Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

Grobmyer, Stephen R.; Reith, John D.; Shahlaee, Amir H.; Bush, Charles H.; Hochwald, Steven N.

doi:10.1002/jso.20971

Cited by 182 publications

(190 citation statements)

References 91 publications

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“…Further, the presentation of MPNSTs can vary greatly, making diagnosis challenging [7]. Magnetic resonance imaging can be particularly useful for determining the features of a malignancy such as illdefined margins, heterogeneity, and peripheral reactive edema surrounding the lesion.…”

Section: Discussionmentioning

confidence: 99%

“…Surgical resection is the treatment of choice for MPNSTs [7]. As with other soft tissue sarcomas, en bloc resection is often ideal for MPNSTs.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, in our cohort, only one patient received postoperative radiotherapy. The effectiveness of adjuvant chemotherapy for MPNST remains controversial [7]. However, it is worth noting that relatively high overall response rates (45%) have been found in paediatric patients with MPSNTs who received adjuvant chemotherapy [14].…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis for patients with MPNST is relatively poor, with recurrence rates estimated to range from 20 to 40% [7] and 5-year survival rates ranging from 34 to 52% [2,5]. A number of factors are known to influence prognosis including tumour size, location, and histological grade, whether removal is en bloc (or not), resection margin, the presence of recurrence, and metastasis [7].…”

Section: Discussionmentioning

confidence: 99%

“…A number of factors are known to influence prognosis including tumour size, location, and histological grade, whether removal is en bloc (or not), resection margin, the presence of recurrence, and metastasis [7]. Of these, having a negative surgical resection margin is thought to be the most significant prognostic factor for survival [5].…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionmentioning

confidence: 99%

“…Surgical resection is the treatment of choice for MPNSTs [7]. As with other soft tissue sarcomas, en bloc resection is often ideal for MPNSTs.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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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Complete, Pupil-Sparing Third Nerve Palsy in a Patient With a Malignant Peripheral Nerve Sheath Tumor

Fard

Montgomery²,

Miller³

2011

Arch Ophthalmol

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Natamycin and Voriconazole in Fungal Keratitis—Reply

2011

Arch Ophthalmol

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Comment. Malignant peripheral nerve sheath tumors are rare tumors that originate from peripheral or cranial nerves or result from malignant transformation of a benign schwannoma or neurofibroma. 2-4 They may sometimes be confused with amelanotic melanomas. In our case, the lack of KIT mutations and the absence of melanosomes in any of the cells were consistent with a diagnosis of MPNST rather than amelanotic melanoma. 4 Although MPNSTs can invade the paranasal sinuses, origin within the sinuses is extremely rare. 2 These tumors almost never affect the cranial nerves, but when they do, the nerve most commonly affected is the facial nerve. 3 We are unaware of any cases in which an OMNP was the manifesting sign. This case is even more unusual in that neither the size nor the reactivity of the pupil was affected.What was the cause of third nerve palsy in our case? One possibility is that it was related not to the tumor but to the patient's underlying vascular disease. This would be consistent with the painless onset of the palsy and its complete resolution after about 7 months, although this is somewhat longer than one would expect in such a setting. Although lesions of the sphenoid sinus do not commonly produce third nerve palsies, a few such examples have been reported. 5,6 Thus, the mass could have affected the oculomotor nerve in the anterior cavernous sinus by compression, by producing an inflammatory reaction, or by causing focal ischemia. Any of these mechanisms could account for the rapid resolution of the palsy following resection of the lesion. In any event, this case provides support for the recommendation that a patient with an isolated, complete, pupil-sparing OMNP be evaluated with neuroimaging when there is no evidence of improvement after several months. A u t h o r A f f i l i a t i o n s : D e p a r t m e n t o f N e u r oophthalmology, Wilmer Eye Institute (Drs Fard and Miller

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Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

Cited by 182 publications

References 91 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

Complete, Pupil-Sparing Third Nerve Palsy in a Patient With a Malignant Peripheral Nerve Sheath Tumor

Natamycin and Voriconazole in Fungal Keratitis—Reply

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