Adrenocortical carcinoma (ACC) usually present with either hormone excess (Cushing’s syndrome, virilization or symptoms associated with low potassium) or a local mass effect when the tumor is in excess of 10 cm.
Adrenocortical carcinoma with functional tumors involves hormonal dysfunction and is characterized
• Cushing’s syndrome – due to High serum Cortisol Levels,
• Conn syndrome - due to high Aldosterone levels
• Virilization or feminization – due to high levels of testosterone in women and oestrogen in men.
Adrenocortical carcinoma presents itself differently in children (virilization - presenting symptom) and adults (Cushing’s syndrome-presenting symptom).
The prominent symptoms and signs of Adrenocortical carcinoma are - Cushing syndrome -
due to increased cortisol levels or glucocorticoids.
- weight gain,
- purple lines on abdomen,
- muscle wasting,
- fatty ‘buffalo’ hump on neck, thin fragile skin,
- ‘moon-like’ face - flushed rounded face with pudgy cheeksConn syndrome
due to High levels of Aldosterone or mineralocorticoids-
- High Blood Pressure
- Hypokalemia (low potassium),
- Muscle weakness,
- Increased thirst
- Increased urinationVirilism
- due to androgen or testostosterone excess – affects women
- Excess facial and body hair,
- Enlarged clitoris,
- Coarse facial features,
- Deep voice,
- due to estrogen excess seen in men -
- Breast enlargement,
- Low libido,
Non-functional carcinomas can present as abdominal mass and flank pain. They can often present late. They are often detected on routine investigations such as ultrasound. Pathobiology / Causes
The main cause of adrenocortical cancer is unknown. However several causes are implicated.
• A family history of Fraumeni syndrome generates an increased risk
• Tumor suppressor genes such as p53 and RB(retinoblastoma) protein are altered.
• The expressions of genes h19 and insulin-like growth factor II ( essential for fetal development) are decreased and increased respectively.
• The expression of the c-myc gene is relatively high in these cancers and is associated with poor prognosis. Diagnosis
Diagnosis of adrenocortical cancer is based on the following tests -
• Blood Pressure is High
• Serum Cortisol levels are High
• Serum Aldosterone level is High
• Serum ACTH level is Low
• Serum Potassium is Low
• CT scan of the abdomen
• Abdominal MRI
Microscopic examinations of biopsied samples confirms the diagnosis.