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 (Adrenal Cortex Cancer)

Rare and aggressive malignancy of the adrenal cortex with frequent hormonal hypersecretion.

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 (Adrenal Cortex Cancer)?

Adrenocortical carcinoma (ACC) is a rare malignant tumor arising from the adrenal cortex with an annual incidence of 0.7-2 per million. Bimodal age distribution shows peaks in childhood (associated with Li-Fraumeni and Beckwith-Wiedemann syndromes) and the fourth to fifth decade. Up to 60% are functional, secreting cortisol (Cushing syndrome), androgens (virilization), aldosterone, or estrogens.

Most patients present with abdominal mass, pain, or rapid-onset hormonal symptoms. Imaging features include large size (over 4 cm), heterogeneous appearance, calcifications, necrosis, and absolute contrast washout below 50%. Pathology relies on Weiss criteria, Ki-67 index, and immunohistochemistry (SF-1, melan-A, inhibin).

Staging by ENSAT system guides treatment. Complete R0 resection is the cornerstone for stages I-III. Adjuvant mitotane reduces recurrence in high-risk cases. For stage IV or unresectable disease, mitotane plus EDP chemotherapy (etoposide, doxorubicin, cisplatin) is standard. Prognosis depends on stage, Ki-67, and resection completeness, with 5-year survival of 60-80% for stage I-II and below 15% for stage IV.

Symptoms

Abdominal or flank pain
Palpable abdominal mass
Cushing syndrome (weight gain, moon face, hypertension)
Virilization (hirsutism, deep voice, acne in women)
Feminization (gynecomastia in men)
Hypertension and hypokalemia (aldosterone-secreting)
Rapid weight changes
Muscle weakness
Diabetes or hyperglycemia
Menstrual irregularity
Easy bruising, skin striae
Fatigue
Back pain (large or invasive tumors)
Symptoms of metastasis (bone, lung, liver)

Risk Factors

Li-Fraumeni syndrome (TP53 mutation)
Beckwith-Wiedemann syndrome
Lynch syndrome
MEN1 syndrome
Familial adenomatous polyposis (rare)
Childhood adrenocortical tumors (often TP53 R337H in Brazil)
Female sex slightly more affected
Adrenal incidentaloma over 4 cm with suspicious imaging

When to See a Doctor?

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

  • Rapid-onset Cushing or virilization features
  • Newly discovered adrenal mass over 4 cm
  • Severe hypertension with hypokalemia
  • Persistent abdominal or flank pain
  • Unexplained weight loss with hormonal symptoms
  • Pediatric virilization or precocious puberty
  • Abnormal contrast washout on adrenal CT
  • Family history of Li-Fraumeni or related syndromes

Treatment Methods

01
Multidisciplinary endocrine oncology evaluation
02
Hormonal workup: dexamethasone suppression, urinary cortisol, DHEA-S, testosterone, estradiol, aldosterone-renin, plasma metanephrines (rule out pheochromocytoma)
03
Adrenal protocol CT or MRI with washout analysis
04
FDG-PET for staging in high-risk cases
05
Open adrenalectomy by an experienced endocrine surgeon (laparoscopy controversial for large tumors)
06
En bloc resection of adjacent organs if invasion present
07
Avoid percutaneous biopsy unless metastatic and unresectable
08
Mitotane adjuvant therapy for high-risk resected disease (Ki-67 >10%, R1 margins, stage III)
09
EDP plus mitotane for advanced or recurrent disease
10
Radiotherapy for tumor bed in selected R1/R2 cases
11
Bone-targeted therapy for skeletal metastases
12
Locoregional therapies (RFA, TACE) for liver-dominant disease
13
Glucocorticoid replacement during mitotane therapy (mitotane induces adrenal insufficiency)
14
Monitor mitotane levels (target 14-20 mg/L), liver enzymes, lipid profile
15
Manage hormone excess: ketoconazole, metyrapone, etomidate for cortisol
16
Surveillance imaging every 3 months for first 2 years, then biannual
17
Genetic counseling for hereditary syndromes
18
Pediatric oncology referral for childhood ACC
19
Clinical trials for second-line therapy (immunotherapy, targeted agents)
20
Palliative care for symptom control in advanced disease

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.