Agnostic Therapy in Rare Solid Tumors

Inclusion Criteria

• •1. Age 18 years or older.
• •2. Patients with immunohistochemistry for PD-L1 with a combined positive score (CPS) of 10 or higher.
• •3. Patients with progression or intolerance to already approved and accessible treatments for the specific neoplasm and population.
• •4. Documented disease progression radiologically after the last routine treatment.
• •5. Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1.
• •6. Measurable lesion per RECIST v1.1. Lesions previously treated with radiotherapy can only be used as target lesions if they are confirmed to be progressing by imaging before enrollment.
• •7. Male participants must meet at least one of the following conditions:
• •1. Considered infertile;
• •2. No fertile partner;
• •3. Has a fertile partner who agrees to follow contraceptive guidance throughout the study period and for at least 6 months after the last dose of Nivolumab;
• •and
• •4. Agrees to abstain from sperm donation throughout the study period and for at least 6 months after the last dose of Nivolumab.
• •8. Female participants must meet at least one of the following conditions:
• •1. Considered infertile;
• •2. Agrees to follow contraceptive guidance throughout the study period and for at least 6 months after the last dose of Nivolumab;
• •9. Estimated life expectancy greater than 12 weeks, as determined by the investigator or delegated sub-investigator.
• •10. Preserved organ functions defined by:
• •* Absolute neutrophil count ≥ 1,000;
• •* Hemoglobin ≥ 8.0 g/dL (patients may receive transfusions to reach this level);
• •* Platelet count ≥ 100,000;
• •* Total bilirubin ≤ 1.5 × Upper Limit of Normal (ULN), or ≤ 3.0 × ULN for patients with Gilbert's syndrome;
• •* Aspartate Aminotransferase (AST) and Alanine Aminotransferase (ALT) ≤ 2.5 × ULN (≤ 5 × ULN in the presence of liver metastases);
• •* Creatinine clearance \> 30 mL/min (estimated by Cockcroft-Gault).
• •11. Diagnosis of rare cancer (List I) confirmed by histopathological examination, with the possibility of including other types of rare tumors (incidence of less than 6 in every 100,000) after careful evaluation and approval by the study board.
• •* List I:
• •* Urachal adenocarcinoma
• •* Parathyroid carcinoma
• •* Nasopharyngeal epithelial tumors
• •* Fibrolamellar carcinoma of any primary site
• •* Angiosarcoma of any primary site
• •* Secretory breast carcinoma
• •* Anal cancer
• •* Metaplastic breast carcinoma
• •* Chromophobe renal carcinoma, Microphthalmia-associated Transcription Factor (MiT) family translocation renal carcinoma; renal carcinoma with Fumarate Hydratase (FH) or Succinate Dehydrogenase (SDH) deficiency
• •* Carcinosarcoma of any primary site
• •* Small intestine cancer
• •* Cholangiocarcinoma
• •* Sertoli-Leydig cell tumors
• •* Cervical cancer of non-epidermoid histology
• •* Tracheal epithelial tumors
• •* Non-cystadenoma salivary gland tumors
• •* Mesothelioma of any site
• •* Neuroblastoma
• •* Adrenal cancer
• •* Penile cancer
• •* Apocrine carcinoma
• •* Fibrosarcoma of any primary site
• •* Cancer of unknown primary site
• •* Hemangioblastoma of any primary site
• •* Thyroid cancer
• •* Hepatoblastoma
• •* Fallopian tube cancer
• •* Leiomyosarcoma of any primary site
• •* Vaginal cancer
• •* Neurofibrosarcoma of any primary site
• •* Gallbladder cancer
• •* Osteosarcoma of any primary site
• •* Bile duct cancer
• •* Clear cell endometrial carcinoma
• •* Yolk sac tumor of any primary site
• •* Non-epidermoid bladder cancer
• •* Vulvar cancer
• •* Kaposi's sarcoma
• •* Epithelial ovarian cancer
• •* Soft tissue sarcoma
• •* Urethral cancer
• •* Granulosa cell tumor of any primary site
• •* Cystadenoma carcinoma
• •* Primitive neuroectodermal tumor of any primary site
• •* Pure or mixed neuroendocrine tumors with neuroendocrine component
• •* Trophoblastic tumor
• •13. Presence of meningeal carcinomatosis.
• •14. Worsening renal and liver function in the 14 days prior to enrollment.
• •15. History of solid organ transplantation with or without immunosuppression.
• •16. Patients with untreated acquired immunodeficiency. Immunocompromised patients may be included as long as they do not have active opportunistic disease and/or active infection, after thorough clinical evaluation by the investigator or sub-investigator. HIV-positive patients must have documented undetectable viral load prior to inclusion.
• •17. Chronic use of corticosteroids at doses greater than 10 mg/day of prednisone or equivalent. Patients with adrenal insufficiency of non-autoimmune etiology (e.g., previous bilateral adrenalectomy) may be included if they are clinically compensated with 10 mg/day of prednisone or equivalent or less.