This slide contains general information about Adrenocortical Carcinoma, how patients presents with it and information regarding how it can be Hormonally diagnosed.

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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