Velvet Lamb was diagnosed in 2015 with stage 4 adrenal cortical carcinoma. A one in a million diagnosis with a poor prognosis, listen to Velvet's story of hope.
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Matthew Campbell, MD, The University of Texas MD Anderson Cancer Center, Houston, TX, talks on an Phase II study (NCT03370718) exploring the role of carbozantinib, a tyrosine kinase inhibitor, in adrenocortical carcinoma (ACC) management. 18 patients were enrolled with advanced ACC, with the primary endpoint being progression-free survival (PFS) at 4 months (PFS4). The median follow-up was 19.4 months and the PFS4 was 72%, median PFS was 7.2 months. 16 patients had restaging with ORR including 2 partial response, 12 stable disease, and 2 progressive disease as best response; disease control rate was 78%. The median PFS was 7.2 months and median OS was 23.9 months. Overall, cabozantinib was shown to be able to provide sustained disease control in patients with advanced ACC and a manageable safety profile in the majority of patients. This interview took place at the European Society for Medical Oncology (ESMO) 2022 Congress in Paris, France.

Outcomes for pediatric patients with unresectable, metastatic, or relapsed adrenocortical carcinoma (ACC) are dismal, and CAR-T cell therapy is a promising approach to improve outcomes for patients with this disease. Rebecca Epperly, MD, St. Jude Children's Research Hospital, Memphis, TN, provides an overview of an evaluation into B7-H3 expression in primary pediatric ACC samples and B7-H3-CAR T activity in pediatric patient-derived xenograft (PDX) models. B7-H3 expression in 44 pediatric adrenocortical tumors (ACTs) was assessed, demonstrating a range of B7-H3 positivity, including 55% with expression above the threshold for eligibility for an active B7-H3-CAR T clinical trial (NCT04897321). Strikingly, B7-H3-CAR T eradicated 100% of tumors in mice and mice survived long term compared to controls. In conclusion, B7-H3 is expressed in a majority of pediatric ACTs and B7-H3-CAR T have potent anti-ACC activity. This interview took place at the American Association for Cancer Research (AACR) Annual Meeting 2023 in Orlando, FL.

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Alfredo Berruti, MD, University of Brescia, Brescia, Italy, talks on the rationale behind the Adjuvo trial investigating adjuvant therapy with mitotane in patients with adrenocortical carcinoma who are at low-intermediate risk of relapse following surgery. According to retrospective data, approximately 40-70% of patients with adrenocortical carcinoma will inevitably relapse following radical surgery. This produces a need for adjuvant therapy to reduce risk of relapse. The current guidelines follow that only those patients who have high-risk disease undergo adjuvant therapy, with uncertainties surrounding the use of adjuvant therapy in patients who are low-intermediate risk, which provided the rationale for the Adjuvo trial. This interview took place at the American Society of Clinical Oncology (ASCO) Genitourinary Cancers Symposium 2022 in San Francisco, CA.

Alfredo Berruti, MD, University of Brescia, Brescia, Italy, provides an overview of the Adjuvo study comparing the efficacy of adjuvant mitotane treatment versus observation on prolonging recurrence-free survival (RFS) in patients with adrenocortical carcinoma at low-intermediate risk of recurrence. Results do not support the routine use of adjuvant mitotane in this subset of patients, who may thus avoid a potentially toxic treatment. This interview took place at the American Society of Clinical Oncology (ASCO) Genitourinary Cancers Symposium 2022 in San Francisco, CA.