Management of NF2-associated vestibular schwannomas in children and young adults: influence of surgery and clinical factors on tumor volume and growth rate

OBJECTIVEThe authors’ aim was to evaluate the tumor volume and growth rate of neurofibromatosis type 2 (NF2)–associated vestibular schwannomas (VSs) and the clinical factors or type of mutations before and after surgery in children and adults younger than 25 years at the time of diagnosis.METHODSA total of 579 volumetric measurements were performed in 46 operated tumors in 28 NF2 patients, using thin-slice (< 3 mm) T1-weighted contrast-enhanced MRI. The follow-up period ranged from 21 to 167 months (mean 75 months). Growth rate was calculated using a multilinear regression model. Mutation analysis of the NF2 gene was performed in 25 patients.RESULTSSurgery significantly (p = 0.013) slowed the VS growth rate from 0.69 ± 1.30 cm3/yr to 0.23 ± 0.42 cm3/yr. Factors significantly associated with a higher growth rate of VSs were increasing patient age (p < 0.0005), tumor volume (p = 0.006), tumor size (p = 0.001), and constitutional truncating mutations in the NF2 gene (p = 0.018). VS growth rates tended to be higher in patients with spinal ependymomas and in right-sided tumors and lower in the presence of peripheral schwannomas; however, no statistical significance was achieved.CONCLUSIONSDecompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rate in children and young adults with NF2-associated VS. Patients with potential risk factors for accelerated growth (e.g., large volume, truncating mutations) and with increasing age should be monitored more closely before and after surgery.

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Managing NF2-associated vestibular schwannomas in children and young adults: review of an institutional series regarding effects of surgery and bevacizumab on growth rates, tumor volume, and hearing quality

Child s Nervous System ◽

10.1007/s00381-020-04728-x ◽

2020 ◽

Vol 36 (10) ◽

pp. 2471-2480

Author(s):

Isabel Gugel ◽

Julian Zipfel ◽

Philip Hartjen ◽

Lan Kluwe ◽

Marcos Tatagiba ◽

...

Keyword(s):

Growth Rate ◽

Young Adults ◽

Growth Rates ◽

Tumor Volume ◽

Tumor Resection ◽

Internal Auditory Canal ◽

Hearing Preservation ◽

Data Sets ◽

Bevacizumab Treatment ◽

Children And Young Adults

Abstract We reviewed our experience in managing of NF2-associated vestibular schwannoma (VS) in children and young adults regarding the effect of surgery and postoperative bevacizumab treatment. A total of 579 volumetric and hearing data sets were analyzed. The effect of surgery on tumor volume and growth rate was investigated in 46 tumors and on hearing function in 39 tumors. Long-term hearing follow-up behavior was compared with 20 non-operated ears in additional 15 patients. Sixteen operated VS were treated with bevacizumab. Mutation analysis of the NF2 gene was performed in 25 patients. Surgery significantly slowed down VS growth rate. Factors associated with a higher growth rate were increasing patient age, tumor volume, and constitutional truncating mutations. Immediately after surgery, functional hearing was maintained in 82% of ears. Deterioration of hearing was associated with initial hearing quality, larger tumor volumes, and larger resection amounts. Average hearing scores were initially better in the group of non-operated VS. Over time, hearing scores in both groups worsened with a similar dynamic. During bevacizumab treatment of residual tumors, four different patterns of growth were observed. Decompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rates. Carefully tailored BAEP-guided surgery does not cause additional hearing deterioration. Secondary bevacizumab treatment showed heterogenous effects both regarding tumor size and hearing preservation. It seems that postoperative tumor residuals, that grow slower, behave differently to bevacizumab than reported for not-operated faster growing VS.

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Cystic vestibular schwannomas: neuroimaging and growth rate

The Journal of Laryngology & Otology ◽

10.1017/s0022215100126854 ◽

1994 ◽

Vol 108 (5) ◽

pp. 375-379 ◽

Cited By ~ 33

Author(s):

Samih Charabi ◽

Margit Mantoni ◽

Mirko Tos ◽

Jens Thomsen

Keyword(s):

Growth Rate ◽

Obstructive Hydrocephalus ◽

Internal Auditory Canal ◽

Vestibular Schwannomas ◽

Annual Growth Rate ◽

Residual Tumour ◽

Shunt Insertion ◽

Trigeminal Schwannoma ◽

Tumour Extension ◽

Primary Evaluation

AbstractIn a series of 571 vestibular schwannomas (VS) operated on in the period 1976–1992, via the translabyrinthine approach, 23 tumours (4 per cent) from 23 patients were radiologically, peroperatively and histologically identified as cystic VS. Neuroimaging examinations (CT and MRI) revealed 15 tumours (2.6 per cent) with extracystic formations and in eight cases (1.4 per cent) intratumoural cysts. In 15 cases (65 per cent), tumours were located extracanalicularly with no involvement of the internal auditory canal (IAC). The extracanalicular tumour extension including the cystic elements ranged between 10 and 60 mm with a mean of 45 mm. In eight cases out of 23 (35 per cent) other diagnostic possibilities such as trigeminal schwannoma, epidermoid cyst and meningioma were proposed at the primary evaluation. Deformation, displacement and/or impression of the fourth ventricle was demonstrated in 17 cases (74 per cent). Three patients (13 per cent) had obstructive hydrocephalus which demanded shunt insertion. Residual tumour was detected in two patients (9 per cent). Growth rate in four cases was more than 10 times higher when compared to the annual growth rate of noncystic VS. The results of this study provide data on cystic VS attenuation demonstrated by CT or MRI which may be valuable in neuroradiological diagnosis of these cystic lesions and emphasizes that the wait-and-see policy should not be applied to the cystic variant of VS.

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What Is the Best Method for Reporting Tumor Diameter in Vestibular Schwannoma?

Neurosurgery ◽

10.1227/01.neu.0000080062.61335.a5 ◽

2003 ◽

Vol 53 (3) ◽

pp. 634-638 ◽

Cited By ~ 27

Author(s):

Yuichiro Tanaka ◽

Kazuhiro Hongo ◽

Tsuyoshi Tada ◽

Shigeaki Kobayashi

Keyword(s):

Tumor Volume ◽

Three Dimensional ◽

Internal Auditory Canal ◽

Vestibular Schwannomas ◽

Maximum Diameter ◽

Tumor Diameter ◽

Constructive Interference ◽

Group Iii ◽

Group I ◽

Group Ii

Abstract OBJECTIVE Various methods have been used to report the tumor diameter of vestibular schwannomas. To clarify the most appropriate method to represent the tumor volume, tumor diameters according to various measuring methods were statistically compared with the actual tumor volume. METHODS Tumor volume was measured by three-dimensional constructive interference in steady state images in 52 unselected vestibular schwannomas. Pearson's correlation coefficient was obtained between the tumor volume and various tumor diameters, such as diameter parallel to the petrous edge (a); a pons-to-petrous diameter (b); √ab, a maximum diameter of the portion in the cerebellopontine angle cistern (max CPA); a maximum diameter of the whole tumor (Max); and a diameter through an axis of the internal auditory canal (Axis). The tumors were divided into three groups on the basis of tumor volume, as follows: Group I (small, &3x003C;0.5 cm3), Group II (medium, 0.5–2 cm3), and Group III (large, >2 cm3). RESULTS Max and Axis correlated best with the tumor volume in Group I and correlated least with the tumor volume in Group II. Any of these measurements was acceptable in Group III tumors. The max CPA consistently revealed good correlation with the tumor volume in all three tumor groups. CONCLUSION The max CPA measurement is the simplest and most appropriate way to represent the tumor volume in unselected tumors. Max or Axis is better only when small tumors (<0.5 cm3 in volume) are being assessed—that is, those with a max CPA of less than 1 cm.

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Exercise Assessment of Cardiac Function in Children and Young Adults Before and After Bone Marrow Transplantation

PEDIATRICS ◽

10.1542/peds.89.4.722 ◽

1992 ◽

Vol 89 (4) ◽

pp. 722-729

Author(s):

Ranae L. Larsen ◽

Gerald Barber ◽

Charles T. Heise ◽

Charles S. August

Keyword(s):

Bone Marrow ◽

Young Adults ◽

Bone Marrow Transplantation ◽

Cardiac Function ◽

Marrow Transplantation ◽

Cardiac Toxicity ◽

Intensive Therapy ◽

Left Ventricular ◽

Before And After ◽

Children And Young Adults

Cardiac toxicity is a potential complication of bone marrow transplantation because recipients frequently receive cardiotoxic chemotherapy and/or irradiation before transplantation. Most studies indicate that transient cardiac toxicity occurs within weeks of transplantation, but few studies have evaluated either cardiac status before or late after transplantation. Cardiac performance was assessed via cycle ergometry in 20 children and young adults before transplantation and 31 other children and young adults after transplantation. Mean survival time in the group post-transplantation was 3.9 years with a range of 11 months to 12.1 years. Left ventricular size and shortening fraction at rest were assessed via echocardiography. Data were compared to those of 70 healthy subjects from our laboratory. Patients before and after transplantation had normal oxygen consumptions and cardiac indices at rest. During exercise, however, patients treated for cancer both before and after bone marrow transplantation had reduced exercise times, reduced maximal oxygen consumptions, and reduced ventilatory anaerobic thresholds. Cardiac reserve, as judged by the response of the cardiac output during exercise, was reduced severely. There were no significant differences between the groups tested before and after transplantation. Patients who had been treated for aplastic anemia, who had received less intensive therapy before transplantation, performed significantly better than did patients treated for cancer. Despite these findings, only four patients had abnormalities by echocardiography. In conclusion, before transplantation patients with oncologic diagnoses had serious limitations in exercise performance, most likely as a result of the effects of the cardiotoxic therapy given as part of their conventional cancer therapy. Long-term survivors of bone marrow transplantation also had similar abnormalities. Since the same patients were not studied before and after transplantation, one cannot draw definite conclusions about the effect of the transplantation itself upon cardiac function. However, exercise testing is a sensitive, noninvasive method of assessing patients at risk for cardiac dysfunction secondary to potentially cardiotoxic agents.

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Percent reduction of growth hormone levels correlates closely with percent resected tumor volume in acromegaly

Journal of Neurosurgery ◽

10.3171/2014.10.jns14496 ◽

2015 ◽

Vol 122 (4) ◽

pp. 798-802 ◽

Cited By ~ 15

Author(s):

Lucia Schwyzer ◽

Robert M. Starke ◽

John A. Jane ◽

Edward H. Oldfield

Keyword(s):

Growth Hormone ◽

Tumor Volume ◽

Tumor Resection ◽

Volume Growth ◽

Residual Tumor ◽

Close Correlation ◽

Tumor Removal ◽

Hormone Levels ◽

Before And After ◽

Plasma Gh

OBJECT Correlation between tumor volume and hormone levels in individual patients would permit calculation of the fraction of tumor removed by surgery, by measuring postoperative hormone levels. The goals of this study were to examine the relationship between tumor volume, growth hormone (GH), and insulin-like growth factor–1 (IGF-1) levels, and to assess the correlation between percent tumor removal and the reduction in plasma GH and IGF-1 in patients with acromegaly. METHODS The 3D region of interest–based volumetric method was used to measure tumor volume via MRI before and after surgery in 11 patients with GH-secreting adenomas. The volume of residual tumor as a fraction of preoperative tumor volume was correlated with GH levels before and after surgery. Examination of this potential correlation required selection of patients with acromegaly who 1) had incomplete tumor removal, 2) had precise measurements of initial and residual tumor, and 3) were not on medical therapy. RESULTS Densely granulated tumors produced more peripheral GH per mass of tumor than sparsely granulated tumors (p = 0.04). There was a correlation between GH and IGF-1 levels (p = 0.001). Although there was no close correlation between tumor size and peripheral GH levels, after normalizing each tumor to its own plasma GH level and tumor volume, a comparison of percent tumor resection with percent drop in plasma GH yielded a high correlation coefficient (p = 0.006). CONCLUSIONS Densely granulated somatotropinomas produce more GH per mass of tumor than do sparsely granulated tumors. Each GH-secreting tumor has its own intrinsic level of GH production per mass of tumor, which is homogeneous over the tumor mass, and which varies greatly between tumors. In most patients the fraction of a GH-secreting tumor removed by surgery can be accurately estimated by simply comparing plasma GH levels after surgery to those before surgery.

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Impact of COVID-19 Lockdown on Pulmonary and Nutritional Status in Children and Young Adults With Cystic Fibrosis, in Greece

Journal of Patient Experience ◽

10.1177/23743735211008295 ◽

2021 ◽

Vol 8 ◽

pp. 237437352110082

Author(s):

Ioanna Loukou ◽

Maria Moustaki ◽

Argyri Petrocheilou ◽

Ioanna Zarkada ◽

Konstantinos Douros

Keyword(s):

Body Mass Index ◽

Cystic Fibrosis ◽

Young Adults ◽

Lung Function ◽

Nutritional Status ◽

Body Mass ◽

Somatic Growth ◽

Standard Of Care ◽

Before And After ◽

Children And Young Adults

During the first wave of the coronavirus pandemic in 2020, Greece adopted strict lockdown measures. We aimed to investigate the effects of lockdown and the resultant changes in the standard of care, on the lung function and somatic growth of cystic fibrosis (CF) patients. We analyzed data on body mass index and lung function of 103 CF patients 5.0- to 23.0-years-old before and after the lockdown period. Body mass index did not change significantly, but there was a significant improvement in lung function after the end of the lockdown period.

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Immune Responses to Exercise and Training: A Comparison of Children and Young Adults

Pediatric Exercise Science ◽

10.1123/pes.10.3.210 ◽

1998 ◽

Vol 10 (3) ◽

pp. 210-226 ◽

Cited By ~ 7

Author(s):

Susan Shore ◽

Roy J. Shephard

Keyword(s):

Young Adults ◽

Immune Responses ◽

Acute Exercise ◽

Aerobic Training ◽

Ventilatory Threshold ◽

Before And After ◽

Children And Young Adults ◽

Exercise Induced ◽

Exercise And Training ◽

And Training

Immune responses have been examined in 11 children aged 10.3 ± 0.6 years before and after 12 weeks of aerobic training. Initial resting data showed high total lymphocyte, CD3+ and CD8+ counts, a low CD4+/CD8+ ratio and a low CD25+ count relative to young adults. Acute exercise (30 min at ventilatory threshold) initially increased CD4+, CD8+, and CD56+ counts, and decreased CD4+/CD8+ ratio, but CD56+ count did not decrease during recovery. After training, relative aerobic power remained unchanged at 50 ±3 ml · kg−1 · min−1. However, resting leukocyte, CD3’ and CD25’ counts were decreased, and acute exercise induced smaller changes in leukocyte and subset counts. We conclude that immune responses to exercise are generally similar in children and young adults.

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Yoga in Psychiatry: feasibility of a yoga-intervention for severely affected children and young adults with non-internalizing psychiatric disorders

10.31234/osf.io/tvr6k ◽

2021 ◽

Author(s):

Myrte J. M. van Langen ◽

Bob Oranje ◽

Anneke Sips ◽

Sarah Durston

Keyword(s):

Young Adults ◽

Psychiatric Disorders ◽

Treatment Program ◽

Behavioral Problems ◽

Outpatient Treatment ◽

Developmental Disorders ◽

Psychiatric Diagnoses ◽

Before And After ◽

Children And Young Adults ◽

Psychiatric Department

Yoga-based interventions are increasingly being introduced to improve the lives of individuals with widely varying psychiatric diagnoses and symptoms. We developed a yoga-program for two severely affected populations at our psychiatric department: young adults with psychosis from our inpatient clinic, and children with severe developmental disorders and/or behavioral problems from our inpatient clinic or outpatient treatment program. The participants, clinic staff and yoga-instructors assessed the feasibility of our yoga program. They participated in evaluation meetings and gave feedback. Participants also filled in an evaluation form before and after every session. Results showed that our yoga-program is feasible for severely affected populations. The program can and should be tailored to meet the differing needs of individuals. Participants benefitted from and enjoyed sessions and reported feeling calmer, more goal-oriented and more relaxed after the sessions.

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Magnetic resonance imaging characteristics and the prediction of outcome of vestibular schwannomas following Gamma Knife radiosurgery

Journal of Neurosurgery ◽

10.3171/2016.9.jns161510 ◽

2017 ◽

Vol 127 (6) ◽

pp. 1384-1391 ◽

Cited By ~ 6

Author(s):

Chih-Chun Wu ◽

Wan-Yuo Guo ◽

Wen-Yuh Chung ◽

Hisu-Mei Wu ◽

Chung-Jung Lin ◽

...

Keyword(s):

Gamma Knife ◽

Tumor Volume ◽

Gamma Knife Radiosurgery ◽

Reduction Ratio ◽

Volume Reduction ◽

Vestibular Schwannomas ◽

Tumor Control ◽

Mri Features ◽

Before And After

OBJECTIVEGamma Knife surgery (GKS) is a promising treatment modality for patients with vestibular schwannomas (VSs), but a small percentage of patients have persistent postradiosurgical tumor growth. The aim of this study was to determine the clinical and quantitative MRI features of VS as predictors of long-term tumor control after GKS.METHODSThe authors performed a retrospective study of all patients with VS treated with GKS using the Leksell Gamma Knife Unit between 2005 and 2013 at their institution. A total of 187 patients who had a minimum of 24 months of clinical and radiological assessment after radiosurgery were included in this study. Those who underwent a craniotomy with tumor removal before and after GKS were excluded. Study patients comprised 85 (45.5%) males and 102 (54.5%) females, with a median age of 52.2 years (range 20.4–82.3 years). Tumor volumes, enhancing patterns, and apparent diffusion coefficient (ADC) values were measured by region of interest (ROI) analysis of the whole tumor by serial MRI before and after GKS.RESULTSThe median follow-up period was 60.8 months (range 24–128.9 months), and the median treated tumor volume was 3.54 cm3 (0.1–16.2 cm3). At last follow-up, imaging studies indicated that 150 tumors (80.2%) showed decreased tumor volume, 20 (10.7%) had stabilized, and 17 (9.1%) continued to grow following radiosurgery. The postradiosurgical outcome was not significantly correlated with pretreatment volumes or postradiosurgical enhancing patterns. Tumors that showed regression within the initial 12 months following radiosurgery were more likely to have a larger volume reduction ratio at last follow-up than those that did not (volume reduction ratio 55% vs 23.6%, respectively; p < 0.001). Compared with solid VSs, cystic VSs were more likely to regress or stabilize in the initial postradiosurgical 6–12-month period and during extended follow-up. Cystic VSs exhibited a greater volume reduction ratio at last follow-up (cystic vs solid: 67.6% ± 24.1% vs 31.8% ± 51.9%; p < 0.001). The mean preradiosurgical maximum ADC (ADCmax) values of all VSs were significantly higher for those with tumor regression or stabilization at last follow-up compared with those with progression (2.391 vs 1.826 × 10−3 mm2/sec; p = 0.010).CONCLUSIONSLoss of central enhancement after radiosurgery was a common phenomenon, but it did not correlate with tumor volume outcome. Preradiosurgical MRI features including cystic components and ADCmax values can be helpful as predictors of treatment outcome.

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The Feasibility of a Modified Exclusive Endoscopic Transcanal Transpromontorial Approach for Vestibular Schwannomas

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0038-1667061 ◽

2018 ◽

Vol 80 (01) ◽

pp. 082-087 ◽

Cited By ~ 3

Author(s):

In Moon ◽

Dongchul Cha ◽

Sung-Il Nam ◽

Hyun-Jin Lee ◽

Jae Choi

Keyword(s):

Short Form ◽

Tumor Resection ◽

Internal Auditory Canal ◽

Operation Time ◽

Vestibular Schwannomas ◽

Profound Hearing Loss ◽

Hospitalization Period ◽

The Mean

Objective We evaluated the feasibility of an exclusive endoscopic transcanal transpromontorial approach (EETTA) for the treatment of small vestibular schwannomas (VSs) limited to the internal auditory canal (IAC), and introduced a modification without external auditory canal closure. Methods Between June 2016 and June 2017, seven patients with VS underwent surgery using a modified EETTA. Treatment outcomes, including efficacy of tumor resection, preservation of function, operation time, and quality of life (QOL), were evaluated. Results The patients preoperatively exhibited Koos Grade I/II tumors and severe-to-profound hearing loss. Gross total resection was accomplished in all cases. There were no major complications, and all patients exhibited normal facial nerve function immediately after surgery. The mean follow-up period was 12.9 months. The operation time (average 196.3 ± 64.9 minutes) and hospitalization period (average 7.4 ± 1.0 days) were favorable. Short Form-36 scores for QOL showed unremarkable results compared with previous reports. Conclusions The modified EETTA was effective in the removal of VSs in the IAC. It can be an alternative surgical option for small VSs.

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