Related Experiment Video
Updated: Mar 22, 2026
Generation of CAR T Cells for Adoptive Therapy in the Context of Glioblastoma Standard of Care
Published on: February 16, 2015
The challenge of developmental therapeutics for adrenocortical carcinoma
Ricardo Costa1,2, Benedito A Carneiro1,2, Fabio Tavora3
1Northwestern Medicine Developmental Therapeutics Institute, Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, IL, USA.
Abstract:
Adrenocortical carcinoma (ACC) is a rare disease with an estimated incidence of only 0.7 new cases per million per year. Approximately 30-70% of the patients present with advanced disease with very poor prognosis and without effective therapeutic options. In the recent years, unprecedented progresses in cancer biology and genomics have fostered the development of numerous targeted therapies for various malignancies. Immunotherapy has also transformed the treatment landscape of malignancies such as melanoma, among others. However, these advances have not brought meaningful benefits for patients with ACC. Extensive genomic analyses of ACC have revealed numerous signal transduction pathway aberrations (e.g., insulin growth factor receptor and Wnt/β-catenin pathways) that play a central role in pathophysiology. These molecular alterations have been explored as potential therapeutic targets for drug development. This manuscript summarizes recent discoveries in ACC biology, reviews the results of early clinical studies with targeted therapies, and provides the rationale for emerging treatment strategies such as immunotherapy.
Insights
Adrenocortical carcinoma (ACC) remains a challenge with limited treatments. Recent genomic insights reveal molecular targets and immunotherapy potential for this rare cancer.
Area of Science:
- Oncology
- Genomics
- Cancer Biology
Background:
- Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a high incidence of advanced disease and poor prognosis.
- Current therapeutic options for advanced ACC are limited, highlighting an unmet clinical need.
- Recent advances in cancer genomics and targeted therapies have shown success in other malignancies, but not yet for ACC.
Purpose of the Study:
- To summarize recent discoveries in adrenocortical carcinoma biology.
- To review early clinical studies of targeted therapies for ACC.
- To provide a rationale for emerging treatment strategies, including immunotherapy.
Main Methods:
- Review of recent scientific literature on adrenocortical carcinoma.
- Analysis of genomic data revealing key signaling pathway aberrations.
- Evaluation of early-phase clinical trial results for targeted agents and immunotherapies.
Main Results:
- Genomic analyses identified critical aberrant signaling pathways in ACC, including IGF receptor and Wnt/β-catenin.
- Targeted therapies exploiting these molecular alterations are under investigation.
- Immunotherapy has shown promise in other cancers but has yet to demonstrate significant benefit in ACC.
Conclusions:
- Understanding ACC molecular pathophysiology is crucial for developing effective treatments.
- Targeted therapies and immunotherapy represent promising avenues for future ACC treatment strategies.
- Further clinical investigation is warranted to translate these findings into improved patient outcomes.
Related Concept Videos
02:57Targeted Cancer Therapies
There are several types of targeted therapies against...
02:57Targeted Cancer Therapies
01:27Adrenal Gland Disorders
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
02:56Treatment Resistant Cancers
01:27Tumor Immunotherapy
02:56Treatment Resistent Cancers