VERY-RARE CANCERAdrenal CancerWHO 5th Edition Classification

Adrenocortical Carcinoma (ACC)

Very Rare Malignancy (1–5 cases per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A rare cancer of the adrenal cortex. Around 60% are functional, secreting cortisol, androgens or both, so patients may present with hormonal syndromes before any mass effect. It has a bimodal age distribution with a small childhood peak, and childhood cases carry a high rate of germline TP53 alteration.

Request Rare Cancer Tumor Board Find NCI Treatment CentersAsk Vani AI Navigator

Emergent Referral AdvisoryImmediate Action

Certain rare malignancies progress rapidly or carry acute risk of airway obstruction, acute spinal compression, hydrocephalus, or biopsy-seeding. Do not perform needle biopsy or surgery outside of an NCI-designated specialty sarcoma or neuro-oncology unit without multidisciplinary tumor board review.

Report immediately to an emergency department or tertiary oncologist upon:

  • Paediatric ACC - test germline TP53 and refer for genetic counselling
Who It Affects

Bimodal - children under 5 and adults 40-50; female predominance in adults

Annual Incidence

Approximately 0.7-2 per million per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

Rapid weight gain, round face, purple stretch marks and muscle weakness (cortisol excess)
New facial hair, acne, deepening voice or menstrual disturbance in women (androgen excess)
Flank or abdominal pain and a palpable mass
New or worsening high blood pressure and low potassium
Precocious puberty in children

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

TP53 (including germline - Li-Fraumeni)CTNNB1ZNRF3IGF2 overexpressionKi-67 index

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

Complete open surgical resection by a high-volume adrenal surgeon

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Adjuvant mitotane for high-risk resected disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

EDP-M chemotherapy (etoposide, doxorubicin, cisplatin plus mitotane) for advanced disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

Radiotherapy to the tumour bed in selected high-risk cases

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Blockade of steroid excess - metyrapone, ketoconazole - as supportive care

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

Stage-dependent and generally guarded. Five-year survival is roughly 60-80% for completely resected localised disease but under 15% for metastatic disease. Ki-67 index and resection margin status are the strongest pathological predictors.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

Immunotherapy has shown modest single-agent activity; IGF1R inhibition was unsuccessful in phase III. Current focus is on combination immunotherapy, and on trials stratified by the recognised molecular subgroups from comprehensive genomic profiling.

Need Help Matching to an Active Rare Cancer Trial?

ByOnco scans ClinicalTrials.gov, NCI trial networks, and institutional registries for open patient cohorts.

Request Trial Match
Expert Clinicians

Verified Specialists for Adrenocortical Carcinoma

Browse All Oncologists

Dr. Gary D. Hammer

Endocrine Oncologist

25+ yrs exp

University of Michigan

Ann Arbor, USA

Endocrine OncologyACCClinical Trials
Vetted DirectoryConsult Specialist

Dr. Khoo Kei Siong

Medical Oncologist

30+ yrs exp

Parkway Cancer Centre

Singapore, Singapore

Medical OncologyACCMitotane Therapy
Vetted DirectoryConsult Specialist

Prof. Martin Fassnacht

Endocrinologist

20+ yrs exp

University of Würzburg

Würzburg, Germany

EndocrinologyACC ResearchEuropean Guidelines
Vetted DirectoryConsult Specialist

Dr. Rajeev Agarwal

Surgical Oncologist

30+ yrs exp

Private Practice

New Delhi, India

Adrenal Tumor SurgeryACCMultidisciplinary Management
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

Related Rare Malignancies in Catalog

very-rare

Angioimmunoblastic T-cell Lymphoma (AITL)

very-rare

Appendiceal Carcinoma

very-rare

Esthesioneuroblastoma

very-rare

Adenoid Cystic Carcinoma (ACC)

Independent Tumor Board Review

Facing a Diagnosis of Adrenocortical Carcinoma?

Rare cancers demand rare expertise. Connect your medical records to US NCI-fellowship trained oncologists, subspecialist pathologists, and active clinical trial investigators within 12 to 72 hours.

Upload Scans for Second Opinion Schedule Virtual Teleconsult