Diagnosis

Adrenal cortical carcinoma

Etiology

Cause [sih]

• Sporadic (most common): genetic mutation at specific genomic loci --> inactivation of tumor suppressor genes and activation of different oncogenes --> tumor genesis

• Familial cancer syndrome: Li-Fraumeni syndrome, familial adenomatous polyposis (FAP) or Gardner syndrome, Beckwith-Wiedemann syndrome, Multiple endocrine neoplasia type 1

Pathophysiology [sih]

• Adrenal cortex:

  • Glucocorticoids: affect carbohydrate, protein, and fat metabolism

    • Cortisol --> hypercortisolism

  • Mineralocorticoids: affect Na/K balance and the fluid homeostasis

    • Aldosterone

    • Androgen dehydroepiandrosterone

• Adrenal medulla:

  • Catecholamines:

    • Dopamine

    • Epinephrine

    • Norepinephrine

Epidemiology

Incidence per 100.000 [wtb][kuf][sea][sif]

Approach

• Blood test: cortisol, aldosterone, testosterone, estrogen

• Urine collection: cortisol, aldosterone, catecholamine, metanephrine, vanillylmandelic acid, 17-OH corticosteroids, 17-ketosteroids

• CT abdomen

• MR abdomen

• PET-CT

Treatment

1. Treatment of endocrine excess syndromes [sih]

2. Surgery: total resection remains the only potentially curative therapy

3. Radiation therapy

4. Chemotherapy

5. New types of treatment are being tested in clinical trials:

   • Immunotherapy

   • Targeted therapy

Differential diagnoses

Congenital adrenal hyperplasia, Cushings disease, Cushing syndrome, Pituitary adenoma, Polycystic ovary syndrome