Table 1: Current clinical trials for adrenocortical cancer (from http://www.cancer.gov).

TrialsAgents UsedAdditional Details Organization

Phase I

Study of Sorafenib and Bevacizumab in patients with refractory, metastatic, or unresectable solid tumorsSorafenib plus Bevacizumab
Determine biochemical changes in the Ras-Raf-MAPK, and VEGF signal transduction pathways in tumor and stromal cells from patients treated with this regimen.NCI—Center for Cancer Research  (NCT00098592)

IMC-A12 in combination with Temsirolimus (CCI-779) in patients with advanced cancerscombination of IMC-A12 and TemsirolimusResearchers will also perform biomarker tests to study how IMC-A12 and Temsirolimus affect genesMD Anderson Cancer Center (NCT00678769)

Phase II

Study of combination comprising Tariquidar, Mitotane, Doxorubicin, Vincristine, and Etoposide and surgery in patients with recurrent, metastatic, or primary unresectable adrenocortical cancer Tariquidar, Mitotane, Doxorubicin, Vincristine, and EtoposidePrimary outcome: response rate (partial or complete)progression-free survival
NCI—Center for Cancer Research  (NCT00073996)

Trial with Taxotere and Cisplatin in nonoperable adrenocortical carcinoma
Cisplatin plus TaxotereThe primary objective: to investigate response rate. Secondary endpoints are survival, time to progression, best overall response rate, and duration of response.Rigshospitalet-Copenhagen University Hospital    (NCT00324012)

Randomized study of Mitotane with versus without anti-IGF-1R recombinant monoclonal antibody IMC-A12 in patients with recurrent, metastatic, or primary unresectable adrenocortical carcinomaMitotane with IMC-A12 versus Mitotane without IMC-A12Primary outcome: progression-free survival, objective response rates, changes in tumor size over time, and overall survival
University of Chicago Cancer Research Center    (NCT00778817)

Study of Cixutumumab in patients with relapsed or refractory solid tumorsCixutumumabPrimary outcome: response rate toxicityChildren's Oncology Group    (NCT00831844)

Study of R-(-)-gossypol acetic acid in patients with recurrent, metastatic, or primary unresectable adrenocortical carcinomaR-(-)-gossypol acetic acid.Proportion of patients who achieve a confirmed objective response (complete or partial response by RECIST criteria) to treatmentMayo Clinic Cancer Center    (NCT00848016)

Study of axitinib (AG-013736) with evaluation of the VEGF-pathway in metastatic, recurrent or primary unresectable adrenocortical cancerAxitinib (AG-013736)Determine the response rate of Axitinib (AG-013736) in recurrent, metastatic, or primary unresectable ACC
National Cancer Institute    (NCT01255137)

Sorafenib plus Paclitaxel in adreno-cortical-cancer patientsSorafenib plus weekly Paclitaxel Primary outcome: progression-free survival rate ≥ 40% after 4 months. Response rate evaluation.University of Turin, Italy    (NCT00786110)

Phase III

GALACCTIC: a study of OSI-906 in patients with locally advanced or metastatic adrenocortical carcinomaOSI-906
A multicenter, randomized, double-blind, and placebo-controlled phase 3 study of single-agent OSI-906 in patients with locally advanced/metastatic ACC who received at least one but no more than two prior drug regimenOSI Pharmaceuticals Incorporated    (NCT00924989)

Study of neoadjuvant and adjuvant Cisplatin-based chemotherapy and/or surgical resection in young patients with stage I–IV adrenocortical tumorOral mitotane; cisplatin IV; Etoposide IV and doxorubicin hydrochloride IVChildren's Oncology Group    (NCT00304070)