scholarly journals Neurofibromatosis type 2 and central neurofibromatosis

1998 ◽  
Vol 4 (3) ◽  
pp. E2 ◽  
Author(s):  
Leonard I. Malis
Keyword(s):  
United States ◽  
Nervous System ◽  
Genetic Research ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
The Past ◽  
Acoustic Neuromas ◽  
Central Neurofibromatosis ◽  
Considerable Range

Neurofibromatosis type 2 (NF2) is a rare disease, affecting only approximately 1000 patients in the entire United States. The diagnosis requires the presence of bilateral acoustic neuromas, but many other tumors of the nervous system are also present. It is a very different disease from von Recklinghausen's neurofibromatosis, NF1. The remarkable genetic research in recent years has defined the origin of NF2 to be the lack of a specific suppressor protein, known as Merlin. While we await a method to replace this protein, the neurosurgical care of these patients is a formidable problem. The author reviews his personal series of 41 patients with NF2 treated during the past 30 years and presents 10 cases in detail to demonstrate their considerable range of differences and the treatment problems they have posed.

scholarly journals Brief report on similar mutational changes in neurofibromatosis type 2 gene in minute pulmonary meningothelial-like nodule and meningioma of the central nervous system

Oncotarget ◽  
2018 ◽  
Vol 9 (89) ◽  
pp. 36012-36016
Author(s):  
Mitsunori Higuchi ◽  
Masayuki Watanabe ◽  
Takuya Inoue ◽  
Takumi Yamaura ◽  
Tomoko Suzuki ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
The Central Nervous System

scholarly journals Long-term Follow-up and Histological Correlation of Peripheral Nervous System Alterations in Neurofibromatosis Type 2

2021 ◽  
Author(s):  
Tim Godel ◽  
Philipp Bäumer ◽  
Said Farschtschi ◽  
Klaus Püschel ◽  
Barbara Hofstadler ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Nerve ◽  
Peripheral Nervous System ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Lesions ◽  
Peripheral Nerve Lesions

Abstract Purpose To examine long-term alterations of the dorsal root ganglia (DRG) and the peripheral nerve in patients with neurofibromatosis type 2 (NF2) by in vivo high-resolution magnetic resonance neurography (MRN) and their correlation to histology. Methods In this prospective study the lumbosacral DRG, the right sciatic, tibial, and peroneal nerves were examined in 6 patients diagnosed with NF2 and associated polyneuropathy (PNP) by a standardized MRN protocol at 3 T. Volumes of DRG L3–S2 as well as peripheral nerve lesions were assessed and compared to follow-up examinations after 14–100 months. In one patient, imaging findings were further correlated to histology. Results Follow-up MRN examination showed a non-significant increase of volume for the DRG L3: +0.41% (p = 0.10), L4: +22.41% (p = 0.23), L5: +3.38% (p = 0.09), S1: +10.63% (p = 0.05) and S2: +1.17% (p = 0.57). Likewise, peripheral nerve lesions were not significantly increased regarding size (2.18 mm2 vs. 2.15 mm2, p = 0.89) and number (9.00 vs. 9.33, p = 0.36). Histological analyses identified schwannomas as the major correlate of both DRG hyperplasia and peripheral nerve lesions. For peripheral nerve microlesions additionally clusters of onion-bulb formations were identified. Conclusion Peripheral nervous system alterations seem to be constant or show only a minor increase in adult NF2. Thus, symptoms of PNP may not primarily attributed to the initial schwannoma growth but to secondary long-term processes, with symptoms only occurring if a certain threshold is exceeded. Histology identified grouped areas of Schwann cell proliferations as the correlate of DRG hyperplasia, while for peripheral nerve lesions different patterns could be found.

Growth Rate Characteristics of Acoustic Neuromas Associated With Neurofibromatosis Type 2

1996 ◽  
Vol 106 (6) ◽  
pp. 694-699 ◽  
Author(s):  
Mona M. Abaza ◽  
Erini Makariou ◽  
Mark Armstrong ◽  
Anil K. Lalwani
Keyword(s):  
Growth Rate ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Acoustic Neuromas

Multiple central nervous system tumors, neurofibromatosis type 2, a case report

2015 ◽  
Vol 8 (1) ◽  
pp. 55-58
Author(s):  
Nedim Ongun ◽  
Eylem Değirmenci ◽  
Çağdaş Erdoğan ◽  
Attila Oğuzhanoğlu
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Central Nervous System Tumors ◽  
Nervous System Tumors

scholarly journals Targeted Therapies for the Neurofibromatoses

Cancers ◽  
2021 ◽  
Vol 13 (23) ◽  
pp. 6032
Author(s):  
Lauren D. Sanchez ◽  
Ashley Bui ◽  
Laura J. Klesse
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
The Past ◽  
Therapeutic Trials ◽  
Nerve Sheath

Over the past several years, management of the tumors associated with the neurofibromatoses has been recognized to often require approaches that are distinct from their spontaneous counterparts. Focus has shifted to therapy aimed at minimizing symptoms given the risks of persistent, multiple tumors and new tumor growth. In this review, we will highlight the translation of preclinical data to therapeutic trials for patients with neurofibromatosis, particularly neurofibromatosis type 1 and neurofibromatosis type 2. Successful inhibition of MEK for patients with neurofibromatosis type 1 and progressive optic pathway gliomas or plexiform neurofibromas has been a significant advancement in patient care. Similar success for the malignant NF1 tumors, such as high-grade gliomas and malignant peripheral nerve sheath tumors, has not yet been achieved; nor has significant progress been made for patients with either neurofibromatosis type 2 or schwannomatosis, although efforts are ongoing.

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