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adrenal cortex carcinoma - Clinical Profile, Treatments & Trials | Kisho | Kisho

Features include always present findings: Adrenocortical carcinoma; and common findings: Abnormality of reproductive system physiology, Irritability, Anxiety, and Diabetes mellitus and others. 29 total HPO annotations.

Organ System	Phenotype Count	Example Features
Hormones	4	Diabetes mellitus, Increased circulating cortisol level, Paradoxical increased cortisol secretion on dexamethasone suppression test
Lab test results	4	Increased circulating aldosterone concentration, Increased circulating androstenedione concentration, Elevated serum 11-deoxycortisol
Brain and nerves	2	Irritability, Anxiety
Heart and blood vessels	1	Hypertension
Skin	1	Excessive sweating (hyperhidrosis)
Muscles	1	Muscle weakness
Growth and development	1	Weight loss
Metabolism	1	Abnormality of metabolism/homeostasis
Digestive system	1	Abdominal pain
Kidneys and urinary system	1	Increased urinary cortisol level
Lungs and breathing	1	Lung adenocarcinoma

Age of onset: infancy.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

1 FDA-approved treatment is available for adrenal cortex carcinoma, including MITOTANE (LYSODREN, approved 1970). An additional 4 compounds hold orphan drug designation.

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved	Market Status
LYSODREN	MITOTANE	—	1970	Available

Orphan Drug Designations

The following drugs have received orphan drug designation from the FDA for adrenal cortex carcinoma. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
Acetyl-D-tyrosylglycyl-D-arginyl-D-lysyl-D-lysyl-D-arginyl-D-arginyl-D-glutaminyl-D-arginyl-D arginyl-D-arginylglycyl-D-lysyl-D-threonyl-D-leucyl-D-arginyl-D-valyl-D-alanyl-D-lysyl-D-alanyl-D-isoleucyl-D-tyrosyl-D-lysyl-D-arginyl-D-tyrosyl-D-isoleucyl-D-isoglutamine, acetate salt	Acetyl-D-tyrosylglycyl-D-arginyl-D-lysyl-D-lysyl-D-arginyl-D-arginyl-D-glutaminyl-D-arginyl-D arginyl-D-arginylglycyl-D-lysyl-D-threonyl-D-leucyl-D-arginyl-D-valyl-D-alanyl-D-lysyl-D-alanyl-D-isoleucyl-D-tyrosyl-D-lysyl-D-arginyl-D-tyrosyl-D-isoleucyl-D-isoglutamine, acetate salt	Cytovation AS	2024	—	Designated
linsitinib	linsitinib	Astellas Pharma Global Development, Inc.

Gene therapy approaches for adrenal cortex carcinoma have been reported in the published literature.

Clinical Trials

35 trials found

Explore Trials

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

35 clinical trials registered, 9 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 1 PHASE3, 18 PHASE2, 5 PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT06831175](https://clinicaltrials.gov/study/NCT06831175)	Phase II Study of PD-1 Inhibitor Combined With Apatinib and Mitotane in the Treatment of Advanced Adrenal Cortical Carcinoma	PHASE2	West China Hospital	RECRUITING
[NCT04897321](https://clinicaltrials.gov/study/NCT04897321)	B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)	PHASE1	St. Jude Children's Research Hospital	RECRUITING
[NCT06006013](https://clinicaltrials.gov/study/NCT06006013)	Cabozantinib in Combination With Pembrolizumab for the Treatment of Patients With Locally Advanced, Metastatic, or Unresectable Adrenal Cortical Cancer	PHASE2	Emory University	RECRUITING
[NCT05913427](https://clinicaltrials.gov/study/NCT05913427)	Evaluation of the Efficacy of Addition of Progesterone to Standard Chemotherapy in Adrenocortical Carcinoma (ACC)	PHASE2	Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia	RECRUITING
[NCT00669266](https://clinicaltrials.gov/study/NCT00669266)	Adrenal Tumors - Pathogenesis and Therapy	—	University of Wuerzburg	RECRUITING

16 publications have been identified in PubMed for adrenal cortex carcinoma. Research spans Case Report / Case Series (38%), Review / Meta-Analysis (19%), and Basic Science / Preclinical (19%).

Research Type	Count	% of Total
Patient case studies	6	38%
Research summaries	3	19%
Laboratory research	3	19%
Testing and diagnosis research	1	6%
Clinical study results	1	6%
Disease patterns and progression	1

Recent Publications

Li S (2026). [PMID: 41800051](https://pubmed.ncbi.nlm.nih.gov/41800051/). *Frontiers in oncology*. [Case Report / Case Series]
Shatadal A (2026). [PMID: 42068377](https://pubmed.ncbi.nlm.nih.gov/42068377/). *Pediatr Radiol*. [Review / Meta-Analysis]
Meet S (2026). [PMID: 41735791](https://pubmed.ncbi.nlm.nih.gov/41735791/). *Diagnostic cytopathology*. [Case Report / Case Series]
Erickson LA (2026). [PMID: 41384706](https://pubmed.ncbi.nlm.nih.gov/41384706/). *Histopathology*. [Review / Meta-Analysis]
Yamashita SN (2026). [PMID: 41617449](https://pubmed.ncbi.nlm.nih.gov/41617449/). *Anticancer research*. [Clinical Trial Publication]
Feng R (2025). [PMID: 40638989](https://pubmed.ncbi.nlm.nih.gov/40638989/). *Annals of diagnostic pathology*. [Review / Meta-Analysis]
Mao Q (2025). [PMID: 41339227](https://pubmed.ncbi.nlm.nih.gov/41339227/). *Archivos espanoles de urologia*. [Basic Science / Preclinical]
Falcon-de Legal E (2025). [PMID: 40558276](https://pubmed.ncbi.nlm.nih.gov/40558276/). *Current oncology (Toronto, Ont.)*. [Epidemiology / Natural History]
Cantini G (2025). [PMID: 41362288](https://pubmed.ncbi.nlm.nih.gov/41362288/). *The journal of liquid biopsy*. [Basic Science / Preclinical]
Cao L (2025). [PMID: 41255605](https://pubmed.ncbi.nlm.nih.gov/41255605/). *Frontiers in medicine*. [Diagnostic / Biomarker]

Kisho

adrenal cortex carcinoma

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Treatment

Approved Therapies

Orphan Drug Designations

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Latest News & Developments

Current clinical management and outcome of patients with adrenal cortical carcinoma (ACC) with rare histological subtypes-an ENSAT cohort study.

adrenal cortex carcinoma

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Treatment

Approved Therapies

Orphan Drug Designations

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Latest News & Developments

Current clinical management and outcome of patients with adrenal cortical carcinoma (ACC) with rare histological subtypes-an ENSAT cohort study.