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About ACC

Epidemiology

Adrenocortical carcinoma (ACC) is a rare malignancy often with poor outcomes. It arises from the cortex of the adrenal gland with an estimated reported incidence in adults of 0.7-2.0 cases per million per year.1

ACC affects more women than men and has a bimodal age distribution with a peak before the age of 5 years and another peak in patients 40-60 years of age.2 Most cases of ACC occur sporadically, with a few being linked to genetic syndromes.2

 

 

INCIDENCE Incidence rate of 0.7 to 2.0 cases per million per year1
AGE Peak incidence between 40-60 years of age, but can occur at any age2
SEX Females are affected 55%-60% more frequently than males3

Clinical Presentation

The presence of ACC is not always obvious.3

There are 3 main scenarios in which patients with ACC may present.4

50%-60%
present with signs and symptoms of hormone excess3
◀

In approximately 50%-60% of patients, symptoms related to excessive hormone secretion are the main reason for seeking medical attention. Biochemical hormone testing reveals that up to 60% of tumors are functioning (vs nonfunctioning).4

30%-40%
present with symptoms from a nonspecific abdominal mass3
◀

The second most common symptoms at time of initial presentation are unspecific abdominal symptoms, such as abdominal pain or fullness.4

10%-15%
are incidentally discovered3
◀

While the likelihood of an adrenal incidentaloma being an ACC is low, an increasing number of cases are diagnosed within the group of incidentally discovered adrenal masses (incidentalomas).3

ACC and Cushing’s syndrome

  • The most common syndrome of hormone excess in patients with functioning ACC is Cushing’s syndrome, which occurs in -45% of patients and may result in central weight gain, plethora, proximal muscle atrophy, diabetes mellitus, and easy bruising5
  • Conversely, it’s estimated that ACC is the cause of Cushing’s syndrome in ~10% of patients6-8

Diagnosing ACC4,5

In addition to evaluating the clinical presentation of the patient and past and family medical history, initial evaluation is centered around detailed hormonal workup to reveal any excessive hormone production by the tumor (ie, is the tumor functioning vs nonfunctioning), which can serve as a tumor target during therapy.

Staging should include imaging of the primary site by computed tomography (CT) and/or magnetic resonance imaging (MRI) of the abdomen. Additional imaging may be performed if metastases are suspected. The final diagnosis is confirmed by a histopathologist.

Clinical assessment
Clinical assessment
Detailed hormonal workup
Detailed hormonal workup
Imaging
Imaging
Histopathology
Histopathology

Lysodren® is indicated for the treatment of patients with inoperable, functional or nonfunctional, adrenocortical carcinoma (ACC).

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Staging Systems for ACC

Most patients with ACC present with advanced disease.5 Some patients with ACC may be diagnosed earlier from the use of improved imaging techniques.4

Several staging systems for ACC are in use, including the American Joint Committee on Cancer (AJCC) and the European Network for the Study of Adrenal Tumors (ENSAT), which is widely used internationally.

Staging Systems for ACC4,9

AJCC*ENSAT*
Stage IT1, N0, M0T1, N0, M0
Stage IIT2, N0, M0T2, N0, M0
Stage IIIT1-T2, N1, M0 T3-T4, Any N, M0T1-T2, N1, M0 T3-T4, N0, M0
Stage IVAny T, Any N, M1Any T, N0-N1, M1

*Tumors are classified as follows: T1: tumor ≤5 cm; T2: tumor >5 cm; T3: infiltration into surrounding tissue; T4: tumor invasion into adjacent organs or venous tumor thrombus in vena cava or renal vein; N0: no positive lymph node; N1: positive lymph node; M0: no distant metastasis; M1: presence of distant metastasis.

General Prognosis

ACC has a high recurrence rate with poor overall 5-year survival.5 The prognosis is, however, heterogeneous.3

Prognosis for ACC when treated10

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

Multidisciplinary Team Management of ACC

The multidisciplinary team managing patients with ACC includes endocrinologists, specialized surgeons, medical and radiation oncologists, specialized pathologists, and genetic counselors. In addition to providing dedicated care to patients with ACC, these expert multidisciplinary teams exchange clinical and scientific information and may support broader patient engagement throughout the treatment journey.1

 

 

Nurses, pharmacists, mental health professionals, and other
healthcare providers are also involved in the management of ACC.

Complete surgical resection of the tumor is the only curative treatment option3 and is the treatment of choice for patients with stage I and II ACC.4 In stages III and IV ACC, in addition to surgical resection of the tumor if possible (stage III) and removal of localized metastases when appropriate (stage IV), Lysodren® with or without chemotherapy may be recommended.3

  1. Kiseljak-Vassiliades K, Bancos I, Hamrahian A, et al. American association of clinical endocrinology disease state clinical review on the evaluation and management of adrenocortical carcinoma in an adult: a practical approach. Endocr Pract. 2020;26(11):1366-1383. doi:10.4158/DSCR-2020-0567
  2. Al-Ward R, Zsembery C, Habra MA. Adjuvant therapy in adrenocortical carcinoma: prognostic factors and treatment options. Endocr Oncol. 2022;2(1):R90-R101. doi:10.1530/EO-22-0050
  3. Fassnacht M, Dekkers OM, Else T, 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. doi:10.1530/EJE-18-0608
  4. Adrenocortical Carcinoma Treatment (PDQ®): Health Professional Version. PDQ Adult Treatment Editorial Board. August 25, 2022. Accessed April 11, 2024. In: PDQ Cancer Information Summaries. https://www.ncbi.nlm.nih.gov/books/NBK65956/
  5. Shariq OA, McKenzie TJ. Adrenocortical carcinoma: current state of the art, ongoing controversies, and future directions in diagnosis and treatment. Ther Adv Chronic Dis. 2021;12:20406223211033103. doi:10.1177/20406223211033103
  6. Lacroix A, Feelders RA, Stratakis CA, Nieman LK. Cushing’s syndrome. Lancet. 2015;386(9996):913-927.
    doi: 10.1016/S0140-6736(14)61375-1
  7. Newell-Price, J. (2010). Etiologies of Cushing’s Syndrome. In: Bronstein, M. (eds) Cushing’s Syndrome. Contemporary Endocrinology. Humana Press, Totowa, NJ. doi:10.1007/978-1-60327-449-4_2
  8. Valassi E, Santos A, Yaneva M, et al. The European Registry on Cushing’s syndrome: 2-year experience. Baseline demographic and clinical characteristics. Eur J Endocrinol. 2011;165(3):383-392. doi:10.1530/EJE-11-0272
  9. Else T, Kim AC, Sabolch A, et al. Adrenocortical carcinoma. Endocr Rev. 2014;35(2):282-326. doi:10.1210/er.2013-1029
  10. Fassnacht M, Johanssen S, Quinkler 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):243-250. doi:10.1002/cncr.24030
INDICATION AND IMPORTANT SAFETY INFORMATION FOR LYSODREN©
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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.
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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Last updated: October 2025; 0097-10/2026@ESTEVE

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