Diagnosis and Treatment > Medication > Octreotide

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Naweed Alzaman Division of Endocrinology, Diabetes, and Metabolism, Tufts Medical Center, 800 Washington Street, Boston, Massachusetts, 02111, USA

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Anastassios G Pittas Division of Endocrinology, Diabetes, and Metabolism, Tufts Medical Center, 800 Washington Street, Boston, Massachusetts, 02111, USA

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Miriam O'Leary Department of Otolaryngology, Tufts Medical Center, 800 Washington Street, Boston, Massachusetts, 02111, USA

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Lisa Ceglia Division of Endocrinology, Diabetes, and Metabolism, Tufts Medical Center, 800 Washington Street, Boston, Massachusetts, 02111, USA

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Summary

Transient hypocalcemia after thyroidectomy is not uncommon and the risk increases with the extent of neck surgery. We report a case of severe and prolonged hypocalcemia after total thyroidectomy complicated by thoracic duct injury. Hypoparathyroidism and thoracic duct injury are potential complications following total thyroidectomy with extensive lymph node dissection. This case suggested that having both conditions may complicate treatment of hypoparathyroid-induced hypocalcemia by way of losses of calcium and vitamin D in the chyle leak.

Learning points

  • This report highlights chyle leak as an uncommon cause of prolonged hypocalcemia in patients who have undergone extensive neck surgery.

  • Chyle has an electrolyte concentration similar to that of plasma.

  • Medical treatment options for a chyle leak include fat-free oral diet or parenteral nutrition without oral intake, pharmacological treatment (primarily octreotide).

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Sally K Abell Department of Endocrinology and Diabetes, St Vincent's Hospital, PO Box 2900, Fitzroy, Melbourne, 3065 Victoria, Australia

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Jessie Teng Department of Endocrinology and Diabetes, St Vincent's Hospital, PO Box 2900, Fitzroy, Melbourne, 3065 Victoria, Australia

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Anthony Dowling Department of Oncology, St Vincent's Hospital, PO Box 2900, Fitzroy, Melbourne, 3065 Victoria, Australia

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Michael S Hofman Department of Medicine, University of Melbourne, Parkville, Melbourne, Victoria, Australia
Molecular Imaging, Centre for Cancer Imaging, Peter MacCallum Cancer Centre, East Melbourne, Melbourne, Victoria, Australia

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Richard J MacIsaac Department of Endocrinology and Diabetes, St Vincent's Hospital, PO Box 2900, Fitzroy, Melbourne, 3065 Victoria, Australia
Department of Medicine, University of Melbourne, Parkville, Melbourne, Victoria, Australia

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Nirupa Sachithanandan Department of Endocrinology and Diabetes, St Vincent's Hospital, PO Box 2900, Fitzroy, Melbourne, 3065 Victoria, Australia

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Summary

This paper details the case of a 77-year-old male with refractory hypoglycaemia due to inoperable metastatic pancreatic neuroendocrine tumour (pNET) co-secreting insulin and gastrin. Multiple medical therapies were trialled with limited success, and we describe the complications experienced by our patient. Somatostatin analogues can ameliorate hypoglycaemia and may have tumour-stabilising effects; however, in our case resulted in paradoxical worsening of hypoglycaemia. This rendered our patient hospital dependent for glycaemic support including continuous dextrose infusion. Although this is a reported adverse effect with initiation of therapy, we describe successful initiation of short-acting octreotide as an inpatient followed by commencement of long-acting octreotide. Hypoglycaemic collapse occurred only after dose titration of long-acting octreotide. We outline the pitfalls of somatostatin analogue therapy and the mechanisms that may contribute to worsening hypoglycaemia. This rare side effect cannot be reliably predicted, necessitating close supervision and glucose monitoring during therapy. Our patient achieved disease stabilisation and gradual resolution of hypoglycaemia with peptide receptor radionuclide therapy (PRRT), an emerging therapeutic option for metastatic neuroendocrine tumours with high efficacy and low toxicity. We present a brief but comprehensive discussion of currently available and novel therapies for insulin secreting pNETs.

Learning points

  • Hypoglycaemia due to malignant insulin secreting pNET is frequently severe and may be life-threatening despite supportive therapies.

  • Octreotide can ameliorate hypoglycaemia, and may have anti-proliferative and tumour-stabilising effects in malignant pNETs that are surgically unresectable.

  • Paradoxical worsening of hypoglycaemia may occur with octreotide initiation and dose titration, necessitating close supervision and glucose monitoring.

  • PRRT is emerging as a therapeutic option with high efficacy and low toxicity.

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Philip C Johnston Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Amir H Hamrahian Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Richard A Prayson Patholgy and Laboratory Medicine Institute, Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio 44195, USA

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Laurence Kennedy Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Robert J Weil Department of Neurosurgery and the Neurological Institute, Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio 44195, USA

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Summary

A 54-year-old woman presented with bi-temporal hemianopia, palpitations, and diaphoresis. An invasive pituitary macroadenoma was discovered. The patient had biochemical evidence of secondary hyperthyroidism and GH excess; however, she did not appear to be acromegalic. Surgical removal of the pituitary mass revealed a plurihormonal TSH/GH co-secreting pituitary adenoma. TSH-secreting adenomas can co-secrete other hormones including GH, prolactin, and gonadotropins; conversely, co-secretion of TSH from a pituitary adenoma in acromegaly is infrequent.

Learning points

  • This case highlights an unusual patient with a rare TSH/GH co-secreting pituitary adenoma with absence of the clinical features of acromegaly.

  • Plurihormonality does not always translate into the clinical features of hormonal excess.

  • There appears to be a clinical and immunohistochemical spectrum present in plurihormonal tumors.

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Kun Zhang Department of Cardiology and Angiology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Lukas J Lehner Department of Nephrology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Damaris Praeger Department of Cardiology and Angiology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Gert Baumann Department of Cardiology and Angiology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Fabian Knebel Department of Cardiology and Angiology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Marcus Quinkler Department of Endocrinology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Torsten K Roepke Department of Cardiology and Angiology, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10115 Berlin, Germany

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Summary

Neuroendocrine tumours (NETs) represent a broad spectrum of tumours, of which the serotonin-producing carcinoid is the most common and has been shown to cause right ventricular heart failure. However, an association between heart failure and NETs other than carcinoid has not been established so far. In this case report, we describe a 51-year-old patient with a glucagon-producing NET of the pancreas who developed acute heart failure and even cardiogenic shock despite therapy. Heart failure eventually regressed after initialising i.v. treatment with the somatostatin analogue octreotide. Chromogranin A as a tumour marker was shown to be significantly elevated, and it decreased with clinical improvement of the patient. The effects of long-time stimulation of glucagon on the myocardium have not been studied yet; however, sarcoplasmic reticulum calcium leak can be discussed as a possible mechanism for glucagon-induced heart failure.

Learning points

  • Glucagonoma can be a cause for heart failure.

  • i.v. infusion of octreotide can be successfully used to treat glucagonoma-induced acute heart failure.

  • We suggest that cardiac function should be monitored in all NET patients.

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Chiara Baratelli Dipartimento di Oncologia, Oncologia Medica

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Maria Pia Brizzi Dipartimento di Oncologia, Oncologia Medica

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Marco Tampellini Dipartimento di Oncologia, Oncologia Medica

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Giorgio Vittorio Scagliotti Dipartimento di Oncologia, Oncologia Medica

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Adriano Priola SCDU Radiologia

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Massimo Terzolo Dipartimento di Scienze Cliniche e Biologiche, Medicina Interna, Università di Torino, Azienda Ospedaliero Universitaria San Luigi Gonzaga, Regione Gonzole 10, 10043 Orbassano, Italy

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Anna Pia Dipartimento di Scienze Cliniche e Biologiche, Medicina Interna, Università di Torino, Azienda Ospedaliero Universitaria San Luigi Gonzaga, Regione Gonzole 10, 10043 Orbassano, Italy

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Alfredo Berruti Dipartimento di Specialità Medico-Chirurgiche, Scienze Radiologiche e Sanità Pubblica Università di Brescia, Oncologia Medica, Azienda Ospedaliera Spedali Civili, Brescia, Italy

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Summary

Insulinoma is a rare form of insulin-secreting pancreatic islet cell neuroendocrine (NE) tumor. The medical treatment of the malignant NE disease of the pancreas deeply changed in the last years, thanks to the introduction of new target molecules, as everolimus. Even if the exact mechanism is not actually known, one of the side effects of everolimus, hyperglycemia, has been demonstrated to be useful to contrast the typical hypoglycemia of the insulinoma. We report the case of a patient with a metastatic malignant insulinoma treated with intermittent everolimus, obtaining an important improvement in the quality of life; this suggests the necessity of preclinical studies to analyze the cellular pathways involved in insulin-independent gluconeogenesis.

Learning points

  • Effect of somatostatin analogs is long-lasting in the control of functioning NE tumors.

  • Persistent everolimus control of hypoglycemia despite serum insulin levels and disease progression.

  • Open issue: are disease progression and the increase in serum markers the only valid criteria to reject a treatment?

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Omayma Elshafie Department of Medicine, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Yahya Al Badaai Department of Surgery, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Khalifa Alwahaibi Department of Surgery, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Asim Qureshi Department of Pathology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Samir Hussein Department of Radiology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Faisal Al Azzri Department of Radiology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Ali Almamari Department of Medicine, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Nicholas Woodhouse Department of Medicine, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

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Summary

A 48-year-old hypertensive and diabetic patient presented with a 10-year history of progressive right facial pain, tinnitus, hearing loss, sweating, and palpitations. Investigations revealed a 5.6 cm vascular tumor at the carotid bifurcation. Her blood pressure (BP) was 170/110, on lisinopril 20 mg od and amlodipine 10 mg od and 100 U of insulin daily. A catecholamine-secreting carotid body paraganglioma (CSCBP) was suspected; the diagnosis was confirmed biochemically by determining plasma norepinephrine (NE) level, 89 000 pmol/l, and chromogranin A (CgA) level, 279 μg/l. Meta-iodobenzylguanidine and octreotide scanning confirmed a single tumor in the neck. A week after giving the patient a trial of octreotide 100 μg 8 h, the NE level dropped progressively from 50 000 to 25 000 pmol/l and CgA from 279 to 25 μg/l. Treatment was therefore continued with labetalol 200 mg twice daily (bid) and long-acting octreotide-LA initially using 40 mg/month and later increasing to 80 mg/month. On this dose and with a reduced labetalol intake of 100 mg bid, BP was maintained at 130/70 and her symptoms resolved completely. CgA levels returned to normal in the first week and these were maintained throughout the 3 month treatment period. During tumor resection, there were minimal BP fluctuations during the 10 h procedure. We conclude that short-term high-dose octreotide-LA might prove valuable in the preoperative management of catecholamine-secreting tumors. To the best of our knowledge, this is the first report on the successful use of octreotide in a CSCBP.

Learning points

  • The value of octreotide scanning in the localization of extra-adrenal pheochromocytoma.

  • Control of catecholamine secretion using high-dose octreotide.

  • This is a report of a rare cause of secondary diabetes and hypertension.

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M A W Hermans
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B M L Stelten Department of Neurology, Canisius Wilhelmina Hospital, Weg door Jonkerbos 100, 6532SZ, Nijmegen, The Netherlands

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H R Haak
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W W de Herder Department of Internal Medicine, Erasmus University Medical Centre, Gravendijkwal 230, 3015CE, Rotterdam, The Netherlands

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M W Dercksen
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Summary

This paper reports on two patients with a long-standing diagnosis of an ENETS stage IV neuroendocrine tumour (NET) of the small intestine who developed neurological symptoms. The first patient only had bulbar symptoms and tested positive for acetylcholine receptor antibodies. The second patient had more classical symptoms of fatigable diplopia and muscle weakness of the legs, but no detectable antibodies. The diagnosis of paraneoplastical myasthenia gravis (MG) was postulated. Both patients were treated with pyridostigmine for MG and octreotide for the NETs. Interestingly, treatment of the NETs resulted in improvement of myasthenic symptoms. Paraneoplastic MG has been described to occur with certain malignancies, mainly thymoma. Herein, we prove that the association with gastrointestinal NETs, however, rare, is also one to be considered by clinicians dealing with either of these diseases. The pathogenesis has yet to be elucidated.

Learning points

  • NETs are rare malignancies with a wide variety of symptoms.

  • Paraneoplastic MG can occur with various types of malignancies.

  • Herein, we provide evidence of paraneoplastic MG in association with a grade IV NET of the small intestine.

  • Treatment of the NETs resulted in remission of myasthenic symptoms in one patient.

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Ramez Ibrahim Royal Hallamshire Hospital, Sheffield, UK

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Atul Kalhan University Hospital of Wales, Cardiff, UK

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Alistair Lammie Cardiff University, Cardiff, UK

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Christine Kotonya Bronglais Hospital, Aberystwyth, UK

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Ravindra Nannapanenni University Hospital of Wales, Cardiff, UK

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Aled Rees Institute of Molecular and Experimental Medicine, Cardiff University, Cardiff, UK

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Summary

A 30-year-old female presented with a history of secondary amenorrhoea, acromegalic features and progressive visual deterioration. She had elevated serum IGF1 levels and unsuppressed GH levels after an oral glucose tolerance test. Magnetic resonance imaging revealed a heterogeneously enhancing space-occupying lesion with atypical extensive calcification within the sellar and suprasellar areas. Owing to the extent of calcification, the tumour was a surgical challenge. Postoperatively, there was clinical, radiological and biochemical evidence of residual disease, which required treatment with a somatostatin analogue and radiotherapy. Mutational analysis of the aryl hydrocarbon receptor-interacting protein (AIP) gene was negative. This case confirms the relatively rare occurrence of calcification within a pituitary macroadenoma and its associated management problems. The presentation, biochemical, radiological and pathological findings are discussed in the context of the relevant literature.

Learning points

  • Calcification of pituitary tumours is relatively rare.

  • Recognising calcification in pituitary adenomas on preoperative imaging is important in surgical decision-making.

  • Gross total resection can be difficult to achieve in the presence of extensive calcification and dictates further management and follow-up to achieve disease control.

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Suresh Chandran Departments of Neonatology, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

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Fabian Yap Kok Peng Paediatric Endocrinology, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

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Victor Samuel Rajadurai Departments of Neonatology, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

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Yap Te Lu Paediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

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Kenneth T E Chang Children's Pathology, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

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S E Flanagan Institute of Biomedical and Clinical Science, University of Exeter Medical School, Exeter EX2 5DW, UK

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S Ellard Institute of Biomedical and Clinical Science, University of Exeter Medical School, Exeter EX2 5DW, UK

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Khalid Hussain Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK

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Summary

background: Congenital hyperinsulinism (CHI) is a rare genetic disorder characterised by inappropriate insulin secretion in the face of severe hypoglycaemia. There are two histological subtypes of CHI namely diffuse and focal. Diffuse CHI is most common due to recessive mutations in ABCC8/KCNJ11 (which encode the SUR/KIR6.2 components of the pancreatic β-cell KATP channel) whereas focal CHI is due to a paternally inherited ABCC8/KCNJ11 mutation and somatic loss of heterozygosity for the 11p allele inside the focal lesion. Fluorine-18-l-dihydroxyphenylalanine positron emission tomography/computed tomography (18F-DOPA-PET/CT) is used in the pre-operative localisation of focal lesions prior to surgery. Diffuse CHI if medically unresponsive will require a near total pancreatectomy whereas focal CHI will only require a limited lesionectomy, thus curing the patient from the hypoglycaemia.

Aims: To report the first case of genetically confirmed CHI in Singapore from a heterozygous paternally inherited ABCC8 mutation.

Methods/Results: A term male infant presented with severe hyperinsulinaemic hypoglycaemia (HH) after birth and failed medical treatment with diazoxide and octreotide. Genetic testing (paternally inherited mutation in ABCC8/p.D1472N) suggested focal disease, but due to the unavailability of 18F-DOPA-PET/CT to confirm focal disease, a partial pancreatectomy was performed. Interestingly, histology of the resected pancreatic tissue showed changes typical of diffuse disease.

Conclusion: Heterozygous paternally inherited ABCC8/KCNJ11 mutations can lead to diffuse or focal CHI.

Learning points

  • HH is a cause of severe hypoglycaemia in the newborn period.

  • Paternal mutations in ABCC8/KCNJ11 can lead to diffuse or focal disease.

  • 18F-DOPA-PET/CT scan is the current imaging of choice for localising focal lesions.

  • Gallium-68 tetra-aza-cyclododecane-N NNN-‴-tetra-acetate octreotate PET scan is not a useful imaging tool for localising focal lesions.

  • The molecular mechanism by which a heterozygous ABCC8 mutation leads to diffuse disease is currently unclear.

  • Focal lesions are curable by lesionectomy and so genetic studies in patients with HH must be followed by imaging using 18F-DOPA-PET/CT scan.

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F Serra Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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S Duarte Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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S Abreu Department of Endocrinology, Hospital Central do Funchal, Funchal, Portugal

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C Marques Departments of Neurosurgery

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J Cassis Pathology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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M Saraiva Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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Summary

Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature.

Learning points

  • Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies.

  • Most paraganglioma of the head and neck are not hormonally active.

  • Nasal paraganglioma, especially ACTH producing, is a very rare tumour.

Open access