An understanding of the role of inherited cancer predisposition syndromes in pediatric tumor
diagnoses continues to develop as more information is learned through the application of …

Background Rhabdomyosarcoma (RMS) occasionally occurs in a context of a predisposition
syndrome. The most common predisposition syndromes include germline TP53 mutations …

Abstract Neurofibromatosis type 1 (NF1) is a complex neurocutaneous disorder with an
increased susceptibility to develop both benign and malignant tumors but with a wide …

Background DICER1 alterations are associated with intracranial tumors in the pediatric
population, including pineoblastoma, pituitary blastoma, and the recently described “primary …

Background Many children with neurofibromatosis type 1 (NF1) have focal abnormal signal
intensities (FASI) on brain MRI, whose full clinical impact and natural history have not been …

Background Focal abnormal signal intensities (FASI) on brain MRI occur commonly in
patients with neurofibromatosis type 1 (NF1). The natural history of cerebellar FASI and their …

Malignant peripheral nerve sheath tumors are rare tumors that originate from Schwann cells.
Patients with neurofibromatosis type 1 are prone to develop these tumors. Due to their rarity …

Objective: We report on the incidence and the gender, age and ethnic distribution of
sarcomas diagnosed between 1980 and 2008 in the multi-ethnic Republic of Suriname …

Abstract Neurofibromatosis Type 1 (NF1) is a neurocutaneous syndrome characterized by
multiple café-au-lait macules, neurofibromas, and predisposition to malignancies, including …

Summary Neurofibromatosis type 1 (NF1) is the most common phakomatosis and
neurofibromatosis, with an incidence of 1 per 2500 births. This autosomal dominant disease …