Adrenal Cortical Cancer

The goal of this clinical trial is to learn if Ivonescimab works to treat advanced rare tumors including cohort 1: PAGET's disease of scrotum with infiltrating sweat gland carcinoma. cohort 2: Metastatic paraganglioma and pheochromocytoma. cohort 3: Metastatic renal angiomyolipoma and malignant perivascular epithelioid cell tumor. cohort 4: Rhabdomyosarcoma and Ewing's sarcoma cohort 5: Collecting duct carcinoma cohort 6: Urachal carcinoma. cohort 7: Neuroendocrine cancer. cohort 8: Basal cell carcinoma and sarcomatoid carcinoma. cohort 9: Penile cancer. cohort 10: Adrenal cortical cancer. cohort 11: Metastatic germ cell tumors, failure of standard cisplatin based therapy (mostly testicular cancer). cohort 12: Non-clear cell renal carcinoma (including renal papillary renal carcinoma); Renal cancer cannot be classified). cohort 13: Non-clear cell renal carcinoma (including chromophobe renal carcinoma) cohort 14: Other rare tumors that cannot be classified (such as testicular reticulum adenocarcinoma, etc.). cohort 15: Prostate cancer. cohort 16: Clear cell renal carcinoma. (16.1: received PD-1; 16.2: no PD-1 received) cohort 17: Urothelial carcinoma. cohort 18: Kidney cancer with brain metastases. cohort 19: Brain metastases of urothelial carcinoma. cohort 20: Rare tumors with brain metastases. It will also learn about the safety of Ivonescimab. The main questions it aims to answer are: Does Ivonescimab improve the objective response rate and prolong the survival of participants? What medical problems do participants have when taking Ivonescimab? Participants will: Receive Ivonescimab 20mg/kg intravenously every 21 days until disease progression, intolerable toxicity, or full 2 years of treatment, whichever occurs first. Be performed imaging evaluation according to RECIST 1.1 every 9 weeks for 1 year of treatment and every 12 weeks after 1 year Be recorded any adverse events in the whole study period including type, incidence, grade, severity, duration, and association with the study drug according to NCI-CTCAE V5.0 criteria

Adrenocortical carcinoma (ACC) is a rare aggressive malignant tumor. According to the literature, the 5-year survival rate of ACC is 12%-47%. For patients with advanced ACC, mitotane alone or combined with traditional chemotherapy was the first-line standard treatment, but its progression-free survival was only about 1 year. The efficacy of mitotane monotherapy is approximately 10% to 30%. FIRM-ACT trial reported an objective response rate (ORR) of 23.2% for etoposide, doxorubicin, cisplatin, and mitotane (EDP-M) chemotherapy regimen. Our phase II study found that PD-1 inhibitor camrelizumab and apatinib showed impressive clinical data in the second-line treatment of relapsed and metastatic ACC patients. The aim of this study is to evaluate the efficacy and safety of PD-1 inhibitor camrelizumab combined with apatinib and mitotane in advanced ACC, and to explore a new treatment strategy for patients with advanced ACC.