Browse

You are looking at 1 - 8 of 8 items

Takuya Higashitani Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Takuya Higashitani in
Google Scholar
PubMed
Close
,
Shigehiro Karashima Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Shigehiro Karashima in
Google Scholar
PubMed
Close
,
Daisuke Aono Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Daisuke Aono in
Google Scholar
PubMed
Close
,
Seigoh Konishi Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan
Department of Internal Medicine, Keiju Medical Center, Nanao, Ishikawa, Japan

Search for other papers by Seigoh Konishi in
Google Scholar
PubMed
Close
,
Mitsuhiro Kometani Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Mitsuhiro Kometani in
Google Scholar
PubMed
Close
,
Rie Oka Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Rie Oka in
Google Scholar
PubMed
Close
,
Masashi Demura Department of Hygiene, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Masashi Demura in
Google Scholar
PubMed
Close
,
Kenji Furukawa Health Care Center, Japan Advanced Institute of Science and Technology, Nomi, Ishikawa, Japan

Search for other papers by Kenji Furukawa in
Google Scholar
PubMed
Close
,
Yuto Yamazaki Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

Search for other papers by Yuto Yamazaki in
Google Scholar
PubMed
Close
,
Hironobu Sasano Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

Search for other papers by Hironobu Sasano in
Google Scholar
PubMed
Close
,
Takashi Yoneda Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Takashi Yoneda in
Google Scholar
PubMed
Close
, and
Yoshiyu Takeda Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

Search for other papers by Yoshiyu Takeda in
Google Scholar
PubMed
Close

Summary

Renovascular hypertension (RVHT) is an important and potentially treatable form of resistant hypertension. Hypercortisolemia could also cause hypertension and diabetes mellitus. We experienced a case wherein adrenalectomy markedly improved blood pressure and plasma glucose levels in a patient with RVHT and low-level autonomous cortisol secretion. A 62-year-old Japanese man had been treated for hypertension and diabetes mellitus for 10 years. He was hospitalized because of a disturbance in consciousness. His blood pressure (BP) was 236/118 mmHg, pulse rate was 132 beats/min, and plasma glucose level was 712 mg/dL. Abdominal CT scanning revealed the presence of bilateral adrenal masses and left atrophic kidney. Abdominal magnetic resonance angiography demonstrated marked stenosis of the left main renal artery. The patient was subsequently diagnosed with atherosclerotic RVHT with left renal artery stenosis. His left adrenal lobular mass was over 40 mm and it was clinically suspected the potential for cortisol overproduction. Therefore, laparoscopic left nephrectomy and adrenalectomy were simultaneously performed, resulting in improved BP and glucose levels. Pathological studies revealed the presence of multiple cortisol-producing adrenal nodules and aldosterone-producing cell clusters in the adjacent left adrenal cortex. In the present case, the activated renin-angiotensin-aldosterone system and cortisol overproduction resulted in severe hypertension, which was managed with simultaneous unilateral nephrectomy and adrenalectomy.

Learning points:

  • Concomitant activation of the renin-angiotensin-aldosterone system and cortisol overproduction may contribute to the development of severe hypertension and lead to lethal cardiovascular complications.

  • Treatment with simultaneous unilateral nephrectomy and adrenalectomy markedly improves BP and blood glucose levels.

  • CYP11B2 immunohistochemistry staining revealed the existence of aldosterone-producing cell clusters (APCCs) in the adjacent non-nodular adrenal gland, suggesting that APCCs may contribute to aldosterone overproduction in patients with RVHT.

Open access
Yasufumi Seki Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Yasufumi Seki in
Google Scholar
PubMed
Close
,
Satoshi Morimoto Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Satoshi Morimoto in
Google Scholar
PubMed
Close
,
Naohiro Yoshida Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Naohiro Yoshida in
Google Scholar
PubMed
Close
,
Kanako Bokuda Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Kanako Bokuda in
Google Scholar
PubMed
Close
,
Nobukazu Sasaki Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Nobukazu Sasaki in
Google Scholar
PubMed
Close
,
Midori Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Midori Yatabe in
Google Scholar
PubMed
Close
,
Junichi Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Junichi Yatabe in
Google Scholar
PubMed
Close
,
Daisuke Watanabe Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Daisuke Watanabe in
Google Scholar
PubMed
Close
,
Satoru Morita Departments of Diagnostic Imaging and Nuclear Medicine, Tokyo, Japan

Search for other papers by Satoru Morita in
Google Scholar
PubMed
Close
,
Keisuke Hata Departments of Urology, Kidney Center, Tokyo, Japan

Search for other papers by Keisuke Hata in
Google Scholar
PubMed
Close
,
Tomoko Yamamoto Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

Search for other papers by Tomoko Yamamoto in
Google Scholar
PubMed
Close
,
Yoji Nagashima Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

Search for other papers by Yoji Nagashima in
Google Scholar
PubMed
Close
, and
Atsuhiro Ichihara Departments of Endocrinology and Hypertension, Tokyo, Japan

Search for other papers by Atsuhiro Ichihara in
Google Scholar
PubMed
Close

Summary

Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (LH) receptor in patients with PA has been reported; however, its physiological role on the development of PA is still unknown. Herein, we report two unique cases of PA in patients with untreated Klinefelter’s syndrome, characterized as increased serum LH, suggesting a possible contribution of the syndrome to PA development. Case 1 was a 39-year-old man with obesity and hypertension since his 20s. His plasma aldosterone concentration (PAC) and renin activity (PRA) were 220 pg/mL and 0.4 ng/mL/h, respectively. He was diagnosed as having bilateral PA by confirmatory tests and adrenal venous sampling (AVS). Klinefelter’s syndrome was suspected as he showed gynecomastia and small testes, and it was confirmed on the basis of a low serum total testosterone level (57.3 ng/dL), high serum LH level (50.9 mIU/mL), and chromosome analysis. Case 2 was a 28-year-old man who had untreated Klinefelter’s syndrome diagnosed in his childhood and a 2-year history of hypertension and hypokalemia. PAC and PRA were 247 pg/mL and 0.3 ng/mL/h, respectively. He was diagnosed as having a 10 mm-sized aldosterone-producing adenoma (APA) by AVS. In the APA, immunohistochemical analysis showed co-expression of LH receptor and CYP11B2. Our cases of untreated Klinefelter’s syndrome complicated with PA suggest that increased serum LH levels and adipose tissues, caused by primary hypogonadism, could contribute to PA development. The possible complication of PA in hypertensive patients with Klinefelter’s syndrome should be carefully considered.

Learning points:

  • The pathogenesis of primary aldosteronism is still unclear.

  • Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma.

  • Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism.

Open access
Alessandro Rossini Endocrinology and Diabetes Unit, ASST Papa Giovanni XXIII, Bergamo, Italy

Search for other papers by Alessandro Rossini in
Google Scholar
PubMed
Close
,
Francesca Perticone Endocrine Unit, Department of Internal Medicine

Search for other papers by Francesca Perticone in
Google Scholar
PubMed
Close
,
Laura Frosio Endocrine Unit, Department of Internal Medicine

Search for other papers by Laura Frosio in
Google Scholar
PubMed
Close
,
Marco Schiavo Lena Department of Pathology, San Raffaele Hospital, Milan, Italy

Search for other papers by Marco Schiavo Lena in
Google Scholar
PubMed
Close
, and
Roberto Lanzi Endocrine Unit, Department of Internal Medicine

Search for other papers by Roberto Lanzi in
Google Scholar
PubMed
Close

Summary

ACTH-secreting pheochromocytoma is a very rare cause of Cushing’s syndrome, with a high morbidity and mortality risk due to both cortisol and catecholamines excess. We report the case of a 45-year-old female patient with a 3 cm, high-density, left adrenal mass, diagnosed as an ACTH-secreting pheochromocytoma. The biochemical sensitivity of the tumor to somatostatin analogues was tested by a 100 μg s.c. octreotide administration, which led to an ACTH and cortisol reduction of 50 and 25% respectively. In addition to alpha and beta blockers, preoperative approach to laparoscopic adrenalectomy included octreotide, a somatostatin analogue, together with ketoconazole, in order to achieve an adequate pre-surgical control of cortisol release. Histopathological assessment confirmed an ACTH-secreting pheochromocytoma expressing type 2 and 5 somatostatin receptors (SSTR-2 and -5).

Learning points:

  • ACTH-secreting pheochromocytomas represent a rare and severe condition, characterized by high morbidity and mortality risk.

  • Surgical removal of the adrenal mass is the gold standard treatment, but adequate medical therapy is required preoperatively to improve the surgical outcome and to avoid major complications.

  • Somatostatin analogs, in addition to other medications, may represent a useful therapeutic option for the presurgical management of selected patients.

  • In this sense, the octreotide challenge test is a useful tool to predict favorable therapeutic response to the treatment.

Open access
Eka Melson Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Eka Melson in
Google Scholar
PubMed
Close
,
Sidra Amir University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Sidra Amir in
Google Scholar
PubMed
Close
,
Lisa Shepherd Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Lisa Shepherd in
Google Scholar
PubMed
Close
,
Samina Kauser University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Samina Kauser in
Google Scholar
PubMed
Close
,
Bethan Freestone University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Bethan Freestone in
Google Scholar
PubMed
Close
, and
Punith Kempegowda University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Punith Kempegowda in
Google Scholar
PubMed
Close

Summary

Although pheochromocytoma classically presents with headaches, palpitations and paroxysmal hypertension, atypical presentations such as cardiomyopathy, stroke and subarachnoid haemorrhage have been infrequently documented. We present in this case report, an uncommon presentation of pheochromocytoma with myocardial infarction with normal coronary arteries (MINOCA). A 79-year-old woman presented with central crushing chest pain radiating to left arm associated with headache, palpitations, sweating and difficulty in breathing. For 2 years, she experienced brief episodes of headache, tinnitus, dizziness, palpitations, and sweating that spontaneously resolved. Clinical examination was unremarkable except for high blood pressure (210/105 mmHg). Her electrocardiogram showed T wave inversions from V1 to V6 and elevated troponins (774 ng/L at baseline and 932 ng/L 3 h from baseline (normal <16 ng/L) in keeping with a diagnosis of non-ST elevated myocardial infarction. Coronary angiography showed normal coronary arteries. Patient was hence treated as myocardial infarction with normal coronaries (MINOCA). Despite appropriate treatment for MINOCA, she continued to experience episodic headaches, palpitations, dizziness and erratic blood pressures (particularly severe hypertension shortly after beta-blocker administration). Further investigations revealed raised urine noradrenaline of 4724 nmol/24 h (<554 nmol/24 h) and urine adrenaline of 92863 nmol/24 h (<77 nmol/24 h). Computerised tomography demonstrated a well-defined rounded mass in right adrenal gland morphological of pheochromocytoma. She underwent laparoscopic right adrenalectomy with histology confirming pheochromocytoma. This case highlights the importance of thorough investigation for the underlying cause for MINOCA. In patients with unexplained erratic blood pressure control, pheochromocytoma should be considered as a differential diagnosis.

Learning points:

  • Pheochromocytoma is rare tumour that often presents with non-specific symptoms.

  • It is important to investigate underlying cause of MINOCA.

  • Thorough history is the key to diagnosis.

Open access
Mohammed Faraz Rafey Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

Search for other papers by Mohammed Faraz Rafey in
Google Scholar
PubMed
Close
,
Arslan Butt Galway University Hospitals, Galway, Ireland

Search for other papers by Arslan Butt in
Google Scholar
PubMed
Close
,
Barry Coffey Galway University Hospitals, Galway, Ireland

Search for other papers by Barry Coffey in
Google Scholar
PubMed
Close
,
Lisa Reddington Galway University Hospitals, Galway, Ireland

Search for other papers by Lisa Reddington in
Google Scholar
PubMed
Close
,
Aiden Devitt Galway University Hospitals, Galway, Ireland

Search for other papers by Aiden Devitt in
Google Scholar
PubMed
Close
,
David Lappin Galway University Hospitals, Galway, Ireland

Search for other papers by David Lappin in
Google Scholar
PubMed
Close
, and
Francis M Finucane Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

Search for other papers by Francis M Finucane in
Google Scholar
PubMed
Close

Summary

We describe two cases of SGLT2i-induced euglycaemic diabetic ketoacidosis, which took longer than we anticipated to treat despite initiation of our DKA protocol. Both patients had an unequivocal diagnosis of type 2 diabetes, had poor glycaemic control with a history of metformin intolerance and presented with relatively vague symptoms post-operatively. Neither patient had stopped their SGLT2i pre-operatively, but ought to have by current treatment guidelines.

Learning points:

  • SGLT2i-induced EDKA is a more protracted and prolonged metabolic derangement and takes approximately twice as long to treat as hyperglycaemic ketoacidosis.

  • Surgical patients ought to stop SGLT2i medications routinely pre-operatively and only resume them after they have made a full recovery from the operation.

  • While the mechanistic basis for EDKA remains unclear, our observation of marked ketonuria in both patients suggests that impaired ketone excretion may not be the predominant metabolic lesion in every case.

  • Measurement of insulin, C-Peptide, blood and urine ketones as well as glucagon and renal function at the time of initial presentation with EDKA may help to establish why this problem occurs in specific patients.

Open access
Valeria de Miguel Departments of Endocrinology, Metabolism and Nuclear Medicine

Search for other papers by Valeria de Miguel in
Google Scholar
PubMed
Close
,
Andrea Paissan Departments of Endocrinology, Metabolism and Nuclear Medicine

Search for other papers by Andrea Paissan in
Google Scholar
PubMed
Close
,
Patricio García Marchiñena Departments of Urology, Metabolism and Nuclear Medicine

Search for other papers by Patricio García Marchiñena in
Google Scholar
PubMed
Close
,
Alberto Jurado Departments of Urology, Metabolism and Nuclear Medicine

Search for other papers by Alberto Jurado in
Google Scholar
PubMed
Close
,
Mariana Isola Pathology, Buenos Aires, Argentina

Search for other papers by Mariana Isola in
Google Scholar
PubMed
Close
,
José Alfie Hypertension Unit of Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

Search for other papers by José Alfie in
Google Scholar
PubMed
Close
, and
Patricia Fainstein-Day Departments of Endocrinology, Metabolism and Nuclear Medicine

Search for other papers by Patricia Fainstein-Day in
Google Scholar
PubMed
Close

Summary

We present the case of a 25-year-old male with a history of neurofibromatosis type 1 and bilateral pheochromocytoma 4 years after kidney transplantation that was successfully treated with simultaneous bilateral posterior retroperitoneoscopic adrenalectomy.

Learning points:

  • Hypertensive patients with NF1 should always be screened for pheochromocytoma.

  • Pheochromocytoma is rarely associated with transplantation, but it must be ruled out in patients with genetic susceptibility.

  • Posterior retroperitoneoscopic adrenalectomy (PRA) allows more direct access to the adrenal glands, especially in patients with previous abdominal surgeries.

Open access
Pedro Marques Department of Endocrinology, St. Bartholomew’s Hospital, West Smithfield, London, UK

Search for other papers by Pedro Marques in
Google Scholar
PubMed
Close
,
Nicola Tufton Department of Endocrinology, St. Bartholomew’s Hospital, West Smithfield, London, UK

Search for other papers by Nicola Tufton in
Google Scholar
PubMed
Close
,
Satya Bhattacharya Hepatobiliary and Pancreatic Surgery Unit, The Royal London Hospital, London, UK

Search for other papers by Satya Bhattacharya in
Google Scholar
PubMed
Close
,
Mark Caulfield Clinical Pharmacology, William Harvey Research Institute, Queen Mary University of London, London, UK

Search for other papers by Mark Caulfield in
Google Scholar
PubMed
Close
, and
Scott A Akker Department of Endocrinology, St. Bartholomew’s Hospital, West Smithfield, London, UK

Search for other papers by Scott A Akker in
Google Scholar
PubMed
Close

Summary

Mineralocorticoid hypertension is most often caused by autonomous overproduction of aldosterone, but excess of other mineralocorticoid precursors can lead to a similar presentation. 11-Deoxycorticosterone (DOC) excess, which can occur in 11-β hydroxylase or 17-α hydroxylase deficiencies, in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. We report a 35-year-old woman who in the third trimester of pregnancy was found to have a large adrenal mass on routine obstetric ultrasound. On referral to our unit, persistent hypertension and long-standing hypokalaemia was noted, despite good compliance with multiple antihypertensives. Ten years earlier, she had hypertension noted in pregnancy which had persisted after delivery. A MRI scan confirmed the presence of a 12 cm adrenal mass and biochemistry revealed high levels of DOC and low/normal renin, aldosterone and dehydroepiandrosterone, with normal catecholamine levels. The patient was treated with antihypertensives until obstetric delivery, following which she underwent an adrenalectomy. Histology confirmed a large adrenal cortical neoplasm of uncertain malignant potential. Postoperatively, blood pressure and serum potassium normalised, and the antihypertensive medication was stopped. Over 10 years of follow-up, she remains asymptomatic with normal DOC measurements. This case should alert clinicians to the possibility of a diagnosis of a DOC-producing adrenal tumours in patients with adrenal nodules and apparent mineralocorticoid hypertension in the presence of low or normal levels of aldosterone. The associated diagnostic and management challenges are discussed.

Learning points:

  • Hypermineralocorticoidism is characterised by hypertension, volume expansion and hypokalaemic alkalosis and is most commonly due to overproduction of aldosterone. However, excess of other mineralocorticoid products, such as DOC, lead to the same syndrome but with normal or low aldosterone levels.

  • The differential diagnosis of resistant hypertension with low renin and low/normal aldosterone includes congenital adrenal hyperplasia, syndrome of apparent mineralocorticoid excess, Cushing’s syndrome, Liddle’s syndrome and 11-deoxycorticosterone-producing tumours.

  • DOC is one intermediate product in the mineralocorticoid synthesis with weaker activity than aldosterone. However, marked DOC excess seen in 11-β hydroxylase or 17-α hydroxylase deficiencies in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension.

  • Excessive production of DOC in adrenocortical tumours has been attributed to reduced activity of the enzymes 11-β hydroxylase and 17-α hydroxylase and increased activity of 21-α hydroxylase.

  • The diagnosis of DOC-producing adrenal tumours is challenging because of its rarity and poor availability of DOC laboratory assays.

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

Search for other papers by Vasileios Chortis in
Google Scholar
PubMed
Close
,
Christine J H May Departments of Endocrinology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

Search for other papers by Christine J H May in
Google Scholar
PubMed
Close
,
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

Search for other papers by Kassiani Skordilis in
Google Scholar
PubMed
Close
,
John Ayuk 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 John Ayuk in
Google Scholar
PubMed
Close
,
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

Search for other papers by Wiebke Arlt in
Google Scholar
PubMed
Close
, and
Rachel K Crowley St. Vincent’s University Hospital and University College Dublin, Dublin, Ireland

Search for other papers by Rachel K Crowley in
Google Scholar
PubMed
Close

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.

Open access