neurofibromatosis type 1, also known as von recklinghausen disease, is an autosomal dominant disorder with an incidence of 1 per 4000. neurofibromas are benign, heterogeneous tumors arising from the connective tissue of peripheral nerve sheaths, especially the endoneurium. visceral involvement in disseminated neurofibromatosis is rare. neurofibroma occurs most frequently in the stomach and jeju...

Neurofibromatosis type 1 and type 2, affecting both children and adults, often results in devastating complications. The rapid unravelling of the genetic underpinnings of these unique disorders has led to the development of novel therapies, especially molecular-targeted therapies. To facilitate clinical trial development, the Neurofibromatosis Clinical Trial Consortium (NFCTC) was established i...

In a group of 28 children with neurofibromatosis type 1 aged between 4 and 16 years, neuroradiological findings were correlated with intelligence as measured by the Wechsler scales. The presence or specific location in the brain of T2 weighted prolonged signals on MRI was not associated with cognitive problems. No other physical characteristics associated with neurofibromatosis type 1 were foun...

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...

Neurofibromatosis (NF) is a genetic and multisystemic disease with autosomal dominant transition. It can affect anesthesia applications by affecting more than one system. In the presence of neurofibromas in airway, it cause airway obstruction difficulties respiratory delivery during general anesthesia. The tumors central nervous system makes spinal risky. Anesthesiologists should act awareness ...

3. Side, L, Taylor B, Cayouette M , et al. Homozygous inactivation of the NF1 gene in bone marrow cells from children with neurofibromatosis type 1 and malignant myeloid disorders. N Engl J Med 1997; 336: 1713-20. 4. Matsui I, Tanimura M, Kobayashi N, Sawada T, Nagahara N, Akatsuka J. Neurofibromatosis type 1 and childhood cancer. Cancer 1993; 72:2746-54. 5. Friedman JM, Birch PH. Type 1 neurof...

BACKGROUND In Neurofibromatosis type 1, cerebral Unidentified Bright Objects are a well-known benign entity that has been extensively reported in the literature. In our case series, we wish to focus on a further possible location of such lesions, the spinal cord, which we have defined as medullary Unidentified Bright Objects. These have been, to our knowledge, scarcely described in previous wor...

Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle c...