Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS‐2005 prospective study

Noesel, Max M. van; Orbach, Daniel; Brennan, Bernadette; Kelsey, Anna; Zanetti, Ilaria; Salvo, Gian Luca De; Gaze, Mark; Craigie, Ross; McHugh, Kieran; Francotte, Nadine; Collini, Paola; Bisogno, Gianni; Casanova, Michela; Ferrari, Andrea

doi:10.1002/pbc.27833

Cited by 53 publications

(79 citation statements)

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“…Although survival rates in pediatric MPNST from previous studies show comparable results as in adult MPNST, another study using SEER data showed that children had a better prognosis when controlling for known confounders . Few other studies have found factors associated with survival in pediatric MPNST . NF1 status has previously been reported as well to be independently associated with worse survival in children .…”

Section: Discussioncontrasting

confidence: 69%

“…Few other studies have found factors associated with survival in pediatric MPNST . NF1 status has previously been reported as well to be independently associated with worse survival in children . It is not yet completely clear what NF1‐related factors cause this difference.…”

Section: Discussionmentioning

confidence: 85%

“…Anthracycline‐based regimens with or without additional ifosfamide have shown superior results in a large cohort of pediatric patients in a study published in 2005 . The European Pediatric Soft‐Tissue Sarcoma Group (EpSSG) consequently published results of their 2005‐2016 cohort in which doxorubicin and ifosfamide was used whenever chemotherapy was administered . The study by the EpSSG showed higher five‐year survival rates compared with the earlier publication of the Italian and German Soft‐Tissue Sarcoma Cooperative Group.…”

Section: Discussionmentioning

confidence: 91%

“…In part, it may be due to lower chemosensitivity, which has been suggested in NF1 patients . However, in the EpSSG study, similar response rates were seen between NF1 and non‐NF1 children . The impact of NF1 status on survival in adults has been controversial as well.…”

Section: Discussionmentioning

confidence: 99%

“…To date, surgery remains the key to improve survival in any localized MPNST . However, MPNSTs have been reported unresectable in 17% to 53%, which is higher than other NRSTS . Also, when unresectable, clinical response to neoadjuvant chemotherapy is lowest in MPNSTs compared with other NRSTS, especially in NF1 patients .…”

Section: Introductionmentioning

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: Discussioncontrasting

confidence: 69%

Section: Discussionmentioning

confidence: 85%

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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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The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

et al. 2023

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Background Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data. Methods The INSTRuCT non‐rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors. Results and Conclusion This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research.

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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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Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS‐2005 prospective study

Cited by 53 publications

References 24 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

The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

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

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