Adrenal and Neuroendocrine Tumours 1

ea0063p1 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A novel heterozygous mutation in exon 3 of VHL gene leading to Von Hippel-Lindau disease in a Turkish family

Yildirim Ozge Tasgin , Yildiz Ismail , Horozoglu Fatih , Gonen Aysun , Yazici Cenk Murat , Elbuken Gulsah , Zuhur Sayid

Background: Von Hippel-Lindau disease (VHL) is an autosomal dominant disease, characterized by haemangioblastomas of the retina and CNS, renal cell carcinomas (RCC) and renal cysts, pheochromocytomas, pancreatic neuroendocrine tumors and cysts, and endolymphatic sac tumors. VHL is associated with a germline mutation of the VHL tumor suppressor gene on the short arm of chromosome 3. The type-1 disease is caused by mutations, leading to severe disruption of protein acti...

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ea0063p2 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Long evolution of Connshing syndrome?

Elfekih Hamza , Sayadi Hanene , El Hak Abid Dhia , Kechida Malak , Sallem Om Kalthoum , Marmouche Hela , Klii Rim , Khochtali Ines

Introduction: Primary hyperaldosteronism is the main endocrine cause of secondary hypertension. Its association with an autonomous cortisol secretion is rarely described in the literature. Here we report the case of a patient having a unilateral adrenal adenoma responsible for both mineralocorticoids and glucocorticoids excess.Observation: A 54-years-old female diagnosed at the age of 26 years with hypertension. She received up to four antihypertensive t...

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ea0063p3 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Effect of Glucocorticoid receptor antagonist administration in a Cushing’s syndrome model rat

Seki Toshiro , Yasuda Atsushi , Kitajima Natsumi , Seki Masami , Oki Masayuki , Fukagawa Masafumi

In Cushings syndrome (CS), hypercortisolemia due to cortisol-producing adrenal adenomas suppresses the hypothalamic-pituitary-adrenal (HPA) axis, so the normal adrenal tissue is atrophied without ACTH stimulation and its function impairs. As a result of surgical resection of cortisol-secreting adenoma, postoperative adrenal insufficiency occurs. Therefore, we thought that administering glucocorticoid receptor antagonist (mifepristone) in order to release suppression of H...

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ea0063p4 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Ectopic adrenocorticotropic hormone (ACTH-ectopic) syndrome and osteoporosis: Rare cases and difficult treatment

Krylov Vadim , Dobreva Ekaterina , Kharnas Sergey , Kuznetsov Nikolay

Objective: Ectopic adrenocorticotropic hormone (ACTH-ectopic) syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze the clinical, biochemical, and radiological features, management, and treatment outcome of patients with EAS.Methods: It was a retrospective case-record study of 47 patients with EAS. Clinical, biochemical, and radiological features and response to therapy and survival rate ...

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ea0063p5 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Paraganglioma of the urinary bladder wall: 2 cases with 2 different presentations

Salloum Cynthia , Sawan Carla , Assaf George

Objective: We present two cases of patients with Paraganglioma of the urinary bladder wall, illustrating the differences in diagnosis and management.Case 1: An 80-year-old female known to have diabetes and hyperthyroidism (treated) presented to her urologist with painless hematuria. Cystoscopy was done and bladder polyps were biopsied. Results of the tumors sampled showed paraganglioma of the urinary bladder wall, although patient was asymptomatic. She p...

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ea0063p6 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Adrenal insufficiency secondary to bilateral adrenal diffuse large B-cell lymphoma: a case report

Garcia Maria Laura , Speroni Romina , Lisdero Ana Paula

Introduction: Primary adrenal lymphoma is extremely rare. It accounts for <1% of extranodal lymphoma. It affects typically old males with bilateral adrenal gland involvement that leads to adrenal hypofunction. Prognosis is very poor due to the lack of optimum chemotherapeutic regimens.Case Report: A 71 yearr old man with prior history of non insulin dependent diabetes, was admitted to the emergency department with symptoms of progressive weakness, fa...

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ea0063p7 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A rare association of Adrenocortical carcinoma with haematological and breast malignancy in a young female

Shafqat Muhammad Murtaza , Khalily Naveed , Deol Herpret , Naeem Aamir , Dar Shujah

A 20-year-old female admitted under haematology team for acute myeloid leukaemia. During her admission, she had a whole body CT scan and was found to have a left breast lesion (proven on biopsy to have invasive ductal carcinoma) and a right adrenal mass measuring 6.5 × 7.5 cm with radiological features suggestive of an adrenocortical cancer. Biochemical workup showed raised adrenal androgens: DHA Sulphate 14.9 umol/l (4.011.0), 17-OH Progesterone 7.6 nmol/l (0.6&#15...

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ea0063p8 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Metabolic and cardiovascular profile of 143 adrenal incidentalomas

Araujo-Castro Marta , Nunez Miguel Sampedro , Ramirez Paola Parra , Lazaro Cristina Robles , Hernandez Martin Cuesta , Marazuela Monica

Objective: To study the differences in the metabolic profile of patients with non-functioning adrenal adenomas (NFA) and autonomous cortisol secretion (ACS).Methods: 143 patients diagnosed of adrenal incidentaloma (AI) between 2010 and 2018 were retrospectively analyzed. AI was defined as an adrenal mass≥1 cm, accidentally discovered by radiologic examination. ACS was confirmed by serum cortisol post-dexamethasone suppression test (Nugent)≥3 ...

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ea0063p9 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

HNRNP Q suppresses polyglutamine huntingtin aggregationby post-transcriptional regulation of Vaccinia-related kinase 2

Kim Kyong-Tai , Ryu Hye Guk

The misfolded proteins with abnormal polyglutamine (polyQ) expansion cause neurodegenerative disorders including Huntingtons disease (HD). Recently, Vaccinia-related kinase 2 (VRK2) were found to accumulate polyQ aggregates by controlling TCP-1 ring complex (TRiC)/Chaperonin-containing TCP-1 (CCT), which has an essential role in preventing against the aggregation and cytotoxicity of polyQ proteins. Interestingly, VRK2 expression is known to be much higher in actively pro...

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ea0063p10 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Study of body composition and phase angle in relation to nutritional parameters in patients with neuroendocrine tumors

Hernandez-Garcia Carmen , Cornejo-Pareja Isabel Maria , Damas-Fuentes Miguel , Martinez-Montoro Jose Ignacio , Diaz-Perdigones Cristina Maria , Bandera-Merchan Borja , Garcia-Almeida Jose Manuel , Tinahones-Madueno Francisco Jose

Introduction: Neuroendocrine tumors are an heterogeneous group of neoplasms with increasing incidence and high survival. The therapies, could be have a negative impact on nutrition and pronogsis of these patients.Objetives: To analyze analytical and anthropometric parameters in relation to disease stage and survival.Material and methods: An observational, retrospective and cross-sectional study that includes patients with neuroendo...

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ea0063p11 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A case series of two patients with intrapericardial paragangliomas

Puri Gaurav , Farquhar Hannah , Mundy Julie , Sinha Ashim Kumar

Two patients have presented to a regional Australian Hospital with intrapericardial paragangliomas in the last decade. In 2011, a 28-year-old Indigenous male presented with a hypertensive crisis. He had a two-year history of symptoms during physical exertion, including abdominal pain, nausea, and vomiting. He had also experienced paroxysmal light-headedness and sweats, and had been previously diagnosed with hypertension. Urinary and serum catecholamine testing revealed markedl...

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ea0063p12 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Combined autophagy and mTOR inhibition reduces cells proliferation and induces apoptosis in a lung carcinoid in-vitro model

Knigin Adi , Avniel-Polak Shani , Gross David J , Grozinsky-Glasberg Simona

Introduction: Treatment options for patients with metastatic lung carcinoids (LC) are limited. Everolimus (RAD001), an mTOR inhibitor (mTORi) which suppresses tumor cells growth & proliferation, appears to be efficient in these patients; however, it promotes autophagy, thereby paradoxically supporting tumor cell survival and development of drug-resistance. We have previously demonstrated in a BON1 pancreatic NEN model that adding chloroquine (CQ, an autophagy inhibitor) to...

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ea0063p13 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Pheochromocytoma – 3 sides of the same story

Ene Cristina , Nour-Dinca Angelica , Acatrinei Eliza , Casian Marioara , Enyedi Mihaly , Terzea Dana , Istrate Adrian , Grozavu Constantin

Background: Pheochromocytomas are rare tumors, with prevalence less than 0.2% of hypertensive patients, in general population being unknown. With increasing access to imaging and hormonal workup, more pheochromocytomas are diagnosed. This may have changed the occurrence of the classic presentation with hypertension and the classic triad.Methods: We present 3 cases of pheochromocytoma diagnosed in our departments over the last 2 years, with 3 different ph...

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ea0063p14 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Analysis of the 22-years experience of surgical treatment of adrenal tumor in single specialized endocrinological center

Tarasenko Sergii , Tovkay Olersander , Kunatovskiy Mikhailo , Yefimova Olena

Objectives: To analyze the data of the 22-year-old clinical experience surgical treatment of the adrenal tumor patients in the single specialized endocrinological center.Materials and methods: There were analyzed the diseases stories of the perioperative period in 1256 patients with adrenal gland tumors who underwent adrenalectomy for the period 19952017 in specialized endocrinological center. Average data are given in the format M±σ....

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ea0063p15 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Immunohistochemical characteristics of adrenal tumors in patients with primary aldosteronism

Romanova Natalia , Selivanova Lilya , Platonova Nadezhda , Troshina Ekaterina , Abrosimov Aleksandr

Introduction: CYP11B2 is a key enzyme of primary aldosteronism, and several factors are involved in the regulation of CYP11B2 expression and the overproduction of aldosterone. Somatic mutations in aldosterone-driver genes are strongly associated with CYP11B2 expression and have been only detected in the CYP11B2-positive tumor area, indicating that aldosterone producing adenoma (APA) intratumoral heterogeneity corresponds to non-uniform CYP11B2 expression in neoplastic cells. I...

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ea0063p16 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Cortex protecting surgery in MEN 2A

Dokmetas Hatice Sebile , Kilicli Fatih , Ozdenkaya Yasar , Cavusuglu Gunes , Dokmetas Meric , Erol Bugra , Karaipek Kubra

Cortex protecting surgery for bilateral pheochromocytoma protects the patient from adrenal insufficiency. Risk of relapse is low. 35 years old woman had paroxysmal seizures and hypertension crises for the last year which became more frequent in the last 10 days. She had thyroidectomy and under the use of levothyroxine the patient. Because of a nodule on ultrasonography and paternal history of MEN 2A which led to a check of calcitonin level that resulted 8 times higher than the...

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ea0063p17 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Unilateral non-haemorrhagic adrenal infarction (NHAI) as a cause of abdominal pain during pregnancy

Chasseloup Fanny , Bourcigaux Nathalie , Christin-Maitre Sophie

Adrenal infarction is usually associated with bilateral adrenal hemorrhage in the setting of antiphospholipid syndrome or hemodynamic variation. A few cases of unilateral non-hemorrhagic adrenal infarction (NHAI) have been described in the literature. Here, we report a case of unilateral non-haemorrhagic adrenal infarction occurring during pregnancy and a literature review of this clinical entity. A 30-year-old woman presented at 32 weeks of gestation with sudden-onset right a...

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ea0063p18 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Evaluating the applicability of urinary miR-483-5p as a non-invasive marker in adrenocortical cancer patients

Decmann Abel , Bancos Irina , Thomas Melinda A , Turai Peter , Perge Pal , Toth Miklos , Patocs Attila , Igaz Peter

Introduction: Minimally invasive blood-borne circulating microRNAs might be used for the preoperative differentiation of adrenocortical carcinoma (ACC) and adrenocortical adenoma (ACA). Circulating hsa-miR-483-5p is so far the best microRNA biomarker of ACC. To the best of our knowledge, there have been no studies concerning the potential applicability of urinary has-miR-483-5p as a non-invasive biomarker of ACC and its correlation with plasma hsa-miR-483...

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ea0063p19 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Allgrove syndrome (Triple A syndrome): A case report

Moustafa Heba , El Gohary Amira M , Essam Kareem , Fatah Yasmine Abd El , Salam Randa F

Introduction: Allgrove syndrome is a rare autosomal recessive disorder characterized by a Lacrima, achalasia, adrenal Insufficiency and Neurologic disorders. Mutation in (AAAS) gene on chromosome 12q13, has been implicated.Case report: Eighteen-year-old male referred to the Endocrinology clinic for evaluation of suspected adrenal insufficiency. The patient reported generalized weakness, fatigue, anorexia recurrent fainting attacks, and progressive hyperp...

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ea0063p20 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A case series of metastatic pheochromocytomas and paragangliomas: patients characteristics, therapeutic approach and follow-up

Angelousi Anna , Kassi Eva , Peppa Melpomeni , Chrysoulidou Alexandra , Zografos George , Aggeli Chrysanthi , Kaltsas Grigorios

Background: Metastatic pheochromocytomas (PCs) and paragangliomas (PGLs) are rare neuroendocrine neoplasms with a <1:106 incidence, defined by the presence of metastatic disease besides recurrent or locally invasive disease.Aim: Retrospective analysis of clinical, biochemical/hormonal, imaging, genetic and histopathological features of patients with malignant PCs/PGLs diagnosed over 15 years.Results: Thirteen patient...

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ea0063p21 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Retroperitoneal paraganglioma: clinical case

Volkova Natalya , Davidenko Ilya , Zibarev Alexander , Harlamov Alexey , Shevchenko Anastasia , Raksha Alexandra

Background: Paraganglioma is a rare neuroendocrine tumor from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis. About 1520% of chromaffin-cell tumors are paragangliomas. In most cases paragangliomas produce catecholamines in large concentrations and the lack of treatment can lead to cardiovascular and cerebral catastrophes. That is why early diagnosis of this tumor is so important and, consequently, knowledge of its d...

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ea0063p22 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

The frequency of confirmed primary hyperaldosteronism in patients with high aldosteron renin ratio

Ozdemir Didem , Topaloglu Oya , Aydogan Leyla , Ersoy Reyhan , Cakir Bekir

Introduction: The aldosterone-to-renin ratio (A/R) is the most frequently used screening test for primary hyperaldosteronism (PH) and a confirmation test is recommended when it is high. We aimed to determine the frequency of PH in patients with high A/R and investigated possible clinical features that might help to prevent unnecesary confirmation tests.Method: Patients who underwent saline infusion test because of high A/R (>3.8) were retrospectively...

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ea0063p23 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Evaluation of angiotensin II in primary aldosteronism and other forms of hypertension – a preliminary study

Łebek-Szatańska Agnieszka , Glinicki Piotr , Walczak Maria , Nowak Karolina M , Rdzanek Monika , Zgliczyński Wojciech , Papierska Lucyna

Background: The renin-angiotensin-aldosterone system (RAS) constitutes a key hormonal system in the physiological and pathological regulation of blood pressure. In effort to understand complex and multifunctional aspects of this system, current biochemical approaches target to characterize and define its various components with angiotensin II being in the mainstream.Objective: The aim of this study was to evaluate concentrations of angiotensin II in hype...

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ea0063p24 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Management of adjuvant mitotane therapy for adrenocortical carcinoma: a survey in Italy

Puglisi Soraya , Calabrese Anna , Basile Vittoria , Pivonello Rosario , Ceccato Filippo , Scaroni Carla , Torlontano Massimo , Cannavo Salvatore , Arnaldi Giorgio , Stigliano Antonio , Malandrino Pasqualino , Saba Laura , Altieri Barbara , Casa Silvia Della , Perotti Paola , Berchialla Paola , De Filpo Giuseppina , Canu Letizia , Loli Paola , Reimondo Giuseppe , Terzolo Massimo

Background and objective: Mitotane is widely used as post-operative adjuvant treatment of adrenocortical carcinoma. However, the management of therapy is largely empirical. Mitotane monitoring is recommended but we do not know what impact target concentrations do have on patient outcome. The aim of the study is to evaluate its use in expert Italian centers, analyzing data of Lysosafe Online® database.Methods: Retrospective analysis of patients repor...

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ea0063p25 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Management of mitotane for advanced adrenocortical carcinoma: a survey in Italy

Puglisi Soraya , Saba Laura , Perotti Paola , Calabrese Anna , Basile Vittoria , Arnaldi Giorgio , Cannavo Salvatore , Stigliano Antonio , Canu Letizia , Loli Paola , Pivonello Rosario , Malandrino Pasqualino , Badalamenti Giuseppe , Torlontano Massimo , Della Casa Silvia , Scaroni Carla , Terzolo Massimo

Background and objective: Mitotane is the main option of treatment for advanced adrenocortical carcinoma (ACC). However, limited evidence is available regarding practical management of mitotane treatment and its eventual combination with chemotherapy or radiotherapy. The aim of the study is to do a survey on the use of mitotane for advanced ACC in expert Italian centers, analyzing data of the LYSOSAFE database.Methods: Retrospective analysis of patients ...

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ea0063p26 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

How do sex and BMI affect glucocorticoid treatment in adrenal insufficiency?

Puglisi Soraya , Tabaro Isabella , Cannavo Salvatore , Borretta Giorgio , Pellegrino Micaela , Chiappo Francesca , Pia Anna , Terzolo Massimo , Reimondo Giuseppe

Background and objective: Optimization of glucocorticoid (GC) replacement therapy in adrenal insufficiency (AI) is crucial to avoid consequences of under- or overtreatment. Dosing of GC replacement is mainly based on clinical grounds; however, the impact of patients characteristics on daily GC requirement is poorly evaluated. The aim of the study is to assess the influence of sex (M/F) and BMI on dosing GC in patients with AI of different etiology....

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ea0063p27 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Abstract Unavailable....

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ea0063p28 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

Piskinpasa Hamide , Dogansen Sema Ciftci , Cakır Ilkay , Akdeniz Yasemin Sefika , Esen Ayse , Bozkur Evin , Mert Meral

Introduction: Congenital adrenal hyperplasia (CAH) due to 11 beta-hydroxylase deficiency (11βOHD) is a rare autosomal recessive genetic disorder. It results defects in adrenal cortisol and aldosterone synthesis. Early diagnosis and initiation of treatment in male patients are essential in order to prevent serious complications. We report a male patient with CAH who developed complications because of late diagnosisCase Report: 44-year-old male patien...

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ea0063p29 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Pheocromocytoma, newly diagnosed during pregnancy in a subject with neurofibromatosis type 1: A case report and review of literature

Kermaj Marjeta , Isufi Alma , Misja Adriana , Hoxha Violeta , Fureraj Thanas , Muco Ermira , Guni Mirjeta , Resuli Klotilda , Ylli Agron

Introduction: Pheocromocytoma, is a rare cause of hypertension diagnosed during pregnancy, with a prevalence 0.002%, due to its non-specific presentation and difficulty to distinguish its hypertension from other clinical conditions during pregnancy. Early diagnosis and appropriate treatment have a great impact on maternal and fetal mortality. Pheocromocytoma is sporadic in the majority of cases, but up to 25% may be syndrome-associated.Case report: We re...

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ea0063p30 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Becoming of patients with primary aldosteronism according to their treatment

Belmihoub Ines , Cornu Erika , Nevoret Camille , Jannot Anne-Sophie , Azizi Michel , Amar Laurence

Introduction: Unilateral forms or primary aldosteronism (PA) can be treated by adrenalectomy of by mineralocorticoid receptor antagonists, whereas bilateral forms are always treated by mineralocorticoid receptor antagonists. Most studies find similar results for the control of blood pressure and kalemia, but also to reverse organ damages, however recent studies are discordant and show a benefit of adrenalectomy regarding cardiovascular events and blood pressure (BP). Regarding...

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ea0063p31 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Predictors of bilateral and unilateral primary aldosteronism: a retrospective observational study

Ishikawa Takuya , Suwanai Hirotsugu , Abe Hironori , Ishii Keitaro , Iwasaki Hajime , Yakou Fumiyoshi , Ito Mariko , Shikuma Jumpei , Suzuki Ryo , Hara Kazuo , Miwa Takashi , Takamiya Tomoko , Inoue Shigeru , Saito Kazuhiro , Odawara Masato

Primary aldosteronism is a frequent cause of secondary hypertension, with early diagnosis being important for appropriate treatment and minimizing the risk of organ damage due to excessive aldosterone. Treatment, however, varies for unilateral and bilateral primary aldosteronism, with oral aldosterone antagonist being the treatment of choice for the bilateral form and adrenalectomy, for the unilateral form. As surgical treatment for unilateral primary aldosteronism is effectiv...

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ea0063p32 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

18-oxocortisol synthesis in aldosterone-producing adrenocortical adenoma and significance of KCNJ5 mutation status

Satoh Fumitoshi Yamazaki Yuto , Morimoto Ryo , Kudo Masataka , Gomez-Sanchez Celso E , Ito Sadayoshi , Sasano Hironobu , Satoh Fumitoshi

Peripheral 18-oxocortisol level could contribute to the detection of aldosterone-producing adenoma in patients with primary aldosteronism. However, peripheral 18-oxocortisol varies among such patients, which is a big drawback concerning its clinical application. We studied 48 cases of aldosterone-producing adenoma, 35 harboring KCNJ5 mutation, to clarify the significance of clinical and pathological parameters regarding peripheral 18-oxocortisol. Peripheral 18-oxocort...

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ea0063p33 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Complete remission of a highly aggressive, metastasized, SDHB-related paraganglioma after chemotherapy with CVD and peptide-receptor radiotherapy

Meyer Gesine , Koch Christine , Bojunga Jorg

Case: A 40-year-old woman presented in 04/2015 with pain in the upper abdomen and a history of paroxysmal tachycardia. Plasma normetanephrines were highly elevated. Imaging revealed a large retroperitoneal tumor (15×12×8 cm), which could be completely resected after pre-treatment with urapidil. Histology confirmed diagnosis of a paraganglioma. Genetic diagnostics detected a previously unknown mutation in intron 2 of the SDHB-gene (c.200+5G>C). Six month after ini...

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ea0063p34 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Aldosterone measurement in the Diagnosis of primary aldosteronism – comparison between two automated immunoassays and two liquid chromatography tandem mass-spectrometry methods

Fagotto Valentina , Uhl Olaf , Heinrich Daniel , Nirschl Nina , Sturm Lisa , Kratzsch Jurgen , Bae Yoon Ju , Fries Charlotte , Fenske Wiebke , Reincke Martin , Bidlingmaier Martin

Poor agreement between different analytical methods measuring aldosterone concentrations impedes applicability of uniform cut-offs in the diagnostic work-up of primary aldosteronism (PA). We compared 4 analytical methods (2 immunoassays and 2 LC-MS/MS) in plasma samples before and after saline infusion test (SIT). 80 hypertensive patients underwent the standardized diagnostic work-up within the German Conns registry. Patients (PA n=39/non-PA n=41) were ...

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ea0063p35 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Embolization of the adrenal artery to control severe adrenal hemorrhage

Medenou Lionel

Background: The adrenal hemorrhage is rare and potentially lethal. Its diagnosis is difficult because atypical. We report the case of a patient beautifully treated by embolization of the adrenal artery.Case report: A 68-year-old female patient admitted for abdominal pain, with as background, a diabetes, atrial fibrillation under anticoagulation with recent relay Previscan Innohep and a right adrenal incidentaloma (for 9 years, 43 mm of centerline and den...

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ea0063p36 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Vitamin D deficiency is a predictor marker of tumor aggressiveness in sporadic and MEN1-related well-differentiated, low-grade GEP-NET

Altieri Barbara , Barrea Luigi , Modica Roberta , Circelli Luisa , Bottiglieri Filomena , Cicco Federica De , Savarese Giovanni , Somma Carolina Di , Colao Annamaria , Savastano Silvia , Faggiano Antongiulio

Background: Vitamin D has several noncalcemic implications, including effects on cell signaling and differentiation. Patients with gastroenteropancreatic (GEP) neuroendocrine tumors (NET) have an increased risk of vitamin D deficiency, due to the tumor itself, systemic therapies and abdominal surgery. However, data regarding vitamin D status are limited. Aim of this study was to evaluate 25-hydroxy-vitamin-D (25(OH)D) levels in GEP-NET patients and correlated them ...

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ea0063p37 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Role of KCNJ5 mutations in mediating cell growth in aldosterone-producing adenomas

Yang Yuhong , Meyer Lucie S , Jaquin Diana , Schneider Holger , Ploncynski Maria , Beuschlein Felix , Gomez-Sanchez Celso E , Reincke Martin , Williams Tracy Ann

Background: Primary aldosteronism is commonly caused by an aldosterone-producing adenoma (APA). Somatic mutations in the KCNJ5 gene (encoding an inwardly rectifying potassium channel) are found in around 40% of APAs, KCNJ5 germline mutations cause familial hyperaldosteronism type III. The role of KCNJ5 mutations in excessive aldosterone production is established but their role in cell growth is unclear.Objective: To study the e...

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ea0063p38 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Influence of Tumor Microenviroment in response modulation to treatment in human BP-NENs

Bresciani Giulia , Ditsiou Angeliki , Cilibrasi Chiara , Vella Viviana , Giamas Georgios , Zatelli Maria Chiara , Gagliano Teresa

Introduction: Broncho-Pulmonary Neoplasms (BP-NENs) are rare neoplasms arising from neuroendocrine cells of the respiratory epithelium. Since previous studies in our lab have demonstrated the efficacy ofEverolimus, approved for BP-NENs treatment, and Dinaciclib, a Cyclins and CdKs inhibitor, on monolayer system we have investigated if a more complex tumour system could generate a change in drugs effects and cell resistance. Therefore, through the use of 3D culture model, we ha...

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ea0063p39 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A novel CYP11B1 mutation presenting as a classical congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency

Mazarico Isabel , Gimenez-Palop Olga , Albert Lara , Florencia Luchtenberg , Casamitjana Laia , Capel Ismael , Subias David , Cano Albert , Guitart Miriam , Caixas Assumpta , Rigla Mercedes

Background: Congenital adrenal hyperplasia (CAH) is a rare autosomal recessive disorder, of which 21-hydroxylase deficiency (21OHD) is the most frequent form. 11 beta-hydroxylase deficiency (11bOHD) is the second most common type of CAH. This pathology results from inactivating mutations in CYP11B1 gene.Case presentation: We describe a case of a 48-year-old woman with 11bOHD, presented with hypokalemia hypertension, early adrenarche and mild vir...

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ea0063p40 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Available 3D cultures methods: study on a Pancreatic Neuroendocrine Neoplasm cell line

Bresciani Giulia , Hofland Leo J , Giamas Georgios , Gagliano Teresa , Zatelli Maria Chiara

Background: Pancreatic Neuroendocrine Neoplasms (pNENs) are malignancies arising from the endocrine pancreas. Past in vitro studies have led to a better comprehension and characterization of this malignancy under several points of view. However, effective medical therapies are still not available, therefore, it is essential to continue studying this malignancy in order to identify a successful approach. Due to tumour complexity, techniques such as 3D cultures, able to...

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ea0063p41 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Prevalence and prognosis of bone metastases in pancreatic and small intestinal neuroendocrine tumours

Mammadov Emin , Hughes Nicola , Alnuaimi Asma , Chin Jun Liong , O'Toole Dermot , O 'Shea Donal , Heffernan Eric , Crowley Rachel K

Background: Neuroendocrine tumours (NET) can affect various tissues and organs, have different clinical presentation and prognosis, depending on the primary tumour site, grading, differentiation and functional status. Reported prevalence of bone metastases (BM) from NETs has been increasing with improved imaging modalities, reaching >40% in some reports. The BM diagnosis is associated with reduced overall survival; however, this was not confirmed by all studies.<p clas...

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ea0063p42 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Endocrine hypertension?

Maraver-Selfa Silvia , Damas-Fuentes Miguel , Cornejo-Pareja Isabel Maria , Molina-Vega Maria , Munoz-Garach Araceli , Hernandez-Garcia Carmen , Mancha-Doblas Isabel , Tinahones Francisco

An 18-years-old woman without medical history was referred for study of hypertension and an adrenal incidentaloma. The family history was significant for essential well-controlled hypertension in her father; diagnosed at 39 years-old. The patient complained of headache, asthenia, edema in lower limbs, high blood pressure values (200/150 mmHg) for one year (well controlled with losartan 100 mg/12 h, amlodipine 10 mg/24 h and doxazosin 4 mg/24 h) and marked facial acne previous ...

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ea0063p43 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Ninety-four serum steroids measured by gas chromatography tandem mass spectrometry (GC-MS/MS) in patients with Cushing’s syndrome

Hana Jr Vaclav , Ježkova Jana , Kosak Mikulaš , Kršek Michal , Hana Vaclav , Hill Martin

Introduction: Gas chromatography tandem mass spectrometry (GC-MS/MS) quantitatively detects many steroids at one time in a single serum sample. Cushings syndrome (CS) caused by adrenal adenoma is often associated with decreased DHEAS, whereas low levels of aldosterone in ACTH dependent CS and also low levels of 18-oxocortisol were observed in ectopic CS. We aimed to evaluate steroid differences in various types of CS.Methods: In serum from patients...

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ea0063p44 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Comparative study between autoimmune metaplastic atrophic gastritis (AMAG) and type 1 gastric neuroendocrine tumors (G-NETs) in Hospital Clínico San Carlos (HCSC), Madrid

Ramos Elvira , Vargas-Zuniga Ignacio , Barrio Elvira , Suarez Pablo , Pazos Mario , Pallares Raquel , Aldama Paula , De La Torre Nuria Garcia , Sevilla Concepcion , 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. Endoscopically, they are usually smaller than 1 cm and often multiple. These tumors usually present a non-aggressive evolution. Our goal is to approach the presence of risk fac...

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ea0063p45 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Pheochromocytoma and disorders of carbohydrate metabolism

lmir Siham E , Yaden Yousef , Rouf Siham , Latrech Hanene

Introduction: Pheochromocytoma is a rare tumor secreting catecholamines, which are part of the hormones of carbohydrate counter-regulation. The aim of our work is to evaluate the glycemic balance in patients in pre- and post-operative pheochromocytoma.Material and methods: A retrospective study of 18 cases of pheochromocytoma over a period of 5 years. A pre- and post-operative metabolic assessment was performed as well as an evaluation of the other clini...

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ea0063p46 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Heart and vessels after a pheochromocytoma: which outcomes?

Elmir Siham , Khadija Boujtat , Rouf Siham , Laterch Hanane

Introduction: Pheochromocytoma is a rare tumor. It can be revealed or complicated by cardiovascular manifestations. The aim of our work is to study the cardiovascular manifestations of pheochromocytoma and the evolution of hypertension after surgery.Patients and methods: A retrospective study of 18 cases of pheochromocytoma followed at the endocrinology and metabolic diseases department of Mohammed VI university hospital of Oujda during a period of 5 yea...

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ea0063p47 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

First presentation of Addison’s disease as hyperkalaemia: a rare case report

Ben Yakhlef Salma , Derkaoui Nada , Rouf Siham , Latrech Hanane

Introduction: Addissons disease is a rare endocrine disorder; that continues to be a diagnostic challenge in the 21st century; mainly due to the slow and non specific progression of symptoms. We report the case of a 45-year-old woman who was admitted with acute adrenal insufficiency discovered in the context of a severe hyperkalemia.Case report: A 45-year-old woman was admitted in our institution with a history of extreme weakness and vomiting. On ...

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ea0063p48 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Pharmacokinetic Interaction Study of the Effects of Multiple-dose Gastric Acid Reducers Omeprazole (OMP) and Famotidine (FAM on the Pharmacokinetics (PK) of Single-dose Telotristat Etiprate (TE) in Healthy Subjects

Wilson Alan , Qubeck Christine , Warner Chris , Tesfaye Eshetu , Nguyen Quyen , Wason Suman

Background: TE is indicated for the treatment of carcinoid syndrome diarrhea in patients with metastatic neuroendocrine tumors.Objective: To evaluate the effects of a proton pump inhibitor (OMP) and H2 antagonist (FAM) on the PK of TE and its active metabolite LP-778902 via an open-label study of OMP (40 mg orally QD × 4 days) or FAM (40 mg orally BID × 4 days) with a single 250 mg oral dose of TE. Eligible subjects: healthy, nonsmoking, aged &...

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ea0063p49 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Endocrine hypertension: before and after treatment of the endocrinopathy

Khiari Marwa , Nacef Ibtissem Ben , Rojbi Imene , Khiari Karima , Jerbi M , Mchirgui Nadia , Lakhoua Youssef , Abdallah Nejib Ben

High blood pressure of endocrine origin remains a rare cause of hypertension. The interest of the research of endocrine hypertension lies in the severity of certain forms and its potentially curable and reversible nature, hence the interest of oriented screening. The aim of our work was to study the profile of endocrine hypertension among a population suffering from secondary diabetes. This is a retrospective descriptive study of 55 patients followed in endocrinology departmen...

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ea0063p50 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A seminoma with entrapped nerve ganglion masquerading as a paraganglioma

Rosenblum Rachel Chava , Atlan Karine , Diment Judith , Mazeh Haggi , Rotman-Pikielny Pnina , Twito Orit

Background: The differential diagnosis of retroperitoneal tumors includes lymphoid, germ cell and neurogenic tumors such as paraganglioma. Paragangliomas are rare neuroendocrine tumors of the autonomic nervous system, which may secrete catecholamines and their metabolites. Clinical features include sustained or paroxysmal hypertension, headaches, sweating and palpitations. Here we present an unusual case of a retroperitoneal tumor entrapping a sympathetic nerve ganglion and mi...

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ea0063p51 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Magnetic resonance imaging in the initial diagnosis of pancreatic insulinoma: primary data analysis

Yukina Marina , Nuralieva Nurana , Troshina Ekaterina , Vorontsov Aleksandr , Vladimirova Victoria , Tarbaeva Natalia , Rebrova Olga

Introduction: Insulinoma visualization is the most difficult stage of diagnosis of this disease. Currently, in most cases the contrast-enhanced multislice computer tomography of abdomen and retroperitoneal space (CE-MSCT) is the method of choice in topical diagnosis of insulinoma. This method is associated with radiation exposure and administration of contrast agents, so it is invasive. Thus, an alternative highly informative and safe method for initial topical diagnosis of in...

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ea0063p52 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Exome analysis of pituitary adenoma tissue derived cell cultures

Rovite Vita , Peculis Raitis , Mandrika Ilona , Petrovska Ramona , Megnis Kaspars , Balcere Inga , Konrade Ilze , Stukens Janis , Breiksa Austra , Nazarovs Jurijs , Pirags Valdis , Klovins Janis

Introduction: Pituitary adenomas (PA) are tumours of the anterior pituitary. Despite the benign nature these neoplasms cause increased mortality and morbidity. Clinically relevant PAs affect around 0.1% of population during their lifetime. Currently, there is no human PA cell culture models. Tissue cultures derived from PA surgery materials depending on harvesting conditions can form free floating aggregates called pituispheres (PS) or adherent mesenchymal stromal cells (MSC)....

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ea0063p53 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A rare association of neuroendocrine tumor with adenocarcinoma of ampulla of vater: difficulties for diagnosis and effects of multi therapeutic approach

Stanca Oana Stefania , Olariu Cristina , Badiu Corin

Introduction: The neuroendocrine tumor of the ampulla of Vater represent a very rare disease, corresponding up to 2% of the periampullary malignancies and less than 1% of gastrointestinal NET. Less than 130 patients have been reported until 2013. The biological and clinical behavior is very unpredictable especially if its associated with another tumor, like adenocarcinoma. We present a case of a fifty-one-years-old woman who complained of weight loss, jaundice, abdominal...

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ea0063p54 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Continuous glucose monitoring during the prolonged fast test for the diagnosis of hypoglycemic diseases: primary data analysis

Yukina Marina , Nuralieva Nurana , Troshina Ekaterina

Introduction: To diagnose the hypoglycemic diseases (HD), firstly, proving the hypoglycemia presence is necessary. For this purpose, the prolonged fast test, which can reach 72 h, is performed. In case of late completion this test has a potential risk of hypoglycemic coma. Repeated glycemia control by glucometer brings discomfort to patients and increases the labor costs of medical workers. In this regard, it was relevant to conduct a study on the comparison of continuous gluc...

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ea0063p55 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Clinical case of a repeated life-threatening upper gastrointestinal bleeding due to previously unknown primary hyperparathyroidism

Budul Natalia , Komerdus Irina

Background: Peptic ulcer bleeding due to primary hyperparathyroidism is extremely rare and only a few cases have been reported in the literature.Material and methods: Man with repeated upper gastrointestinal bleeding, with a history of kidney stones and previously unknown primary hyperparathyroidism.Case: Man M., 46 y.o., with a history of 4 episodes of gastrointestinal bleeding and a ten-year history of multiple kidney stones. In ...

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ea0063p56 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A difficult diagnosis: pheochromocytoma or methamphetamine abuse?

Constantinescu Georgiana , Leike Steffen , Kunath Carola , Langton Katharina , Gruber Matthias , Peitzsch Mirko , Deinum Jaap , Lenders Jacques , Eisenhofer Graeme

Introduction: Pheochromocytomas represent rare but potential lethal tumors arising from the adrenal medulla. Early recognition and diagnosis represent a challenge due to the non-specific character of signs and symptoms. The classic presentation includes headache, sweating, palpitations and other signs and symptoms of apparent catecholamine excess occurring in paroxysms. Sympathomimetics may result in a similar presentation, potentially complicating the diagnosis.<p class="...

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ea0063p57 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Association of the BclI glucocorticoid receptor polymorphism with body composition and metabolic parameters in female patients with adrenal incidentalomas

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

Interindividual variations in tissue sensitivity to glucocorticoids (GC) have been partly attributed to polymorphisms in the glucocorticoid receptor (GR) gene. The aim of this study was to investigate whether BclI variant of the GR gene may contribute to metabolic abnormalities frequently present in patients with adrenal incidentaloma (AI). Biochemical tests and hormonal evaluation were performed in 106 consecutive women with AI. Non-diabetic patients underwent an ora...

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ea0063p58 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

A case of Von Hippel-Lindau disease with bilateral pheochromocytoma and ectopic hypersecretion of intact parathyroid hormone

Bennour Marwa , Oueslati Ibtissem , Rejeb Ons , Mekni Sabrine , Yazidi Meriem , Grira Wafa , Chaker Fatma , Chihaoui Melika

Introduction: Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited syndrome predisposing to a variety of highly vascularised tumors in different organs. Although bilateral pheochromocytoma was reported in patient with VHL disease, the coexistence of primary hyperparathyroidism is not a common condition. Herein we report a case of a primary hyperparathyroidism secondary to an ectopic secretion of intact parathyroid hormone (PTH) in a patient with VHL disease and b...

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ea0063p59 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Adrenal pheocromocytoma during childhood – a case report

Silva Eugenia , Cunha Clara , Ferrinho Catia , Ferreira Rute , Oliveira Manuela

Pheochromocytoma is a rare tumor made up of chromaffin cells from the adrenal medulla that secrete catecholamines. It can occur at any age, with a peak incidence between the fourth and fifth decade of life, and only 10% of cases occur in children. The majority of cases are sporadic and approximately 10% are malignant. The existence of familial syndrome, multiple or extra adrenal tumors is more common at younger ages. The clinic is very variable, and hypertension (HTA) is the m...

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ea0063p60 | Adrenal and Neuroendocrine Tumours 1 | ECE2019

Adrenal morphology in a large cohort of adult subjects with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Oriolo Claudia , Gasparini Daniela Ibarra , Altieri Paola , Ruffilli Francesca , Corzani Francesca , Dalmazi Guido Di , Vicennati Valentina , Pagotto Uberto , Gambineri Alessandra

Introduction: The adrenal morphology in congenital adrenal hyperplasia (CAH) is poorly described in the literature, so that adrenal radiological evaluation is not recommended in patients with CAH. The aim of this study was to evaluate the adrenal morphology in a large cohort of adult patients with CAH due to 21-hydroxylase deficiency and its correlation with the subtype of CAH (non-classical-NCAH, simple virilizing-SV, or salt wasting-SW), the hormonal status and the treatment...

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21st European Congress of Endocrinology

BiosciAbstracts