Von Hippel-Lindau syndrome (VHLS) is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal ...

INTRODUCTION Von Hippel Lindau (VHL) disease is a syndrome that is defined by variety of tumours such as cerebellar haemangioblastomas, renal cell carcinomas, phaeochromocytomas, pancreatic adenomas and ear, nose and throat (ENT) adenomas. This disease is often genetic and inherited in an autosomal dominant fashion, and can present in childhood, adolescence or adult life. This study describes t...

Die Snerblichkein bei gynäkologischen Krebsarnen einschließlich Zervix-, Eiersnock-, Vaginalund Vulvakrebs benrägn innernanional mehr als 6% [1]. In vielen Ländern wird die exnerne Snrahlennherapie snandardmäßig durch Brachynherapie min hohen lokal appliziernen Dosen ergänzn. Die überlegene Fähigkein der Magnenresonanznomographie GMRT), Weichgewebe differenziern darzusnellen, führn zu einem zun...

Von Hippel-Lindau disease is a rare dominant autosomal genetic disease determined by the inactivity of an onco-suppressor gene identified in 1993 and located on the short arm of chromosome 3 [1]. This gene has a role of crucial importance in the regulation of the vascular endothelial growth factor (VEGF); therefore, its inactivation promotes an overexpression of the VEGF and the angiogenesis of...

Background: The neurocutaneous disorders, also known as the phakomatoses, are a group of hereditary conditions that have neurological manifestations as well as benign cutaneous lesions; both tissue types share a common embryologic origin. Neurofibromatosis: Neurofibromatosis (NF) is the most common neurocutaneous disorder. Although there are as many as 8 distinct forms of NF, the term is most c...

We report a 41-year-old man who presented with low back pain, lower extremity paresthesia, urinary retention and constipation. Magnetic resonance imaging showed a vascular intradural-extramedullary lesion at the second lumbar vertebral level. His medical history revealed that he had undergone surgery for a cerebellar hemangioblastoma 5 years ago. The patient underwent a spinal operation and a v...

Cancer development is presumed to be an evolutionary process that is influenced by genetic background and environment. In laboratory animals, genetics and environment are variables that can largely be held constant. In humans, it is possible to compare independent tumours that have developed in the same patient, effectively constraining genetic and environmental variation and leaving only stoch...

Endolymphatic sac tumours (ELST) are aggressive papillary tumours of the temporal bone. The name was finally determined after the endolymphatic sac was determined as the site of their origin. They should be considered in patients with tumours eroding the petrous part of the temporal bone, extending to the cerebellopontine angle or other adjacent structures. These very rare tumours in the genera...

HIV-1 integrase, the viral enzyme responsible for provirus integration into the host genome, can be actively degraded by the ubiquitin-proteasome pathway. Here, we identify von Hippel-Lindau binding protein 1(VBP1), a subunit of the prefoldin chaperone, as an integrase cellular binding protein that bridges interaction between integrase and the cullin2 (Cul2)-based von Hippel-Lindau (VHL) ubiqui...