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Endocrine Abstracts (2017) 48 CP6 | DOI: 10.1530/endoabs.48.CP6

SFEEU2017 National Clinical Cases Poster Presentations (26 abstracts)

The long search for an occult ectopic ACTH-producing tumour

Edouard Mills , Ali Naqvi , Florian Wernig & Jeannie Todd


Imperial College Healthcare NHS Trust, London, UK.


Case history: Ectopic adrenocorticotropic hormone (ACTH) production accounts for 10–20% of all endogeneous Cushing’s syndrome. The ideal treatment is curative surgery of the underlying tumour. In difficult cases bilateral adrenalectomy is an option. We report a 58-year-old woman with an aggressive ectopic Cushing’s syndrome that required bilateral adrenalectomy undertaken in 1989 at age 32-years.

Investigations: At 16 years post adrenalectomy, two right lung nodules became apparent on subsequent surveillance imaging. Over a 10-year period, these two foci on CT and Octreotide imaging demonstrated only marginal interval growth. The lung multidisciplinary team therefore advised against surgical intervention in 2006.

Results and treatment: However, in 2015, these two foci increased in size with growth noted in proximity to the main vessels and bronchi. She was therefore referred for surgery and underwent a right lung wedge resection. Histology was consistent with two bronchial typical carcinoids with clear resection margins and stained positive for ACTH. Post-operative imaging (CT chest, abdomen and pelvis, and gallium DOTATATE) demonstrated surgical lung changes, but no evidence of new or recurrent DOTATATE avid disease.

Conclusions and discussion: Since surgery, her tan significantly decreased. ACTH fell from a preoperative level of 1465 ng/l to 12 ng/l postoperatively with reduction in pigmentation, suggesting cure from ectopic ACTH-secretion. Ectopic ACTH-secreting tumours present challenges and require careful clinical, biochemical, radiological and pathological investigations. As this case demonstrates, ectopic ACTH-producing tumours can be extremely difficult to localise and often require multiple modalities of imaging. At present, there is no accepted frequency for imaging in occult ACTH-producing tumours. Our patient had an occult and indolent tumour that took almost two decades to present but has now had curative surgery.

Volume 48

Society for Endocrinology Endocrine Update 2017

Society for Endocrinology 

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