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Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2

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Abstract

We retrospectively reviewed characteristics of patients with neurofibromatosis type 2 to identify factors predicting further growth of bilateral vestibular schwannomas. Subjects comprised 27 neurofibromatosis type 2 patients with 54 vestibular schwannomas, followed for 24–204 months (mean, 86 months). This study investigated factors predictive of vestibular schwannoma growth in neurofibromatosis type 2. Features distinguishing actively growing from quiescent VS were determined for untreated course (28 vestibular schwannomas) and posttreatment course (including either resection or radiosurgery; 33 vestibular schwannomas). A general estimation equation was used to identify factors affecting tumor growth. During the untreated course, 19 vestibular schwannomas showed growth and 9 vestibular schwannomas were stable. No factors predictive of growth were shown. During the posttreatment course (23 surgical resections, ten radiosurgeries), ten treatments were followed by growth and 23 were followed by stability, with growth showing an association with onset at an early age (p = 0.007). Multivariate analysis identified no factors predictive of growth. After treatment, close follow-up is warranted for patients with onset at an early age.

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Authors and Affiliations

  1. Department of Neurosurgery, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Eiji Ito & Tetsuya Nagatani

  2. Department of Public Health/Health Information Dynamics, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Hiroshi Yatsuya

  3. Department of Neurosurgery, Fukushima Medical University, Fukushima, Japan

    Kiyoshi Saito

  4. Department of Psychiatry, National Hospital Organization Nagoya Medical Center, Nagoya, Japan

    Goro Otsuka

  5. Department of Neurosurgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima, 960-1295, Japan

    Kiyoshi Saito

Authors
  1. Eiji Ito
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  2. Kiyoshi Saito
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  3. Hiroshi Yatsuya
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  4. Tetsuya Nagatani
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  5. Goro Otsuka
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Corresponding author

Correspondence to Kiyoshi Saito.

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Comments

Cordula Matthies, Würzburg, Germany

Ito and colleagues present a thorough analysis on individual tumor progression in neurofibromatosis type 2. While the stability of vestibular schwannomas (1) under observation, (2) after surgery, or (3) after radiosurgery is the focus of their study, they have investigated the related incidences of further cranial or spinal tumors in the individual cases.

Despite analysis of such individual tumor manifestation and of all general patient parameters, only young age was identified as a reliable parameter predicting some progression. In case of tumor progression, the average age was 16.7 years compared with 30.2 years in tumor stability; this was most obvious in the treated course and, to a milder extent, also clears in the untreated course.

Still, a few parameters might give a hint towards increased risk of progression, though no statistical significance could be found: In NF2 patients with untreated course, the incidence of tumor growth appeared somewhat higher if cranial meningiomas or spinal tumors were present, factors which we associate with a more severe phenotypic NF manifestation. A fundamental difficulty is inherent to this study: comparison of treated and untreated course neglects a preselection as patients with a rather quiet course were more likely to be in the observed group.

In conclusion, it might be interesting to analyze the different genetic mutations in these different observation and treatment groups.

Differing from sporadic schwannomas, treatment in neurofibromatosis type 2 is never curative and often incomplete on purpose with regard to functional preservation of cranial nerves. Therefore, the likelihood of tumor progression or stability will remain a major issue in neurofibromatosis type 2; if factors predicting relative disease stability were identified, surgical decisions on indication, timing, and radicality could be based on more sound grounds.

Comments

Nobutaka Kawahara, Yokohama, Japan

Management of vestibular schwannomas associated with NF 2 is difficult. In particular, how to preserve hearing and when to intervene surgically or radiosurgically are still controversial issues. In addition, some tumors appear to behave differently when compared with solitary VS. Since some VSs are conservatively managed when useful hearing remains, it would be helpful in decision making if factors which predict further tumor growth are available.

In this article, the authors tried to identify these factors in untreated and posttreatment courses. Unfortunately, they could not identify factors predicting tumor growth in untreated courses. However, 19 out of 28 tumors grew (68%). This number may be high when compared with that in solitary VS, which is reported to be 40 % in systematic review (Yoshimoto et al. J Neurosurg 103:59–63, 2005). In the posttreatment course, they identified onset at an early age as a factor associated with growth. This would be a reasonable result when considering that NF 2 is a genetic disease. Somewhat surprising is the negative result of treatment (surgery vs. radiosurgery), though there was a trend for better control of tumor in favor of radiosurgery when residual tumor is present. To clarify these predictive factors, increased number of patients and prospective design of the study would be required as demonstrated in meta-analysis (Yoshimoto et al. J Neurosurg 103:59–63, 2005). The authors are encouraged to continue the study in a prospective way.

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Ito, E., Saito, K., Yatsuya, H. et al. Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2. Neurosurg Rev 32, 425–433 (2009). https://doi.org/10.1007/s10143-009-0223-3

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  • DOI: https://doi.org/10.1007/s10143-009-0223-3

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