Endocrine Abstracts

The usefulness of long-term medical treatment with somatostatin analogs has not been evaluated in insulinomas. 22 patients with hypoglycaemia related to endogenous hyperinsulinism (62±21 years, M±S.D., 27–88 years) were not treated by surgery: aged patients (n=10), patients with malignant unresectable insulinomas (n=6; locally invasive, n=1; multimetastatic, n=5), multiple insulinomas (n=2), diffuse beta cell pat...

Introduction: Abnormalities of glycoregulation are frequent in acromegaly (15-38%) secondary to insulin resistance related to GH/IGF-1 hyper-secretion. The frequency of gestational diabetes (GD) in women with acromegaly was reported in the French multicenter study (JCEM, 2010, 95, 4680) as increased mainly in the absence of pre-gestational control of GH/IGF-1 hyper-secretion. The aim of this study was to evaluate the frequency of GD in a cohort of acromegalic women, and compar...

Introduction: SDH genes mutations are associated with hereditary phaeochromocytoma and paraganglioma syndromes. We describe the case of a patient with SDHC related familial paraganglioma and pituitary adenoma.Case: A 65-year old man consulted for an incidentally discovered 7 cm abdominal mass on CT-scan, lateral to the right kidney, invading inferior vena cava, associated to a retroperitoneal adenomegaly and a lesion on the body of L2 vertebra with spina...

Introduction: In women with GH-secreting pituitary adenoma, a decrease of IGF1 levels has been reported during the first trimester of pregnancy, before placental GH (pGH) secretion inducing a progressive increase in IGF1 levels throughout gestation. This decrease has been related to hepatic GH-resistant state, via JAK2/STAT pathway, due to increased estrogen (E2) levels.Objectives: Evaluate IGF1 change in non-acromegalic women during the first...

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...

Metastases in the thyroid gland are very rare and their true incidence has not been clearly established. There are few reports of metastases to this gland and most of them are not detected in clinical practice.Although detection of metastases to the thyroid gland usually indicates poor prognosis aggressive surgical and medical treatment in isolated thyroid metastases may be effective as described for renal carcinoma.Malignant melan...

Introduction: The French National Institute of Cancer (INCa) launched supported by the Ministry of Health in 2008 a program for the recognition of national networks for the management of rare cancers. Among the 23 selected networks COMETE-Cancer was recognized by INCa in 2009 for Adrenocortical carcinoma (ACC) and malignant pheochromocytoma/paraganglioma (MPP). At that time 60–120 new ACC/year and 30 new MPP/year were expected at the national level....

The cortisol secretion level of adrenocortical adenomas range from hormonally silent to overt hypercortisolism. The mechanisms leading to the autonomous hypersecretion of cortisol are unknown. The aim was to identify the gene expression alterations associated with the autonomous and excessive cortisol secretion of adrenocortical adenomas.Methods: The transcriptome of 22 unilateral adrenocortical adenomas (5 non-secreting, 6 subclinical cortisol-producing...

Background: Cushing syndrome due to PPNAD is the main endocrine disorder of CNC, an autosomal dominant multiple neoplasia caused by germline inactivating mutations of the subunit type 1A (PRKAR1A) of the protein kinase A (PKA). In addition, germline inactivating mutations in the gene encoding phosphodiesterase 11A (PDE11A) have been identified in patients with PPNAD.Aim of the study: To investigate the role of PDE11A genetic alterati...

In a monocentric study on 48 patients with insulinomas treated between 1988 and 2008, we evaluate the results of the radiological and surgical procedures, and determine prognostic factors regarding the recurrence risk. Clinical, radiological, and histopathological findings were analysed along with long-term follow-up after surgery. Kaplan–Meier analysis studies recurrence-free survival, and uni- and multi-variable analyses determine prognostic factors related to recurrenc...