked
by the complex cystic background superimposed by bleeding, degenerated blood clot, proteinaceous
debris, and infection. So the follow-up of ADPKD patient should be very careful due to above reasons.
When we visit a patient with ADPKD, the possibility of RCC in bilateral kidney simultaneously or
sequentially should keep in mind.
395519
·上一篇：!超诊断胎儿十二指肠闭锁合并多囊肾"例
·下一篇：!超诊断胎儿期婴儿型多囊肾并脑膜膨出畸形"例
