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

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

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

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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

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

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

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Masashi Demura Department of Hygiene, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Kenji Furukawa Health Care Center, Japan Advanced Institute of Science and Technology, Nomi, Ishikawa, Japan

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Yuto Yamazaki Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

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Hironobu Sasano Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

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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

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

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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.

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Shamaila Zaman Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Bijal Patel Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Paul Glynne The Physicians’ Clinic, London, UK

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Mark Vanderpump The Physicians’ Clinic, London, UK

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Ali Alsafi Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Sairah Khan Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Rashpal Flora Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Fausto Palazzo Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Florian Wernig Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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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.

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Aisha A Tepede Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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James Welch Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Maya Lee Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Adel Mandl Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Sunita K Agarwal Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Naris Nilubol National Cancer Institute (NCI), National Institutes of Health, Bethesda, Maryland, USA

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Dhaval Patel National Cancer Institute (NCI), National Institutes of Health, Bethesda, Maryland, USA

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Craig Cochran Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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William F Simonds Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Lee S Weinstein Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Abhishek Jha Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD), National Institutes of Health, Bethesda, Maryland, USA

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Corina Millo Clinical Center PET Department (CC PET), National Institutes of Health, Bethesda, Maryland, USA

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Karel Pacak Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD), National Institutes of Health, Bethesda, Maryland, USA

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Jenny E Blau Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)

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Summary

Pheochromocytoma (PHEO) in multiple endocrine neoplasia type 1 (MEN1) is extremely rare. The incidence is reported as less than 2%. We report a case of a 76-year-old male with familial MEN1 who was found to have unilateral PHEO. Although the patient was normotensive and asymptomatic, routine screening imaging with CT demonstrated bilateral adrenal masses. The left adrenal mass grew from 2.5 to 3.9 cm over 4 years with attenuation values of 9 Hounsfield units (HU) pre-contrast and 15 HU post-contrast washout. Laboratory evaluation demonstrated an adrenergic biochemical phenotype. Both 18F-fluorodeoxyglucose (18F-FDG) PET/CT and 123I-metaiodobenzylguanidine (123I-mIBG) scintigraphy demonstrated bilateral adrenal uptake. In contrast, 18F-fluorodihydroxyphenylalanine (18F-FDOPA) PET/CT demonstrated unilateral left adrenal uptake (28.7 standardized uptake value (SUV)) and physiologic right adrenal uptake. The patient underwent an uneventful left adrenalectomy with pathology consistent for PHEO. Post-operatively, he had biochemical normalization. A review of the literature suggests that adrenal tumors >2 cm may be at higher risk for pheochromocytoma in patients with MEN1. Despite a lack of symptoms related to catecholamine excess, enlarging adrenal nodules should be biochemically screened for PHEO. 18F-FDOPA PET/CT may be beneficial for localization in these patients.

Learning points:

  • 18F-FDOPA PET/CT is a beneficial imaging modality for identifying pheochromocytoma in MEN1 patients.

  • Adrenal adenomas should undergo routine biochemical workup for PHEO in MEN1 and can have serious peri-operative complications if not recognized, given that MEN1 patients undergo frequent surgical interventions.

  • MEN1 is implicated in the tumorigenesis of PHEO in this patient.

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Yasufumi Seki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoshi Morimoto Departments of Endocrinology and Hypertension, Tokyo, Japan

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Naohiro Yoshida Departments of Endocrinology and Hypertension, Tokyo, Japan

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Kanako Bokuda Departments of Endocrinology and Hypertension, Tokyo, Japan

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Nobukazu Sasaki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Midori Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Junichi Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Daisuke Watanabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoru Morita Departments of Diagnostic Imaging and Nuclear Medicine, Tokyo, Japan

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Keisuke Hata Departments of Urology, Kidney Center, Tokyo, Japan

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Tomoko Yamamoto Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Yoji Nagashima Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Atsuhiro Ichihara Departments of Endocrinology and Hypertension, Tokyo, Japan

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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.

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N F Lenders Diabetes and Metabolism, Garvan Institute of Medical Research, Sydney, New South Wales, Australia
Department of Endocrinology, St Vincent’s Hospital, Sydney, New South Wales, Australia
St Vincent’s Clinical School, University of New South Wales, Sydney, New South Wales, Australia

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J R Greenfield Diabetes and Metabolism, Garvan Institute of Medical Research, Sydney, New South Wales, Australia
Department of Endocrinology, St Vincent’s Hospital, Sydney, New South Wales, Australia
St Vincent’s Clinical School, University of New South Wales, Sydney, New South Wales, Australia

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Summary

Adrenal oncocytomas are rare tumours, with only approximately 160 cases reported in the literature. We report the use of urinary steroid profiling as part of their diagnostic evaluation and prognostication. A 45-year-old woman presented with clinical features of hyperandrogenism. Serum biochemistry confirmed androgen excess and computed tomography (CT) demonstrated a 3.2 cm adrenal tumour with density 39 HU pre-contrast. Urine steroid profiling showed elevated tetrahydro-11 deoxycortisol (THS), which is associated with adrenal malignancy. Laparoscopic adrenalectomy was performed, and histopathology diagnosed adrenal oncocytoma. Serum and urinary biochemistry resolved post-operatively and remained normal at 1-year follow-up.

Learning points:

  • Differential diagnosis of adrenal masses is challenging. Current techniques for differentiating between tumour types lack sensitivity and specificity.

  • 24-h urinary steroid profiling is a useful tool for reflecting steroid output from adrenal glands. Gas chromatography-mass spectrometry (GC-MS) of urinary steroid metabolites has sensitivity and specificity of 90% for diagnosing adrenocortical carcinoma.

  • Adrenal oncocytoma are rare tumours. Differentiating between benign and malignant types is difficult. Data guiding prognostication and management are sparse.

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Skand Shekhar Section on Endocrinology & Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Sriram Gubbi Diabetes, Endocrinology, and Obesity Branch, National Institute of Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA

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Georgios Z Papadakis Department of Medical Imaging, Heraklion University Hospital, Medical School, University of Crete, Crete, Greece
Computational Biomedicine Laboratory (CBML), Institute of Computer Science (ICS), Foundation for Research and Technology Hellas (FORTH), Heraklion, Greece

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Naris Nilubol Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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Fady Hannah-Shmouni Section on Endocrinology & Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Summary

Adrenococortical carcinoma (ACC) is a rare cancer, occurring at the rate of one case in two million person years. Cushing syndrome or a mixed picture of excess androgen and glucocorticoid production are the most common presentations of ACC. Other uncommon presentations include abdominal pain and adrenal incidentalomas. In the present report, a 71-year-old male presented with abdominal pain and was eventually diagnosed with ACC. He was found to have pulmonary thromboembolism following an investigation for hypoxemia, with the tumor thrombus extending upto the right atrium. This interesting case represents the unique presentation of a rare tumor, which if detected late or left untreated is associated with poor outcomes, highlighting the need for a low index of suspicion for ACC when similar presentations are encountered in clinical practice.

Learning points:

  • ACC is a rare but aggressive tumor.

  • ACC commonly presents with rapid onset of hypercortisolism, combined hyperandrogenism and hypercortisolism, or uncommonly with compressive symptoms.

  • Clinicians should have a low index of suspicion for ACC in patients presenting with rapid onset of symptoms related to hypercortisolism and/or hyperandrogenism.

  • Venous thromboembolism and extension of the tumor thrombus to the right side of the heart is a very rare but serious complication of ACC that clinicans should be wary of.

  • The increased risk of venous thromboembolism in ACC could be explained by direct tumor invasion, tumor thrombi or hypercoagulability secondary to hypercortisolism.

  • Early diagnosis and prompt treatment can improve the long-term survival of patients with ACC.

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

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Francesca Perticone Endocrine Unit, Department of Internal Medicine

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Laura Frosio Endocrine Unit, Department of Internal Medicine

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Marco Schiavo Lena Department of Pathology, San Raffaele Hospital, Milan, Italy

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Roberto Lanzi Endocrine Unit, Department of Internal Medicine

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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.

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Katta Sai Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Amos Lal Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Jhansi Lakshmi Maradana Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Pruthvi Raj Velamala Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Trivedi Nitin Department of Endocrinology, Diabetes, and Metabolism, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Summary

Mifepristone is a promising option for the management of hypercortisolism associated with hyperglycemia. However, its use may result in serious electrolyte imbalances, especially during dose escalation. In our patient with adrenocorticotropic hormone-independent macro-nodular adrenal hyperplasia, unilateral adrenalectomy resulted in biochemical and clinical improvement, but subclinical hypercortisolism persisted following adrenalectomy. She was started on mifepristone. Unfortunately, she missed her follow-up appointments following dosage escalation and required hospitalization at an intensive care level for severe refractory hypokalemia.

Learning points:

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Stephanie Wei Ping Wong Department of Diabetes and Endocrinology, St Helens and Knowsley Teaching Hospitals NHS Trust, Prescot, UK

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Yew Wen Yap Department of Diabetes and Endocrinology, St Helens and Knowsley Teaching Hospitals NHS Trust, Prescot, UK

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Ram Prakash Narayanan Department of Diabetes and Endocrinology, St Helens and Knowsley Teaching Hospitals NHS Trust, Prescot, UK

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Mohammad Al-Jubouri Department of Biochemistry, St Helens and Knowsley Teaching Hospitals NHS Trust, Prescot, UK

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Ashley Grossman Centre for Endocrinology, William Harvey Research Institute, Barts and London School of Medicine and Dentistry, Queen Mary University of London, London, UK

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Christina Daousi Department of Diabetes and Endocrinology, Aintree University Hospital NHS Foundation Trust, Liverpool, UK

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Yahya Mahgoub Department of Diabetes and Endocrinology, St Helens and Knowsley Teaching Hospitals NHS Trust, Prescot, UK

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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.

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E Sanz-Sapera Endocrinology, Vall d’Hebron Hospital, Barcelona, Spain

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S Sarria-Estrada Radiology, Vall d’Hebron Hospital, Barcelona, Spain

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F Arikan Neurosurgery, Vall d’Hebron Hospital, Barcelona, Spain

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B Biagetti Endocrinology, Vall d’Hebron Hospital, Barcelona, Spain

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Summary

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome characterised by ischaemic infarction or haemorrhage into a pituitary tumour that can lead to spontaneous remission of hormonal hypersecretion. We report the case of a 50-year-old man who attended the emergency department for sudden onset of headache. A computed tomography (CT) scan at admission revealed pituitary haemorrhage and the blood test confirmed the clinical suspicion of acromegaly and an associated hypopituitarism. The T1-weighted magnetic resonance imaging (MRI) showed the classic pituitary ring sign on the right side of the pituitary. Following admission, he developed acute-onset hyponatraemia that required hypertonic saline administration, improving progressively. Surprisingly, during the follow-up, IGF1 levels became normal and he progressively recovered pituitary function.

Learning points:

  • Patients with pituitary apoplexy may have spontaneous remission of hormonal hypersecretion. If it is not an emergency, we should delay a decision to undertake surgery following apoplexy and re-evaluate hormone secretion.

  • Hyponatraemia is an acute sign of hypocortisolism in pituitary apoplexy. However, SIADH although uncommon, could appear later as a consequence of direct hypothalamic insult and requires active and individualised treatment. For this reason, closely monitoring sodium at the beginning of the episode and throughout the first week is advisable to guard against SIADH.

  • Despite being less frequent, if pituitary apoplexy is limited to the tumour, the patient can recover pituitary function previously damaged by the undiagnosed macroadenoma.

Open access