Inclusion Criteria

INCLUSION CRITERIA

1. Patients must have genetically or clinically confirmed VHL disease and have symptoms
from VHL that are no longer controllable by conventional approaches.

2. Patients must have at least one of the following lesions :

- Eye : retinal hemangioblastoma that can no longer be treated by laser therapy or
cryotherapy and resulting in progressive loss of vision;

- CNS : cerebellar, bulbar, spinal, or cerebellopontine angle haemangioblastoma or
endolymphatic sac tumor causing neurological symptoms that are not amenable to
further surgery, or have recurred after a first surgery;

- Kidney: multiple or bilateral tumors not accessible to conservative surgery, or
tumors having recurred after surgery and/or radiofrequency ablation or
advanced/metastatic RCC;

- Pancreas: inextirpable or advanced neuroendocrine tumors.

3. Patients previously treated for VHL with surgery, chemotherapy or radiotherapy are
considered eligible for this study under the condition that these treatments were
completed more than 4 weeks prior starting the study treatment. Previously radiated
lesions will be considered as target lesions only if they demonstrate unequivocal
evidence of growth upon imagery.

4. Male or female, at least 18 year-old.

5. Performance status ECOG 0-2

6. Life expectancy = 3 months

7. Biological/clinical values within the following limits:

- Total serum bilirubin = 1.5 x ULN (patients with Gilbert's disease are not
eligible)

- Serum transaminases and alkaline phosphatases = 2.5 x ULN, or in case of
underlying malignancy (hepatic metastasis) = 5x ULN

- Serum creatinine = 1.5 x ULN, creatinine clearance = 80 ml/min

- Absolute neutrophil count = 1500/mm3

- Platelets = 100,000/mm3

- Hemoglobin = 9.0 g/dL

- QTc interval = 450 msec

- Left ventricular ejection fraction (LVEF) = lower limit of institutional normal
as assessed by multigated acquisition (MUGA) scan or echocardiogram

8. Eligibility of patients receiving any medications or substances which may alter the
activity or pharmacokinetics of sunitinib (CYP3A4 inhibitors or inducers among which
ketoconazole, theophylline, phenobarbital, coumadin/warfarin) will be decided after
review by the principal investigator of possibility to interrupt or switch to other
medications. Otherwise, patient is not eligible. Anticoagulants drugs (among which
coumadin/warfarin) may be, either switched to low-molecular-weight heparin, or be
subject to individual dose adaptation in order to maintain INR in the target range
with regard to patient's history, all along his participation in the study.

9. Signed and dated informed consent document stating that the patient, or legally
representative, has been informed of all the aspects of the trial prior to enrollment

10. Willingness and ability to comply with all protocol assessments, schedule of visits,
and procedures that are or could be requested as part of this study.

11. Affiliated to French social security system

EXCLUSION CRITERIA

1. Chemotherapy, radiotherapy, radiofrequency or surgery within 4 weeks prior to
entering the study or not complete recovering from adverse events due to drugs
administered more than 4 weeks earlier.

2. Patients receiving any other investigational agent or having participated in a
clinical trial in the last 30 days.

3. History of allergic reaction attributed to compounds of similar chemical or
biological composition to sunitinib.

4. Previous treatment with sunitinib

5. NCI-CTCAE grade = 3 hemorrhage within 4 weeks prior to study entry.

6. History of known or suspected brain metastases, spinal cord compression,
carcinomatous meningitis, evidence of leptomeningeal disease (excepted leptomeningeal
hemangioblastoma, according to the neurologist) on screening CT scan or MRI.

7. Any of the following within the 6 months prior to study drug administration:
symptomatic congestive heart failure, myocardial infarction or coronary artery
bypass, pulmonary embolism, ongoing severe or unstable angina pectoris, NCI-CTCAE
grade = 2 cardiac dysrhythmia, cerebrovascular accident or transient ischemic attack.

8. Hypertension >140/90 mmHg that cannot be controlled despite optimal antihypertensive
therapy.

9. Pre-existing thyroid abnormality with thyroid function that cannot be maintained in
the normal range despite medication.

10. Other severe acute or chronic medical condition including (but not limited to),
ongoing infection, unstable or uncompensated respiratory, cardiac, hepatic or renal
disease, psychiatric condition, laboratory abnormality that would impart, in the
judgment of the investigator, excess risk associated with study participation or
study drug administration, or which would make the patient inappropriate for entry
into the trial.

11. Any medical condition (gastric or small intestine pathology, malabsorption syndrome)
that might interfere with oral medication absorption.

12. Known HIV-positive patients treated with antiretroviral therapy (potential
pharmacokinetic interactions with sunitinib).

13. Pregnancy or breastfeeding. Patients must agree to use effective contraception during
the study, including oral contraceptives, intrauterine devices, or being unable to
procreate.

14. Any other malignancy within the last 3 years excepted basal cell carcinoma, in situ
cervical carcinoma, squamous cell skin cancer, pT1/a bladder cancer with no evidence
of recurrence during the last 12 months.