Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience

Sobczuk, Paweł; Teterycz, Paweł; Czarnecka, Anna; Świtaj, Tomasz; Koseła-Paterczyk, Hanna; Kozak, Katarzyna; Falkowski, Sławomir; Goryń, Tomasz; Zdzienicki, Marcin; Morysiński, Tadeusz; Rutkowski, Piotr

doi:10.1016/j.suronc.2020.09.011

Cited by 7 publications

(34 citation statements)

References 35 publications

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“…While there are few studies showing no significant association between histologic grade and survival, 10,23 a growing body of research suggests that high FNCLCC grade is associated with unfavorable survival outcomes in MPNST. 3,5,7,11,24 Among recent studies, Valentin et al 3 retrospectively evaluated survival outcomes in a cohort of 353 individuals seen in multiple French cancer centers, demonstrating worse overall and disease-specific survival in patients with FNCLCC grade 3 tumors compared with those with lower grade lesions. In this study, tumors located in the trunk, head and neck, and deep sites also had worse survival outcomes compared with those in the extremities and superficial locations.…”

Section: Discussionmentioning

confidence: 99%

“…1 It arises most frequently in 3 clinical settings: sporadic (40% to 50%), type-1 neurofibromatosis (NF1; 22% to 50%), and in a previously radiated field (3% to 10%). [2][3][4][5] MPNST is generally considered an aggressive neoplasm, with a reported 5-year mortality ranging from 23% to 69%, despite multimodality treatment. 3 A limited number of studies have investigated the prognostic value of clinical and pathologic features of MPNST.…”

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confidence: 99%

“…Malignant peripheral nerve sheath tumor (MPNST) is a relatively uncommon type of sarcoma, with an overall incidence rate of 1.46 per million person‐years 1. It arises most frequently in 3 clinical settings: sporadic (40% to 50%), type-1 neurofibromatosis (NF1; 22% to 50%), and in a previously radiated field (3% to 10%) 2–5. MPNST is generally considered an aggressive neoplasm, with a reported 5-year mortality ranging from 23% to 69%, despite multimodality treatment 3…”

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confidence: 99%

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Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) Grading, Margin Status and Tumor Location Associate With Survival Outcomes in Malignant Peripheral Nerve Sheath Tumors

Wakeman

Zhang

Bandhlish³

et al. 2021

American Journal of Clinical Oncology

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Background: Histologic grading using the Fédération Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) system is not universally accepted as applicable to malignant peripheral nerve sheath tumor (MPNST), as its prognostic value is not well established. Methods:We retrospectively evaluated 99 cases of MPNST to investigate any association between the outcomes overall survival (OS) and progression-free survival (PFS), and predictor variables FNCLCC grade, clinical setting, tumor location, and tumor size at diagnosis using multivariable Cox proportional hazard analysis.Results: Univariable and multivariable analysis demonstrate a statistically significant association between FNCLCC grade and both OS and PFS when comparing tumors by histologic grade. Of note, no deaths were observed in patients with grade 1 MPNST. Other variables associated with unfavorable outcomes include fragmented resection and primary site, with tumors in the extremities having favorable OS, but not PFS, when compared with those in truncal locations. Tumors in the head and neck had favorable PFS, but not OS, compared with those in the trunk. No statistically significant differences in OS or PFS were observed when comparing patient age and sex, tumor size at diagnosis, clinical setting (primary vs. type-1 neurofibromatosis vs. radiation associated) or history of neoadjuvant therapy. Interobserver agreement for FNCLCC grading of these tumors was considered good (S* = 0.77, 95% confidence interval: 0.71-0.84).Conclusions: Association between FNCLCC grading and survival outcomes in MPNST suggests potential value to routinely grading these neoplasms. However, the subjectivity of the grading system, particularly when assigning a tumor differentiation score, may pose a challenge, especially in low and intermediate grade lesions.

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confidence: 99%

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confidence: 99%

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confidence: 99%

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Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) Grading, Margin Status and Tumor Location Associate With Survival Outcomes in Malignant Peripheral Nerve Sheath Tumors

Wakeman

Zhang

Bandhlish³

et al. 2021

American Journal of Clinical Oncology

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“…Some retrospective analyses have shown shorter survival for patients with MPNST associated with NF1 [100][101][102]. However, other studies did not confirm significant differences [103][104][105]. Because of the lack of unequivocal data concerning differences in prognosis, the procedure recommended for treating MPNST associated with NF1 is in agreement with general guidelines for MPNST treatment.…”

Section: Treatmentmentioning

confidence: 99%

“…Because of the lack of unequivocal data concerning differences in prognosis, the procedure recommended for treating MPNST associated with NF1 is in agreement with general guidelines for MPNST treatment. Qualification of patients for treatment should be done by a multispecialist panel [106,107].…”

Section: Treatmentmentioning

confidence: 99%

Recommendations of the Polish Sarcoma Group on diagnostic-therapeutic procedures and control in patients with type 1 neurofibromatosis (NF1) and the associated malignant neoplasm of peripheral nerve sheaths

Rutkowski

Raciborska²,

Szumera‐Ciećkiewicz

et al. 2022

Nowotwory. Journal of Oncology

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This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.According to the authors, this elaboration contains the most justified principles of diagnostic-therapeutic procedures. They should, however, be interpreted in relation to the particular clinical situation. The recommendations do not always correspond to the current bases of refunding treatment in force in Poland (which is noted in the text). In the case of doubt, the current possibilities of refunding particular procedures should be ascertained.

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Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1‐CREM fusion: A case report and literature review

Hong

Wang

et al. 2023

Intl J Gynecology & Obste

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Epithelioid malignant peripheral nerve sheath tumor (EMPNST) is a rare soft tissue sarcoma. The authors report the first case of EMPNST arising in the ovary (OEMPNST). A 7‐year‐old child underwent left salpingo‐oophorectomy due to tumor rupture and the pathology suggested a juvenile granulosa cell tumor (JGCT). Six cycles of bleomycin, etoposide, and carboplatin were administrated. A second surgery was applied due to relapse 4 months after the last cycle of chemotherapy, and the pathology revealed JGCT with extensive abdominopelvic seedings even after interinstitutional consultation in two hospitals. Next‐generation sequencing demonstrated EWSR1 exon12‐CREM exon6 fusion with neurofibromatosis‐2 gene deletion, and no mutation was detected in either FOXL2 or DICER1. However, pathology consultation in two other hospitals suggested the diagnosis of OEMPNST, and additional immunohistochemical (IHC) staining revealed positive H3K27me3. Nonetheless, she was treated with nine courses of chemotherapy but experienced a second recurrence of extensive abdominal metastases approximately 3 months after ceasing chemotherapy. Neither elevated tumor makers nor abnormal sex hormones level was noted since the initial presentation. Repeated cytoreductive surgery was conducted and IHC staining showed expression of SOX10, S‐100, INI‐1, and α‐inhibin in tumor tissue. A final diagnosis of OEMPNST with EWSR1‐CREM fusion was established, indicating that the probability of OEMPNST could not be excluded when treatment for JGCT showed poor response. A comprehensive evaluation including biological characteristics, morphology, IHC staining, and molecular features is vital in the differential diagnosis between JGCT and OEMPNST.

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Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience

Cited by 7 publications

References 35 publications

Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) Grading, Margin Status and Tumor Location Associate With Survival Outcomes in Malignant Peripheral Nerve Sheath Tumors

Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) Grading, Margin Status and Tumor Location Associate With Survival Outcomes in Malignant Peripheral Nerve Sheath Tumors

Recommendations of the Polish Sarcoma Group on diagnostic-therapeutic procedures and control in patients with type 1 neurofibromatosis (NF1) and the associated malignant neoplasm of peripheral nerve sheaths

Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1‐CREM fusion: A case report and literature review

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