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Adreno Cortical Carcinoma - Symptoms and Diagnosis

Last Updated on Feb 07, 2016
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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.

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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
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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
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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.

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