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.
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
Search for other papers by Vasileios Chortis in
Google Scholar
PubMed
Search for other papers by Christine J H May in
Google Scholar
PubMed
Departments of Cellular Pathology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
Search for other papers by Kassiani Skordilis in
Google Scholar
PubMed
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
Search for other papers by John Ayuk in
Google Scholar
PubMed
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
Search for other papers by Wiebke Arlt in
Google Scholar
PubMed
Search for other papers by Rachel K Crowley in
Google Scholar
PubMed
Summary
Context
Adrenal incidentalomas (AI) represent an increasingly common problem in modern endocrine practice. The diagnostic approach to AIs can be challenging and occasionally reveals surprising features. Here we describe two rare cases of complex adrenal lesions consisting of phaeochromocytomas with synchronous metastases from extra-adrenal primaries.
Case descriptions
Patient 1 – a 65-year-old gentleman with a newly diagnosed malignant melanoma was found to harbour an adrenal lesion with suspicious radiographic characteristics. Percutaneous adrenal biopsy was consistent with adrenocortical adenoma. After excision of the skin melanoma and regional lymphatic metastases, he was followed up without imaging. Three years later, he presented with abdominal discomfort and enlargement of his adrenal lesion, associated with high plasma metanephrines. Adrenalectomy revealed a mixed tumour consisting of a large phaeochromocytoma with an embedded melanoma metastasis in its core. Patient 2 – a 63-year-old lady with a history of NF-1-related phaeochromocytoma 20 years ago and previous breast cancer presented with a new adrenal lesion on the contralateral side. Plasma normetanephrine was markedly elevated. Elective adrenalectomy revealed an adrenal tumour consisting of chromaffin cells intermixed with breast carcinoma cells.
Conclusions
Adrenal incidentalomas require careful evaluation to exclude metastatic disease, especially in the context of a history of previous malignancy. Adrenal biopsy provides limited and potentially misleading information. Phaeochromocytomas are highly vascularised tumours that may function as a sieve, extracting and retaining irregularly shaped cancer cells, thereby yielding adrenal masses with intriguing dual pathology.
Learning points:
-
Adrenal incidentalomas require careful evaluation focused on exclusion of underlying hormone excess and malignant pathology.
-
Adrenal biopsy can be misleading and should only be considered in select cases.
-
Phaeochromocytomas harbouring intratumoural metastases from other, extra-adrenal primary malignancies represent rare pathological entities that highlight the complexities that can be presented by adrenal tumours.
