成人多囊性腎臟腫瘤-案例報告

陳建凱、陳俊吉、石宏仁

彰化基督教醫院泌尿外科

Multilocular Cystic Nephroma of adult: a case report

Jian-Kai Chen, Chun Chi Chen, Hung-Jen Shih

Division of Urology, Department of Surgery, Changhua Christian Hospital

Multilocular cystic nephroma is a rare cystic renal tumor, which is benign and has a favorable prognosis. It was initially described in 1892, and there have only been roughly 200 cases published in the associated literature. Here, we presented a case with multilocular cystic nephroma and described the clinical presentation as well as image, management, pathology and follow up condition after nearly half year.

The patient is a 63-years-old married Taiwanese male worker with a history of Right renal stone s/p ESWL on 2023/05/15. He presented with complaints of right flank soreness and nocturia occurring twice per night. A diagnostic work-up in 2023/08 included a KUB and renal sonogram, revealing the presence of a bilateral renal stone and a right renal cyst. Further management was advised as Retrograde Intrarenal Surgery (RIRS), which the patient declined. Subsequently, a computed tomography (CT) scan was arranged, which disclosed multiple cystic lesions occupying the enlarged lower pole and interpolar region of the right kidney. The nature of these cystic lesions was unknown, and cystic renal dysplasia was suspected. These lesions measured 10 x 14 cm in size. A nephrectomy was suggested as a possible course of action. Additionally, a Tc-99m DTPA renal function scan was performed, indicating a total glomerular filtration rate (GFR) of 92 ml/min, with a splitting GFR of 67 ml/min (73%) for the left kidney and 25 ml/min (27%) for the right kidney. This revealed right hydronephrosis with markedly impaired function. On 2023/10/04 the patient was admitted for a scheduled hand-assisted laparoscopic right radical nephrectomy to address the concerning renal condition. Then, the patient was admitted for further care. We prescribed empirical antibiotics with Cefazolin, adequate pain control and intravenous fluid hydration. Flatus passage was noted on post-operatively. There was no discomforts after taking diet. We followed up the wound condition, Jackson-Vac drain and urethral Foley's catheter drainage day by day. Fever with chillness was noted on 2023/10/10, and we adjusted antibiotic use. The wounds showed well-healing without pus formation. Fever subsided. We removed CVC on post-operation day 5, urethral Foley's catheter on post-operation day 8 and Jackson-Vac drain on post-operation day 9. The patient could urinate smoothly by herself. There was no abdominal pain, nausea, vomit after the operation. Under relatively stable condition, the patient was discharged on 2023/10/13. During the post operation following up period, the pathology report revealed that multilobular renal cystic lesion lined by simple layer of flat epithelium with clear fluid with intervening atrophic tubules and fibrous tissue, microscopically. The lining cells showed positive over cytokeratin and PAX8, but negative on CD31, CA9 (-), melan-A (-) and SMA (-). Therefore, multilocular cystic nephroma was diagnosed.