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Atypical evolution of meningiomatosis after discontinuation of cyproterone acetate: clinical cases and histomolecular characterization

  • Original Article - Tumor - Meningioma
  • Published:
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A Correction to this article was published on 09 November 2021

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Abstract

Purpose

The long-term use of cyproterone acetate (CPA) is associated with an increased risk of developing intracranial meningiomas. CPA discontinuation most often induces a stabilization or regression of the tumor. The underlying biological mechanisms as well as the reasons why some meningiomas still grow after CPA discontinuation remain unknown. We reported a series of patients presenting CPA-induced meningiomatosis with opposed tumor evolutions following CPA discontinuation, highlighting the underlying histological and genetic features.

Methods

Patients presenting several meningiomas with opposite tumor evolution (coexistence of growing and shrinking tumors) following CPA discontinuation were identified. Clinical and radiological data were reviewed. A retrospective volumetric analysis of the meningiomas was performed. All the growing meningiomas were operated. Each operated tumor was characterized by histological and genetic analyses.

Results

Four women with multiple meningiomas and opposite tumor volume evolutions after CPA discontinuation were identified. Histopathological analysis characterized the convexity and tentorial tumors which continued to grow after CPA discontinuation as fibroblastic meningiomas. The decreasing skull base tumor was characterized as a fibroblastic meningioma with increased fibrosis and a widespread collagen formation. The two growing skull base meningiomas were identified as meningothelial and transitional meningiomas. The molecular characterization found two NF2 mutations among the growing meningiomas and a PIK3CA mutation in the skull base tumor which decreased.

Conclusion

To our knowledge, this is the first report describing an atypical tumor evolution of CPA-associated meningiomas after CPA discontinuation. The underlying biological mechanisms explaining this observation and especially the close relationship between mutational landscapes and embryologic origins of the meninges in CPA-related meningiomas as well as their clonal origin require further research.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Neurosurgery, Lariboisière Hospital, Assistance Publique – Hopitaux de Paris, University of Paris, Paris, France

    Thibault Passeri, Lorenzo Giammattei, Tuan Le Van, Rosaria Abbritti, Anne-Laure Bernat, Emmanuel Mandonnet & Sébastien Froelich

  2. Department of Genetics, Institut Curie, Paris, France

    Thibault Passeri, Alexandre Perrier, Jennifer Wong, Christine Bourneix & Julien Masliah-Planchon

  3. Department of Anatomopathology, Lariboisière Hospital, Assistance Publique – Hopitaux de Paris, University of Paris, Paris, France

    Marc Polivka & Homa Adle-Biassette

Authors
  1. Thibault Passeri
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Correspondence to Thibault Passeri.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the local ethical committee (Geneva Ethics Committee Board no. 11-233R, NAC 11-085R) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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This article is part of the Topical Collection on Tumor - Meningioma

The original version of this article was revised. Table i is incorrect and is now corrected.

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Passeri, T., Giammattei, L., Le Van, T. et al. Atypical evolution of meningiomatosis after discontinuation of cyproterone acetate: clinical cases and histomolecular characterization. Acta Neurochir 164, 255–263 (2022). https://doi.org/10.1007/s00701-021-05005-9

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