Endocrine Abstracts

Insulinoma, a pancreatic β-cell tumor, comprises 55% of Neuroendocrine tumors with an annual incidence of 3–10/million. Female to male ratio is 3:2 presenting in their 4th decade. The main clinical symptom is severe hypoglycemia irrespective of tumor size.Aim: Presentation of four newly diagnosed insulinoma patients aged 18–60, within an eight month interval.All patients presented with neuroglycopenic symptoms (irrit...

Medullary thyroid carcinoma (MTC) early spreads to cervical lymph nodes and to distant sites: mostly to the liver, lungs or bones. Unusual sites such as breast, skin or orbit have been also described. The aim of the study was to evaluate the frequency, localisation of unusual metastases of MTC and their impact on survival.Eligibility criteria for this study were the presence of a pathological confirmed diagnosis of MTC with distant metastases (other than...

Context: The post-surgical follow-up of malignant insulinomas is often marked by recurrence after initial remission. However, the criteria for the diagnosis of recurrence of these malignant endocrine tumors are not well defined in the literature, unlike diagnostic of criteria for hyperinsulinemic hypoglycaemia (Cryer 2009). We retrospectively reviewed the criteria that allowed the diagnosis of recurrence in four patients with malignant insulinoma after initial post-operative r...

The embryonic transcription factor TBX1 plays a critical role in cell differentiation during organogenesis of the parathyroid glands. Here we demonstrated that TBX1 mRNA and protein were detected in human adult normal parathyroid tissues (n=3). Immunostaining showed that TBX1 protein was expressed in endothelial CD31+, alfa-SMA+ cells. By contrast, TBX1 mRNA and protein was expressed at higher levels in parathyroid typical adenomas (n=23), where TBX1 staining was...

Introduction and objective: Neuroendocrine tumors (NETs) are a rare kind of tumor that can have various locations. Pancreatic location is the most common after the gastrointestinal tract. The aim of this report is to study the characteristics of neuroendocrine tumors located in the pancreatic gland (pNETs) at diagnosis.Material and methods: Descriptive and retrospective study about 22 patients diagnosed of pNET during the period 1993–2010 in our hos...

Introduction: The Birt–Hogg–Dube syndrome (BHDS) is a rare autosomal dominant genodermatosis characterised by presence of at least one of the following: benign skin fibrofolliculomas, lung cysts with recurrent pneumothoraces or renal tumours. The phenotypic constellation is due to heterozygous germline mutations of the tumour suppressor gene – FLCN, located on 17p11.2, encoding a 579 aminoacid protein termed folliculin. Case reports have described endocrine tumo...

Introduction: The adrenal incidentaloma is defined as a mass lesion found unexpectedly in an adrenal gland by an imaging procedure performed for another reason than suspected adrenal pathology.Objective: To estimate the incidence of the adrenal mass and to define the algorithm for managing patients with incidental adrenal mass; a comparative analysis to the treatises data. The observational study was set one’s face towards by the adrenal incidental ...

Only a few cell lines are available for endocrine tumors which furthermore do not reflect heterogenous functional properties and specific therapeutic response rates of individual tumors. To facilitate patient individual treatments and thereby to optimize therapeutic efficacy, we are aiming at the development and characterisation of patient-individual tumor models. Pieces of surgical tumor specimen from four adrenocortical carcinomas, one aldosterone producing adenoma, one pheo...

Medical treatment of adrenocortical carcinoma (ACC) is limited to common cytotoxic agents, which are usually given in combination with mitotane. Recently we have developed a novel therapeutic approach by coupling a monoclonal IGF1 receptor blocking antibody (1H7) to sterically stabilized liposomal doxorubicin (SSLD). 1H7 coupled liposomes showed in vitro high and significant cellular association and furthermore internalization in various human tumor cell lines as well a...

Introduction: The two major long-acting somatostatin analogues (SSA) available on the European market for the treatment of neuroendocrine tumours and acromegaly are Somatuline Autogel (SA) and Sandostatin LAR (LAR). The aim of this study was to gain nurses’ insights on the use and ease of administration of SSA devices, including Somatuline Autogel new device (SA-ND), in Europe and the US.Methods/design: Seventy-seven qualified nurses with ≥3 S...

Introduction: ACTH is measured to confirm impairments in the hypothalamic–pituitary–adrenal axis. Patients with small cell lung cancer (SCLC) can have ectopic adrenocorticotropic secretion. In December 2009, a SCLC patient showed an enormous discrepancy between the ACTH results obtained with two different ACTH assays. The aim of this study was to compare different ACTH assays in SCLC patients.Methods: In a pilot study EDTA plasma samples of nin...

Background: Primary hyperparathyroidism (pHPT) is the most prevalent manifestation of multiple endocrine neoplasia type 1 (MEN1). Surgery is the preferred treatment but which type of surgery offers the best chance of cure combined with the lowest risk of postoperative hypoparathyroidism is still uncertain.Aims: Identifying the optimal surgical technique for MEN1 related pHPT. Describing the course of postoperative hypoparathyroidism. To see if a genotype...

Background: Whereas many papers have reported on clinical and morpho pathological features of thyroid cancer with BRAFV600E mutation, a limited number of studies have been focused on the quantification of BRAF mRNA expression in thyroid tumors.The present study aimed to assess the expression of BRAF transcript in fine needle aspiration specimens, prelevated from cases with nodular thyroid disease.Material and methods: Th...

Introduction: Positron emission tomography (PET) using 6-[F-18]fluoro-L-dihydroxyphenylalanine (18F-DOPA) has an excellent sensitivity to detect carcinoid tumor lesions. 18F-DOPA-tumor uptake and the levels of biochemical tumor markers are mediated by tumor endocrine metabolic activity. Therefore we evaluated whether total 18F-DOPA-tumor uptake on PET, defined as the whole-body metabolic burden (WBMTB) reflects tumor load per pat...

Introduction: Incidence of ectopic parathyroid glands in individuals is ~6%, the most common location being the thymic capsule or the superior mediastinum.Case: We present the case of 21-years-old female with a recent history of osteoclastoma affecting the maxillary bone and the mandible, together with increased values of the parathyroid hormone (over 20 times the normal value), total calcium and alkaline phosphatase and decreased serum phosphorus. A cer...

Introduction: Pancreatic neuroendocrine tumors (PNETs) are generally clinically more indolent than adenocarcinomas. But relevant, clinically applicable prognostic survival factors still need to be more precisely defined.Aim: To identify survival factors in patients with PNETs.Methods: We analyzed 66 patients with PNETs (52.62±13.3 years old) treated at our department during 2003–2010. Patients with insulinomas were not in...

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Objective: To assess outcome of stage III–ENSAT adrenocortical carcinoma (ACC) patients after complete macroscopic resection and prognostic factors for disease-free survival (DFS) and overall survival (OS).Methods: A retrospective review of 68 stage III ACC patients treated surgically between 1994 and 2009 in the French COMETE network was performed. Characteristics of patients were reviewed by a single investigator as well as the Weiss score by expe...

The recent unavailability of pentagastrin (Pg) in several European Countries, prompted us to evaluate the potency and tolerability of the ‘high dose calcium test’ (Ca) in the stimulation of serum human calcitonin (hCt). We compared Pg- and Ca-stimulated hCt in 19 patients already treated for medullary thyroid cancer (MTC) in remission (n=19) or in persistence (n=13), in 18 patients with chronic thyroiditis and multinodular goiter, and in 15 healthy cont...

Introduction: Adrenocortical carcinoma (ACC) is a rare tumor of the adrenals with poor prognosis (survival rate for metastatic patients <15% at 5 years). Mitotane (o,p′-DDD) is the main therapeutic option with up to 66% objective response rates in patients with serum levels between 14 and 20 mg/l. Its two main metabolites are o,p′-DDA and o,p′-DDE which plasma levels are not correlated to therapeutic response. Mitotane is a lipophilic drug that accumulate...

Background and objective: 111In-DPTA-Octreotide (Octreoscan) has limited spatial resolution and a somewhat lower receptor affinity of the radiopeptide compared to DOTATATE, a novel somatostatin analogue, which can be radiolabelled with 68Ga and adapted for PET imaging, resulting in an increased spatial resolution. The aim of this study was to evaluate the diagnostic performance of 68Ga-DOTATATE PET in patients with NET and to compare this tecni...

Ovarian carcinoma (OC) is associated with poor prognosis and low survival rate. Eligible treatment for advanced OC is surgery followed by chemotherapy based on a combination of Platinum and Taxol, but unfortunately long-term outcome of such therapy is disappointing. Moreover, systemic administration of cytotoxic agents is usually accompanied by a series of side effects. On the basis of this evidence, more selective and tumour cell-targeted therapeutic approaches are warranted....

Medullary thyroid carcinoma (MTC) accounts for ~5–10% of thyroid cancers. Intense efforts are currently directed toward the identification of new druggable targets for the treatment of MTC. The aim of this study was to investigate whether drugs acting at voltage-gated T-type calcium channels could affect hormone release and/or cell proliferation in a MTC cell line, the TT cells. The expression of the three isoforms of T-type calcium channels (CaV3.1, CaV3.2 and CaV3.3) wa...

Background: Type 3 neuroendocrine tumors of the stomach (type 3 NETV) are very rare subtypes of NETV with poor prognosis, not fully understood biology and not well investigated diagnostic and therapeutic procedures. The aim of the study was to analyze tumor biology and the outcome of differentiated treatment in patients with type 3 neuroendocrine tumors of the stomach.Methods: A prospective 10 years follow up of five patients with type 3 NETV treated in ...

Introduction: GLP-1 receptors have been fund on different types of neoplasm’ cells and density of these receptors appears to be more frequent in some of them. GLP-1 receptor scanning offers a new approach for successful localization of small or not detectable by other methods insulinomas and other cancers such as MTC, gastrinoma, pheochromocytoma, gastrinoma, etc.Aim of the study: The aim of study was to present first successful experiences with the...

Background: Patients with advanced NET have limited treatment options. In the phase III RADIANT-2 study of patients with advanced low- or intermediate-grade NET and a history of carcinoid syndrome, everolimus, an oral mTOR inhibitor, + octreotide LAR provided a clinically meaningful 5.1-month increase in median progression-free survival (PFS) compared to placebo + octreotide LAR (ESMO 2010 Abstract #LBA8). An updated safety analysis from this trial is presented.<p class="a...

Background: In the phase III RADIANT-3 trial, everolimus, an oral mTOR inhibitor, demonstrated superiority in progression-free survival with a median of 11.0 vs 4.6 months for placebo (hazard ratio, 0.35; 95% confidence interval, 0.27 to 0.45; P<0.0001) in patients with advanced pNET (ESMO 2010, Abstract #LBA9). Here we present an update of the safety analysis from this trial.Methods: Patients with progressive advanced low- or intermediate-gra...

Adrenocortical carcinoma (ACC), a rare tumor, with incidence of 1–2 per million population annually, has a bimodal distribution by age, with cases clustering in children under 6, and in adults 30–40 years old. ACC has a dismal prognosis. The only curative treatment is complete surgical excision of the tumor, but late diagnosis prevents surgical cure, since ACC frequently recurs and metastasize. Chemotherapy is frequently ineffective, due to the overexpression of the ...

Purpose: The efficacy and safety of RAD, an mTOR inhibitor, in patients with well differentiated metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NET).Material and methods: We studied seven male patients (mean age 55 years, range 36–75) with well differentiated non functioning stage IV GEP-NETs (five pancreatic, one hindgut carcinoid, and one NET of unknown primary). All received RAD 10 mg/day in combination with long acting octerotide 3...

Background: The exact indications and/or the type of operation requiring for the treatment of gastric carcinoids (GC) type 1, are not clearly stated. The present study was designed to evaluate the value of the oncological gastrectomy in the treatment of GC type 1.Materials and method: Between January 2004 and December 2010, we advocated gastric resection in cases of GC type 1 when at least one of the following criteria was present: i) multiple recurrent ...

Background: Neuroendocrine tumors awareness is developing. Carcinoid syndrome and hormonal overproduction may be controlled with somatostatin analogues in majority of cases. However, due to unspecific symptomatology in early stages of disease, majority of cases are diagnosed when process is disseminated. Co-morbidities and coexisting diseases are present in most of patients. Bone loss may be accelerated by metastizing tumor, peptides secreted or decreased absorption of vitamin...

Introduction: Neuroendocrine tumors (NET) are rare tumors originating from various types of neuroendocrine cells. The somatostatin receptor type 2 (sst2) is expressed in NET cells and is a target for therapy with somatostatin analogues.Epigenetic changes including methylation of DNA at CpG dinucleotides, and particularly in the promoter region of genes can inhibit transcription. Methylation can be inhibited by cytidine analogues such as 5-aza-...

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by variable combination of more than 20 types of endocrine and non-endocrine tumors: typically parathyroid glands, pancreatic islet cells and anterior pituitary gland are involved. We studied 20 patients (10M and 10F), affered to our Pituitary Unit in a period of 10 years. All patients had clinical diagnostic criteria suspected for MEN1 (presence of two endocrine tumors at least) and und...

PRRT is a treatment choice for inoperable or metastasized neuroendocrine tumours and this therapy seems more effective in the biochemical and volume control of disease than SSA. We investigated the role of medical therapy with SSA and PRRT in patients with well and moderately differentiated neuroendocrine tumours. We evaluated 55 patients with well or moderately differentiated neuroendocrine tumours; 31 patients with either metastatic, unresectable or functioning tumours were ...

The incidental discovery of adrenal tumors in the course of computer tomography scanning for various reasons has led to the more detailed study of this type of tumors.The aim was to study the anatomical distribution of primary adrenal tumors at diagnosis.The archives of all patients with primary adrenal tumors having been hospitalized in the Department of Endocrinology and Metabolism of Metaxa Hospital, Pireaus, Greece from 01.01.2...

Estrogen exposure has been linked to a risk for the development of testicular germ cell cancers. The effects of estrogen are now known to be mediated by estrogen receptor α (ERα) and ERβ subtypes, but only ERβ has been found in human germ cells of normal testis. However, its expression was markedly diminished in seminomas, embryonal cell carcinomas, and in mixed germ cell tumours but remains high in teratomas. PATZ1 is a recently discovered zinc finger prot...

Introduction: The management of a thyroid nodule in a patient who is newly diagnosed with an active cancer depends not only of the thyroid disease its self but also of the prognosis of the second neoplasia.Aim: We present a case of a male who was admitted to an endocrinologist for a thyroid nodule evaluation.Case presentation: B.C., 66-year-old male patient, smoker from the last 17 years, was treated with thyamazol for hyperthyroid...