Results 191 to 200 of about 4,213 (222)

Familial schwannomatosis

Neurology, 2003
Schwannomatosis is a recently recognized disorder, defined as multiple pathologically proven schwannomas without vestibular tumors diagnostic of neurofibromatosis 2 (NF2). Some investigators have questioned whether schwannomatosis is merely an attenuated form of NF2.The authors identified eight families in which a proband met their diagnostic criteria ...
M, MacCollin, C, Willett, B, Heinrich, L B, Jacoby, J S, Acierno, A, Perry, D N, Louis   +6 more
openaire   +2 more sources

Schwannomatosis: Multiple Schwannomas of the Upper Limb

Journal of Hand Surgery, 2006
Schwannomas (also known as neurilemmomas) are neoplasms of the peripheral nerve sheaths. They are the most common benign tumour of peripheral nerves, yet they account for only 5% of all soft tissue tumours. Their incidence in the upper limb is variable, as is their clinical presentation. We present an unusual case of multiple schwannomas in the upper
S, Hasham, P, Matteucci, P R W, Stanley
openaire   +2 more sources

Salvage Microsurgery After Failed Bevacizumab Treatment for NF2-Related Schwannomatosis Vestibular Schwannoma: A Multicentric Retrospective Study.

Neurosurgery
BACKGROUND AND OBJECTIVES Surgery in NF2-related schwannomatosis (NF2-SWN) vestibular schwannoma (VS) carries a higher risk of facial nerve damage, hearing loss, and partial resection, than in sporadic cases.
Benoit Hudelist, A. King, John P. Marinelli, J. T. Roland, O. Pathmanaban, S. Raza-Knight, Michael Bartellas, Daniele Bernardeschi, M. Link, J. Golfinos, Matthew L. Carlson, D. G. Evans, Michel Kalamarides   +12 more
semanticscholar   +1 more source

GsMTx-4 Reduces Mechanical Allodynia in a Model of Schwannomatosis-related Pain.

Journal of Pain
Patients with schwannomatosis (SWN) develop multiple tumors, called schwannomas, along peripheral nerves, with most experiencing significant pain. Neuropathic, nociceptive, and inflammatory pain types have been reported, but many patients describe severe
Carson Gutierrez, Randy Rubright, K. Ostrow   +2 more
semanticscholar   +1 more source

Manejo actual y avances en el tratamiento de la Schwannomatosis Vestibular

Mediciencias UTA
Introducción: la schwannomatosis vestibular (SV), también conocida como neurinoma del acústico o NF2, es una mutación genética en el gen NF2 ubicado en el cromosoma 22, se caracteriza por perdida de la función de una proteína supresora de tumores que ...
Javier Aquíles Hidalgo Acosta, Wagnner Ariol Intriago Zambrano, Israel Luis Villavicencio Quevedo, Evelyn Lucero Capuz Balladares, Mónica Piedad Moran Ayala   +4 more
semanticscholar   +1 more source

INNV-09. Disease stabilization in atypical schwannomatosis with BAP1 and PIK3CA mutations treated with olaparib and alpelisib

Neuro-Oncology
Atypical schwannomatosis is a rare, non-syndromic peripheral nerve sheath tumor disorder characterized by multiple schwannomas, often with uncertain malignant potential. Malignant transformation and metastatic behavior are exceptionally rare, and there
Osama Elzaafarany, Catherine Boldig, Vincent Dlugi, Sepideh Mokhtari   +3 more
semanticscholar   +1 more source

EPID-13. Are patients with LZTR1-related schwannomatosis at increased risk for gliomas?

Neuro-Oncology
Somatic LZTR1 variants were initially identified in glioblastomas (GBM). Schwannomatosis (SWN) is a rare neurogenetic tumor suppressor syndrome in which affected individuals have a predisposition to multiple schwannomas and chronic pain.
Anna Hauswirth, J. Jordan, S. Plotkin
semanticscholar   +1 more source

The Association Between Age and Outcomes of Bevacizumab Treatment in NF2-Related Schwannomatosis.

Otology and Neurotology
OBJECTIVE NF2-related schwannomatosis (NF2-SWN) is an autosomal dominant genetic disorder characterized by the development of schwannomas, meningiomas, and spinal ependymomas. Treatment with bevacizumab, a monoclonal antibody against VEGF, has been shown
Maya G Hatley, K. Yohay, J. T. Roland, Devorah Segal   +3 more
semanticscholar   +1 more source

CTNI-50. UPDATED RESULTS ON BRIGATINIB TREATMENT FOR PROGRESSIVE TUMORS IN PATIENTS WITH NF2 -RELATED SCHWANNOMATOSIS: A SUB-STUDY OF THE INTUITT-NF2 TRIAL

Neuro-Oncology
Brigatinib is an oral ALK inhibitor that inhibits multiple tyrosine kinases. We previously published outcomes of patients with NF2-related schwannomatosis treated with brigatinib for a median of 10.4 months.
S. Plotkin, K. Yohay, P. Nghiemphu, Christine Dinh, D. Babovic-Vuksanovic, Vanessa Merker, Annette Bakker, Geoffrey Fell, L. Trippa, J. Blakeley   +9 more
semanticscholar   +1 more source

[Neurofibromatosis versus schwannomatosis].

Fortschritte der Neurologie-Psychiatrie, 1998
Neurofibromatosis Type 1 and 2 (NF1 und NF2) are different forms of neurofibromatosis, well defined both clinically and genetically. In absence of typical clinical features of NF1 (café-au-lait-spots, cutaneous neurofibromas, Lisch-nodules) or NF2 (vestibular schwanoma) clinical classification is often not possible. Neurofibromas are more common in NF1
V F, Mautner, S, Schröder, S M, Pulst, H, Ostertag, L, Kluwe   +4 more
openaire   +1 more source