Ectopic Cushing syndrome from pulmonary carcinoid tumour

Case contributed by Dr Rajesh Annamalaisamy

Presentation

Severe depression, headaches, weakness and ? bipolar disorder.

Patient Data

Age: 40
Gender: Male

CT shows a small right middle lobe lung nodule and bilateral adrenal hyperplasia.

Octerotide

Modality: Nuclear medicine

Uptake in the right lung nodule. 

Case Discussion

Following CT, patient had serum cortisol and ACTH levels assessed. Cortisol was > 3000 and ACTH was 162. He also had mild diabetes, profound hypokalemia and hypocalcemia. MRI brain was normal. Octreotide scan confirmed uptake in the right lung nodule and a diagnosis of ectopic Cushing syndrome was made.

Following resection, the patient was completely relieved of his symptoms. Pathology confirmed a carcinoid tumor.

Unlike Cushing disease, with an 8:1 female to male preponderance, this syndrome is more common in men. Earlier it has been reported that ectopic secretion of ACTH is most often from small cell carcinomas of the lung. But, later reports indicate that bronchial carcinoid tumour is the single most common cause.

Other well documented cases included thymic carcinoid, medullary thyroid carcinoma, pheochromocytoma, medullary paraganglioma, pancreatic islet cell tumours, and tumours of the ovary, cervix and prostate. The Cushing syndrome due to ‘primary’ hepatic carcinoid is very rare.

Tumours are best localized with CT thorax/abdomen in >80% of cases. However, in case of failure to localize the ectopic source of ACTH secretion with one modality, multiple imaging techniques should be employed to identify the same. CT, MRI and octreotide scan/PET scan all may be required to localize the ectopic source of ACTH secretion.

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Case Information

rID: 19559
Case created: 24th Sep 2012
Last edited: 1st Oct 2016
System: Chest
Inclusion in quiz mode: Included

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