Andrew T King1, Scott A Rutherford1, Charlotte Hammerbeck-Ward1, Simon K Lloyd2, Simon R Freeman2, Omar N Pathmanaban1, Mark Kellett3, Rupert Obholzer4, Shazia Afridi5, Patrick Axon6, Dorothy Halliday7, Allyson Parry8, Owen M Thomas9, Roger D Laitt9, Martin G McCabe10, Stavros Stivaros11, Sara Erridge12, D Gareth Evans13. 1. Department of Neurosurgery, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Manchester, United Kingdom. 2. Department of Otolaryngology, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Manchester, United Kingdom. 3. Department of Neurology, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Manchester, United Kingdom. 4. Department of Otolaryngology, Guy's and St. Thomas' Hospital, London, United Kingdom. 5. Department of Neurology, Guy's and St. Thomas' Hospital, London, United Kingdom. 6. Department of Otolaryngology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom. 7. Department of Clinical Genetics, Oxford University Hospitals NHS Trust, Oxford, United Kingdom. 8. Department of Neurology, Oxford University Hospitals NHS Trust, Oxford, United Kingdom. 9. Department of Neuroradiology, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Manchester, United Kingdom. 10. Centre for Paediatric, Teenage and Young Adult Cancer, Institute of Cancer Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom. 11. Department of Neuroradiology, Manchester Academic Health Science Centre, Central Manchester NHS Foundation Trust, Manchester, United Kingdom. 12. Institute of Cancer Sciences, Edinburgh Cancer Centre, Western General Hospital, Edinburgh, United Kingdom. 13. Manchester Centre for Genomic Medicine, Manchester Academic Health Science Centre, Central Manchester NHS Foundation Trust, Manchester, United Kingdom.
Abstract
BACKGROUND: The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. OBJECTIVE: To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. METHODS: The prospective database consists of 1253 patients with NF2. One thousand and nine are known to be alive at last follow-up. The presence and laterality/pathology of vestibular schwannoma at diagnosis and last follow-up was sought. RESULTS: There were no cases of spontaneous MPNST with 2114 proven (n = 1150) and presumed benign (n = 964) vestibular schwannomas found. Two patients had developed MPNST (1 presumed) after having previously undergone stereotactic radiosurgery for a vestibular schwannoma. CONCLUSION: In this series, and from the literature, malignant transformation of a vestibular schwannoma was not a feature of NF2 in the unirradiated patient. NF2 patients should not be told that they have an increased risk of malignant change in a vestibular schwannoma unless they undergo radiation treatment. However, very much larger datasets are required before it can be determined whether there is any association between NF2 and MPNST in the unirradiated patient.
BACKGROUND: The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. OBJECTIVE: To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. METHODS: The prospective database consists of 1253 patients with NF2. One thousand and nine are known to be alive at last follow-up. The presence and laterality/pathology of vestibular schwannoma at diagnosis and last follow-up was sought. RESULTS: There were no cases of spontaneous MPNST with 2114 proven (n = 1150) and presumed benign (n = 964) vestibular schwannomas found. Two patients had developed MPNST (1 presumed) after having previously undergone stereotactic radiosurgery for a vestibular schwannoma. CONCLUSION: In this series, and from the literature, malignant transformation of a vestibular schwannoma was not a feature of NF2 in the unirradiated patient. NF2 patients should not be told that they have an increased risk of malignant change in a vestibular schwannoma unless they undergo radiation treatment. However, very much larger datasets are required before it can be determined whether there is any association between NF2 and MPNST in the unirradiated patient.
Authors: Roland Goldbrunner; Michael Weller; Jean Regis; Morten Lund-Johansen; Pantelis Stavrinou; David Reuss; D Gareth Evans; Florence Lefranc; Kita Sallabanda; Andrea Falini; Patrick Axon; Olivier Sterkers; Laura Fariselli; Wolfgang Wick; Joerg-Christian Tonn Journal: Neuro Oncol Date: 2020-01-11 Impact factor: 12.300
Authors: Umberto Tosi; Sergio Guadix; Anjile An; Drew Wright; Paul J Christos; Susan Pannullo; Andrew Brandmaier; Jonathan P S Knisely; Philip E Stieg; Rohan Ramakrishna Journal: Neurooncol Pract Date: 2021-02-01