Endocrine Abstracts

Background and objective: Multiple Endocrine Neoplasia type 1 (MEN 1) is an autosomal dominant disease characterized by a broad clinical spectrum. Previous multi-center studies, that analyzed large groups of patients with MEN 1 have been reported before but long term follow up data of these patients focusing exclusively on primary hyperparathyroidism (PHPT) are scarce.Patients and Methods: In this retrospective cohort study we include all patients with P...

Introduction: Etomidate is an imidazole derivative which inhibits several enzymatic steps (11β-hydroxylasey, 17β-hydroxylase, 17,20-lyase, cholesterol side-chain cleavage). Intravenous etomidate at sub-anaesthetic doses remains an important option when i.v. administration is required for rapid treatment of severely ill patients with hypercortisolaemia (Cushing’s syndrome, CS) and is almost always very effective.Case series: A 49-year-old w...

Background: Although sunitinib, an oral multitargeted tyrosine kinase inhibitor of RET, VEGFR, PDGFR, c-KIT used for cancer treatment, has a reported prevalence of hypocalcemia of 35%, this has not been documented in pancreatic neuroendocrine tumour (pNETs).Methods: We present three, out of 12, patients with pNETs treated with sunitinib who developed grade 1, 2 and 4 hypocalcemia according Common Terminology Criteria for Adverse Events v3.0 (CTCAE) crite...

Objective: The prevalence of metastatic bone disease in patients with neuroendocrine tumours (NETs) and their response to first line treatment with biphosphonates.Methods: We studied 271 patients (141 females) with NETs: 54 gastric, nine duodenal, 102 pancreatic, 29 small intestine, 29 appendix, 14 colon, 15 lung, one thymic, 20 unknown primary and ten elsewhere. Since September 2012 all patients with NETs and bone metastases were recruited to receive 4 ...

Introduction: Neuroendocrine neoplasms (NENs) are rare and heterogeneous neoplasms with variable biological behaviour but generally slow progression.Methods/design: 355 patients with NENs registered in our data-base have been studied (166 females, mean age: 52 years; range: 11–88), during the period 2004–2014. TNM system has been used for staging and proliferation index Ki-67 for grading.Results: Out of 355 patients, 35 (...

Introduction: ACTH-secreting pituitary carcinomas are rare neoplasms but there are not factors till now to predict their aggressive evolution nor a standard practice to their management.Case report: We report a 43-year-old female patient presented in 1990 with Cushing’s syndrome due to ACTH-secreting pituitary microadenoma who had been submitted on trans-sphenoidal surgery with cure as defined by post-operative cortisol serum levels <50 nmol/l. ...

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disorder involving mainly parathyroid tumours, pancreatic neuroendocrine neoplasms (pNENs) and pituitary tumours. In the present study we have registered demographic, clinical, imaging, pathological characteristics, therapeutic options, response to treatment and overall outcome of patients with MEN1.Methods/design: 35 patients with MEN1 registered in our data base have been studied (14...

Introduction: Primary adrenal insufficiency (PAI) can be of autoimmune origin (AI), non-autoimmune origin (bilateral adrenalectomy, acute hemorrhage, infection) (NAI) or pharmaceutical (mitotane) origin. Studies have shown that these patients develop long-term morbidities and increased mortality due to excessive glucocorticoid tissue exposure. Data about differences concerning hydrocortisone (HC) needs, cortisol hormonal levels and metabolic parameters in these patients are sc...

Introduction: Neuroendocrine neoplasms (NENs) are neoplasms with a slow progression and a 5-year survival 77–95%. It is well known that secondary deposits of NENs may dedifferentiate during the natural history of the disease, presenting a more aggressive biological behaviour.Aim of the study: The prevalence of secondary foci dedifferentiation of pancreatic NENs (p-NENs).Methods: From the NENs database of 414 patients, 160 (38....

Background: The aim of the study was to assess the clinical features of gastric neuroendocrine neoplasms type 1 (GNEN1).Methods: We have analyzed the clinical data of the first 73 patients with GNEN1 from our registry.Results: The mean age(±S.D.) of the patients (51 (68.9%) females) and the mean follow-up were 55±12.3 years and 28.8±42.2months, respectively. All patients had sporadic tumors and all wer...