The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Adrenocortical Carcinoma: Mitotane and Multimodality Treatment

Comprehensive multimodality management of adrenocortical carcinoma combining surgery, mitotane adjuvant therapy and chemotherapy for advanced disease

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Adrenocortical Carcinoma: Mitotane and Multimodality Treatment?

Adrenocortical carcinoma is rare aggressive malignancy of adrenal cortex with poor prognosis.

Mitotane is unique adrenocorticolytic agent with selective toxicity to adrenocortical cells.

Many ACCs are functional producing Cushing syndrome, virilization or feminization.

Five-year survival is 10-30% reflecting aggressive disease behavior.

Multimodality treatment combining surgery, mitotane and chemotherapy provides best outcomes.

Symptoms

Cushing syndrome with weight gain, hypertension, hyperglycemia, hypokalemia from cortisol excess.
Virilization with hirsutism, voice deepening, menstrual irregularities from androgen excess.
Feminization with gynecomastia from estrogen excess in male patients.
Abdominal pain, mass, weight loss with non-functional or advanced tumors.
Adrenal insufficiency from mitotane therapy requires hormone replacement and education.

Risk Factors

Bimodal age distribution with peaks in childhood and middle age.
Li-Fraumeni syndrome with TP53 germline mutations strongly predisposes to ACC.
Beckwith-Wiedemann syndrome predisposes to pediatric ACC and other cancers.
Carney complex and multiple endocrine neoplasia type 1 are rare predisposing syndromes.
Sporadic ACC is most common form with unknown etiology in most cases.

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Adrenal mass with hormonal excess or imaging features suspicious for malignancy warrants urgent evaluation.
  • Specialized endocrinology and oncology center referral with experienced multidisciplinary teams optimizes outcomes.
  • Severe Cushing syndrome, severe virilization, severe hypertension require management.
  • Mitotane-related adrenal insufficiency requires hormone replacement and patient education.
  • Disease progression on mitotane warrants chemotherapy with EDP regimen or clinical trials.

Treatment Methods

01
Complete surgical resection (R0) by experienced adrenal surgeons represents primary treatment.
02
Adjuvant mitotane for high-risk disease with target plasma levels 14-20 mg/L.
03
EDP-mitotane (etoposide, doxorubicin, cisplatin with mitotane) for advanced disease.
04
Hormonal control of Cushing syndrome with adrenal blockers including ketoconazole, metyrapone.
05
Comprehensive multidisciplinary care with endocrinology, surgical oncology, medical oncology, radiation oncology, hormonal evaluation and management, mitotane administration with careful titration to therapeutic levels and management of toxicities including gastrointestinal, neurological, hepatic, hormonal supplementation including hydrocortisone, fludrocortisone and other replacement hormones, surveillance imaging post-treatment, salvage therapy for recurrence, palliative interventions for hormonal manifestations, clinical trial enrollment for advanced disease, genetic counseling for hereditary syndromes, and survivorship care addressing multimodality treatment effects provides optimal outcomes for patients with adrenocortical carcinoma.

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Onkoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Anaemia

Dahiliye (İç Hastalıkları)

Anaemia is a low haemoglobin level that reduces oxygen delivery, causing fatigue, pallor, and shortness of breath. It is not a disease itself but a sign of many underlying conditions. Most cases are correctable with appropriate diagnosis and treatment.

Iron Deficiency Anaemia

Dahiliye (İç Hastalıkları)

Iron deficiency anaemia develops when dietary intake, absorption, or losses create an iron shortfall, most often affecting women and children. Identifying the underlying cause is the core of management, alongside iron replacement.

Vitamin B12 Deficiency

Dahiliye (İç Hastalıkları)

Vitamin B12 deficiency can cause megaloblastic anaemia, neurological symptoms, and cognitive impairment. Early treatment with intramuscular or oral B12 largely prevents irreversible complications.

Hypertension (High Blood Pressure) Management

Dahiliye (İç Hastalıkları)

Hypertension is often called the silent killer because it progresses symptom-free for years and can damage the heart, brain, kidneys, and eyes. Regular monitoring, lifestyle change, and evidence-based drug therapy dramatically reduce cardiovascular risk.

Chronic Kidney Disease

Dahiliye (İç Hastalıkları)

Chronic kidney disease is one of the most common complications of chronic conditions such as diabetes and hypertension, and can be silent in its early stages.

Hepatitis B (HBV)

Dahiliye (İç Hastalıkları)

Hepatitis B is a DNA virus infection causing acute and chronic hepatitis with risk of cirrhosis and hepatocellular carcinoma; diagnosis integrates HBsAg, HBeAg, anti-HBc, and HBV DNA with management based on disease phase using nucleos(t)ide analogues (entecavir, tenofovir) and universal infant vaccination.

Hepatitis C (HCV)

Dahiliye (İç Hastalıkları)

Hepatitis C is an RNA virus causing chronic hepatitis that may progress to cirrhosis and hepatocellular carcinoma; modern direct-acting antiviral (DAA) pangenotypic regimens (sofosbuvir/velpatasvir, glecaprevir/pibrentasvir) achieve sustained virologic response over 95% in 8–12 weeks with universal adult screening and cure for nearly all patients.

Fatty Liver Disease

Dahiliye (İç Hastalıkları)

Non-alcoholic fatty liver disease (NAFLD) is closely related to obesity and metabolic syndrome and is largely reversible with early treatment.

Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.