Understanding Adrenocortical Carcinoma (ACC)

Adrenocortical carcinoma (ACC) is a rare aggressive form of cancer that grows in the outer part of the adrenal glands.1

What is Adrenocortical Carcinoma (ACC)?1

The adrenal glands sit just above the kidneys and produce hormones, including cortisol, aldosterone and male sex hormones. Tumors in these glands are common, but ACC is a rare aggressive form of cancer that grows in the outer part of the glands. The tumors in the adrenal glands cause too much of multiple types of hormone to be produced.

How common is ACC?

ACC is rare – there are only 0.7–2.0 new cases per million people each year.4

ACC can occur at any age, but it is more common in children younger than 10 years of age and in adults between 40 and 50 years. Women are more frequently affected than men.3 Family history of certain inherited diseases and having certain genes can make it more likely you will develop ACC.2

What are the symptoms of ACC?

Some patients with ACC don’t have symptoms, while others do. Symptoms can mimic other conditions, which makes ACC difficult to diagnose. Up to 20% of patients are diagnosed by accident.3

Symptoms include excess hair growth on the face, chest and back. As well as nausea/vomiting, muscle weakness, back and abdominal pain, passing water more often, weight gain/weight loss, high blood pressure, and high sugar levels.1

How is ACC diagnosed?1

ACC can be difficult to diagnose, because the symptoms are also seen in more common diseases.

The tests used to diagnose ACC depend on the patient’s symptoms, but usually begin with a simple check of the outward signs of the disease. These can include excess hair growth and high blood pressure. More extensive tests, such as measuring the hormone levels in the blood, imaging (including CT and MRI) and a microscopic examination of gland tissue are also carried out. These tests allow the doctor to see how far the tumor has developed and how best to treat the disease.

What treatment options are there for ACC?1

Surgery to remove the affected adrenal gland is usually the first choice of treatment. Medicines, or radiation therapy aim to reduce the risk of recurrence.

If you are a patient, please contact your physician for more information.

REFERENCES:

  1. https://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq/
  2. Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoili EM, Jolly S, Miller BS, Giordano TJ, Hammer GD. Adrenocortical carcinoma. Endocr Rev. 2014 Apr;35(2):282-326
  3. Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment. Front Cell Dev Biol. 2015 Jul 3:45
  4. Fassnacht M, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018;179(4):G1-G46

Additional Resources for adrenocortical carcinoma:

Prognosis for ACC when treated:

The 5-year survival rate for ACC depends greatly on when the tumor is found.

5-year stage-dependent survival %
  • Stage 1
  • Stage 2
  • Stage 3
  • Stage 4

Fassnacht M, et al. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer. 2009;115(2):243-50.

INDICATION AND IMPORTANT SAFETY INFORMATION INCLUDING BOXED WARNING ON ADRENAL CRISIS IN THE SETTING OF SHOCK, SEVERE TRAUMA OR INFECTION

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LYSODREN® is an adrenal cytotoxic agent indicated for the treatment of patients with inoperable, functional or nonfunctional, adrenocortical carcinoma (ACC).

Warning/Adrenal crisis in the setting of shock, severe trauma or infection: Patients treated with LYSODREN are at increased risk for developing adrenal crisis in the setting of shock, severe trauma or infection that may lead to death.
If shock, severe trauma or infection occurs or develops, temporarily discontinue LYSODREN and administer exogenous steroids. Monitor patients closely for infections and instruct patients to contact their physician immediately if injury, infection, or any other concomitant illness occurs.

Adrenal Insufficiency and Adrenal Crisis: Temporarily withhold LYSODREN during shock; trauma, infection or adrenal insufficiency. Steroid replacement may be necessary.

Central Nervous System (CNS) Toxicity: Monitor behavioral and neurologic assessments and mitotane plasma levels at regular intervals. Mitotane plasma levels exceeding 20 mg/L are associated with a greater incidence of toxicity. Advise patients not to drive or operate hazardous machinery if experiencing CNS adverse reactions.

Ovarian Macrocysts in Premenopausal Women: Monitor pelvic ultrasound at baseline and at regular intervals. Withhold, reduce the dose, or permanently discontinue LYSODREN based on severity.

Hematologic Toxicity: Monitor complete blood counts prior to starting LYSODREN, during dose titration and as clinically indicated. Withhold, reduce the dose or permanently discontinue based on severity.

Prolonged Bleeding Time: Prolonged bleeding time has occurred in patients treated with mitotane and this should be taken into account when surgery is considered.

Embryo-Fetal Toxicity: LYSODREN can cause fetal harm. Advise females of reproductive potential of the potential risk to a fetus and to use effective nonhormonal contraception.

Lactation: Advise not to breastfeed.

Hepatic Impairment: LYSODREN is not recommended for patients with severe hepatic impairment. In patients with mild to moderate hepatic impairment, monitor mitotane plasma levels frequently and modify the dosage as needed.

Renal Impairment: LYSODREN® is not recommended for patients with severe renal impairment. In patients with mild or moderate renal impairment, monitor mitotane plasma levels frequently and modify the dosage as needed.

Most common adverse reactions include: anorexia, epigastric discomfort, nausea, vomiting diarrhea, depression, dizziness, vertigo, rash, hypercholesterolemia, hypertriglyceridemia, hypothyroidism and decreased blood free testosterone in males. LYSODREN is a hazardous drug. Advise caregivers to wear disposable gloves when handling LYSODREN tablets.

Avoid concomitant use with spironolactone, certain CYP3A substrates, hormonal contraceptives and warfarin.

Please see Full Prescribing Information, including BOXED WARNING, for LYSODREN® (mitotane) tablets.

To report SUSPECTED ADVERSE REACTIONS, contact Direct Success Pharmacy, Inc. at 1-844-LYSODREN (1-844-597-6373) or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Link to US Prescribing Information including Boxed Warning.

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