Summary von Hippel-Lindau disease is a heritable multisystem cancer syndrome that is
associated with a germline mutation of the VHL tumour suppressor gene on the short arm of …

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with
a neuroendocrine phenotype. The old and the new WHO classifications distinguish between …

The diagnosis of pancreatic neuroendocrine tumours (PanNETs) is increasing owing to
more sensitive detection methods, and this increase is creating challenges for clinical …

Pancreatic endocrine tumors (PETs) have long fascinated clini-cians, because some can
release biologically active hormones that cause distinct syndromes and provide important …

Endocrine pancreatic tumours (EPTs) are uncommon tumours occurring in approximately 1
in 100 000 of the population, representing 1–2% of all pancreatic neoplasms. Some of the …

Understanding of molecular mechanisms of tumor growth has an increasing impact on the
development of diagnostics and targeted therapy of human neoplasia. In this review, we …

Mutations in the von Hippel-Lindau (VHL) gene are responsible for VHL disease, congenital
polycythemia, and are found in many sporadic tumor types as well. Reports of VHL …

Background & Aims: Pancreatic involvement in von Hippel-Lindau (VHL) disease, a genetic
disorder with a dominant mode of inheritance affecting various organs, has rarely been …

Context.—Gastrointestinal (GI) and pancreatobiliary tracts contain a variety of
neuroendocrine cells that constitute a diffuse endocrine system. Neuroendocrine tumors …

Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the
pancreas, are among the most common neuroendocrine tumors. The genetic causes of …