RISK FACTORS IN PEDIATRIC MALIGNANT PERIPHERAL NERVE SHEATH TUMORS (MPNST): RESULTS FROM THE FRENCH PEDIATRIC ONCOLOGY SOCIETY (SFCE) COHORT

Jordane Chaix,Marie Karanian,Nadege Corradini,Maria Merched,Frederique Larrousserie,Louise Galmiche,Brigitte Lacour,Aude Marie-Cardine,Anne-Sophie Defachelles,Veronique Minard-Colin,Angelique Rome,Estelle Thebaud,Valerie Bernier,Herve Brisse,Frederic Hameury,Pierre Wolkenstein,Stephane Ducassou,Daniel Orbach,Cecile Verite

PEDIATRIC BLOOD & CANCER（2022）

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摘要

•The prognosis of Pediatric MPNST is poor: 5y-OS 46.7% (95%CI, 35.8–60.8).•Type 1 neurofibromatosis is an independent risk factor in pediatric patients.•High grade, large and metastatic tumors are other major risk factors.•Histological subtype, such as malignant triton tumor, is not correlate with survival.

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关键词

Malignant Peripheral Nerve Sheath Tumor,Child,Adolescent,Survival rate,Pathology review,Risk factors,Neurofibromatosis type 1

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