Adreno Cortical Carcinoma / Adreno Cortical Cancer / Cancer of the Adrenal Cortex / Adrenal Cortex Cancer

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 characterizedby –

• 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 cheeks

Conn syndrome due to High levels of Aldosterone or mineralocorticoids-

- High Blood Pressure

- Headache,

- Hypokalemia (low potassium),

- Muscle weakness,

- Palpitations

- Confusions

- Increased thirst

- Increased urination

Virilism - due to androgen or testostosterone excess – affects women

- Acne,

- Excess facial and body hair,

- Enlarged clitoris,

- Coarse facial features,

- Deep voice,

- Menopause

Feminization - due to estrogen excess seen in men -

- Breast enlargement,

- Low libido,

- Impotence

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.