Adrenocortical carcinoma (ACC) is a rare and highly malignant tumor with an annual incidence of 1-2 per million, primarily affecting adults in their fourth to fifth decades and more prevalent in females. Clinical features can include incidental detection on CT scans, hormonal overproduction leading to symptoms of Cushing's syndrome, or non-functional symptoms like abdominal pain and weight loss. Hormonal evaluation, such as the dexamethasone suppression test, is essential for diagnosis and determining the cause of hormonal abnormalities.

1. ADRENOCORTICAL CARCINOMA
PRESENTATION & HORMONAL
EVALUATION
Gourav Singh
Dept. of Endocrinology
AIIMS
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2. INTRODUCTION & EPIDEMIOLOGY
• ACC is a rare malignancy with an annual
incidence of 1–2 per million population.
• Around 5% of all Adrenal Incidentaloma.
• Highly malignant tumor.
• Bimodal age distribution –
Children – First decade of life
Adults – Fourth to Fifth decades of life
• F > M (55-60% more in females).
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3. INTRODUCTION & EPIDEMIOLOGY
• ACC usually present as sporadic tumor.
• But it can present as Hereditary Tumor
• Associated with –
 Germline TP53 mutation (Li-Fraumeni syndrome)
 Alterations in the Wnt/β-catenin pathway (FAP)
 IGF2 overexpression (Beckwith Wiedemann Syndrome)
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4. CLINICAL FEATURES
• Detected incidentally on CT.
• 60–70% of ACCs show biochemical
evidence of steroid overproduction –
 Cortisol (Most Common)
 Aldosterone
 Sex Steroids
• Mixed excess production of several
corticosteroids is indicative of
malignancy.
• So may present as Functional/Non-
Functional tumors
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5. CLINICAL FEATURES
• Non-Functional tumor symptoms –
 Local symptoms:
 Abdominal fullness, back pain, nausea & vomiting.
 Systemic/Metastatic symptoms:
 Anorexia, anaemia, asthenia and weight loss.
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6. CLINICAL FEATURES
• Functional tumor symptoms –
 Symptoms like Cushing Syndrome (Cortisol secreting tumor).
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7. CLINICAL FEATURES
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8. CLINICAL FEATURES
• Functional tumor symptoms –
 Symptoms like Cushing Syndrome (Cortisol secreting tumor).
 Symptoms like Hyperaldosteronism.
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9. CLINICAL FEATURES
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10. HORMONAL EVALUATION
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11. HORMONAL EVALUATION
• Dexamethasone suppression test
To confirm the diagnosis of Cushing Syndrome.
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12. HORMONAL EVALUATION
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13. HORMONAL EVALUATION
• Dexamethasone suppression test
To confirm the diagnosis of Cushing Syndrome.
Dexamethasone given in night (11 pm)
Cortisol levels measured on next morning (8 am)
Done in two stages –
 Low dose test
 1mg Dexamethasone given
 To confirm patient actually has
Cushing Syndrome.
 Cortisol > 5gm/dl
 High dose test
 8mg Dexamethasone given
 To know the cause of Cushing
Syndrome.
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14. 11p.m.
11p.m.
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15. REFERENCES AND ILLUSTRATIONS
• Harrison's Principles of Internal Medicine, 20th edition.
• Puglisi S, Perotti P, Cosentini D, Roca E, Basile V, Berruti A, Terzolo M. Decision-
making for adrenocortical carcinoma: surgical, systemic, and endocrine management
options. Expert review of anticancer therapy. 2018 Nov 2;18(11):1125-33.
• Ross NS, Aron DC. Hormonal evaluation of the patient with an incidentally
discovered adrenal mass. New England Journal of Medicine. 1990 Nov
15;323(20):1401-5.
• Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment.
The Journal of urology. 2003 Jan;169(1):5-11.
• Google Image Search.
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