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Natural history of peripheral nerve schwannomas

  • Original Article - Peripheral Nerves
  • Published:
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Abstract

Background

Little information about the natural history of peripheral nerve schwannomas exists in the literature. The aim of this study was to determine the natural history of those tumors both in sporadic and schwannomatosis cases to determine their growth rates and patterns.

Methods

In 44 patients from 3 surgical centers, hospital charts, follow-up records, and imaging studies were reviewed. Of these patients, 7 had sporadic schwannomatosis. Histological diagnosis was obtained in 37 patients (84%). Tumor growth rates were determined by calculating the absolute and relative growth rates.

Results

On the 47 tumors analyzed, the median tumor size at diagnosis was 1.8 cm3, and the majority of tumors were located in the lower limb (62%). The absolute growth rate ranged from − 1.13 to 23.17 cm3/year (mean, 1.69 cm3/year). Relative annual growth rates ranged from − 9 to 166%/year (mean, 33.9%/year). There was no clear correlation between initial tumor size, age at diagnosis, and tumor growth rate. Six patients (13%) harbored “fast-growing” tumors (absolute growth rate > 2 cm3/year and relative growth rate > 35%/year) while 19% of tumors demonstrate no growth or negative growth. In schwannomatosis patients, each tumor displayed a distinct growth pattern.

Conclusion

This study confirms the slow-growing nature of most, but not all, peripheral nerve schwannomas. Additional studies are mandatory to explore the environmental factors influencing growth in sporadic cases and the precise growth patterns in schwannomatosis cases to detect the rare cases of malignant transformation and pave the way to the evaluation of future clinical trials.

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Authors and Affiliations

  1. Hand and Upper Extremity Surgery Unit, European Hospital Georges Pompidou, Assistance publique-Hôpitaux de Paris, 20, rue Leblanc, 75908, Paris Cedex 15, France

    Laila El Sayed & Emmanuel H. Masmejean

  2. Faculté de Pharmacie de Paris, Université Paris Descartes-Sorbonne, Paris Cité, Paris, France

    Laila El Sayed, Emmanuel H. Masmejean, Beatrice Parfait & David Biau

  3. Clinique Blomet - Research Unit, Paris, France

    Emmanuel H. Masmejean

  4. Service de Génétique et Biologie Moléculaires, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France

    Beatrice Parfait

  5. Department of Neurosurgery, Bâtiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, Assistance publique-Hôpitaux de Paris, Sorbonne Universités, 47-83 boulevard de l’Hôpital, 75013, Paris, France

    Michel Kalamarides & Matthieu Peyre

  6. Genetics and Development of Brain Tumors - CRICM INSERM U1127 CNRS UMR 7225, Bâtiment ICM 4ème étage - Hôpital de la Pitié-Salpêtrière, Paris, France

    Michel Kalamarides & Matthieu Peyre

  7. Department of orthopedic surgery, Université Paris-Descartes, Hôpital Cochin, Assistance publique-Hôpitaux de Paris, Paris, France

    David Biau

Authors
  1. Laila El Sayed
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  2. Emmanuel H. Masmejean
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  3. Beatrice Parfait
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  4. Michel Kalamarides
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Corresponding author

Correspondence to Matthieu Peyre.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (name of institute/committee) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

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Comments

We read with interest the manuscript from El Sayed et. al. evaluating the growth rates and patterns of benign schwannomas. The authors have provided a concise evaluation of absolute and relative volumetric growth of sporadic and schwannomatosis-associated schwannomas in patients with short- to mid-term imaging follow-up. They have also demonstrated that different schwannomas within the same patient exhibit unique growth rates.

This study demonstrates that sporadic and schwannomatosis-associated schwannomas exhibit highly variable growth: some grow slowly, a handful exhibit rapid growth, and some remain stable. Anecdotally, there are schwannomas that remain stable or slow growing, but then more rapidly, expand between serial imaging studies; cystic schwannomas may grow at different rates than purely solid tumors. Growth rates of peripheral schwannomas in NF-2 patients are unknown. Additionally, the growth rate of incompletely resected schwannomas that subsequently recur is unknown. Thus, there is an unmet need for additional information regarding the growth rate of the spectrum of schwannomas at long-term follow-up, particularly in those patients whose schwannomas are not resected.

This heterogeneity of growth in schwannomas highlights the importance of an individualized evaluation of patient and tumor characteristics when determining which schwannomas need to be resected. Tumors that are asymptomatic and stable or slow-growing may be observed. Tumors that produce symptoms, are already large (> 3cm) at diagnosis, and those that demonstrate rapid growth may be resected. There is a large subgroup in between. In these patients, age, symptomatology, presence of a tumor-related syndrome and site of the tumor (location, location, location) must all be considered and weighed when considering resection of a presumed benign peripheral nerve sheath tumor.

Courtney Pendleton,

Robert J. Spinner,

Rochester, MN USA

A very interesting article with important clinical implications as well as research opportunities to study the molecular features of multiple tumors with different patterns of growth in a single patient. The presence of nerve tumors with a similar genotype with different phenotypes suggest somatic mutations and/or epigenetic modifications playing a role. It is important to consider the natural history of a disease process when considering the risks and benefits of a surgical intervention.

Michel Kliot

CA, USA

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This article is part of the Topical Collection on Peripheral Nerves

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El Sayed, L., Masmejean, E.H., Parfait, B. et al. Natural history of peripheral nerve schwannomas. Acta Neurochir 162, 1883–1889 (2020). https://doi.org/10.1007/s00701-020-04430-6

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  • DOI: https://doi.org/10.1007/s00701-020-04430-6

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