Inclusion Criteria:

          1. age ≥ 18;

          2. histology characteristics accord with FH-deficient RCC;

          3. gene testing confirms germline and/or somatic FH gene mutation ;

          4. ECOG (Eastern Cooperative Oncology Group)≤2;

          5. expected survival >3 months;

          6. blood routine indexes: neutrophils ≥1.5*109, platelets ≥100*109, hemoglobin ≥90g/L;

          7. liver function: bilirubin ≤ normal upper limit 1.5 times, AST≤ normal upper limit 2.5
             times;Serum creatinine ≤ 1.5 times of normal upper limit;Serum calcium concentration:
             ≤12.0 mg/dL;

          8. coagulation function: PT≤ 1.5 times of normal upper limit;

          9. the following diseases did not appear within 12 months: myocardial infarction, severe
             or unstable angina pectoris, asymptomatic heart failure, cardiovascular and
             cerebrovascular accident or transient ischemic attack, etc.

         10. all patients signed informed consent.

        Exclusion Criteria:

          1. other malignancies previously or at the same time that are different from the primary
             site or histology of the tumor assessed in this study, except cervical carcinoma in
             situ, basal-cell carcinoma that has been fully treated, superficial bladder tumor (Ta,
             Tis, T1) or other malignancies that occurred before the enrollment and have been cured
             for more than 3 years;

          2. renal decompensation requires hemodialysis or peritoneal dialysis;

          3. arrhythmia need anti-arrhythmic treatment, symptomatic coronary artery disease or
             myocardial ischemia (myocardial infarction), nearly six months, or congestive heart
             failure than NYHA Ⅱ level; Hypertension (systolic blood pressure >160 mmHg or
             diastolic blood pressure >100 mmHg) that has been treated with 2 or more
             antihypertensive treatments and still cannot be controlled;

          4. severe active clinical infection;

          5. patients with coagulation disorder or bleeding constitution;

          6. major surgery or severe trauma was performed within 4 weeks before enrollment;

          7. a history of allogeneic organ transplantation or bone marrow transplantation;

          8. drug abuse and medical, psychological or social conditions that may interfere with
             patients' participation in research or affect the evaluation of results;

          9. known or suspected allergy to the study drug;

         10. those who received treatment other than this study within 4 weeks prior to and during
             the study period.