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Background
Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) associated with small cell lung cancer (SCLC) results in worse prognosis, increased risk of infection and decreased response to treatment. The poor survival is considered to be related to the high tumour burden, chemoresistance and higher incidence of infective complications even without neutropenia often resulting in discontinuation of cytotoxic treatment. Anticancer treatment alone rarely provides adequate control of endogenous hypercortisolism and steroid synthesis inhibitors such as ketoconazole and metyrapone have to be used for the optimum control of hormonal abnormalities. It is therefore prudent to control ectopic ACTH hypersecretion, which can be challenging if concomitant chemotherapy can result in potentially increased toxicity due to interaction between ketoconazole and etoposide. Sustained suppression of corticosteroid and improvement of performance status can be achieved while potential toxicity can be avoided when metyrapone is used with chemotherapy for the management of EAS.
Case presentation
A 50-year-old woman with a history of bilateral flank pain, abdominal distension, constipation, lower limb oedema, poor mobility and approximately 10 kg of weight gain was referred to the hospital by her general practitioner. She had a history of osteoporotic crush fractures of lumbar spine and poorly controlled hypertension as reflected by the use of multiple antihypertensives including atenolol, frusemide, irbesartan, perindopril and prazosin. She was also taking methadone, amitryptylline and paracetamol for backache. She lived alone and had experienced a substantial decline in her activities of daily living (Eastern Co-operative Oncology Group, ECOG, status 3). She had a 35-pack years history of smoking and consumed six standard drinks of alcohol per day for the past few years.
On examination, she had cushingoid appearance with facial plethora, centripetal obesity, interscapular fat pad, distended abdomen with purple striae and bilateral lower limb oedema. Her weight was 90 kg.
She was hypertensive with blood pressure of 180/115 mm Hg on multiple occasions. She did not have any hyperpigmentation or proximal muscular weakness.
Investigations
The random morning cortisol (802 nmol/l), random evening cortisol (761 nmol/l), 23:00 salivary cortisol (54 nmol/l) and 24 hours urinary free cortisol (2419 nmol/ 24 h) were all elevated. ACTH was normal. A 1 mg and an 8 mg dexamethasone suppression test failed to suppress with the morning serum cortisol values of 904 nmol/l and 840 nmol/l,...