Neurofibromatosis (NF) is divided into two types, NF type 1 and NF type 2. Optic nerve gliomas have a high degree of association with NF type 1. NF 2, less commonly seen, is a complex of cutaneous and deep neural tumors. It is an autosomal dominant familial disorder in which CNS is affected in about 15% of the cases. Bilateral acoustic neuromas are pathognomonic of NF type 2 which may be associ...

We review the current status of 28 patients with chiasmal glioma who were originally reported on by Hoyt and Baghdassarian in 1969. The median period of follow-up was 20 years. Sixteen patients are now dead, five from chiasmal glioma and the rest from other causes. Four of the five deaths from chiasmal glioma occurred before 1969. The mortality rate in patients with neurofibromatosis and those ...

The effects of treatment of scoliosis in neurofibromatosis type 1 are less satisfactory than other scoliotic types due to the particular pathogenesis and clinical characteristics. Surgical treatment usually included 360 degrees fusion with instrumentation, but optimal strategies are different according to the degree of deformity. High incidence of pseudarthrosis, extensive bleeding and curve pr...

Gliomas are the most common infiltrative neoplasms of the optic nerve and can present as two distinct growth patterns: intraneural glial proliferation and perineural arachnoidal gliomatosis (PAG). It has been suggested that perineural arachnoidal gliomatosis is seen almost exclusively in the setting of neurofibromatosis type 1 (NF1). We describe a child with perineural arachnoidal gliomatosis o...

Neurofibromatosis type 1 is a relatively common inherited disorder. Patients have a high predisposition to develop both benign and malignant tumours. Although many manifestations of neurofibromatosis type 1 affect the nervous system, other organs and tissues can also be affected. Because of the varying features and clinical heterogeneity inherent to this disorder, patients can present to differ...

A variety of neoplasms may occur in association with neurofibromatosis type 1 (NF1). We describe a patient with NF1 and mycosis fungoides. Recommendations for the initial and long-term evaluations of patients with neurofibromatosis are presented.

Segmental neurofibromatosis is a rare subtype of neurofibromatosis type 1 (NF1). Glomus tumors are uncommon benign tumors. The authors report the association between these two rare conditions, not yet reported.

BACKGROUND Glomus tumors are painful benign tumors arising from the neuromyoarterial elements of the glomus body, typically in a subungual location. Historically, glomus tumors have been considered isolated or sporadic, not typically associated with other disease processes. Over the last few years, however, multiple case reports, a molecular genetics study, and an epidemiologic study have confi...

Neurofibromatosis type 1 is a common neurogenetic disorder characterized by significant clinical variability. As such, numerous studies have focused on identifying clinical, radiographic, or molecular biomarkers that predict the occurrence or progression of specific clinical features in individuals with neurofibromatosis type 1. One of these clinical biomarkers, macrocephaly, has been proposed ...

INTRODUCTION Collateral muscular artery aneurysm is exceedingly rare. We report the first case of subscapular artery aneurysm in a patient with type 1 neurofibromatosis and ipsilateral chronic subclavian artery occlusion. CASE PRESENTATION A 74-year-old Caucasian woman with a medical history of type 1 neurofibromatosis, presented a sudden left pectoral mass, later diagnosed as a ruptured aneu...