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G Leksic Division of Endocrinology and Diabetes, Department of Internal Medicine

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A M Alduk Department of Radiology, University Hospital Centre Zagreb, Zagreb, Croatia

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V Molnar Division of Endocrinology and Diabetes, Department of Internal Medicine

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A Haxhiu Division of Endocrinology and Diabetes, Department of Internal Medicine

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A Balasko Division of Endocrinology and Diabetes, Department of Internal Medicine

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N Knezevic Department of Urology, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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T Dusek Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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D Kastelan Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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Summary

Primary aldosteronism (PA) is characterised by aldosterone hypersecretion and represents a common cause of secondary hypertension. During diagnostic evaluation, it is essential to determine the aetiology of PA since the treatment of unilateral and bilateral disease differs significantly. Adrenal vein sampling (AVS) has been implemented as a gold standard test for the diagnosis of PA subtype. However, due to the AVS complexity, costs and limited availability, many patients with PA are being treated based on the computed tomography (CT) findings. In this article, we present two patients with discrepant CT and AVS results, demonstrating that AVS is the only reliable method for localising the source of aldosterone excess.

Learning points:

  • CT is an unreliable method for distinguishing aldosterone-producing adenoma (APA) from bilateral adrenal hyperplasia (BAH).

  • CT can be misleading in defining lateralisation of the aldosterone excess in case of unilateral disease (APA).

  • AVS is the gold standard test for defining the PA subtype.

Open access
Vasileios Chortis Institute of Metabolism and Systems Research, University of Birmingham
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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Christine J H May Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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Kassiani Skordilis Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Cellular Pathology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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John Ayuk Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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Wiebke Arlt Institute of Metabolism and Systems Research, University of Birmingham
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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Rachel K Crowley St. Vincent’s University Hospital and University College Dublin, Dublin, Ireland

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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.

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Michal Barabas Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust

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Isabel Huang-Doran Wellcome-MRC Institute of Metabolic Science, Addenbrooke’s Hospital, Cambridge, UK

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Debbie Pitfield Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust

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Hazel Philips Department of Cardiology, Bedford Hospital NHS Trust, Bedford, UK

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Manoj Goonewardene Department of Cardiology, Bedford Hospital NHS Trust, Bedford, UK

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Ruth T Casey Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust

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Benjamin G Challis Wolfson Diabetes & Endocrine Clinic, Cambridge University Hospitals NHS Foundation Trust
IMED Biotech Unit, Clinical Discovery Unit, AstraZeneca, Cambridge, UK

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Summary

A 67-year-old woman presented with a generalised rash associated with weight loss and resting tachycardia. She had a recent diagnosis of diabetes mellitus. Biochemical evaluation revealed elevated levels of circulating glucagon and chromogranin B. Cross-sectional imaging demonstrated a pancreatic lesion and liver metastases, which were octreotide-avid. Biopsy of the liver lesion confirmed a diagnosis of well-differentiated grade 2 pancreatic neuroendocrine tumour, consistent with metastatic glucagonoma. Serial echocardiography commenced 4 years before this diagnosis demonstrated a progressive left ventricular dilatation and dysfunction in the absence of ischaemia, suggestive of glucagonoma-associated dilated cardiomyopathy. Given the severity of the cardiac impairment, surgical management was considered inappropriate and somatostatin analogue therapy was initiated, affecting clinical and biochemical improvement. Serial cross-sectional imaging demonstrated stable disease 2 years after diagnosis. Left ventricular dysfunction persisted, however, despite somatostatin analogue therapy and optimal medical management of cardiac failure. In contrast to previous reports, the case we describe demonstrates that chronic hyperglucagonaemia may lead to irreversible left ventricular compromise. Management of glucagonoma therefore requires careful and serial evaluation of cardiac status.

Learning points:

  • In rare cases, glucagonoma may present with cardiac failure as the dominant feature. Significant cardiac impairment may occur in the absence of other features of glucagonoma syndrome due to subclinical chronic hyperglucagonaemia.

  • A diagnosis of glucagonoma should be considered in patients with non-ischaemic cardiomyopathy, particularly those with other features of glucagonoma syndrome.

  • Cardiac impairment due to glucagonoma may not respond to somatostatin analogue therapy, even in the context of biochemical improvement.

  • All patients with a new diagnosis of glucagonoma should be assessed clinically for evidence of cardiac failure and, if present, a baseline transthoracic echocardiogram should be performed. In the presence of cardiac impairment these patients should be managed by an experienced cardiologist.

Open access
Naoya Toriu Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Masayuki Yamanouchi Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Rikako Hiramatsu Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Noriko Hayami Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Junichi Hoshino Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Akinari Sekine Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Masahiro Kawada Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Eiko Hasegawa Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Tatsuya Suwabe Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Keiichi Sumida Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Toshiharu Ueno Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Naoki Sawa Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan

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Kenichi Ohashi Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan
Department of Pathology, Yokohama City University, Graduate School of Medicine, Yokohama, Japan

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Takeshi Fujii Department of Pathology, Toranomon Hospital, Tokyo, Japan

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Kenmei Takaichi Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan
Okinaka Memorial Institute for Medical Research, Tokyo, Japan

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Motoko Yanagita Department of Nephrology, Kyoto University Graduate School of Medicine, Japan

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Tetsuro Kobayasi Okinaka Memorial Institute for Medical Research, Tokyo, Japan

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Yoshifumi Ubara Nephrology Center and Department of Rheumatology, Toranomon Hospital, Tokyo, Japan
Okinaka Memorial Institute for Medical Research, Tokyo, Japan

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Summary

We report the case of a 67-year-old Japanese woman with type 1 diabetes mellitus. At 47 years of age, her hemoglobin A1c (HbA1c) was 10.0%, and she had overt nephropathy. The first renal biopsy yielded a diagnosis of diabetic nephropathy. Intensive glycemic control was initiated and her HbA1c improved to 6.0%. Renal dysfunction showed no progression for 15 years. At 62 years of age, a second renal biopsy was performed. Glomerular lesions did not show progression but tubulointerstitial fibrosis and vascular lesions showed progression compared with the first biopsy. Intensive glycemic control can prevent the progression of glomerular lesions, but might not be effective for interstitial and vascular lesions.

Learning points:

  • Intensive control of blood glucose can prevent the progression of glomerular lesions.

  • Intensive control of blood glucose may not be able to prevent progression of interstitial and vascular lesions.

  • CSII reduces HbA1c without increasing the risk of hypoglycemia.

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Kazuyuki Oishi Kochi Health Sciences Center – Divisions of Breast & Thyroid Surgery, Kochi, Japan

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Daisuke Takabatake Kochi Health Sciences Center – Divisions of Breast & Thyroid Surgery, Kochi, Japan

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Yuichi Shibuya Kochi Health Sciences Center – Divisions of Breast & Thyroid Surgery, Kochi, Japan

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Summary

We experienced a case of an 82-year-old woman who presented to our hospital with a 1-month history of dysphagia and dyspnea. Cervical contrast-enhanced computed tomography revealed diffuse thyroid neoplasms causing significant tracheal stenosis with tumors, particularly of the superior mediastinum, which were associated with an embolism of the brachiocephalic vein and suspected invasion to the bilateral common carotid arteries. Anaplastic thyroid cancer (ATC) was diagnosed by fine-needle aspiration; thus, emergency tracheostomy and gastrostomy were performed. We made a definitive diagnosis of ATC (T4bN0M0 Stage IVB) and initiated continuous lenvatinib administration at 24 mg/day. Although several adverse events occurred, the tumor size reduced remarkably over a short period. However, the patient died from rupture of the common carotid artery 30 days after treatment initiation. Here, we report our experience with lenvatinib therapy for ATC and include a literature review.

Learning points:

  • Lenvatinib is extremely effective for ATC.

  • Lenvatinib has a much greater cytoreductive effect than traditional therapies, but it needs dose reduction or withdrawal because of treatment-related side effects.

  • Lenvatinib may cause treatment-related carotid blowout syndrome, resulting in death for patients with invasion to the carotid artery.

Open access
Gautam Das Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Peter N Taylor Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Arshiya Tabasum Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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L N Rao Bondugulapati Department of Diabetes and Endocrinology, Maelor Hospital, Betsi Cadwaldr University Health Board, Wrexham, LL13 7TD, UK

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Danny Parker Department of Histopathology, University Hospital of Wales, Cardiff and Vale University Health Board, Cardiff, CF14 4XW, UK

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Piero Baglioni Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Onyebuchi E Okosieme Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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David Scott Coombes Department of Endocrine Surgery, University Hospital of Wales, Cardiff and Vale University Health Board, Cardiff, CF14 4XW, UK

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Summary

Resistant hypertension is often difficult to treat and may be associated with underlying primary aldosteronism (PA). We describe the case of an elderly gentleman who presented with severe and resistant hypertension and was found to have a left adrenal incidentaloma during evaluation but had aldosterone excess secondary to unilateral adrenal hyperplasia (UAH) of the contralateral gland, which needed surgical intervention. A 65-year-old gentleman was evaluated for uncontrolled high blood pressure (BP) in spite of taking four antihypertensive medications. The high BP was confirmed on a 24-h ambulatory reading, and further biochemical evaluation showed an elevated serum aldosterone renin ratio (ARR) (1577 pmol/l per ng per ml per h). Radiological evaluation showed an adrenal nodule (15 mm) in the left adrenal gland but an adrenal vein sampling demonstrated a lateralization towards the opposite site favouring the right adrenal to be the source of excess aldosterone. A laparoscopic right adrenalectomy was performed and the histology of the gland confirmed nodular hyperplasia. Following surgery, the patient's BP improved remarkably although he remained on antihypertensives and under regular endocrine follow-up. PA remains the most common form of secondary and difficult-to-treat hypertension. Investigations may reveal incidental adrenal lesions, which may not be the actual source of excess aldosterone, but UAH may be a contributor and may coexist and amenable to surgical treatment. An adrenal vein sampling should be undertaken for correct lateralization of the source, otherwise a correctable diagnosis may be missed and the incorrect adrenal gland may be removed.

Learning points

  • Severe and resistant hypertension can often be associated with underlying PA.

  • ARR is an excellent screening tool in patients with suspected PA.

  • Lateralization with adrenal venous sampling is essential to isolate the source and differentiate between unilateral and bilateral causes of hyperaldosteronism.

  • Adrenal incidentalomas and UAH may coexist and the latter may often be the sole cause of excess aldosterone secretion.

  • Decisions about adrenalectomy should be made only after integrating and interpreting radiological and biochemical test findings properly.

Open access