Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: A challenging endocrine case with a happy ending

• Main Authors: Buch, H. (Author), Hatta, S.F.W.M (Author), Lekkakou, L. (Author), Viswananth, A. (Author)
• Format: Article
• Language: English
• Published: BMJ Publishing Group 2019
• Subjects: (3 iodobenzyl)guanidine; abdominal distension; abdominal pain; abdominal radiography; ACTH Syndrome, Ectopic; adrenal disease; adrenal disorders; adrenal incidentaloma; adrenalectomy; Adrenalectomy; adult; analgesia; androstenedione; Article; atenolol; blood; blood pressure; body mass; case report; catecholamine; catecholamine urine level; chronic disease; Chronic Disease; clinical article; colon obstruction; colonoscopy; complication; computer assisted tomography; constipation; Constipation; corticotropin; corticotropin blood level; Cushing syndrome; defecation; dexamethasone; differential diagnosis; digestive tract intubation; disease exacerbation; disease severity; doxazosin; drug megadose; echography; ectopic corticotropin production; endocrine system; enteric feeding; feces impaction; female; fluconazole; gastrointestinal system; gastroscopy; gliclazide; glycemic control; headache; heart palpitation; hemodynamic parameters; hirsutism; histology; hospital admission; hospital discharge; human; Humans; hydrocortisone; hydrocortisone blood level; hydrocortisone urine level; hyperglycemia; hypertension; hypokalemia; hypophysis adrenal system; indapamide; insulin; intestine transit time; isotope labeling; Ki 67 antigen; male; Male; medical history; metadrenalin; metformin; metyrapone; middle aged; Middle Aged; myopathy; nausea; non insulin dependent diabetes mellitus; nutritional status; oxodotreotide ga 68; patient referral; phenoxybenzamine; pheochromocytoma; Pheochromocytoma; physical examination; positron emission tomography-computed tomography; potassium; potassium intake; prasterone sulfate; priority journal; propranolol; radiopharmaceutical agent; sitagliptin; spironolactone; sweating; systolic blood pressure; tachycardia; testosterone; thrush; treatment outcome; Treatment Outcome; unclassified drug
• Online Access: View Fulltext in Publisher; View in Scopus

 Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rarely caused by a phaeochromocytoma. We report a case of a 51-year-old woman with an 8-year history of severe constipation who underwent extensive investigations including gastroscopy, colonoscopy, ultrasonography, colonic transit studies and isotope defeacography, which did not reveal any pathology other than slow colonic transit time. The unifying diagnosis of ectopic ACTH and phaeochromocytoma was made after the case was initially investigated for an adrenal incidentaloma. Multiple challenges had to be overcome prior to surgery for the functioning adrenal adenoma including management of refractory hypokalaemia, poor nutritional status, persistent hyperglycaemia, labile blood pressure and florid hypercortisolaemia driving the metabolic derangements. She underwent an uneventful left-sided adrenalectomy and required no medication thereafter with normal blood pressure, blood glucose and serum potassium and resolution of constipation and abdominal symptoms. In conclusion, patients with EAS related to phaeochromocytoma are rare and present with distinctive diagnostic and management challenges but if diagnosed successfully and managed intensively, they are curable. © 2019 BMJ Publishing Group Limited.