4th Edition

Chapter 22: The Lebanese man with a sweet tooth

A 73-year-old Lebanese man with long-standing hypertension and diabetes mellitus was overly fond of sweet pastries, especially baklava and ma’amoul. He presented with weight loss, proximal muscle weakness and hepatomegaly.

Initial investigations showed hyperglycaemia (Gluc = 15 mmol/L), hypokalaemia (K+ = 2.2 mmol/L) and metabolic acidosis (pH = 7.6, HCO3 = 39 mmol/L, base excess = 16).

  • 1. What investigations should be performed?

    Correct answer:
    The biochemical profile and symptoms are suggestive of excess secretion of adrenal hormones aldosterone and cortisol. The patient with a suspected diagnosis of adrenal cortical cancer will generally be investigated in an endocrinological or surgical outpatient setting where routine blood testing together with specific endocrinological investigations will be arranged. These will include measurement of the adrenal androgens, adrenocorticotrophic hormone (ACTH) levels, 24-hour urinary cortisol levels, plain X-rays and CT scans of the abdomen, pelvis and chest.

Endocrine investigations showed:
24 hour urine cortisol: 3600 nmol/L (normal <340)
24 hour urine catecholamines:
noradrenaline <500 nmol/L,
adrenaline <100 nmol/L
dopamine <2.7 mmol/L
Plasma ACTH: undetectable (<15 pmol/L) Serum AFP and CEA: normal
He had imaging studies.

  • 2. What does the CT-PET scan show?

    Correct answer:

    FDG avid right adrenal carcinoma

  • 3. What does the abdominal CT show?

    Correct answer:

    Large metastatic deposit in liver.

A CT-guided biopsy of the metastatic lesion in the liver was performed.

The biopsy confirmed neoplastic cells in nests with abundant pink cytoplasm and eccentrically placed nuclei and prominent nucleoli. Vimentin stain of the tumour cells was positive. Vimentin are intermediate microfilaments that form part of the cytoskeleton maintaining cell shape and the integrity of the cytoplasm. It is used as a marker of mesenchymal derived tissues including metastatic adrenocortical cancers.

Thus the final diagnosis is Cushing’s syndrome secondary to metastatic adrenocortical cancer. He was initially treated with mitotane and combination cisplatin and etoposide chemotherapy and his diabetes, hypokalaemia and hypertension resolved. Mitotane is structurally related to the insecticide DDT. The environmentalist Rachel Carson wrote in her book Silent Spring in 1962, that DDT was responsible for the loss of songbirds.

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