The Importance of Predicting Factors in the Surgical Outcome of Peripheral Nerve Sheath Tumors

Vetrano, Ignazio G.; Lucarella, F.; Dalolio, Martina; Cristofori, Andrea Di; Nataloni, Ilaria; Tiberio, F.; Gaini, S. M.; Grimoldi, N.

doi:10.1055/s-0033-1348351

Cited by 3 publications

(3 citation statements)

References 21 publications

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“…MPNST are rare and perplexing malignancies. The best clinical outcomes are linked to early diagnosis and treatment [ 8 ]. However, several factors combine to make the diagnosis challenging.…”

Section: Discussionmentioning

confidence: 99%

“…Vetrano et al reviewed the clinical, histologic, and imaging data of 42 patients treated at their institution to identify factors that may improve the surgical outcome of peripheral nerve sheath tumors. The timing of surgery was found to be the most important predictive factor of outcome with MPNSTs having the worst outcomes [ 8 ]. Notwithstanding a larger recurrent lesion, we excised the tumor en bloc without sacrificing critical structures that could affect the functional outcome.…”

Section: Discussionmentioning

confidence: 99%

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Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report

Mencia,

Moonsie

2023

jocr

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Introduction: A malignant peripheral nerve sheath tumor (MPNST) is a rare soft-tissue sarcoma with a high recurrence rate and poor prognosis. Early diagnosis and complete surgical excision are the fundamental principles of treatment. A benign presentation and low clinical suspicion often delay definitive diagnosis, and en bloc excision may not be feasible depending on the size and location of the tumor. We describe a rare case of a recurrent MPNST successfully treated by surgical excision. Case Report: A 35-year-old woman presented with a rapidly growing painful mass 3 months following incomplete removal of a MPNST from her forearm. Staging investigations showed no evidence of metastasis. The patient underwent en-bloc surgical excision, split skin grafting, and adjuvant radiation therapy. Histology and immunohistochemical analysis confirmed a MPNST. Five years after having surgery, the patient shows no evidence of recurrence and has excellent function. Conclusion: MPNST are rare soft tissue sarcomas that can masquerade as benign lumps. There is a paucity of literature on the outcome of surgically-treated recurrent disease. Notwithstanding local recurrence of the tumor, complete surgical excision can yield excellent clinical results. Keywords: Malignant peripheral nerve sheath tumor, soft-tissue sarcoma, en bloc excision, recurrence, radiation therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report

Mencia,

Moonsie

2023

jocr

View full text Add to dashboard Cite

show abstract

“…The mainstay of PNST treatment is surgical removal [ 1 , 3 , 11 , 12 , 13 , 14 , 15 , 16 ], although this approach is not always feasible, as in the case of huge schwannomas or PNFs. Moreover, although the different subtypes of PNST are considered benign tumors according to the biological behavior, PNFs differ from NFs and schwannomas due to their propensity for malignant degeneration.…”

Section: Introductionmentioning

confidence: 99%

The Lipid Asset Is Unbalanced in Peripheral Nerve Sheath Tumors

Vetrano

Dei

Nazzi

et al. 2021

IJMS

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Peripheral nerve sheath tumors (PNSTs) include schwannomas, neurofibromas (NFs), and plexiform neurofibromas (PNFs), among others. While they are benign tumors, according to their biological behavior, some have the potential for malignant degeneration, mainly PNFs. The specific factors contributing to the more aggressive behavior of some PNSTs compared to others are not precisely known. Considering that lipid homeostasis plays a crucial role in fibrotic/inflammatory processes and in several cancers, we hypothesized that the lipid asset was also unbalanced in this group of nerve tumors. Through untargeted lipidomics, NFs presented a significant increase in ceramide, phosphatidylcholine, and Vitamin A ester. PNFs displayed a marked decrease in 34 out of 50 lipid class analyzed. An increased level of ether- and oxidized-triacylglycerols was observed; phosphatidylcholines were reduced. After sphingolipidomic analysis, we observed six sphingolipid classes. Ceramide and dihydroceramides were statistically increased in NFs. All the glycosylated species appeared reduced in NFs, but increased in PNFs. Our findings suggested that different subtypes of PNSTs presented a specific modulation in the lipidic profile. The untargeted and targeted lipidomic approaches, which were not applied until now, contribute to better clarifying bioactive lipid roles in PNS natural history to highlight disease molecular features and pathogenesis.

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The effect of ozone injection within a common peroneal nerve schwannoma: A mistreatment due to a misdiagnosis

Vetrano

Acerbi

Marucci

et al. 2020

Surgical Neurology International

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Background: Peripheral schwannomas can be misdiagnosed or mistreated as they can mimic other subcutaneous lesions, leading to wrong diagnosis and, therefore, to improper treatment. Case Description: A 23-years-old male presented a painful growing nodule at the left popliteal fossa, with distally irradiated pain. A first magnetic resonance imaging depicted a heterogeneous lesion between common peroneal and sural nerves but, surprisingly, the patient was submitted to perilesional injection of ozone-oxygen mixture, causing the onset of intense neuropathic pain. A second MRI showed a morphological change of tumor characteristics. He finally underwent surgery but, intraoperatively, inter-fascicular fibrous adherences were noticed, making the tumor removal more difficult and riskier. The histopathological diagnosis was of schwannoma with areas of foreign body reaction. Conclusion: The injection of ozone or other substances within a subcutaneous swelling should be avoided, before a complete imaging assessment; because of such swelling could be a peripheral nerve schwannoma. The correct assessment of a lesion of the limbs determining radiating pain should be carefully demanded to a thorough history, clinical examination, and appropriate imaging technique. To avoid incorrect management, the treatment of such tumors should be performed in the first place by dedicated equips with proven expertise in this field.

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The Importance of Predicting Factors in the Surgical Outcome of Peripheral Nerve Sheath Tumors

Abstract: The timing of surgery is the most important predictive factor of surgical outcome, being the only factor that allows to improve the outcome. With the current study, we want to stress the importance of treating PNSTs as soon as possible to provide the best outcome possible.

Cited by 3 publications

References 21 publications

Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report

Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report

The Lipid Asset Is Unbalanced in Peripheral Nerve Sheath Tumors

The effect of ozone injection within a common peroneal nerve schwannoma: A mistreatment due to a misdiagnosis

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