NDP067: Extraperitoneal laparoscopic repair of huge inguinoscrotal bladder hernia: A case report and literature review
針對延胡索酸水合酶缺陷型腎細胞癌的免疫治療：三個病例報告
陳思如，楊晨洸, 裘坤元
台中榮民總醫院泌尿科
Immunotherapy for Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC): three cases report
Szu-Ju Chen, Cheng-Kuang Yang, Kun-Yuan Chiu
Division of Urology, Department of Surgery, Taichung Veteran General Hospital
Introduction:
Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) is a rare and highly aggressive renal malignancy. Atezolizumab (AZ) is a monoclonal antibody medication used to treat many cancers included urothelial carcinoma. It is a fully humanized, engineered monoclonal antibody of IgG1 isotype against the protein programmed cell death-ligand 1 (PD-L1). We reported here with experience of surgical treatment and AZ immunotherapy for 3 patients with DH-deficient RCC.
Patients and Methods:
We had 3 male patients with FH-deficient RCC. Their age was 40,42 and 59 separately (mean 47-year-old). All patients underwent radical nephrectomy and pathological staging above T3a. They all treated by AZ immunotherapy after surgery. However, there were tumor progression after AZ therapy and therapy was shifted to target therapy in two patients. One patient exerted progress free after AZ therapy with 2 years follow-up.
Discussion:
Hereditary leiomyomatosis-RCC syndrome (HLRCC) is thought to be associated with a germ-line mutation of FH gene which is mapped to chromosome 1q43. FH is an enzyme with two functions: to facilitate a transition step in the production of energy and to metabolize fumarate. FH-deficient RCC has aggressive nature and presentats frequently manifested in a status of metastasis even in a small size. Complete surgical resection and retroperitoneal lymph node resection should be performed promptly when the tumor is discovered. In our limited experience, FH-deficient RCC characteristics rapid progression and partial response to AZ. More patients experience is warrant for clinical confirm the pharmacological effect of AZ.