Where adrenocortical carcinoma treatment is heading

Adrenocortical carcinoma is very rare, a few hundred US cases a year, and often makes excess hormones. Complete surgery is the best chance of cure, usually followed by mitotane. For advanced disease, chemotherapy helps few people, so immunotherapy and targeted pills are being tested in small trials.

• 31studies open or running
• 1phase 3 trials
• 270papers published this year
• 19NIH-funded projects

1. Now

   Nov 2026

   Immunotherapy plus a targeted pillHappened 52% tumors shrank

   In a Chinese trial, camrelizumab with apatinib shrank about half of tumors that had grown on earlier treatment.

   52%: had their tumors shrink.

   • Camrelizumab-apatinib trial

   Two immunotherapies help some for yearsHappened 33% shrank or stable 6+ months

   Ipilimumab plus nivolumab helped about 1 in 3, a few for more than 4 years.

   33%: had their cancer shrink or stay stable for more than 6 months.

   • DART trial (SWOG S1609)

   Treatment after surgeryHappened

   Mitotane and radiation after surgery were each linked to better outcomes in pooled studies.

   • Mitotane and radiation review

2. 6 months

   by May 2027

   Radiation plus pembrolizumabPossible

   A small Memorial Sloan Kettering trial combines targeted radiation with pembrolizumab. Due September 2026.

   • MSK trial

   Surgery with heated chemotherapyLong shot

   A Columbia trial tests heated chemotherapy in the abdomen during surgery. Due December 2026.

   • Columbia trial

3. 12 months

   by Nov 2027

   Pembrolizumab with lenvatinibPossible 52% tumors shrank, similar pairing

   A Korean trial of 30 people is due February 2027.

   52%: had tumors shrink with a similar immunotherapy-plus-pill pairing.

   • National Cancer Center Korea trial
   • Camrelizumab-apatinib trial

   Progesterone added to chemotherapyLong shot

   An Italian trial of 80 people tests progesterone with standard chemotherapy. Due June 2027.

   • Italian trial

4. 3 years

   by late 2029

   Cabozantinib plus nivolumab nationwidePossible 6 months before growth, alone

   A US trial adds cabozantinib to nivolumab at 81 sites. It finishes in 2029.

   6: months median before the cancer grew with cabozantinib alone.

   • NCI trial
   • Cabozantinib trial

5. 5 years

   by late 2031

   Chemotherapy after surgery for high-risk cancerPossible

   A 240-person randomized trial tests adding cisplatin and etoposide to mitotane after surgery. It finishes in 2029.

   • ADIUVO-2 trial

   A first approved immunotherapy combinationLong shot

   If combinations hold up in larger trials, one could become a standard after chemotherapy.

   • Camrelizumab-apatinib trial
   • NCI trial

The research paths

Borrowed from other cancers

Open to join now

• After surgery, high-risk

  Mitotane with or without chemotherapy

  Phase 3 · 33 sites

  Locations and eligibility →

• Advanced adrenal cancer, US

  Cabozantinib plus nivolumab

  Phase 2 · 81 sites

  Locations and eligibility →

• Mismatch-repair deficient cancers, France

  Dostarlimab

  Phase 2 · 22 sites

  Locations and eligibility →

• Advanced adrenal cancer, Italy

  Cemiplimab maintenance

  Phase 2 · 1 site

  Locations and eligibility →

See every recruiting adrenal cancer trial →

When the trials finish

Completion dates the trials registered. Results usually follow 3 to 12 months later.

Knowledge on the way

Not treatments yet: detection, causes and how care is delivered. Mostly NIH-funded.

• AreaQuestionFunded throughNews likely
• Care A national network for rare tumors in children and adults (MyPART)National Cancer Institute Funded through Ongoing News likely Continuous

What just happened

Standard treatment today

• LocalizedOpen surgery by an experienced adrenal surgeon; mitotane afterwards for most; radiation for some.
• AdvancedEtoposide, doxorubicin and cisplatin with mitotane (EDP-M).
• Hormone excessMedicines to control cortisol or other hormones, alongside cancer treatment.

See adrenal cancer trials recruiting now →