2014
DOI: 10.1155/2014/368920
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Malignant Peripheral Nerve Sheath Tumors of the Head and Neck: A Case Series and Literature Review

Abstract: Background. Malignant peripheral nerve sheath tumors (MPNSTs) of the head and neck are rare aggressive neoplasms with a poor prognosis. This study describes the management and outcomes of 3 of our patients with MPNSTs of the head and neck. Methods. We identified 3 patients presenting with MPNST of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1963 to 2014. Results. Mean follow-up was 31 months. Average age at diagnosis was 44.7 years of age. … Show more

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Cited by 17 publications
(32 citation statements)
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References 34 publications
(60 reference statements)
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“…Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive malignant mesenchymal tumors that usually arise from peripheral nerves or cells of the peripheral nerve sheath and show variable differentiation toward one of the cellular components of the nerve sheath. MPNSTs account for approximately 5–10% of all soft tissue sarcomas, of which only approximately 8–16% occur in the head and neck region [ 41 ]. They occur mainly in adults; the age at presentation may vary widely, although the peak incidence is in the fifth decade of life.…”
Section: Sinonasal Malignanciesmentioning
confidence: 99%
See 1 more Smart Citation
“…Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive malignant mesenchymal tumors that usually arise from peripheral nerves or cells of the peripheral nerve sheath and show variable differentiation toward one of the cellular components of the nerve sheath. MPNSTs account for approximately 5–10% of all soft tissue sarcomas, of which only approximately 8–16% occur in the head and neck region [ 41 ]. They occur mainly in adults; the age at presentation may vary widely, although the peak incidence is in the fifth decade of life.…”
Section: Sinonasal Malignanciesmentioning
confidence: 99%
“…They occur mainly in adults; the age at presentation may vary widely, although the peak incidence is in the fifth decade of life. They are commonly associated with neurofibromatosis type 1 (NF1) but can also arise through sporadic mutation [ 41 ]. The cases associated with NF1 occur in younger patients (mean patient age in the third to fourth decades).…”
Section: Sinonasal Malignanciesmentioning
confidence: 99%
“…Malignant peripheral nerve sheath tumor (MPNST) is a kind of highly aggressive neoplasm originated from Schwann cells. As the name implies, MPNST develops in the peripheral nerves and scarcely affects the cranial nerves [ 1 ].The total incidence is extremely low, about 0.001 % of the population [ 2 ].About 20–30 % of MPNST are seen in patient with neurofibromatosis type 1(NF-1) [ 3 ], with mortality up to 4.6–13 % [ 4 ]. Unresectable because of adjacency to big vessel or nerve trunk, frequent recurrence and distant metastasis of this tumor can be fatal.…”
Section: Introductionmentioning
confidence: 99%
“…In fewer cases, head and neck may be involved [1]. These tumors generally present as an enlarging palpable mass [8]. In our patient, MPNST was confined to the left frontal sinus area.…”
Section: Discussionmentioning
confidence: 59%
“…To the best of our knowledge, only few cases of MPNSTs involving frontal sinus have been reported. Surgery is used to treat MPNSTs of the head and neck [8]. Previous studies have noticed the role of radiotherapy as adjunctive treatment [11,12].…”
Section: -Year Old/ Malementioning
confidence: 99%