1504 | Ampullary Tumor Caused by Metastatic Re- nal Cell Carcinoma and Literature Review Wang Haidong, Wang Jianwei, Li Guizhong, Liu Ning, He Feng, Man Libo Abstract:‎We‎present‎a‎case‎of‎a‎50-year-old‎man‎with‎a‎metastasis‎to‎the‎ampulla‎of‎vater‎that‎ led‎to‎the‎discovery‎of‎renal‎cell‎carcinomas.The‎man‎was‎referred‎to‎us‎because‎of‎jaundice.‎ Computed‎tomography‎(CT)‎scan‎of‎the‎abdomen‎showed‎irregular‎masses‎in‎the‎right‎kidney.‎ Magnetic‎resonance‎imaging‎(MRI)‎revealed‎dilatation‎of‎the‎bile‎duct.‎The‎patient‎underwent‎ right‎nephrectomy‎and‎pancreatoduodenectomy.‎Postoperative‎histopathologic‎examination‎re- vealed‎clear‎cell‎carcinoma‎in‎both‎the‎renal‎and‎ampullary‎lesions.‎After‎a‎5-year‎follow-up‎,‎ the‎patient‎was‎alive‎with‎no‎evidence‎of‎recurrent‎disease. Keywords:‎ampullary‎neoplasm,‎renal‎cell‎carcinoma,‎metastasis INTRODUCTION Renal‎cell‎carcinomas‎account‎for‎3-4%‎of‎all‎cancers‎and‎have‎a‎predilection‎to‎metastasize‎to‎rare‎locations.‎The‎ampullary‎region‎isn’t‎a‎usual‎site‎of‎metastatic‎malignancy.‎The‎ampulla‎of‎vater‎metastatic‎lesion‎from‎renal‎cell‎carcinoma‎is‎ an‎extremely‎rare‎occurrence.‎This‎is‎the‎first‎case‎that‎metastatic‎tumor‎of‎ampulla‎of‎vater‎ was‎disclosed‎before‎renal‎cell‎carcinoma.‎The‎objective‎of‎this‎case‎report‎is‎to‎present‎the‎ unusual‎clinical‎case‎and‎assess‎the‎role‎of‎surgical‎management. Corresponding Author: Man Libo, MD Beijing jishuitan Hospital, Beijing 100035, China. Tel: +86 010 58398240 E-mail: doctorwhd@163.com Received August 2012 Accepted March 2013 Beijing jishuitan Hospital, Beijing 100035, China. CASE REPORT Case Report 1505Vol. 11 | No. 02 | March- April 2014 |U R O LO G Y J O U R N A L Ampullary Tumor Caused by Metastatic RCC | Haidong et al CASE REPORT Here‎we‎report‎an‎unusual‎clinical‎case‎of‎a‎50-year-old‎man‎ with‎metastasis‎from‎renal‎cell‎carcinoma‎to‎the‎ampulla‎of‎ vater.‎The‎patient‎was‎hospitalized‎with‎a‎one-month‎history‎of‎ fever,‎malaise,‎fatigue,‎and‎jaundice.‎The‎patient‎suffered‎from‎ diarrhea‎sometimes‎and‎lost‎2‎kilograms‎in‎recent‎few‎months.‎ On‎admission,‎the‎patient‎was‎pale‎and‎anemic.‎The‎hemo- globin‎was‎88g/L.‎Stool‎was‎positive‎for‎occult‎blood.‎Results‎ of‎pertinent‎laboratory‎studies‎on‎admission‎showed:‎serum‎ glutamic-oxaloacetic‎ transaminase(GOT),‎ 146‎ IU/L;‎ glutam- icpyruvic‎transaminase‎(GPT),‎324‎IU/L;‎total‎bilirubin‎(TBIL),‎ 41‎μmol/L;‎carcinoembryonic‎antigen‎(CEA),‎5.6‎ng/mL.‎MRI‎ disclosed‎dilatation‎of‎bile‎duct‎(Figure‎1).‎Ultrasonic‎sound‎ showed‎that‎there‎is‎a‎low‎echo-level‎tumor‎in‎right‎kidney.‎A‎ CT‎scan‎confirmed‎that‎the‎irregularly‎shaped‎tumor‎was‎situ- ated‎in‎the‎upper‎part‎of‎right‎kidney‎(Figures‎2‎and‎3).‎ The‎tentative‎preoperative‎diagnosis‎was‎synchronous‎pri- mary‎cancers‎of‎the‎kidney‎and‎the‎ampulla‎of‎vater.‎The‎ patient‎underwent‎open‎right‎radical‎nephrectomy‎and‎stand- ard‎pancreatoduodenectomy.‎The‎postoperative‎course‎was‎ uneventful.‎The‎renal‎lesion‎was‎4‎‎3‎‎2‎cm,‎and‎microscopy‎ was‎consistent‎with‎clear-cell‎carcinoma‎(Figure‎4).‎The‎final‎ pathological‎diagnosis‎of‎the‎ampullary‎lesions‎was‎metastat- ic‎clear‎cell‎carcinoma‎of‎the‎kidney‎(Figure‎5).‎All‎lymph‎ nodes‎and‎the‎margins‎of‎resection‎of‎the‎common‎bile‎duct,‎ pancreas,‎duodenum,‎jejunum,‎and‎ureter‎were‎negative‎for‎ tumor.‎The‎patients‎received‎systemic‎therapy‎(IFN-α-2b)‎af- ter‎the‎radical‎nephrectomy.‎Follow-up‎studies,‎including‎CT‎ scan‎of‎the‎abdomen,‎chest‎radiography,‎and‎laboratory‎stud- ies,‎were‎done.‎Five‎years‎later,‎the‎patient‎was‎alive‎without‎ evidence‎of‎recurrent‎disease. DISCUSSION In‎2010‎kidney‎cancer‎accounted‎for‎4%‎and‎3%‎of‎all‎newly‎ diagnosed‎malignancies‎in‎men‎and‎women,‎respectively.‎From‎ 80%‎to‎85%‎of‎kidney‎cancers‎are‎renal‎cell‎carcinomas.‎ (1) The‎tumor‎is‎commonly‎large‎at‎presentation‎and‎symptoms‎ may‎not‎occur‎until‎relatively‎late‎in‎the‎disease.‎(2) At the time‎of‎diagnosis,‎20-30%‎of‎patients‎present‎with‎metastatic‎ Figure 1. MRI showing dilatation of bile duct. Figure 2. CT scan showing a mass in the right kidney Figure 3. CT scan showing that the tumor was situated in the upper part of right kidney 1506 | Case Report disease,‎40-50%‎of‎renal‎cell‎carcinomas‎patients‎develop‎ metastatic‎disease‎eventually‎and‎20-30%‎ patients‎ relapse‎ distantly‎after‎radical‎nephrectomy.(3)‎Kidney‎cancer‎is‎one‎ of‎the‎most‎deadly‎urological‎tumors.‎The‎5-year‎survival‎ rate‎for‎all‎stages‎is‎approximately‎69.5%.(4)‎If‎detected‎early,‎ renal‎cell‎carcinomas‎can‎be‎treated‎surgically,‎and‎5-year‎ survival‎rates‎approaching‎85%‎can‎be‎achieved‎for‎patients‎ with‎organ-confined‎disease‎(stages‎T1,‎T2,‎and‎N0).(1)‎The‎ prognosis‎ of‎ metastatic‎ renal‎ cell‎ carcinomas‎ is‎ generally‎ poor;‎median‎survival‎is‎10‎months‎and‎five-year‎survival‎is‎ less‎than‎5%.(5)‎Until‎recently,‎very‎few‎systemic‎therapeutic‎ options‎existed‎for‎locally‎advanced‎or‎metastatic‎renal‎cell‎ carcinoma‎patients.‎Immunotherapy‎with‎IFN-α‎can‎be‎con- sidered‎a‎treatment‎option‎to‎modestly‎improve‎survival‎and‎ disease control in patients.(6)‎Our‎patient‎has‎survived‎for‎5‎ years‎after‎surgery‎with‎the‎treatment‎of‎IFN-α-2b.‎The‎pa- tient‎was‎admitted‎every‎3‎months‎for‎routine‎examinations,‎ and‎there‎is‎no‎evidence‎of‎recurrent. Renal‎cell‎carcinoma‎represents‎a‎potentially‎lethal‎cancer‎that‎ has‎a‎propensity‎for‎metastatic‎spread.‎The‎most‎frequent‎sites‎ of‎metastasis‎are‎the‎lung,‎lymph‎nodes,‎liver,‎bone‎and‎adre- nal glands.(7)‎Metastasis‎of‎renal‎cell‎carcinoma‎to‎Pancreatic‎ and‎gallbladder‎is‎rare.‎Ampulla‎of‎vater‎metastasis‎from‎renal‎ cell‎carcinoma‎is‎extremely‎rare,‎and‎very‎few‎cases‎have‎been‎ reported‎ in‎ literatures.‎The‎ epidemiology,‎ clinical‎ presenta- tion,‎and‎treatment‎of‎ampulla‎of‎vater‎metastases‎from‎renal‎ cell‎carcinoma‎are‎less‎known.‎The‎efficiency‎of‎surgery‎in‎ the‎management‎of‎these‎patients‎has‎not‎been‎clearly‎defined.‎ According‎to‎document(8)‎and‎our‎experience,‎one‎thing‎is‎for‎ sure,‎that‎is‎the‎relatively‎good‎prognosis. The‎symptoms‎of‎patient‎presented‎here‎is‎indistinguishable‎ from‎those‎of‎primary‎cancer‎of‎the‎ampulla‎of‎vater.‎Only‎a‎ few‎patients‎with‎renal‎cell‎carcinoma‎can‎get‎early‎diagno- sis,‎which‎results‎in‎a‎high‎proportion‎of‎patients‎with‎metas- tases.(9)‎In‎recent‎years,‎the‎widespread‎application‎of‎CT‎and‎ ultrasonography‎for‎other‎indications‎has‎led‎to‎the‎increased‎ detection‎of‎renal‎cell‎carcinoma‎as‎an‎incidental‎finding.‎Tu- mors‎found‎incidentally‎are‎typically‎smaller‎than‎those‎that‎ produce‎symptoms,‎and‎are‎more‎likely‎to‎be‎resected‎for‎ cure.(10)‎In‎our‎patient,‎the‎renal‎tumor‎was‎also‎found‎inci- dentally‎through‎CT‎and‎ultrasonography,‎and‎the‎tumor‎was‎ not‎large.‎The‎kidney‎and‎tumor‎was‎removed‎completely‎ and‎smoothly,‎which‎leads‎to‎long‎survival‎of‎the‎patient. CONCLUSION Renal‎cell‎carcinoma‎represents‎a‎potentially‎lethal‎cancer‎as- sociated‎with‎aggressive‎behavior.‎The‎pattern‎of‎metastases‎ from‎renal‎cell‎carcinoma‎to‎the‎ampulla‎of‎vater‎is‎rare,‎and‎ radical‎nephrectomy‎and‎standard‎pancreatoduodenectomy‎ can‎be‎a‎therapy‎of‎choice.‎The‎prognosis‎is‎relatively‎good. Figure 4. Histopathologic appearance of renal cell carcinoma (Hematoxylin and Eosin stain, ×40) Figure 5. Histopathological view of the ampullary tumor (Hematoxylin and Eosin stain, ×40) 1507Vol. 11 | No. 02 | March- April 2014 |U R O LO G Y J O U R N A L REFERENCES 1. Jemal A, Siegel R, Xu J, Ward E. Cancer statistics, 2010. CA Cancer J Clin. 2010;60:277-300. 2. Furniss D, Harnden P, Ali N, Royston P, Eisen T, Oliver RT, Hancock BW. National Cancer Research Institute Renal Clinical Studies Group. Prognostic factors for renal cell carcinoma. Cancer Treat Rev. 2008;34:407-26. 3. Bukowski RM. Prognostic factors for survival in metastatic renal cell carcinoma: update 2008. 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