Binimetinib in Patients With BRAF Fusion-positive Low-grade Glioma or Pancreatic Cancer (Perfume)

Inclusion Criteria

• •1. BRAF fusion or rearrangement is detected by reimbursed NGS-based cancer gene panel tests, cancer gene panel tests performed under advanced medical treatment, or clinical study (including liquid biopsy).
• •2. Unresectable or recurrent
• •3. No symptomatic brain metastasis, carcinomatous meningitis or spinal metastasis requiring surgical intervention or radiotherapy
• •4. No cardiac effusion, pleural effusion, or ascites requiring treatment
• •5. Not received anti-cancer drug within 14 days before registration, nor received other study drug (molecular targeting drug, immune therapy) within 21 days before registration
• •6. Not received operation under general anesthesia within 28 days before registration
• •7. Not received radiation therapy (including gamma knife, cyber knife) within 14 days before registration
• •8. Left ventricular ejection fraction \>= 50% by echocardiography or MUGA (multigated acquisition scan) within 28 days before registration
• •9. Having all laboratory tests performed within 14 days before registration and the values are within the following range. Patients should not receive administration of G-CSF and/or blood transfusion within 14 days before the blood collection (1) Absolute neutrophil count \>= 1.500/mm3 (2) Platelet count \>= 10.0 X 10(4))/mm3 (3) Hemoglobin \>= 8.0 g/dL (4) Total bilirubin \<= 1.5 g/dL (5) Aspartate aminotransferase (AST) \<= 100 U/L (6) Alanine aminotransferase (ALT) \<= 100 U/L (7) Serum creatinine \<= 1.5 mg/dL
• •10. Patients who are able to swallow orally administered medication.
• •11. Consent to at least 30 days of contraception and limited egg donation (including egg retrieval for future egg transfer) after last administration of study drug for child-bearing status women. Consent to 90 days of contraception and limited sperm donation after last administration of study drug for men.
• •12. Written informed consent (When registering patient under 18, a signed consent form must be obtained from both the patient and the parent or legal guardian.)
• •Cohort A
• •13. Histopathologically diagnosed as low-grade glioma, based on WHO classification of 2007, 2016 and 2021. The grade is WHO grade 1 or 2.
• •14. Age at the time of registration is 12 years or older (When registering a patient under 18, a signed consent form must be obtained from both the patient and the parent or legal guardian), and patients who are 12-17 years old have to be 40 kg or over in body weight. There is no limitation in body weight for patients who are 18 years or older.
• •15. Lansky Performance Status (LPS) \>= 70 for patients 12-15 years old Karnofsky Performance Status (KPS) \>= 70 for patients 16 years or older
• •16. Having measurable disease within 28 days before registration
• •17. Patients suffice the following. (1) Having adequate initial treatment depending on the primary central nervous tumor including surgery if recommended treatment is available. (2) Neurologically stable.
• •(3) Multiple lesion or dissemination is not detected with MRI at the registration.
• •18\) Not increased steroid for low-grade glioma within 14 days before registration and the dosage of steroid in equivalent to 50 mg prednisolone or less.
• •Cohort B 19) Histopathologically diagnosed as pancreatic cancer (histologically not specified).
• •20\) Having progression after at least one regimen of chemotherapy excluding adjuvant therapy.
• •21\) Age at the time of registration is 18 years or older. 22) Performance Status (ECOG) is 0 or 1 23) Having measurable disease within 28 days before registration detected by enhanced CT (Head, chest, abdominal, pelvic: under 5 mm in slice)