AL101 Before Surgery for the Treatment of Notch Activated Adenoid Cystic Cancer

Inclusion Criteria: * Capable of giving signed informed consent which includes compliance with the requirements and restrictions listed in the informed consent and in this protocol * Age \>= 18 years old * Histologically/cytological confirmed adenoid cystic carcinoma (ACC) of any primary site * Evidence of NOTCH1 pathway activation as determined by NICD1 IHC nuclear staining in \>= 70% of tumor cells * Patients must have surgically resectable disease, either with a curative intent or for local control in the setting of metastatic disease, in the opinion of the treating physician * Patients must be willing to undergo baseline biopsy to obtain tumor material * Disease must be measurable by RECIST 1.1 * Performance status Eastern Cooperative Oncology Group (ECOG) 0 or 1 * Neutrophils \< 1500/mm\^3 * Platelet count \< 100,000/mm\^3 * Hemoglobin \< 9 g/dL * Total bilirubin \> 1.5 upper limit of normal (ULN) (except known Gilbert's syndrome) * Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) \> 2.5 of upper limit of normality (ULN) OR \> 5 ULN for patients with liver metastases * Creatinine clearance \< 40 mL/min (Calculation of creatinine clearance \[CrCl\] will be based on acceptable institution standard) * Female patients with reproductive potential must practice two effective contraceptive measures for the duration of study drug therapy and for at least 90 days after completion of AL101 therapy. The two birth control methods can be either two barrier methods…