Type II Neurofibromatosis
Conditions
Interventions
Case series:Bevacizumab therapy
Sponsors
Shanghai Ninth People's Hospital affiliated to Shanghai JiaoTong University, School of Medicine
Eligibility
Sex/Gender
All
Age
6 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. Aged 6 years or greater; 2. Patients must have a confirmed diagnosis of type II neurofibromatosis; 3. Life expectancy of greater than 1 year; 4. Evidence of progressive hearing loss (with decrease in speech discrimination score) related to VS with a speech discrimination score of 3,000/mcL; Absolute neutrophil count > 1,500/mcL; Platelets > 100,000/mcL; Total bilirubin within normal institutional limits; AST (SGOT)/ALT (SGPT) <2.5 X institutional upper limit of normal; Creatinine clearance=60ml/min/1.73 m2 or a normal serum creatinine; 24-hour urine protein level should be <1000 mg; 6. Subjects must have a VS not amenable to surgery or have refused surgery due to high risk for permanent complications related to surgery as determined by a surgeon with experience in management of NF2; 7. Subjects must have had a discussion of all available treatment options including surgery, radiation therapy, observation, other clinical trials and expressed their preference for participation in this trial in the informed consent process.
Exclusion criteria
Exclusion criteria: 1. Patients who have had chemotherapy or radiotherapy within 4 weeks prior to entering the study or those who have not recovered from adverse events due to agents administered more than 4 weeks earlier; 2. Patients with known hypersensitivity of Chinese hamster ovary cell products, other recombinant human antibodies, or compounds of similar chemical or biologic composition to bevacizumab; 3. Inability to tolerate periodic MRI scans; 4. Uncontrolled intercurrent illness including ongoing or active infection, or psychiatric illness/social situations that would limit compliance with study requirements; 5. Pregnant or fertile women; 6. Participating in any other clinical trial; 7. History of cardiovascular disease,such as: Inadequately controlled HTN; History of CVA within 12 months; Myocardial infarction or unstable angina within 12 months; New York heart association grade II or greater congestive heart failure; Serious and inadequately controlled cardiac arrhythmia; Significant vascular disease (e.g., aortic aneurysm, history of aortic dissection); Clinically significant peripheral vascular disease; 8. Concurrent use of anti-coagulant drugs (not including prophylactic doses), history of coagulopathy, or evidence of bleeding diathesis or coagulopathy; 9. Imaging (CT or MRI) evidence of newly identified hemorrhage (new within the last in the 6 months prior to enrollment), any history of symptomatic intracranial hemorrhage, or any history of spontaneous intracranial hemorrhage; 10. Serious or non-healing wound, ulcer or bone fracture; 11. History of abdominal fistula, gastrointestinal perforation or intra-abdominal abscess within 6 months; 12. Invasive procedures be defined as Major surgical procedure; Brain biopsy within 28 days; Anticipation of need for major surgical procedures during the course of the study; Core biopsy within 7 days; 13. Prior treatment with bevacizumab or other VEGF targeting therapies; 14. Personal history of autoimmune coagulopathy, including idiopathic thrombocytopenia purpura (ITP); 15. Patients couldn't finish follow-up.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Speech Discrimination Score in the target ear; | — |
Secondary
| Measure | Time frame |
|---|---|
| 0??13??25??49??72?;Volume Decreased Rate of vestibular schwannomas in the contralateral ear;Volume Decreased Rate of other tumors; | — |
Countries
China
Contacts
Public ContactWANG Zhaoyan
Shanghai Ninth People's Hospital affiliated to Shanghai JiaoTong University, School of Medicine
Outcome results
None listed