Malignant Peripheral Nerve Sheath Tumors

Gupta, Gaurav; Mammis, Antonios; Maniker, Allen H.

doi:10.1016/j.nec.2008.07.004

Cited by 131 publications

(160 citation statements)

References 64 publications

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“…Reports in the literature comprise either individual case reports or small case series'. Given that MPNSTs are highly malignant and that the associated survival rate is very low [4], we believe that sharing information regarding the diagnosis, treatment, and prognosis of patients with spinal MPNSTs is imperative to improving treatment outcomes.…”

Section: Introductionmentioning

confidence: 99%

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Zhu

Liu

et al. 2011

Eur Spine J

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Background and objectives To summarise our experience treating patients with spinal malignant peripheral nerve sheath tumours (MPNSTs). Methods We retrospectively reviewed the records of patients diagnosed with spinal MPNSTs who received surgical treatment from January 1998 to December 2009. Results Postoperative follow-up data were available for 14/16 patients with spinal MPNSTs (7 men, 7 women; median age = 44 years [range: 23-68 years]). Eight of 14 (57.1%) patients had primary and 6/14 (42.9%) recurrent MPNSTs. A total of 12/14 (85.7%) patients underwent total tumour resection, whereas 2/14 (14.3%) patients underwent subtotal tumour resection. Malignancies were graded low in 4 (28.6%) and high in 10 (71.1%) cases. A total of 12/14 (85.7%) patients experienced tumour recurrence and 10/14 (71.4%) patients died during the course of follow-up. The 0.5-1-, 3-, and 5-year survival rates were 64.3, 48.2, 32.1, and 21.4%, respectively. Overall survival was significantly associated with tumour malignant degree (P = 0.012).Conclusion Diagnosis of spinal MPNSTs should be made with reference to clinical, radiological, and pathological findings. Surgical resection is the best available option for treating spinal MPNST; however, postoperative prognosis is poor.

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Section: Introductionmentioning

confidence: 99%

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Zhu

Liu

et al. 2011

Eur Spine J

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“…[5][6][7]9,25,43,[45][46][47]50 Although schwannomas tend to occur in individuals with NF2 and schwannomatosis, studies have documented an association of intracerebral schwannomas with NF1. 8,13,16,33,35 Most reported cases have been seen in children or young adults.…”

Section: Discussionmentioning

confidence: 99%

Intracerebral schwannomas: a rare disease with varying natural history

Scott

Koral²,

Margraf

et al. 2013

PED

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Although intracerebral schwannomas are typically regarded as benign intracranial tumors, malignancy and recurrence have been reported among patients harboring such neoplasms. The available literature consists of case reports and small series that present variable characteristics distinguishing these unusual lesions. Little advancement has been made to further the understanding and management of these tumors. The authors present 3 cases from their institution that highlight the difference between typical benign intracerebral schwannomas and histopathological variants that may portend more aggressive behavior. Also provided is a review of the literature in the hope of gaining a better understanding of these rare tumors.

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“…9,12 The majority of these cases involve the trunk and extremities. 31 There is generally no sex or race predilection, but MPNSTs are more commonly found in older patients.…”

Section: Malignant Peripheral Nerve Sheath Tumorsmentioning

confidence: 99%

“…31 There is generally no sex or race predilection, but MPNSTs are more commonly found in older patients. 12 Although it is widely accepted that uncontrolled growth of Schwann cells is the cause of these tumors, it has been suggest ed that they occur de novo and do not arise as a result of malignant transformation from a benign schwanno ma. 1,3,23,31,33 However, some reports have suggested that ra diation may play a role in the development of MPNSTs.…”

Section: Malignant Peripheral Nerve Sheath Tumorsmentioning

confidence: 99%

“…Patients with NF also typically have a much younger age of onset. 9,12,31 Al though the presence of NF is known to greatly increase the odds of developing MPNSTs in general, it is interest ing to note that it has not been shown to play a significant role in MPNSTs of the cranial nerves. 1,33 Of the reports we identified for patients with a trigeminal MPNST, only one patient was described as having NF1 and one as hav ing NF2.…”

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confidence: 99%

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Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Schmidt¹,

Yick²,

Boghani³

et al. 2013

FOC

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Object Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease. Methods A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included. Results From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone. Conclusions Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.

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Malignant Peripheral Nerve Sheath Tumors

Cited by 131 publications

References 64 publications

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Intracerebral schwannomas: a rare disease with varying natural history

Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

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