Neurofibromatosis type 1 is a complex autosomal dominant disorder caused by germline
mutations in the NF1 tumour suppressor gene. Nearly all individuals with neurofibromatosis …

Neurofibromatosis type 1 is a relatively common genetic disease, with a prevalence ranging
between 1/3000 and 1/6000 people worldwide. The disease affects multiple systems with …

Summary Background Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder,
predisposing development of benign and malignant tumours. Given the oncogenic potential …

This volume covers tumors of the pituary, the thyroid and parathyroid, the adrenal gland, the
endocrine pancreas, and inherited tumor syndromes. Each entity is extensively discussed …

The etiology of central precocious puberty (CPP) is multiple and heterogeneous, including
congenital and acquired causes that can be associated with structural or functional brain …

Neurofibromatosis type 1 (NF1) is one of the most common brain tumor predisposition
syndromes, in which affected children are prone to the development of low-grade gliomas …

Optic pathway glioma (OPG), seen in 15% to 20% of individuals with neurofibromatosis type
1 (NF1), account for significant morbidity in young children with NF1. Overwhelmingly a …

Background Suspicion of a CNS tumour is classically raised by symptoms of raised
intracranial pressure, focal deficits (including seizures), or papilloedema. Development of …

Purpose Optic pathway gliomas (OPGs) are the most common CNS tumor in
neurofibromatosis 1 (NF1) patients. We evaluated the long-term risk of second tumors in …

Low-grade gliomas (LGGs) are the most common childhood brain tumor in the general
population and in individuals with the Neurofibromatosis type 1 (NF1) cancer predisposition …