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Malignant peripheral nerve sheath tumour transformation of histological benign vestibular schwannoma after stereotactic radiosurgery in patients without neurofibromatosis
  1. Orlando De Jesus1,
  2. José G Sánchez Jiménez1,
  3. George Santiago Quiñones2 and
  4. Román Vélez2
  1. 1Neurosurgery, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
  2. 2Pathology and Laboratory Medicine, University of Puerto Rico School of Medicine, San Juan, Puerto Rico
  1. Correspondence to Dr Orlando De Jesus; drodejesus{at}


Stereotactic radiosurgery (SRS) poses a minimal but important risk for tumour transformation, typically occurring 8–10 years after the treatment. Malignant peripheral nerve sheath tumour (MPNST) is the most common tumour arising from a vestibular schwannoma treated with SRS, with only 14 cases previously reported. We present the fifteenth case and describe its evolution and clinical course. A 56-year-old man without a history of neurofibromatosis was diagnosed 9 years prior with a vestibular schwannoma. SRS to the residual tumour was given 3 months later. During the current hospitalisation, he was reoperated where histology confirmed a MPNST. All 15 MPNST cases were analysed, showing a 77% female predominance presenting a malignant transformation at a mean age of 51. The diagnosis was made at a mean time of 74 months after SRS. The mean survival time after diagnosis was 16 months. MPNST arising from benign vestibular schwannoma after SRS treatment is an uncommon but devastating complication.

  • neuroimaging
  • neurooncology
  • CNS cancer
  • radiotherapy
  • neurosurgery

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  • Contributors Manuscript conception was done by ODJ and JGSJ. Drafting the manuscript was done by ODJ, JGSJ, GSQ and RV. Review and approval of the final version was given by all the authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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