scholarly journals Long-term natural history of neurofibromatosis Type 2–associated intracranial tumors

2012 ◽  
Vol 117 (1) ◽  
pp. 109-117 ◽  
Author(s):  
Michael S. Dirks ◽  
John A. Butman ◽  
H. Jeffrey Kim ◽  
Tianxia Wu ◽  
Keaton Morgan ◽  
...  
Keyword(s):  
Growth Rate ◽  
Natural History ◽  
Growth Pattern ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Intracranial Tumors ◽  
History Of

Object Neurofibromatosis Type 2 (NF2) is a heritable tumor predisposition syndrome that leads to the development of multiple intracranial tumors, including meningiomas and schwannomas. Because the natural history of these tumors has not been determined, their optimal management has not been established. To define the natural history of NF2-associated intracranial tumors and to optimize management strategies, the authors evaluated long-term clinical and radiographic data in patients with NF2. Methods Consecutive NF2 patients with a minimum of 4 years of serial clinical and MRI follow-up were analyzed. Results Seventeen patients, 9 males and 8 females, were included in this analysis (mean follow-up 9.5 ± 4.8 years, range 4.0–20.7 years). The mean age at initial evaluation was 33.2 ± 15.5 years (range 12.3–57.6 years). Patients harbored 182 intracranial neoplasms, 164 of which were assessable for growth rate analysis (18 vestibular schwannomas [VSs], 11 nonvestibular cranial nerve [CN] schwannomas, and 135 meningiomas) and 152 of which were assessable for growth pattern analysis (15 VSs, 9 nonvestibular CN schwannomas, and 128 meningiomas). New tumors developed in patients over the course of the imaging follow-up: 66 meningiomas, 2 VSs, and 2 nonvestibular CN schwannomas. Overall, 45 tumors (29.6%) exhibited linear growth, 17 tumors (11.2%) exhibited exponential growth, and 90 tumors (59.2%) displayed a saltatory growth pattern characterized by alternating periods of growth and quiescence (mean quiescent period 2.3 ± 2.1 years, range 0.4–11.7 years). Further, the saltatory pattern was the most frequently identified growth pattern for each tumor type: meningiomas 60.9%, VSs 46.7%, and nonvestibular schwannoma 55.6%. A younger age at the onset of NF2-related symptoms (p = 0.01) and female sex (p = 0.05) were associated with an increased growth rate in meningiomas. The identification of saltatory growth in meningiomas increased with the duration of follow-up (p = 0.01). Conclusions Neurofibromatosis Type 2–associated intracranial tumors most frequently demonstrated a saltatory growth pattern. Because new tumors can develop in NF2 patients over their lifetime and because radiographic progression and symptom formation are unpredictable, resection may be best reserved for symptom-producing tumors. Moreover, establishing the efficacy of nonsurgical therapeutic interventions must be based on long-term follow-up (several years).

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

Natural history of neurofibromatosis type 2 with onset before the age of 1 year

Neurogenetics ◽  
2013 ◽  
Vol 14 (2) ◽  
pp. 89-98 ◽  
Author(s):  
Martino Ruggieri ◽  
Anna Lia Gabriele ◽  
Agata Polizzi ◽  
Vincenzo Salpietro ◽  
Francesco Nicita ◽  
...  
Keyword(s):  
Natural History ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
History Of

scholarly journals Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

2014 ◽  
Vol 35 (1) ◽  
pp. e50-e56 ◽  
Author(s):  
Scott R. Plotkin ◽  
Vanessa L. Merker ◽  
Alona Muzikansky ◽  
Fred G. Barker ◽  
William Slattery
Keyword(s):  
Natural History ◽  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Newly Diagnosed ◽  
History Of

Cochlear Implantation in the Neurofibromatosis Type 2 Patient: Long-Term Follow-up

2007 ◽  
Vol 117 (6) ◽  
pp. 1069-1072 ◽  
Author(s):  
Brian A. Neff ◽  
R Mark Wiet ◽  
John M. Lasak ◽  
Noel L. Cohen ◽  
Harold C. Pillsbury ◽  
...  
Keyword(s):  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Long Term Follow Up

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.

Variations and natural history of primary intraparenchymal lesions associated with neurofibromatosis type 2

2021 ◽  
Author(s):  
Yukitomo Ishi ◽  
Taisuke Harada ◽  
Hiroyuki Kameda ◽  
Hiromi Okada ◽  
Isao Yokota ◽  
...  
Keyword(s):  
Natural History ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
History Of

scholarly journals Impact of Surgery on Long-Term Results of Hearing in Neurofibromatosis Type-2 Associated Vestibular Schwannomas

Cancers ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1376 ◽  
Author(s):  
Gugel ◽  
Grimm ◽  
Liebsch ◽  
Zipfel ◽  
Teuber ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Auditory Evoked Potential ◽  
Long Term Results ◽  
Data Sets ◽  
Guided Surgery

Hearing preservation is a major goal in the treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannoma (VS), particularly in children and adolescents. In this study, we retrospectively reviewed hearing and volumetry data sets of 39 operated tumors (ears) in 23 patients under the age of 25 and in a follow-up period of 21 to 167 months. Hearing data over a compatible period on 20 other tumors, which did not receive surgery due to their less aggressive nature, were included for comparison. Surgery was carried out via a retrosigmoid approach with the brainstem auditory evoked potential (BAEP) guide. Immediately after surgery, functional hearing was maintained in 82% of ears. Average hearing scores were better in the non-surgery ears. However, the hearing scores in both groups worsened gradually with a similar dynamic during the 42-month postoperative follow-up period. No accelerated impairment of hearing was evident for the operated cases. Rather, the gap between the two hearing deterioration lines tended to close at the end of the follow-up period. Our result suggested that the BAEP-guided surgery did not cause additional hearing deterioration in the long-term and seemed to slow down hearing deterioration of those tumors that were initially more aggressive.

Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients: Hearing and Tumor Growth Results

Neurosurgery ◽  
2013 ◽  
Vol 72 (6) ◽  
pp. 907-914 ◽  
Author(s):  
Matthieu Peyre ◽  
Stéphane Goutagny ◽  
Alpha Bah ◽  
Daniele Bernardeschi ◽  
Béatrice Larroque ◽  
...  
Keyword(s):  
Growth Rate ◽  
Tumor Growth ◽  
Conservative Management ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Growth Rate ◽  
Tumor Diameter ◽  
The Mean

Abstract BACKGROUND: As new treatment modalities develop for the management of vestibular schwannomas (VS) in patients with neurofibromatosis type 2, it remains crucial to ascertain the natural history of the disease. OBJECTIVE: To determine the relationship between hearing and tumor growth in patients undergoing conservative VS management. METHODS: Patients harboring bilateral VS with at least 1 year of radiological follow-up were selected. Conservative management was proposed based on the small tumor size and/or serviceable hearing at presentation. Tumor size was calculated by using the 2-component box model and reported as mean tumor diameter. Hearing was evaluated by using pure-tone average and the American Academy of Otololaryngologists and Head and Neck Surgery classification. RESULTS: Forty-six patients harboring 92 VS were included. The mean clinical and radiological follow-up times were 6.0 and 4.2 years, respectively. The mean tumor diameter was 13 mm at presentation and 20 mm at the end of follow-up. Mean tumor growth rate was 1.8 mm/year. During follow-up, 17 patients (37%) underwent surgery for VS. Surgery-free rate for VS was 88% at 5 years. The number of patients with at least 1 serviceable ear was 39 (85%) at presentation and 34 (74%) at the end of follow-up, including 22 (66%) with binaural serviceable hearing maintained. There was no statistical correlation between tumor growth rate and preservation of serviceable hearing. Tumor growth rates and age at presentation were inversely correlated. CONCLUSION: This study illustrates the high variability among neurofibromatosis type 2 patients regarding hearing status and VS growth rate and justifies the choice of initial conservative management in selected cases.

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