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21st European Congress of Endocrinology

Lyon, France
18 May 2019 - 21 May 2019

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18-21 May 2019, Lyon, France

Poster Presentations

Adrenal and Neuroendocrine Tumours 2

ea0063p407 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

ACTH-dependent hypercortisolism: always follow your nose

Decaestecker Karen , Wijtvliet Veerle , Coremans Peter , Doninck Nike Van

A 41-year-old woman presented with a puffy face since five months. She experienced alopecia, hirsutism, easy bruisability, amenorrhea and proximal muscle weakness. Clinical examination revealed a moonface, centripetal obesity, proximal muscle atrophy, thinned scalp hair, hyperpigmentation in sun-exposed neck region, ecchymosis and arterial hypertension grade 1. Blood analysis showed elevated morning cortisol, elevated morning ACTH of 66.1 pg/ml (normal 10–60), hypokalemic...

ea0063p408 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Metyrapone test in secondary adrenal insufficiency – is ACTH measurement alone sufficient for a proper diagnosis?

Papierska Lucyna , Glinicki Piotr , Nowak Karolina , Łebek-Szatańska Agnieszka , Rdzanek Monika , Rabijewski Michał , Zgliczyński Wojciech

In secondary adrenal insufficiency (SAI), the Synacthen test can give a false negative result. If disease is strongly suspected, an insulin hypoglycaemia test or Metyrapone (Metopirone) test should be carried out. Insulin test could be hazardous for patients with adrenal insufficiency (due to high risk of severe hypoglycaemia), so in our clinic we use overnight single-dose Metyrapone test. Although this test is easy to perform, measurement of 11-deoxycortisol concentration is ...

ea0063p409 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Diagnostic accuracy of the aldosterone to active renin ratio in detecting primary aldosteronism: the graz endocrine causes of hypertension (GECOH) study

Pilz Stefan , Keppel Martin , Trummer Christian , Theiler-Schwetz Verena , Pandis Marlene , Borzan Valentin , Pittrof Matthias , Obermayer-Pietsch Barbara , Grubler Martin R , Verheyen Nicolas , Stepan Vinzenz , Meinitzer Andreas , Voelkl Jakob , Marz Winfried , Tomaschitz Andreas

Context: The aldosterone to active renin ratio (AARR) is the recommended screening test for primary aldosteronism (PA), but prospective study data on its sensitivity and specificity are sparse.Objective: We investigated the diagnostic accuracy of the AARR for detecting PA.Design: This is a prospective diagnostic accuracy study.Setting: This study was conducted from February 2009 to August 2015 at the outpatie...

ea0063p410 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Large adrenal incidentalomas require a dedicated diagnostic procedure

Amodru Vincent , Taieb David , Guerin Carole , Paladino Nuncia Cinzia , Brue Thierry , Sebag Frederic , Castinetti Frederic

Introduction: The management of large non secreting adrenal tumors (at least 4 cm) is still a matter of debate as it is unclear whether imaging, especially 18F-FDG, can be used to characterize their potential malignancy. Moreover, the risk of new hypersecretion in non-operated tumors is uncertain. Our aim was to better characterize these large adrenal incidentalomas.Methods: Patients followed in our Center for a non-secreting large (at least 4 cm) adrena...

ea0063p411 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Necessity of educational programs for healthcare givers in preclinical settings of acute adrenal insufficiency in patients with chronic hypocortisolism

Dolle Michael , Dolle Rodica Mia , Schneidewind Sabine , Manns Michael Peter , Terkamp Christoph , Leitolf Holger , Zender Steffen

Adrenal crisis is a life-threatening complication in patients with adrenal insufficiency. In order to prevent critical situations, patients are supposed to increase their glucocorticoid dose in distressing situations. If dose adjustment is not sufficient, sooner or later patients will fall into a coma, followed by death. While health condition worsens patients usually call paramedics, followed by a decision for primary therapy and transportation to the nearest hospital. We ass...

ea0063p412 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Challenges in diagnosing and treating a glucagon secreting tumor – case report

Bojoga Irina , Enache Oana , Parfeni Ovidiu , Mihai Mara Mădălina , Giurcăneanu Călin , Martin Sorina , Nit¸ipir Cornelia , Fica Simona

Introduction: Glucagonoma is a rare pancreatic neuroendocrine functional tumor, with an estimated incidence of 1 in 20 million people. At the moment of diagnosis, the majority of patients present locoregional and liver metastases.Case report: We present the case of a 46 years old male patient, diagnosed with glucagon secreting pancreatic neuroendocrine tumor in February 2018. As part of the glucagonoma syndrome, the patient was also suffering from insuli...

ea0063p413 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Phenotypic variability within a family with multiple endocrine neoplasia type 1 (MEN1) syndrome

Ahmad Tariq , Sharma Jayant , Crowley Rachel , Kyithar Ma Pyeh

MEN1 is an autosomal dominant endocrine tumour syndrome, caused by inactivating mutations of the MEN1 tumour suppressor gene at 11q13 locus and characterised by occurrence of hyperparathyroidism, pancreatic tumours and pituitary adenomas. We describe a family with MEN1 syndrome. The index case was a 71-year-old man, referred to Endocrinology clinic in Midland Regional Hospital Portlaoise for hypercalcemia (calcium 3 mmol/l, phosphate 0.5 mmol/l, PTH 350 pg/ml, creatin...

ea0063p414 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

High levels of chromogranin A in connection with proton pump inhibitors

Zavrsnik Matej

72 years old patient was send to endocrinological outpatients clinic with suspect to nevroendocrine tumor (NET) with chromogranin A (CgA) level 379 μg/l (range 19.4–98.1). She described abdominal colic and diarrhoea lasting 2–3 hours after meals accompanied by dizziness, drenching sweat and lower blood pressure for the last 2 years. Comorbidities: arterial hypertension, chronic gastritis, heart failure and osteoporosis. Medications: omeprazole, losartan, pravast...

ea0063p415 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A case of pheochromocytoma left untreated for twelve years: Role of the endocrinologist

Caric Bojana , Stankovic Valentina Soldat , Kordic Ozren , Beric Predrag

Pheochromocytomas are rare chromafin, catecholamine-producing neuroendocrine tumors that arise from the adrenal medulla. Nearly half of these tumors are presented as an unexplained death and autopsy reports indicate higher incidence than it is proposed. We report the case of patient with pheochromocytoma treated only with minimal dose prazosin for twelve years. The operation was cancelled twice, on the day of surgery because the patient was not prepared with alpha-blockers and...

ea0063p416 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

High prevalence of autonomous aldosterone secretion in patients with type 2 Diabetes Mellitus and arterial hypertension. The effect of SGLT2 inhibitors on renin-angiotensin-aldosterone system

Tyfoxylou Ernestini , Papanastasiou Labrini , Piaditis George , Voulgaris Nikolaos , Chrousos George , Kounadi Theodora

Introduction: The causes of arterial hypertension in diabetic and non-diabetic hypertensive patients are still not clear. However, in recent studies in non-diabetic hypertensive patients (NDHP) we observed dysregulation of aldosterone (ALD) secretion either in the form of autonomous secretion or as hyperesponse to stress. To the best of our knowledge, a similar study in hypertensive patients with type 2 Diabetes Mellitus (DHP) has not been conducted yet....

ea0063p417 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Extreme improvement in metabolic status with remarkable weight loss of 64 kg after adrenalectomy due to Cushing syndrome, and development of rheumatoid arthritis eight months later

Obradovic Angelina , Nemet Tucic Klara

A 48-year - old woman was admitted in september 2015 for Cushing clinical sings obesity, weight was 120 kg, BMI 44,4 kg/m2, arterial hypertension 160/120 mmHg, hyperlipidemia, moon face, purplish abdominal strie, amenorrhoea, marked swelling of bouth lags, more right leg with varices. Because high D dimer and factor VIII there was suspicion of deep venous thrombosis, Doppler of veins was done immediately wich excludes deep vein thrombosis, and as part of preoperativ...

ea0063p418 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Contribution of morphological and functional liver imaging to differentiate liver metastases of adrenocortical carcinoma from those of neuroendocrine tumors in a woman with a final diagnosis of multiple endocrine neoplasia type 1

Cirederf Ingrid , Paepegaey Anne-Cecile , Glaude Eddy , Belia Lyonel , Diedhiou Abdoulaye , Libe Rossella , Velayoudom Fritz-Line

A 44-year-old woman was admitted for diabetic ketoacidosis and severe hypertension. She had aggressive clinical features related to Cushing’s syndrome (CS). ACTH-independent CS was diagnosed based on undetectable ACTH (0.65 pmol/L) and unsuppressed cortisol levels by dexamethasone (6363 nmol/L). Adrenal contrast-enhanced computed tomography (CT) scans showed a 15x12x20 cm heterogeneous mass of the left adrenal that pushed back the spleen and the left kidney with thrombosi...

ea0063p419 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Prolonged hypoaldosteronism after unilateral adrenalectomy for primary aldosteronism: a report of two cases

Ming Loh Lih , Bingfeng Chuah Matthew , Chandran Suresh Rama

Introduction: Prolonged hypoaldosteronism is an uncommon complication following unilateral adrenalectomy for primary aldosteronism. There are only 5 case reports in literature. However, a retrospective series involving 110 patients found that it occurred in 5% of patients. We present our experience with two patients.Case presentation: Patient 1 was a 51-year-old male presenting with a 7-year history of difficult-to-manage hypertension. He was intolerant ...

ea0063p420 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Premature ovarian failure in a patient with schmidt syndrome

Valea Ana , Moldovan Cristina , Hritiuc Andreea , Agachi Irina , Carsote Mara , Emanuela Georgescu Carmen

Introduction: Premature ovarian failure (POF) may be a part of autoimmune polyglandular syndromes that involves multiple endocrine and systemic conditions due to autoimmunity.Material and methods: We aim to present the case of a young women diagnosed with POF and Schmidt syndrome. The patient was followed in different tertiary centers of endocrinology. The informed consent was obtained.Case report: A 31-year old patient with no pri...

ea0063p421 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

RET Y791F carriers’ medical history – experience from one center

Hasse-Lazar Kornelia , Kotecka-Blicharz Agnieszka , Oczko-Wojciechowska Małgorzata , Pawlaczek Agnieszka , Michalik Barbara , Gawlik Tomasz , Jurecka-Lubieniecka Beata , Krajewska Jolanta , Krol Aleksandra , Legutko Marta , Czarniecka Agnieszka , Zeman Marcin , Jarzęb Barbara

Introduction: Germline mutations of RET oncogene result in development of multiple endocrine neoplasia type 2 (MEN 2). There is a strong correlation between type of the RET sequence changes and the aggressiveness of main syndrome feature, medullary thyroid carcinoma (MTC), and the incidence of remaining manifestations, mainly pheochromocytoma (PHEO). For many of the RET germline mutations, the clinical risk have been precisely defined, but there are ...

ea0063p422 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Association between new anthropometric parameters and new inflammation indicators in non-functioning adrenal incidentalomas

Kowalska Joanna , Zieleń-Zynek Iwona , Nowak Justyna , Zubelewicz-Szkodzińska Barbara

Introduction: Studies demonstrate that non-functioning adrenal incidentalomas are assosiated with increased risk of diabetes mellitus and cardiovascular diseases. Chronic inflammation plays key role in developing type 2 diabetes, as well as cardiovascular diseases.Aim: The purpose of the study was to evaluate new anthropometric parameters (BAI, LAP, VAI, BRI, ABSI, RFM) and its relationship with inflammation indicators (PLR, MPVLR, SII) among patients wi...

ea0063p423 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Comparison of visualization methods in ACTH-ectopic lung tumor: a case report

Petrushkina Alexandra , Pigarova Ekaterina , Dzeranova Larisa , Rumyantsev Pavel , Slaschuk Konstantin , Sheremeta Marina , Melnichenko Galina

Background: ACTH-ectopic syndrome is a rare cause of endogenous hypercortisolism and may pose serious difficulties in topical diagnosis.Case description: Clinical features of hypercortisolism manifested at the age of 18 years (2012). A year later he was referred to an endocrinologist: ACTH 113 pg/ml (0–46), cortisol in 24-h urine 2915 μg/day (4.3–176), 1 mg DX test cortisol 1020 nmol/l, 8 mg DX test 764 nmol/l, intact pituitary MRI with co...

ea0063p424 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Paraneoplastic Cushing’s syndrome related to recurrence of a malignant ovarian teratoma

Jemel Manel , Kandara Hajer , Mimita Wafa , Ben Jemaa Marwa , Jemni Houda , Kammoun Ines

A 38-year-old woman presented with symptoms suggesting Cushing’s syndrome. She has a history of surgery of mature ovarian teratoma (hysterectomy, ovarectomy) associated to radio and chemotherapy 15 years ago. The malignant teratoma relaps with hepatic and grelic metastases, and was unresectable. Main complaints were weight gain with centripetal fat distribution, muscle weakness, melanodermia and purpule striae on the skin of the abdomen, thighs, breasts and arms....

ea0063p425 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Insulinoma: a case series of a tertiary care center

Jemel Manel , Jemel Manel , Kandara Hajer , Adel Meriem , El Guich Dorra , Jemni Houda , Kammoun Ines

Introduction: Insulinoma is rare tumor with an incidence of 1 in 250,000 patient-years. It presents with repeated episodes of hypoglycemia due to endogenous hyperinsulinemia, which occurs mostly in the fasting state. Insulinomas are usually sporadic, solitary, benign and encapsulated small lesions and majority of them measure <2 cm in diameter. They pose a challenge for pre-operative localization.Materials and methods: A retrospective study of patien...

ea0063p426 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Validation of 24-hour human adrenal steroid rhythms measured by ULTRADIAN interstitial automated microdialysis: Comparison with plasma

Upton Thomas , Methlie Paal , Russell Georgina , Henne Nina , Tsagarakis Stelios , Kampe Olle , Lightman Stafford , Husebye Eystein

Background: Hormones oscillate in circadian and ultradian rhythms. Consequently, single time point measurements are very difficult to interpret. To address this, we developed an automated system of 24-hour ambulatory microdialysis. This allows measurement of free hormone concentrations in subcutaneous interstitial fluid collected while participants continue normal daily activities. To validate the technique for adrenal steroid hormones, we simultaneously sampled interstitial f...

ea0063p427 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Genetic mutations in bladder paragangliomas - not just SDHB-related disease

Anandappa Samantha , Breen Louise , Thurairaja Ramesh , Christodoulou Dimitra , Jacques Audrey , Velusamy Anand , McGowan Barbara , Izatt Louise , Carroll Paul

Bladder Paragangliomas (PGLs) are rare forms of neuroendocrine tumours arising from sympathetic paraganglionic tissue. They account for <1% of all Pheochromocytomas and Paragangliomas (PPGLs) and < 0.06% of all bladder tumours. All patients with PPGLs are recommended to be considered for genetic testing as ~ 40% of PPGLs are associated with a germline mutation, even if there is no prior family history of disease. Identifying an inherited PPGL predisposition has importa...

ea0063p428 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

PAI-BEL: the Belgian registry on Primary Adrenal Insufficiency

Driessens Natacha , Corvilain Bernard , De Block Christophe , T'Sjoen Guy , Nobels Frank , Ghys Christophe , Vroonen Laurent , Maiter Dominique

Introduction: Primary Adrenal Insufficiency (PAI or Addison’s disease) is a rare disease with an increasing prevalence that may be complicated by life-threatening acute adrenal crisis. Valid epidemiological data are difficult to obtain. In Belgium, a national PAI registry had not been established yet.Objective: We collected epidemiological and clinical data in a large cohort of adult patients with a known Addison’s disease to have a better know...

ea0063p429 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Urinary steroid profiling by gas chromatography-mass spectrometry (GC-MS) in Cushing’s syndrome

Bonnet-Serrano Fideline , Vaczlavik Anna , Assie Guillaume , Bertherat Jerome , Menet Marie-Claude

Introduction: Steroid profiling by mass spectrometry approaches consists in the simultaneous measurement of several steroid molecules in a biological sample, allowing an optimal characterization of steroidogenesis alterations, particularly in the context of adrenal tumors. Twenty-four hours urine samples have the advantage of being non-invasive and of giving an integrated view of steroidogenesis. Urinary steroid profiling has thus been shown to be particularly useful in the di...

ea0063p430 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Efficacy of DHEA in improving Quality of Life in prolonged HPA axis suppression from exogenous steroids

Anandappa Samantha , Kumar Rakshit , Breen Louise , Thakali Sonu , Tam Maria , Magtoto Cynthia , Carroll Paul , Velusamy Anand

Dehydroepiandrosterone (DHEA) has been a subject of controversy for more than a decade being called ‘a miracle hormone’, the ‘elixir of life’ and ‘an anti-aging supplement’. However, recent literature has proven mild to moderate benefit in selected group of patients. Data is mostly limited to primary adrenal failure patients with adequate glucocorticoid and mineralocorticoid replacement and in hypopituitarism from variable aetiology associated wit...

ea0063p431 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Residual adrenocortical function in autoimmune addison’s disease: interim results of a cross-sectional study

Saevik AEse Bjorvatn , AEkermann Anna-Karin , Methlie Paal , Gunnar Nedrebo Bjorn , Dahle Anne Lise , Carlsen Siri , Tomkowicz Aneta , Holte Synnove Emblem , Debowska Aleksandra , Sollid Stina Therese , Jorgensen Anders , Nermone Ingrid , Gronning Kaja , Finnes Trine , Grimnes Guri , Hoybye Charlotte , Skov Jakob , Dahlqvist Per , Wahlberg Jeanette , Isaksson Magnus , Quinkler Marcus , Bensing Sophie , Oksnes Marianne , Husebye Eystein

Background: Current dogma states that in autoimmune Addison’s disease (AAD), all adrenocortical function eventually is lost. Yet growing evidence suggests that a subgroup of patients retain some self-production.Aim: To explore whether residual production of adrenocortical steroids is present in a subgroup of AAD patients.Material and methods: In an open non-randomized cross-sectional study, an interim of 22 AAD patients delive...

ea0063p432 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Case report: giant adrenal myelolipoma in CAH – should all patients be screened by ultrasound for adrenal masses?

Mondorf Antonia , Flugel Anna Katharina , Meyer Gesine , Bojunga Jorg

Introduction: Myelolipomas account for 5–15% of all resected adrenal masses and are considered as benign lesions consisting of fat and myeloid tissue. Their pathogenesis is mostly unknown, though altered mesenchymal stem cell function or a sustained respond to increased ACTH stimulation are discussed. Even though these findings are partly inconclusive, an increased incidence of myelolipoma in patients with congenital adrenal hyperplasia (CAH) has been reported, which may ...

ea0063p433 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Molecular events in a large series of advanced stage III-IV adrenocortical cancer: looking for new therapeutic options

De Martino Maria Cristina , Lacroix Ludovic , Aubert Sebastien , Libe Rossella , Al Ghuzlan Abir , de la Fouchardiere Christelle , Hescot Segolene , Assie Guillaume , Honore Aurelie , Deschamps Fred , Lombes Marc , Borson-Chazot Francoise , Pattou Francois , Pivonello Rosario , Borget Isabelle , Schlumberger Martin , Leboulleux Sophie , Scoazec Jean-Yves , Bertherat Jerome , Cao Christine Do , Baudin Eric

Adrenocortical cancer (ACC) is a rare cancer with poor prognosis and scant treatment options.Purpose: To look for new therapeutic approaches issued from the screening for common genetic variants in a large series of advanced ACC.Experimental design: Whole exome sequencing have been performed in 10 advanced (stage III and IV) ACC samples to identify the recurrent variants. The presence and the frequency of most interesting variants ...

ea0063p434 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A severe ACTH-dependent Cushing syndrome associated to a possible concomitant primary adrenal-dependent hypercortisolism: a challenging case

Paragliola Rosa Maria , Corsello Andrea , Cese Francesca Delle , Papi Giampaolo , Pontecorvi Alfredo , Corsello Salvatore Maria

Background: Cushing syndrome (CS) represents a challenging disease. The major difficulty is to distinguish between the two main etiologies of ACTH-dependent hypercortisolism. However, in rare cases, the concomitant presence of both an ACTH-dependent and a primary adrenal disease should be considered.Case report: We evaluated a 67-year-old woman for a severe ACTH-dependent CS (midnight serum cortisol 332 ng/ml; urinary free cortisol [UFC] 3000 mcg/day; co...

ea0063p435 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

One-hour glucose level of the oral glucose tolerance test: the new valuable indicator of insulin resistance in patients with adrenal incidentalomas

Sojat Antoan Stefan , Marina Ljiljana , Ivovic Miomira , Gajic Milina Tancic , Arizanovic Zorana , Milin-Lazovic Jelena , Pandurevic Srdjan , Kendereski Aleksandra , Vujovic Svetlana

One-hour (1 h) glucose level of the oral glucose tolerance test (OGTT) was proven to be a better predictor of insulin resistance (IR) in nondiabetic population than the two-hour (2 h) postload glucose level. Specifically, the 8.6 mmol/l cut-off was shown to be an early marker of impaired glucose tolerance (IGT). Previous studies have shown that nondiabetic patients with adrenal incidentalomas (AI) have higher IR than healthy control (HC). However, despite the subtle cortisol s...

ea0063p436 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Measuring steroids hormones by liquid chromatography–tandem mass spectrometry in 21-hydroxylase deficiency (21OHD) reveal large interindividual differences in hormone levels amongst patient with the same genetic mutation

Thorsby Per Medboe , Dahl Sandra R. , Bronstad Ingeborg , Lovas Kristian , Nermoen Ingrid

Background: We have previously shown that LC-MS/MS analyses of steroid hormones are superior to immunoassays monitoring 21OHD, due to better specificity and the possibility to multiplexing several steroid hormones in the same assay. This may lead to better understanding of the individual steroid hormone profile in patients with CAH. Here we present four patients cases with the same genotype in the CYP21A2 gene, and evaluate their steroid profile by LC-MS/MS analyses.<...

ea0063p437 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Hypoadrenalism in Advanced HIV

Ross Ian , Millar Robert , Mofokeng Thabiso , Grossman Ashley , Dave Joel , Levitt Naomi , Pillay Tahir , Erasmus Rajiv , Raubenheimer Peter , Dandara Collet , Johannsson Gudmundur

Background: Large populations of HIV and tuberculosis occur in South Africa and, amongst these patients, Addison’s disease is probably underdiagnosed. Preliminary data in 60 HIV-positive patients with a CD4 count less than 100 cells/mm3 showed that the overall prevalence of hypoadrenalism was 6.7%, with 1 patient having primary hypoadrenalism and 3 patients having central hypoadrenalism. This report describes the prevalence of hypoadrenalism and its association...

ea0063p438 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A case of late recognition of the malignant cause of chronic pancreatitis

Christidis Grigorios , Garaiman Alexandru , Holz Robert , Krawczyk Marcin , Lammert Frank

Pancreatic neuroendocrine tumors constitute a disease with steadily increasing prevalence worldwide in part owing to the increased detection of early stage disease. Here we present the case of a 49 year old male patient, who was admitted with clinical, laboratory and CT features of acute pancreatitis. A clear cause for the pancreatitis could not be detected. Excessive alcohol intake was reliably denied, and the patient did not take any drugs. Gallbladder and bile ducts were in...

ea0063p439 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Imaging studies of a parathyroid carcinoma case

Nguyen Thi Ngoc Huyen , Mathey Celine , Sitoris Georgiana , Kleynen Pierre

Parathyroid carcinoma is a rare neuroendocrine tumor, responsible for less than 1% of all cases of primary hyperparathyroidism. The sole curative treatment is complete surgical removal and thus, an accurate preoperative diagnosis is necessary. Due to the rarity of this disease, there is no staging guidelines. Imaging of parathyroid glands is usually obtained with ultrasonography, computed tomography, scintigraphy and positron emission tomography. These imaging modalities have ...

ea0063p440 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Neuroendocrine neoplasms (NEN) arising in uncommon sites: epidemiological and clinical features

Modica Roberta , de Cicco Federica , Bottiglieri Filomena , Di Maio Silvana , Altieri Barbara , Rainone Carmen , Faggiano Antongiulio , Colao Annamaria

Background and aim: Neuroendocrine neoplasms (NEN) show increasing incidence and varying biology, but epidemiological and clinical data for other than gastroenteropancreatic (GEP) and thoracic NEN are scattered. Furthermore the best therapeutic approach in NEN arising in uncommon sites is still debated. Aim of this study is to assess the epidemiological and clinical features of NEN arising in uncommon sites in a monocentric series of a referral center.Ma...

ea0063p441 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Microwave ablation of the adrenal gland for treatment of functioning adrenocortical tumours: porcine in vivo study

Donlon Padraig , Fallahi Hojjatollah , Beard Warren Beard , Shazad Atif , Heflin Lindsay , Cox Whitney , Bloomberg Brooke , Lillich James , Ganta Chanran , O'Sullivan Gerard , Ruvio Giuseppi , O'Shea Paula , O'Halloran Martin , Prakash Punit , Dennedy Michael

Objective: To evaluate MTA as a precision ablation methodology for adrenocortical tissue, simultaneously evaluating effects on adjacent non-targeted tissue.Methodology: A directional MTA applicator was used in vivo on adrenals of 8 male pigs: (i) sham (n=2); (ii) 45 watts for 60 seconds (n=3); (iii) 70 watts for 60 seconds (n=3). Blood was drawn intraprocedurally and at 48h for measurement of metanephrines, cortisol, A...

ea0063p442 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Simplified model for diagnosis of secondary adrenal insufficiency using non-fasting cortisol and clinical parameters

Kim Tae Ho , Oh Jung Kyun , Kim Hyun Mok , Beom Sun Hee

Objective: Although rapid adrenocorticotrophic hormone (ACTH) stimulation test is a standard test for primary and secondary adrenal insufficiency (AI), it is considered a costly and time-consuming method due to sampling three times in an hour. We aimed to develop a simpler diagnostic model of secondary AI using non-fasting basal cortisol and clinical parameters related to AI such as history of exogenous steroid use or hyponatremia.Methods: Total 430 pati...

ea0063p443 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Type 1 gastric neuroendocrine tumor study in Hospital Clínico San Carlos (HCSC), Madrid

Ramos Elvira , Barrio Elvira , Vargas-Zuniga Ignacio , Pallares Raquel , Pazos Mario , Suarez Pablo , Aldama Paula , Sevilla Concepcion , De Miguel Paz , Diaz Jose Angel

Introduction: Type 1 Gastric neuroendocrine tumors account for 70 to 80 percent of all gastric neuroendocrine tumors (G-NETs) and they are found more commonly in older adults, particularly women. They are associated with autoimmune metaplastic atrophic gastritis (AMAG) with or without pernicious anemia. They are usually smaller than 1 cm and often multiple. Our goal is to describe different characteristics of type 1 G-NETs and to study possible prognostic factors related to th...

ea0063p444 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

The Arg244His missense mutation in SDHB-1 leads to altered metabolism in Caenorhabditis elegans: a new disease model

Saskoi Eva , Hujber Zoltan , Liko Istvan , Meszaros Katalin , Sarkadi Balazs , Matyasi Barbara , Kovacs Attila L , Patthy Laszlo , Nyiro Gabor , Svab Gergely , Tretter Laszlo , Mehta Anil , Sebestyen Anna , Patocs Attila , Takacs-Vellai Krisztina

The succinate dehydrogenase (SDH) enzyme complex consisting of four subunits (SDHA, SDHB, SDHC and SDHD) has a dual function in the process of mitochondrial energy generation. It converts succinate to fumarate as part of the TCA cycle and also transfers electrons to ubiquinone as part of the electron transport chain. Mutation in any subunit of the enzyme complex increases the risk for the development of neuroendocrine tumors including paraganglioma (PGL) and pheochromocytoma (...

ea0063p445 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

11C-Metomidate PET/CT Identifies Unilateral Primary Aldosteronism in a Multi-ethnic Cohort

Puar Troy , Tan Colin , Tong Aaron , Zhang Meifen , Khoo Chin Meng , Tan Alvin , Htoo Htoo , Reilhac Anthonin , Weekes Ashley , Robins Ed , Foo Roger

Introduction: Patients with unilateral primary aldosteronism (PA) can be cured with adrenalectomy, and adrenal vein sampling (AVS) remains the gold-standard test. However, AVS is invasive, technically-challenging and criteria for determining lateralization differ between centres. 11C-Metomidate PET/CT (MTO) imaging offers a promising non-invasive alternative for identifying unilateral PA.Methods: All patients with confirmed primary aldosteronism who were...

ea0063p446 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Prevalence of non-carcinoid syndrome (non-CS) diarrhoea in patients with neuroendocrine tumours (NETs): a systematic literature review (SLR)

Khan Mohid S , Walter Thomas , Buchanan-Hughes Amy , Keeber Lucie , Feuilly Marion , Grande Enrique

Background: Approximately 20% of NET patients develop CS, characterised by diarrhoea and flushing. However, CS is not the only cause of diarrhoea among NET patients. Non-CS causes should be considered to allow for appropriate management. We investigated the reported occurrence of diarrhoea from various non-CS causes in patients with gastroenteropancreatic NETs (GEP-NETs), to explore the need for differential diagnosis (DDx) of NET diarrhoea.Methods: MEDL...

ea0063p447 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Down-regulation of the Ataxia Telangiectasia Mutated Gene (ATM) is associated with increased metastatic potential and decreased overal survival in patients diagnosed with gastroenteropancreatic neuroendocrine tumors (GEP-NETs)

Katalinic Darko , Aleric Ivan , Vcev Aleksandar , Mirat Jure , Soerensen Lilly , Bildat Stephan

Introduction: Neuroendocrine tumors (NETs) are rare tumours that develop in cells of the neuroendocrine system. They comprise a heterogeneous group of neoplasms that range from the benign and multi-focal to the highly malignant and metastatic. Here we conduct itegrated genome and immunohistochemistry analysis (IHC) of the Ataxia Telangiectasia Mutated Gene (ATM) as well as analysis of overal survival in patients diagnosed with NETs.Material and Methods: ...

ea0063p448 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

GEP-NET tumors as the first geno-phenotypic association in MEN1 syndrome – the possibilities for a European MEN1 network

Kovesdi Annamaria , Grolmusz Vince Konrel , Patocs Attila

Objective: Phenotypic variability and lack of genotype-phenotype correlations still represent a major challenge in the surveillance of patients carrying germline MEN1 (Multiple Endocrine Neoplasia type 1) mutations. MEN1 associated gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are highly penetrant and show an indolent course. However, they are still the leading cause of death in MEN1 syndrome. Some single-center and national studies reported higher prevalence of GEP-...

ea0063p449 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Prevalence of neuroendocrine tumors in a subgroup of young patients: single center experience

Isailovic Tatjana , Macut Duro , Popovic Bojana , Ognjanovic Sanja , Kovacevic Valentina Elezovic , Milicevic Ivana , Antic Ivana Bozic , Bogavac Tamara , Ilic Dusan , Damjanovic Svetozar

Neuroendocrine tumors encompass a heterogeneous group of tumors arising from the diffuse neuroendocrine system. The incidence of NETs rises with age, with the peak after 65 years. However, all NETs observed in adults may be diagnosed in children too. There are a very few studies addressing NETs in young patients. This study summarizes clinical, histopatological and genetic characteristics of young patients with NETs. This is a retrospective study describing clinical, histopath...

ea0063p450 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Prognostic role of angiogenesis-associated factors Tie2 and Angiopoietin 2 in patients with neuroendocrine neoplasms: The NETTARE Unit experience

Puliani Giulia , Sesti Franz , Feola Tiziana , Sbardella Emilia , Cozzolino Alessia , Pofi Riccardo , Faggiano Antongiulio , Lenzi Andrea , Anastasi Emanuela , Venneri Mary A , Giannetta Elisa , Isidori Andrea M

Introduction: The role of Angiopoietin (Ang)/Tie2 kinase pathway in neuroendocrine neoplasms (NENs) has recently received increasing attention, but its clinical role remains unclear. Tie2-receptor inactivation favours chronic inflammation. The balance between its agonist and antagonist regulates its signalling. Aim of this study was to measure serum soluble Tie2 (sTie2) and Ang-2 in patients followed at the NETTARE Unit (NeuroEndocrine TAsk foRcE of ‘Sapienza’ Univer...

ea0063p451 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Attributes valued by patients, health-care practitioners (HCPs) and caregivers using pre-filled delivery systems: a systematic literature review

Thanh Xuan-Mai Truong , Rohban Thomas , Ribeiro-Oliveira Jr Antonio

Background: The development of a new syringe for lanreotide Autogel, in conjunction with patients, caregivers and HCPs, identified and integrated several upgrades to the previous design that could improve patient care.Aims: To better understand which attributes are pertinent to users, and how user preferences can be optimally assessed, we conducted a systematic literature review of relevant studies of pre-filled devices.Methods: Pu...

ea0063p452 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Identification of a molecular signature of hypercortisolism by whole blood methylome analysis

Armignacco Roberta , Septier Amandine , Gaspar Cassandra , Jouinot Anne , Neou Mario , Perlemoine Karine , Zennaro Maria Christina , Reincke Martin , Bertherat Jerome , Beuschlein Felix , Assie Guillaume

The prolonged exposure to an excess of circulating cortisol (Cushing’s syndrome) causes various complications. An accurate and early diagnosis is critical for effective surgical management and optimal prognosis. However, the current diagnostic approach based on hormonal assays can be complex and requires multiple tests. The identification of novel, specific and easily measurable biomarkers of hypercortisolism may help to improve the diagnosis and to evaluate the complicat...

ea0063p453 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Risk factors for gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs): a case-control study from NETTARE Unit

Feola Tiziana , Sesti Franz , Puliani Giulia , Sbardella Emilia , Cozzolino Alessia , Lenzi Andrea , Faggiano Antongiulio , Isidori Andrea M , Giannetta Elisa

Introduction: GEP-NENs represent a heterogeneous group of neoplasms with increasing incidence in the last years. Pathogenesis and risk factors of sporadic GEP-NENs are not clear and still debated. The aim of this study is to evaluate the main risk factors for sporadic GEP-NENs in patients followed by the NETTARE Unit (NeuroEndocrine TAsk force of of ‘Sapienza’ University of Rome).Methods: We performed a case-control study including 32 consecuti...

ea0063p454 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Selective arterial calcium stimulation test in two cases with occult insulinoma

Ahmadova Konul , Tam Abbas Ali , Topaloglu Oya , Akkan Koray , Bostancı Birol , Ersoy Reyhan , Cakir Bekir

Introduction: 80–90% of insulinomas are smaller than 2 cm, equally located in the head, body and tail of pancreas. Computer tomography (CT) is 75%, magnetic resonance imaging (MRI) is 55–90%, endoscopic ultrasonography (EUS) is 85–95% and selective arterial calcium stimulation test (SACST) is 95–100% sensitive in the diagnosis of insulinomas. Here, we presented two cases of insulinoma which could not be located by conventional methods and evaluated with SAC...

ea0063p455 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Can pancreatic tumor in von Hippel-Lindau syndrome be a prognostic factor?– a case study

Zwolak Agnieszka , Tywanek Ewa , Świrska Joanna , Dudzińska Marta , Tarach Jerzy

Introduction: Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by formation of tumors and cysts in various organs. Renal cancer and central nervous system angiomas (whose frequencies in VHL disease are estimated for 70% and 76% respectively) are considered main prognostic factors, with renal cancer being the most common cause of death. Neuroendocrine tumors of the pancreas occur in only 10 to 20% of VHL patients and are benign in the majority of ...

ea0063p456 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

An investigation into the role of parathyroid hormone in the regulation of aldosterone secretion in hypertensive cardiovascular patients

Jannat Saba , Akram Maleeha , Jahan Sarwat , Kazmi Ali Raza , Afshan Kiran , Tahir Fahim , Naseem Afzaal Ahmed , Qayyum Mazhar , Rafi Muhammad , Rizvi Syed Shakeel Raza

Hypertension or elevated blood pressure (BP) is quantitatively the most important risk factor for development of cardiovascular diseases (CVDs), whereas aldosterone contributes significantly in the development and severity of hypertension. Interestingly, parathyroid hormone (PTH) stimulates aldosterone synthesis by regulating renin-angiotensin-aldosterone system (RAAS). Conversely, RAAS controls PTH secretion, as angiotensin receptor is expressed by human parathyroid tissues a...

ea0063p457 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Plasma levels and regulation by the hypothalamic–pituitary–adrenal axis of Androst-5-ene-3β,7α/β,17β-triols in human

Pospišilova Hana , Hill Martin , Kolatorova Lucie , Duškova Michaela , Starka Luboslav

Dehydroepiandrosterone is the most abundant steroid hormone circulating in the human body, we focused on its 7-hydroxylated derivatives. Many observations in rodents have demonstrated the anti-inflammatory and immune modulating activity of 7β-hydroxy-androst-5-enes, and on the basis of these experiments androst-5-ene-3β,7β,17β-triol is considered as a potential agent in the treatment of autoimmune diseases. In contrast to the fairly abundant information on ...

ea0063p458 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A case of non metastatic pheochromocytoma in a patient with multiple sclerosis

Lio Serafino , Taschin Elisa , Schiavi Francesca

Pheochromocytoma is a rare neuroendocrine tumor; many cases are sporadic but 1/3 are familial or syndromic and associated with many susceptibility genes including germline mutations of the gene encoding succinate dehydrogenase (SDH) subunits. We describe the case of a 70-years-old woman with arterial hypertension poorly controlled by therapy in a patient with secondary progressive multiple sclerosis started at 25 years of age, associated with euthyroid autoimmune thyroiditis, ...

ea0063p459 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Bilateral gynecomastia revealing a secreting adrenal adrenoma on a 5-year-old boy

Kebaili Raoudha , Hasni Yosra , Belgacem Hajer Ben , Alilech Sana , Hassan Samia Ben , Tej Amel , Eleuch Koussay , Nouri Abdelatif , Boughamoura Lamia

Introduction: Gynecomastia is a benign proliferation of the human mammary gland that results from an imbalance between androgens and estrogens. It can be physiological at different stages of life (birth, adolescence, senescence) or secondary to drugs or diseases leading to androgen decline or estrogen elevation. We report a case of an important gynecomasty in a 5 year old boy.Case report: A boy 5-year-old with no medical history consulted for a gynecomas...

ea0063p460 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Steroid hormones and cancer immunity - insights into adrenocortical carcinoma

Landwehr Laura-Sophie , Dexneit Thomas , Herterich Sabine , Sbiera Iuliu , Schreiner Jochen , Kroiss Matthias , Fassnacht Martin , Sbiera Silviu

Context: Adrenocortical carcinoma (ACC) are endocrine malignant neoplasms associated with severe aggressiveness. By applying of ‘multiple omics’ approach, we recently categorized ACC patients based on their steroidogenic activity and expression of immune activation marker, which is along with prognosis; an ‘immune’ phenotype with good and a ‘steroid’ phenotype with bad outcome.Hypothesis: Our central hypothesis focuses on th...

ea0063p461 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Multiple endocrine neoplasia: A case report of a pancreatic neuroendocrine tumor in a long evolving MEN1 patient

Rotarescu Alexandra , Baciu Ionela , Marinescu Mihai , Dumitrascu Anda , Terzea Dana , Poiana Catalina

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant inherited syndrome caused by mutations in the MEN1 tumor suppressor gene and is characterized by the occurrence of parathyroid, pancreatic islet and anterior pituitary tumors. We present the case of a female patient known to have pituitary and parathyroid tumors in a MEN1 syndrome evolving for more than 20 years before associating pancreatic neuroendocrine tumor.Case r...

ea0063p462 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A clinical case of adrenal incidentaloma: inconsistency of a prediction to reality

Volkova Natalya , Ganenko Liliya , Degtyareva Yuliya

Introduction: The incidence of adrenal incidentalomas is 5% among patients undergoing radiological imaging. Differential diagnosis is extremely important, as it determines the tactics of both therapeutic treatment and surgical intervention. Currently, algorithms for the differential diagnosis of adrenal masses have been developed and are widely used. Nevertheless, the compliance with strictly regulated diagnostic protocols does not always allow to make an accurate diagnosis.</...

ea0063p463 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

InfeCtious disease in patients with Adrenal endocRinOpathies: the ICARO prospective observational study

Minnetti Marianna , Hasenmajer Valeria , Sbardella Emilia , Angelini Francesco , Bonaventura Ilaria , Simeoli Chiara , Chiloiro Sabrina , Pozza Carlotta , Gianfrilli Daniele , De Marinis Laura , Pivonello Rosario , Lenzi Andrea , Isidori Andrea M

Introduction: Cushing’s Syndrome (CS) is associated with an increased frequency and severity of infections. Mild hypercortisolism (MI) from adrenal incidentalomas has been associated with various complications, but data on infections are scarce. Retrospective data on patients with adrenal insufficiency (AI) showed an increased mortality attributed to infections. The approach used was very heterogeneous for all these studies. The aim of the study is to prospectively evalua...

ea0063p464 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

EGFR and IGF1R affect Sunitinib activity: novel targets for Broncho-Pulmonary Neuroendocrine Neoplasm treatment

Bresciani Giulia , Ditsiou Angeliki , Cilibrasi Chiara , Vella Viviana , Rea Federico , Schiavon Marco , Cavallesco Narciso Giorgio , Giamas Georgios , Zatelli Maria Chiara , Gagliano Teresa

Background: Available medical treatments for Broncho-Pulmonary Neuroendocrine Neoplasm (BP-NENs) derived from clinical trials are not specific for the management of this malignancy. Sunitinib, a multi-receptor tyrosine-kinases (RTKs) inhibitor, mainly described as VEGFR inhibitor, has shown its efficacy in Pancreatic NENs but there are not available data about its action in BP-NENs. Our aim was to understand the effects of RTKs inhibition promoted by Sunitinib in order to eval...

ea0063p465 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

Measurement of metanephrine in adrenal venous sampling may help subtyping primary aldosteronism

Baron Stephanie , Bailly Clement , Cornu Erica , Blanchard Anne , Bergerot Damien , Pagny Jean-Yves , Laffy Pierre-Yves , Carrere Thierry , Grataloup Christine , Zinzindohoue Franck , Reznik Yves , Azizi Michel , Billaud Eliane , Amar Laurence

Objective: Adrenal venous sampling (AVS) is the gold standard method to assess lateralization of aldosterone secretion in patients with primary aldosteronism (PA). The selectivity index (SIcortisol, the adrenal to peripheral vein ratio of cortisol concentrations) determines correct catheter positioning during AVS. The lateralization index (LIcortisol, the aldosterone to cortisol ratios between adrenal veins) distinguishes unilateral aldosterone producing ...

ea0063p466 | Adrenal and Neuroendocrine Tumours 2 | ECE2019

A case of metastatic pheochromocytoma diagnosed at a young age with hypertensive attack

Cankurtaran Yılmaz , Oruk Guzide Gonca , Cağlar Sevinc , Yazır Merve Gulec , Onder Pamuk Barış , Cengiz Fevzi , Etit Demet Arıkan

Introduction: Although the radiographic and histological features are the same as benign pheochromocytomas, malignant and metastatic pheochromocytoma is differentiated by invasion of the surrounding tissues and organ metastasis. Here, we present a young patient with a diagnosis of metastatic pheochromocytoma presenting with hypertension attacks, headache and flushing.Case presentation: A 22-year-old woman with a history of hypertension who had been suffe...