Symptoms and Diagnosis

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 by –

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 -