ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2011) 25 P168

Ectopic ACTH syndrome: experience of a tertiary referral centre: from diagnosis to outcome

Andreia Veloza, Georgia Ntali, John Wass & Niki Karavitaki


Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK.


Introduction: Ectopic Cushing’s syndrome (ECS) accounts for approximately 10% cases of Cushing’s syndrome. Its recognition may be delayed and its diagnosis and treatment remain challenging.

Aim: To analyze the clinical, biochemical, radiological features, as well as the outcome of patients with ECS presenting in a tertiary referral centre.

Material and methods: The records of patients with ECS followed presenting in our Department between 1/1996–1/2010 were reviewed.

Results: Thirteen patients were identified (7 men). The mean (±S.D.) age of onset of symptoms was 42.7±13.8 years (range 13–67). The period between onset of symptoms and diagnosis of hypercortisolism was 10.5±8 months (1–24). The most frequent reported manifestations were muscle weakness (85%), weight gain (85%), easy bruising (69%), hypertension (62%), hypokalemia (62%). 100% (8/8) of the patients did not respond to CRH test and 89% (8/9) did not suppress on the high dose Dexamethazone suppression test. No subject (n=5) showed gradient on the bilateral inferior petrosal sinus sampling. The localization of the source of the ECS was achieved between 1 and 90 months; in 9 it was of lung origin (8 pulmonary carcinoid, 1 small cell lung cancer), 1 had gastric adenocarcinoma, 1 prostate adenocarcinoma, 1 intestinal carcinoid tumour and 1 occult tumour. The median duration of follow-up was 27 months (1–148). 39% (5/13) of the subjects died within a median period of 6 months (1 pulmonary carcinoid, 1 small cell lung cancer, 1 gastric adenocarcinoma, 1 prostate adenocarcinoma, 1 occult tumour). Amongst the 8 patients with pulmonary carcinoid, tumour resection was performed in 7 (with lymph node removal/biopsy in 5 and proven metastatic disease in 2); no evidence of recurrence was documented in 71% (5/7).

Conclusions: ECS is a heterogeneous disease mainly attributed to lung origin. Tumour localization may be delayed necessitating regular imaging. Mortality primarily attributed to the original tumour is significant.

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