Cranial nerve and intramedullary spinal malignant peripheral nerve sheath tumor associated with neurofibromatosis-1.
Intramedullary
Malignant peripheral nerve sheath tumor
Neoplasm
Spinal cord
Trigeminal nerve
Journal
Surgical neurology international
ISSN: 2229-5097
Titre abrégé: Surg Neurol Int
Pays: United States
ID NLM: 101535836
Informations de publication
Date de publication:
2021
2021
Historique:
received:
14
06
2021
accepted:
01
12
2021
entrez:
30
3
2022
pubmed:
31
3
2022
medline:
31
3
2022
Statut:
epublish
Résumé
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon but aggressive neoplasms associated with radiation exposure and neurofibromatosis Type I (NF1). Their incidence is low compared to other nervous system cancers, and intramedullary spinal lesions are exceedingly rare. Only a few case reports have described intramedullary spinal cord MPNST. We describe the clinical findings, management, and outcome of a young patient with NF1 who developed aggressive cranial nerve and spinal MPNST tumors. This 35-year-old patient had familial NF1 and a history of optic glioma treated with radiation therapy (RT). She developed a trigeminal MPNST that was resected and treated with RT. Four years later, she developed bilateral lower extremity deficits related to an intramedullary cervical spine tumor, treated surgically, and found to be a second MPNST. To the best of our knowledge, this is the first report of cranial nerve and intramedullary spinal MPNSTs manifesting in a single patient, and only the third report of a confined intramedullary spinal MPNST. This unusual case is discussed in the context of a contemporary literature review.
Sections du résumé
Background
UNASSIGNED
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon but aggressive neoplasms associated with radiation exposure and neurofibromatosis Type I (NF1). Their incidence is low compared to other nervous system cancers, and intramedullary spinal lesions are exceedingly rare. Only a few case reports have described intramedullary spinal cord MPNST.
Case Description
UNASSIGNED
We describe the clinical findings, management, and outcome of a young patient with NF1 who developed aggressive cranial nerve and spinal MPNST tumors. This 35-year-old patient had familial NF1 and a history of optic glioma treated with radiation therapy (RT). She developed a trigeminal MPNST that was resected and treated with RT. Four years later, she developed bilateral lower extremity deficits related to an intramedullary cervical spine tumor, treated surgically, and found to be a second MPNST.
Conclusion
UNASSIGNED
To the best of our knowledge, this is the first report of cranial nerve and intramedullary spinal MPNSTs manifesting in a single patient, and only the third report of a confined intramedullary spinal MPNST. This unusual case is discussed in the context of a contemporary literature review.
Identifiants
pubmed: 35350820
doi: 10.25259/SNI_595_2021
pii: 10.25259/SNI_595_2021
pmc: PMC8942193
doi:
Types de publication
Case Reports
Langues
eng
Pagination
630Informations de copyright
Copyright: © 2021 Surgical Neurology International.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
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