Abstract:

This article reports a case of bilateral multifocal renal carcinoma associated with hyperthyroid heart disease caused by Von Hippel-Lindau （VHL） syndrome.The patient was admitted with a history of "chest tightness and palpitations for 3 months，accompanied by shortness of breath for 3 days，" and was initially diagnosed with hyperthyroid heart disease.Imaging studies revealed multiple renal masses，and after receiving medical treatment，his symptoms improved and he was discharged，the renal mass was untreated.Subsequently，the patient underwent renal mass biopsy and genetic testing at another hospital，which confirmed the diagnosis of VHL syndrome with bilateral clear cell renal carcinoma.Due to the patient's III-level heart failure，he was unable to tolerate surgery and was treated with oral axitinib，but the response was poor.Then the patient was readmitted to our hospital again，after multidisciplinary consultation，underwent catheter-based radiofrequency ablation for rapid atrial fibrillation，resulting in improvement of heart function.A follow-up CT scan revealed locally advanced renal cancer in both kidneys with a right renal vein thrombus.The patient insisted on preserving kidneys and first underwent laparoscopic left partial nephrectomy.After three cycles of axitinib combined with tiragolumab treatment，the right renal cancer was stable on imaging.Through the multidisciplinary consultation by Guangdong Urological Association，it was recommended that the patient undergo right radical nephrectomy due to the complexity and high risk of kidney-preserving surgery for the right kidney.However，the patient insisted on preserving the right kidney.After thorough communication，the multidisciplinary team performed "laparoscopic right nephrectomy + tabletop renal tumor resection + autologous kidney orthotopic transplantation." Postoperatively，follow-up showed no residual tumor in both kidneys，and renal function was well preserved.The patient began receiving atezolizumab-assisted immunotherapy.The tabletop kidney-preserving surgery provided a new treatment option for patients with locally advanced renal carcinoma who strongly wish to preserve kidney function，although future treatment and follow-up still pose challenges.

Key words: VHL syndrome, Renal cell carcinoma, Locally advanced, Tabletop kidney-preserving surgery, Autologous kidney transplantation