Endocrine Abstracts

Introduction: Bone metabolism is impaired in several lipid storage diseases. At now no data are available in Fabry disease (FD), a genetic lipid storage disease characterized by systemic accumulation of glicolipids. The aim of this study was to evaluate bone metabolism and calcium/vitamin-D pathway in patients with FD.Methods/design: Study population included 15 FD patients (8 M and 7 F, age 28–59 years) and 15 sex-, age- and BMI-matched normal subj...

Octreoscan is currently the gold standard for diagnosis of somatostatin receptor positive NET but it is limited by a lower spatial resolution and physiological uptake noises. DOTATATE is a somatostatin analogue, radiolabelled with 68Ga and adapted for PET imaging.Aim of this study was to evaluate the diagnostic performance of 68Ga-DOTATATE PET compared to Octreoscan in NET.Fifty-one patients with NET (40 sporadic, 17 MEN1) of diffe...

Somatostatin analogs (SSA) effectively control symptoms in neuroendocrine tumours (NET), besides showing antiproliferative activity. In progressive or metastatic NET, increasing SSA dose or shortening the dosing interval are common clinical practice, though empirical. Aim of this study is to evaluate efficacy and safety of high-dose SSA treatment in patients with progressive disease under standard SSA dose. Twenty-one patients (median age 56.8 years) with NET of different orig...

Introduction: Recent data suggest that metformin has antineoplastic properties in different type of cancer. Effects of metformin have never been investigated in neuroendocrine tumors (NET).We aim to determine the role of metformine on recurrence-free survival (RFS) in NET patients.Materials and methods: A retrospective analysis was conducted comparing NET patients with recent diagnosis (<3 year) of diabetes mellitus (with HbA1c...

Introduction: PHPT is a common endocrine disease characterized by hypercalcemia and different degree of osteoporosis and nephrolithiasis. PHPT arises in the context of an inherited disease in 5% of cases. Multiple endocrine neoplasia type 1 (MEN1) is the commonest cause of inherited PHPT. The main therapeutic approach for PHPT is surgery, however many patients refuse or have contraindications for surgery while others, in particular those with MEN1 PHPT, have persistent/relapsi...

Introduction: Targeted therapy with the multi-kinase inhibitor sorafenib is effective for treatment of differentiated thyroid cancer (DTC) unresponsive to RAI. Although kinase-inhibitors (KIs) are usually well tolerated, severe and even fatal adverse events are reported. Aim of the study was to assess incidence and characteristics of fatal events in patients with RAI-refractory DTC responsive to treatment with sorafenib.Design: A retrospective analysis o...

Introduction: Paragangliomas are neuroectodermal tumors that arise from adrenal medulla or extra-adrenal ganglia and are characterized by high vascularisation. A high rate of these tumours is genetically inherited. For malignant paragangliomas, chemo- and radio-therapy are potentially effective, but tumor response is of short duration and patient prognosis is quite poor. Sunitinib is a tyrosine-kinase inhibitor, targeting VEGFR1, -2, PDGFRα, -β, RET and c-Kit. Recent...

Introduction: Kinase-inhibitors (KIs) are effective for treatment of most aggressive endocrine cancers. The crucial point about treatment with KIs is that these agents are not curative and their effects are at best transitory and are always followed by a restoration of tumour growth and progression. Several retrospective and phase II studies demonstrated efficacy of both sorafenib and sunitib for treatment of iodine refractory differentiated thyroid cancer although results fro...

Introduction: Medullary thyroid cancer (MTC) is a well-differentiated neuroendocrine tumor in which somatostatin receptor (sst) expression is higher for sst1 and sst5 than for sst2. This may explain why the available sst2–selective analogues do not work in these patients and why pasireotide (SOM230), a novel, multi-receptor targeted somatostatin analogue with high-binding affinity for sst1,2,3 and sst5 coul...

Context: Primary hyperparathyroidism (PHPT) is a common endocrine disease, characterized by the chronic elevation of serum calcium (Ca) levels induced by a long-standing increase of PTH concentrations. PHPT includes mild-asymptomatic and symptomatic forms. Cinacalcet is effective in lowering serum Ca levels in PHPT, but is indicated solely in mild-asymptomatic PHPT meeting surgery criteria. Management of non-surgical mild-asymptomatic PHPT is still a debated issue.<p class...