Introduction: Ectopic Cushings syndrome (ECS) accounts for approximately 10% cases of Cushings 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 1/19961/2010 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 1367). The period between onset of symptoms and diagnosis of hypercortisolism was 10.5±8 months (124). 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 (1148). 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.