Endocrine Abstracts

Pancreatic endocrine tumors (EPT) may occur sporadically or in association with the rare autosomal dominantly inherited tumor syndromes; multiple endocrine neoplasia type 1 (MEN1) and von Hipple Lindau (VHL). The genes causing these syndromes have been identified, and genotyping is possible which enables the laborious clinical investigations for diagnosis of lesions to be restricted to 50% of family members. For MEN1, no clinically useful genotype-phenotype correlation as been...

Introduction: Longitudinal changes in pancreatic neuroendocrine tumor (panNET) cell proliferation correlate with fast disease progression and poor prognosis. The optimal treatment strategy for secondary panNET grade (G)3, that has progressed from a previous low- or intermediate-grade to high-grade panNET G3 is currently unknown. Methods: This was a single center retrospective cohort study, aimed to characterize treatment patterns and outcomes among patie...

Background: Homozygous inactivation of the MEN1 tumor suppressor gene frequently occurs in endocrine pancreatic tumors (EPT); however, a heterozygous germ line inactivation of the gene seems to lead to development of an increase amount of endocrine pancreatic cells. The Notch signaling cascade plays a vital role in sustaining the balance between cell proliferation, differentiation and apoptosis during pancreatic development. Whether Notch signaling is MEN1 depend...

Background: Apart from inactivation of the MEN1 gene, molecular events essential for tumorigenesis of the endocrine pancreas are poorly characterized. A potentially useful approach for understanding tumor progression is to study transcription factors operating in fetal pancreatic development. The Notch signaling cascade with expression of the transcription factors Hes1, Hey1, and Ascl1 plays a vital role in sustaining the balance between cell prolif...

Background: No randomized trials have been conducted in adrenocortical carcinoma (ACC) patients. Treatment recommendations for this rare but aggressive disease have been based on data from small phase II trials. We have now performed the first randomized phase III trial, comparing etoposide, doxorubicin, cisplatin, plus mitotane (EDP-M) against streptozotocin plus mitotane (Sz-M).Methods: Three-hundred and four chemotherapy-naive patients with ACC not am...