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Malignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient

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... Full description

Journal Title: Neurosurgery 2018, Vol.83 (1), p.38-42
Main Author: King, Andrew T
Other Authors: Rutherford, Scott A , Hammerbeck-Ward, Charlotte , Lloyd, Simon K , Freeman, Simon R , Pathmanaban, Omar N , Kellett, Mark , Obholzer, Rupert , Afridi, Shazia , Axon, Patrick , Halliday, Dorothy , Parry, Allyson , Thomas, Owen M , Laitt, Roger D , McCabe, Martin G , Stivaros, Stavros , Erridge, Sara , Evans, D Gareth
Format: Electronic Article Electronic Article
Language: English
Subjects:
Publisher: United States: Oxford University Press
ID: ISSN: 0148-396X
Link: https://www.ncbi.nlm.nih.gov/pubmed/28973692
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recordid: cdi_wolterskluwer_health_10_1093_neuros_nyx368
title: Malignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient
format: Article
creator:
  • King, Andrew T
  • Rutherford, Scott A
  • Hammerbeck-Ward, Charlotte
  • Lloyd, Simon K
  • Freeman, Simon R
  • Pathmanaban, Omar N
  • Kellett, Mark
  • Obholzer, Rupert
  • Afridi, Shazia
  • Axon, Patrick
  • Halliday, Dorothy
  • Parry, Allyson
  • Thomas, Owen M
  • Laitt, Roger D
  • McCabe, Martin G
  • Stivaros, Stavros
  • Erridge, Sara
  • Evans, D Gareth
subjects:
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cell Transformation, Neoplastic - pathology
  • Child
  • Child, Preschool
  • Databases, Genetic
  • Female
  • Humans
  • Infant
  • Male
  • Malignant peripheral nerve sheath tumours
  • Manchester criteria
  • Middle Aged
  • Nerve Sheath Neoplasms - epidemiology
  • Nerve Sheath Neoplasms - genetics
  • Neurofibromatosis
  • Neurofibromatosis 2 - complications
  • Neurofibromatosis 2 - pathology
  • Neurofibromatosis Type 2
  • Neuroma, Acoustic - genetics
  • Neuroma, Acoustic - pathology
  • Neurosurgery
  • otorhinolaryngologic diseases
  • Patients
  • Prospective Studies
  • Radiosurgery
  • Tumors
  • Young Adult
ispartof: Neurosurgery, 2018, Vol.83 (1), p.38-42
description: 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.
language: eng
source:
identifier: ISSN: 0148-396X
fulltext: no_fulltext
issn:
  • 0148-396X
  • 1524-4040
url: Link


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titleMalignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient
creatorKing, Andrew T ; Rutherford, Scott A ; Hammerbeck-Ward, Charlotte ; Lloyd, Simon K ; Freeman, Simon R ; Pathmanaban, Omar N ; Kellett, Mark ; Obholzer, Rupert ; Afridi, Shazia ; Axon, Patrick ; Halliday, Dorothy ; Parry, Allyson ; Thomas, Owen M ; Laitt, Roger D ; McCabe, Martin G ; Stivaros, Stavros ; Erridge, Sara ; Evans, D Gareth
creatorcontribKing, Andrew T ; Rutherford, Scott A ; Hammerbeck-Ward, Charlotte ; Lloyd, Simon K ; Freeman, Simon R ; Pathmanaban, Omar N ; Kellett, Mark ; Obholzer, Rupert ; Afridi, Shazia ; Axon, Patrick ; Halliday, Dorothy ; Parry, Allyson ; Thomas, Owen M ; Laitt, Roger D ; McCabe, Martin G ; Stivaros, Stavros ; Erridge, Sara ; Evans, D Gareth ; English Specialist NF2 research group ; The English Specialist NF2 research group
descriptionAbstract 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.
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subjectAdolescent ; Adult ; Aged ; Aged, 80 and over ; Cell Transformation, Neoplastic - pathology ; Child ; Child, Preschool ; Databases, Genetic ; Female ; Humans ; Infant ; Male ; Malignant peripheral nerve sheath tumours ; Manchester criteria ; Middle Aged ; Nerve Sheath Neoplasms - epidemiology ; Nerve Sheath Neoplasms - genetics ; Neurofibromatosis ; Neurofibromatosis 2 - complications ; Neurofibromatosis 2 - pathology ; Neurofibromatosis Type 2 ; Neuroma, Acoustic - genetics ; Neuroma, Acoustic - pathology ; Neurosurgery ; otorhinolaryngologic diseases ; Patients ; Prospective Studies ; Radiosurgery ; Tumors ; Young Adult
ispartofNeurosurgery, 2018, Vol.83 (1), p.38-42
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7Obholzer, Rupert
8Afridi, Shazia
9Axon, Patrick
10Halliday, Dorothy
11Parry, Allyson
12Thomas, Owen M
13Laitt, Roger D
14McCabe, Martin G
15Stivaros, Stavros
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17Evans, D Gareth
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descriptionAbstract 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.
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9Humans
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12Malignant peripheral nerve sheath tumours
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15Nerve Sheath Neoplasms - epidemiology
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19Neurofibromatosis 2 - pathology
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28Tumors
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authorKing, Andrew T ; Rutherford, Scott A ; Hammerbeck-Ward, Charlotte ; Lloyd, Simon K ; Freeman, Simon R ; Pathmanaban, Omar N ; Kellett, Mark ; Obholzer, Rupert ; Afridi, Shazia ; Axon, Patrick ; Halliday, Dorothy ; Parry, Allyson ; Thomas, Owen M ; Laitt, Roger D ; McCabe, Martin G ; Stivaros, Stavros ; Erridge, Sara ; Evans, D Gareth
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13Laitt, Roger D
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2Hammerbeck-Ward, Charlotte
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8Afridi, Shazia
9Axon, Patrick
10Halliday, Dorothy
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13Laitt, Roger D
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atitleMalignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient
jtitleNeurosurgery
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abstractAbstract 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.
copUnited States
pubOxford University Press
pmid28973692
doi10.1093/neuros/nyx368
oafree_for_read