Endocrine Abstracts

Bilateral laparoscopic adrenalectomy is a treatment option for ACTH-dependent Cushing's syndrome after failed pituitary surgery (Cushing's disease) or when the ACTH source cannot be resected or localized (ectopic ACTH syndrome). It has not, to our knowledge, been reported in the treatment of ectopic ACTH secretion in patients with small cell lung cancer.

A 47 year old presented with haemoptysis and a right hilar mass, biopsy of which confirmed inoperable small cell carcinoma of the lung. During chemotherapy and radiotherapy the patient became pigmented, cushingoid, hypokalaemic, hypertensive (200/110), hypercholesterolaemic (22.3 mmol/l), obese, diabetic, and developed proximal myopathy and peripheral oedema. A diagnosis of Cushing's syndrome secondary to ectopic ACTH production was made based on a urinary free cortisol (UFC) of 22 701 nmol/24h and plasma ACTH of 314ng/l.

Medical treatment with metyrapone and ketoconazole had little clinical impact. Despite escalating medical therapy the patient deteriorated with worsening myopathy, persistently elevated UFC and admission with neutropenic sepsis. Mitotane, antihypertensive, and hypoglycaemic therapy were added. Dysphagia secondary to radiotherapy, multiple side-effects and polypharmacy (Metyrapone, Ketoconazole, Mitotane, Spironalactone, Lisinopril, Sando K, Aspirin, Glimeperide, Frusemide) made compliance difficult.

Laparoscopic bilateral adrenalectomy was therefore performed, following which serum cortisol was undetectable. The patient became normotensive, normoglycaemic, with normal electrolytes on steroid replacement alone. Symptomatically there was a dramatic improvement, the patient remained ambulant with a good quality of life and survived a further 6 months.

Patients with small call carcinoma of the lung and ectopic ACTH production usually present in a poor general state; 82% die within 2 weeks of starting chemotherapy compared to 25% of those without Cushing's syndrome. Medical treatment of ectopic ACTH is unsatisfactory and rarely leads to clinical and biochemical remission. Given the low morbidity of laparoscopic surgery, and the potential for improved quality of life, this approach should be evaluated prospectively.