Modified CV Regimen in Optic Pathway Glioma

Inclusion Criteria:

• Age ≥ 3months and ≤21years;

• Patients with optic pathway gliomas diagnosed by histopathology or characteristicbrain MRI and clinical features;

• Measurable lesions, surgical resection degree < 95% or postoperative residual tumor ≥1.5cm^2;

• KPS score ≥50 (age >12 years) or Lansky score ≥50 (age ≤12 years);

• Clinical symptoms such as decreased visual acuity, visual field defect, optic discedema, exophthalmia, increased intracranial pressure, diencephalic syndrome, etc;

• No dysfunction of major organs.

Exclusion Criteria:

• MRI examination is not available.

• Failing to comply with the visual examination.

• H3K27 mutations, even histopathological grade 1/2.

• Receiving any other investigational agent.

• History of allergic reactions attributed to compounds of similar chemical orbiologic composition to the drugs used in this study.

• Patients who have received organ transplants.

• Patients infected with HIV or treponema pallidum.

• Suffering from serious cardiovascular disease;T wave inversion or elevation or STsegment changes.

• Patients who had coagulation disorder and were being treated with thrombolytic oranticoagulant drugs. Patients with significant clinical bleeding symptoms or clearbleeding tendency occurred within 3 months before enrollment, such asgastrointestinal bleeding, hemorrhagic gastric ulcer, gastrointestinal perforation,baseline fecal occult blood ++ or above, intratumoral or intracranial bleeding, orvasculitis, etc. Arteriovenous thrombosis events (such as cerebrovascular accident (including transient ischemic attack, cerebral hemorrhage and cerebral infarction),deep vein thrombosis and pulmonary embolism) occurred within 6 months beforeenrollment.

• Pregnant or breastfeeding.

• Other conditions considered inappropriate by the researcher for inclusion.