A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours

Martin, Enrico; Coert, J. Henk; Flucke, Uta; Slooff, Willem-Bart M.; Ho, Vincent K.Y.; Graaf, Winette Ta van der; Dalen, Thijs van; Sande, Michiel A.J. van de; Houdt, Winan J. van; Grünhagen, Dirk J.; Verhoef, Cornelis

doi:10.1016/j.ejca.2019.10.014

Cited by 65 publications

(102 citation statements)

References 35 publications

(53 reference statements)

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“…42 Although R1 resections have been associated with increased risks for local recurrence, they have not been associated with worse survival in both adult and pediatric MPNST. 3,6,17,21 This may provide an opportunity for the adoption of planned positive margins in MPNSTs as well, thus decreasing morbidity in some patients. 43,44 The role of both chemotherapy and radiotherapy is controversial in MPNST, even more so in pediatric patients.…”

Section: Treatment Of Pediatric Mpnstmentioning

confidence: 99%

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Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Martin

Coert

Flucke

et al. 2019

Pediatric Blood & Cancer

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Background Malignant peripheral nerve sheath tumors (MPNST) are rare and aggressive non‐rhabdomyoblastic soft‐tissue sarcomas (NRSTS) in children. This study set out to investigate clinical presentation, treatment modalities, and factors associated with survival in pediatric MPNST using Dutch nationwide databases. Methods Data were obtained from the Netherlands Cancer Registry (NCR) and the Dutch Pathology Database (PALGA) from 1989 to 2017. All primary MPNSTs were collected. Demographic differences were analyzed between adult and pediatric (age ≤18 years) MPNST. In children, demographic and treatment differences between neurofibromatosis type 1 (NF1) and non‐NF1 were analyzed. A Cox proportional hazard model was constructed for localized pediatric MPNSTs. Results A total of 70/784 MPNST patients were children (37.1% NF1). Children did not present differently from adults. In NF1 children, tumor size was more commonly large (> 5 cm, 92.3% vs 59.1%). Localized disease was primarily resected in 90.6%, and radiotherapy was administered in 37.5%. Non‐NF1 children tended to receive chemotherapy more commonly (39.5% vs 26.9%). Overall, estimated five‐year survival rates of localized NF1‐MPNST was 52.4% (SE: 10.1%) compared with 75.8% (SE: 7.1%) in non‐NF1 patients. The multivariate model showed worse survival in NF1 patients (HR: 2.98; 95% CI, 1.17‐7.60, P = 0.02) and increased survival in patients diagnosed after 2005 (HR: 0.20; 95% CI, 0.06‐0.69, P = 0.01). No treatment factors were independently associated with survival. Conclusion Pediatric MPNSTs have presentations similar to adult MPNSTs. In children, NF1 patients present with larger tumors, but are treated similarly to non‐NF1 MPNSTs. In localized pediatric MPNST, NF1 is associated with worse survival. Promisingly, survival has increased for pediatric MPNSTs after 2005.

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Section: Treatment Of Pediatric Mpnstmentioning

confidence: 99%

“…Recently, the influence of NF1 on survival has been subject of debate as studies report conflicting results. 11,15,20,21 As…”

Section: Introductionmentioning

confidence: 99%

Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Martin

Coert

Flucke

et al. 2019

Pediatric Blood & Cancer

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“…Seven studies (including subgroups) [16,[23][24][25]29] comparing the overall survival between male and female were included. Values of I 2 = 55.3% and p= 0.037 were obtained after the HR values of OS were merged, indicating that heterogeneity existed.…”

Section: Sexmentioning

confidence: 99%

“…Ten studies [13,14,17,22,24,25,[29][30][31]38] evaluated tumor size (the large vs. the small) as a risk factor for OS. Values of I 2 = 44.4% and p= 0.063 were obtained after the HR values of OS were merged, indicating that heterogeneity existed.…”

Section: Tumor Sizementioning

confidence: 99%

“…Given the rarity of MPNST, there are sporadic published studies [14,16,[21][22][23] reporting the prognosis and related factors. Although poor survival may result from large tumor size [13,24,25], inadequate margin [13,15,24], high-level tumor grade [13][14][15], or presence of distant metastasis [13,25,26], prognostic factors have not reach an agreement in literatures. Besides, few prognostic factors were reported in each study, and the population of these studies was not enough to indicate more detail factors.…”

Section: Introductionmentioning

confidence: 99%

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Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Cai

Tang

Liang

et al. 2020

Preprint

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Background: No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate and LR rate for MPNST, and to assess potential risk factors for prognosis.Methods: Electronic articles published between January 1, 1966 and February 29, 2020, were searched and critically evaluated. The authors independently reviewed the abstracts and extracted data for 5-year OS rate, 5-year EFS rate, LR rate, and potential risk factors for prognosis.Results: Twenty-eight literatures were finally included for meta-analysis. The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 37%, respectively. The significant prognostic factors for survival were NF-1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival.Conclusion: Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1 mutation, large size, deep to fascia, high grade, metastases and location (trunk and head & neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies.

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Pancreas, Adrenal Glands, and Retroperitoneum

Hryhorczuk

Paltiel

2021

Pediatric Ultrasound

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A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours

Cited by 65 publications

References 35 publications

Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Pancreas, Adrenal Glands, and Retroperitoneum

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