Malignant peripheral nerve sheath tumors: clinicopathological aspects, expression of p53 and survival

Cunha, Karin Soares Gonçalves; Caruso, Anabela Cunha; Faria, Paulo Antônio Silvestre de; Silva, Licínio Esmeraldo da; Pires, Andrea; Geller, Mauro; Lopes, Vânia Silami

doi:10.6061/clinics/2012(08)18

Cited by 15 publications

(12 citation statements)

References 20 publications

(34 reference statements)

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“…Considering the patients who received curative treatment, the 5-year diseasefree survival rate was 39%. All these data were showed in the previous study in which we used the same sample (Cunha et al 2012). In that study, we evaluated the influence of clinical and pathological features on overall and disease free survival in patients with MPNSTs: sex, presence of NF1, tumor location, treatment performed, status of the surgical margins, local recurrence, metastasis, histological grade, mitotic index, necrosis, epithelioid variant, Triton tumor and chondrosarcoma differentiation.…”

Section: Data Regarding the Immunostaining For Bcl-2mentioning

confidence: 98%

“…The samples of the tumors were included in two tissue microarray (TMA) paraffin blocks. For detailed information about the construction method for TMA block, please refer to the previous published paper (Cunha et al 2012) All the cases were reviewed by two pathologists. For neurofibromas, only cases with heterogeneous and diffuse immunostaining for S-100 protein were included.…”

Section: Samplementioning

confidence: 99%

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Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

Cunha

Caruso²,

Faria³

et al. 2013

An. Acad. Bras. Ciênc.

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AIMS:To study the expression of Bcl-2, Bcl-x, as well the presence of cleaved caspase-3 in neurofibromas and malignant peripheral nerve sheath tumors. The expression of Bcl-2 and Bcl-x and the presence of cleaved caspase 3 were compared to clinicopathological features of malignant peripheral nerve sheath tumors and their impact on survival rates were also investigated. MATERIALS AND METHODS: The evaluation of Bcl-2, Bcl-x and cleaved caspase-3 was performed by immunohistochemistry using tissue microarrays in 28 malignant peripheral nerve sheath tumors and 38 neurofibromas. Immunoquantification was performed by computerized digital image analysis. CONCLUSIONS: Apoptosis is altered in neurofibromas and mainly in malignant peripheral nerve sheath tumors. High levels of cleaved caspase-3 are more common in tumors with more aggressive histological features and it is associated with lower disease free survival of patients with malignant peripheral nerve sheath tumors.

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Section: Data Regarding the Immunostaining For Bcl-2mentioning

confidence: 98%

Section: Samplementioning

confidence: 99%

Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

Cunha

Caruso²,

Faria³

et al. 2013

An. Acad. Bras. Ciênc.

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“…The French system (Fédération Nationale des Centres de Lutte Contre le Cancer [FNCLCC]) and the WHO grading system are the most commonly used systems for grading soft tissue sarcomas. However, these systems have no prognostic value for some histological subtypes, including MPNSTs 24,28. The WHO grading system may need refinement for MPNST grading, particularly in high-grade categories.…”

Section: Discussionmentioning

confidence: 99%

Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors

L’Heureux-Lebeau¹,

Saliba²

2013

OTT

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Background:Malignant peripheral nerve sheath tumors (MPNSTs) are rare entities and MPNSTs of intracranial nerves are even more sporadic. MPNSTs present diagnosis and treatment challenges since there are no defined diagnosis criteria and no established therapeutic strategies.Methods:We reviewed literature for MPNST-related articles. We found 45 relevant studies in which 60 cases were described.Results:We identified 60 cases of intracranial nerve MPNSTs. The age ranged from 3 to 75 years old. Male to female ratio was 1.5:1. The most involved cranial nerves (CNs) were CN VIII (60%), CN V (27%), and CN VII (10%). Most of the MPNSTs reported (47%) arose sporadically, 40% arose from a schwannoma, 8% arose from a neurofibroma, and 6% arose from an unspecified nerve tumor. Twenty patients had a history of radiation exposure, four patients had neurofibromatosis type 1 (NF1), four patients had neurofibromatosis type 2 (NF2), and NF2 was suspected in two other patients. Twenty-two patients were treated with radiotherapy and presented a higher survival rate. Seventy-two percent of patients died of their disease while 28% of patients survived. One-year survival rate was 33%. Forty-five percent of tumors recurred and 19% of patients had metastases.Conclusion:MPNSTs involving CNs are very rare. Diagnosis is made in regards to the histological and pathological findings. Imaging may help orient the diagnosis. A preexisting knowledge of the clinical situation is more likely to lead to a correct diagnosis. The mainstay of treatment is radical surgical resection with adjuvant radiotherapy. Since these tumors are associated with a poor prognosis, a close follow-up is mandatory.

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“…Korin et al 2012, reported 5-year overall survival rate of 46% in patients with MPNSTs [20]. Though multimodality therapy, including surgical resection and adjuvant radiotherapy, the prognosis remains dismal [21].…”

Section: H M Hendam Et Al Open Journal Of Modern Neurosurgerymentioning

confidence: 99%

Surgical Approaches to Large Peripheral Nerve Sheets Tumors

Hendam¹,

Samouly²,

Behairy³

2018

OJMN

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Peripheral nerve sheath tumors (PNSTs) are not rare tumors. Its sound management is complete surgical excision. The aim of this study was to describe clinical presentation, accuracy of different investigations and ideal surgical approaches in relation to outcomes. The study is a prospective and included 26 surgically treated large PNSTs in 22 patients operated through different surgical approaches according to the site of the tumors. The fellow up period was 6 -60 months. The mean age was 41.8 years, 9 were males and 13 were females. The common presentations were pain in 12 lesions, swelling in 8 lesions, neural deficits in 4 lesions and positive Tinel's test in 2 lesions. These lesions were excised through 6 surgical approaches. Histopathological examination showed 20 benign and 6 malignant lesions. Five cases of malignant peripheral nerve sheet tumors (MPNSTs) had local recurrent, 4 cases had distant metastasis, 4 cases received chemotherapy and radiotherapy, and 4 cases were died in the postoperative fellow up period. It was concluded that surgical approaches to the PNSTs, depends on their locations. Adequate exposure with minimal dissection minimized the neurological deficit and decreased rate of local recurrence.

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Malignant peripheral nerve sheath tumors: clinicopathological aspects, expression of p53 and survival

Cited by 15 publications

References 20 publications

Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors

Surgical Approaches to Large Peripheral Nerve Sheets Tumors

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