Diana Loreta Paun and Lavinia Vija contributed equally to this work.
The authors declare that they have no competing interests.
DLP and LV led the conception and design, acquisition of data, review of literature, and drafted the manuscript. CP, CB and SP critically reviewed the manuscript. DT provided the paraffin embedded samples and advised on the pathology pictures. ES, AB, EB contributed to the patient diagnosis and management and critically reviewed the manuscript. All authors read and approved the manuscript.
DLP is Associate Professor at “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania and Endocrinology Consultant at “C. I. Parhon” National Institute of Endocrinology. LV is Practitioner at Department of Nuclear Medecine at “C. I. Parhon” National Institute of Endocrinology. CP and CB are Professors at “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania and Endocrinology Consultants at “C. I. Parhon” National Institute of Endocrinology.
Ectopic production of adrenocorticotropic hormone (ACTH) by neuroendocrine tumours (NET) is a rare condition, occult presentations often hampering the diagnosis. Although NET are relatively frequent in the ileon and Meckel diverticulum, we describe the first Cushing’s syndrome due to ectopic adrenocorticotropic syndrome (CS-EAS) arising from a Meckel diverticulum.
A 44-year-old man was admitted with recent onset of diabetes, myopathy, edema and hypokalemic metabolic alkalosis consistent with Cushing’s syndrome. Both basal and dynamic laboratory evaluation suggested CS-EAS. Laboratory testing also showed high serum levels of chromogranin A (CgA) and urinary 5-hydroxyindoleacetic acid (5HIAA). Pituitary and neck/thorax/abdomen/pelvis imaging proved to be normal, while somatostatin analogue (99mTc-HYNIC-TOC) scintigraphy revealed increased focalized ileum uptake on the right iliac fossa. Pre-operative ketoconazole and sandostatin treatment controlled the hypercortisolism within a month. Pathological analysis of the resected submucosal 1.8 cm tumour of the Meckel diverticulum and a metastatic local lymph node confirmed a well differentiated neuroendocrine tumour (grade I), whereas immunohistochemistry was positive for ACTH, chromogranin A and synaptophysin. Post-operative clinical and biochemical resolution of Cushing’s syndrome was followed by normalization of both CgA and 5HIAA, which were maintained at the 6 month follow-up.
The identification, characterization and follow-up of this rare cause of ectopic ACTH secretion is important in order to assess the long-term prognostic and management.
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- Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion from a Meckel diverticulum neuroendocrine tumor: case report
Diana Loreta Paun
- BioMed Central
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