Diagnosis and Treatment > Medication
Search for other papers by Shamaila Zaman in
Google Scholar
PubMed
Search for other papers by Bijal Patel in
Google Scholar
PubMed
Search for other papers by Paul Glynne in
Google Scholar
PubMed
Search for other papers by Mark Vanderpump in
Google Scholar
PubMed
Search for other papers by Ali Alsafi in
Google Scholar
PubMed
Search for other papers by Sairah Khan in
Google Scholar
PubMed
Search for other papers by Rashpal Flora in
Google Scholar
PubMed
Search for other papers by Fausto Palazzo in
Google Scholar
PubMed
Search for other papers by Florian Wernig in
Google Scholar
PubMed
Summary
Ectopic adrenocorticotropic hormone (ACTH) production is an uncommon cause of Cushing’s syndrome and, rarely, the source can be a phaeochromocytoma. A 55-year-old man presented following an episode of presumed gastroenteritis with vomiting and general malaise. Further episodes of diarrhoea, joint pains and palpitations followed. On examination, he was hypertensive with no clinical features to suggest hypercortisolaemia. He was subsequently found to have raised plasma normetanephrines of 3.98 nmol/L (NR <0.71) and metanephrines of 0.69 nmol/L (NR <0.36). An adrenal CT showed a 3.8 cm right adrenal nodule, which was not MIBG-avid but was clinically and biochemically consistent with a phaeochromocytoma. He was started on alpha blockade and referred for right adrenalectomy. Four weeks later, on the day of admission for adrenalectomy, profound hypokalaemia was noted (serum potassium 2.0 mmol/L) with non-specific ST-segment ECG changes. He was also diagnosed with new-onset diabetes mellitus (capillary blood glucose of 28 mmol/L). He reported to have gained weight and his skin had become darker over the course of the last 4 weeks. Given these findings, he underwent overnight dexamethasone suppression testing, which showed a non-suppressed serum cortisol of 1099 nmol/L. Baseline serum ACTH was 273 ng/L. A preliminary diagnosis of ectopic ACTH secretion from the known right-sided phaeochromocytoma was made and he was started on metyrapone and insulin. Surgery was postponed for 4 weeks. Following uncomplicated laparoscopic adrenalectomy, the patient recovered with full resolution of symptoms.
Learning points:
-
Phaeochromocytomas are a rare source of ectopic ACTH secretion. A high clinical index of suspicion is therefore required to make the diagnosis.
-
Ectopic ACTH secretion from a phaeochromocytoma can rapidly progress to severe Cushing’s syndrome, thus complicating tumour removal.
-
Removal of the primary tumour often leads to full recovery.
-
The limited literature suggests that the presence of ectopic Cushing’s syndrome does not appear to have any long-term prognostic implications.
Search for other papers by Stephanie Wei Ping Wong in
Google Scholar
PubMed
Search for other papers by Yew Wen Yap in
Google Scholar
PubMed
Search for other papers by Ram Prakash Narayanan in
Google Scholar
PubMed
Search for other papers by Mohammad Al-Jubouri in
Google Scholar
PubMed
Search for other papers by Ashley Grossman in
Google Scholar
PubMed
Search for other papers by Christina Daousi in
Google Scholar
PubMed
Search for other papers by Yahya Mahgoub in
Google Scholar
PubMed
Summary
We report our experience on managing a case of florid Cushing’s disease with Methicillin-resistant Staphylococcus aureus (MRSA) sepsis using intravenous etomidate in the intensive care unit of a UK district general hospital.
Learning points:
-
Severe Cushing’s syndrome is associated with high morbidity and mortality.
-
Etomidate is a safe and effective medical therapy to rapidly lower cortisol levels even in the context of severe sepsis and immunosuppression.
-
Etomidate should ideally be administered in an intensive care unit but is still feasible in a district general hospital.
-
During treatment with etomidate, accumulation of serum 11β-deoxycortisol (11DOC) levels can cross-react with laboratory cortisol measurement leading to falsely elevated serum cortisol levels. For this reason, serum cortisol measurement using a mass spectrometry assay should ideally be used to guide etomidate prescription.
Search for other papers by Ayanthi A Wijewardene in
Google Scholar
PubMed
Kolling Institute of Medical Research
Sydney Medical School, University of Sydney, Sydney, Australia
Search for other papers by Sarah J Glastras in
Google Scholar
PubMed
Sydney Medical School, University of Sydney, Sydney, Australia
Search for other papers by Diana L Learoyd in
Google Scholar
PubMed
Sydney Medical School, University of Sydney, Sydney, Australia
Search for other papers by Bruce G Robinson in
Google Scholar
PubMed
Sydney Medical School, University of Sydney, Sydney, Australia
Search for other papers by Venessa H M Tsang in
Google Scholar
PubMed
Summary
Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing’s syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Tyrosine kinase inhibitors (TKI) previously used for the management of metastatic MTC have become an important therapeutic option for the management of ectopic ACTH in metastatic MTC. The article describes three cases of ectopic ACTH secretion in MTC and addresses the significant diagnostic and management challenges related to Cushing’s syndrome in metastatic MTC.
Learning points:
-
Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour.
-
Cushing’s syndrome is a rare complication of MTC that has a significant impact on patients’ morbidity and mortality.
-
Tyrosine kinase inhibitors (TKI) provide an important therapeutic option for the management of ectopic ACTH in metastatic MTC.
