Nivolumab with or without Stereotactic Radiosurgery in Treating Patients with Recurrent or Metastatic Chordoma

Inclusion Criteria

• Patients must have histologically confirmed diagnosis of chordoma; the pathologic confirmation may be from another metastatic site
• Patients may have metastases, with newly identified peripheral metastases
• Cross-sectional imaging evidence of progression of recurrent/metastatic disease
• Previous treatment information (name of agent, treatment starting date, and date of progression) must be available for review
• Measurable disease in one or more sites (Per Response Evaluation Criteria in Solid Tumors [RECIST] criteria: Measurable lesions are defined as those that can be accurately measured in at least one dimension [longest diameter to be recorded] as >= 10 mm with computed tomography [CT] scan, magnetic resonance imaging [MRI], or calipers by clinical exam. All tumor measurements must be recorded in millimeters [or decimal fractions of centimeters])
• Karnofsky performance scale >= 70%
• White blood cells (WBC) >= 3,000/mcL
• Absolute neutrophil count >= 1,500/mcL
• Platelets >= 100,000/mcL
• Total bilirubin =< 2 x institutional upper limit of normal
• Aspartate aminotransferase (AST) (serum glutamic oxaloacetic transaminase [SGOT])/alanine aminotransferase (ALT) (serum glutamic pyruvic transaminase [SGPT]) =< 2.5 x institutional upper limit of normal
• Creatinine within normal institutional limits OR according to institutional MRI policy
• Women of child bearing potential (WOCBP) should use an adequate method to avoid pregnancy for 5 months plus the time required for nivolumab to undergo approximately five half-lives) after the last dose of investigational drug; in order for a woman to be determined not of child-bearing potential, she must have >= 12 months of non-therapy-induced amenorrhea or be surgically sterile
• Men receiving nivolumab and who are sexually active with WOCBP will be instructed to adhere to contraception for a period of 7 months after the last dose of investigational product
• Ability to understand and the willingness to sign written informed consent document(s)

Exclusion Criteria

• Prior chemotherapy within 21 days or 5 half-lives (whichever is shorter) before starting treatment
• Prior therapy with investigational agent within 21 days or 5 half-lives (whichever is shorter) before starting treatment
• Prior therapy with an anti-PD-1, anti-PD-L1, or anti-PDL-2 antibody
• Neurologic dysfunction that would confound the evaluation of neurologic and other adverse events
• Known allergy to compounds of similar chemical or biologic composition to nivolumab
• Pregnant or breastfeeding women
• Known history of human immunodeficiency virus
• Active infection requiring therapy, including positive tests for hepatitis B surface antigen or hepatitis C ribonucleic acid (RNA)
• Active autoimmune disease, history of autoimmune disease or history of syndrome that required systemic steroids or immunosuppressive medications, e.g. organ, tissue, or allogenic hematopoietic stem cell transplant (HSCT) recipients; exceptions include those with resolved childhood asthma/atopy; subjects with asthma who require intermittent use of bronchodilators (such as albuterol) will not be excluded from this study; subjects are also permitted to enroll if they have vitiligo, type I diabetes mellitus, residual hypothyroidism due to autoimmune condition only requiring hormone replacement, psoriasis not requiring systemic treatment, or conditions not expected to recur in the absence of an external trigger
• Patients should be excluded if they have a condition requiring systemic treatment with either corticosteroids (> 10 mg daily prednisone equivalents) or other immunosuppressive medications within 14 days of study drug administration; inhaled or topical steroids and adrenal replacement doses > 10 mg daily prednisone equivalents are permitted in the absence of active autoimmune disease
• Use of live vaccines against infectious diseases up to 4 weeks (28 days) before receiving nivolumab. (NOTE: Inactivated seasonal influenza vaccines are permitted and do not require a 4 week waiting period before starting study treatment)
• Prisoners or subjects who are compulsorily detained for treatment of either a psychiatric or physical (e.g. infectious disease) illness
• Prior radiation doses equivalent to, or greater than, 8000 cGy to the target lesions at 200 cGy fractions at any timepoint
• Any radiation to the target lesions within 6 months of enrollment
• Unable to meet radiation treatment plan parameters
• Unavailable for follow-up visits after treatment
• Prior malignancy unless disease free for >= 2 years. Curatively treated basal or squamous cell carcinoma of the skin, totally excised melanoma of stage IIA or lower, low- or intermediate-grade localized prostate cancer (Gleason sum =< 7), and curatively-treated carcinoma in situ of the cervix, breast, or bladder are allowed regardless