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Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor

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Abstract

Central nervous system primitive neuroectodermal tumors (CNS PNETs) predominantly occur in children and rarely in adults. Because of the rarity of this tumor, its outcomes and prognostic variables are not well characterized. The purpose of this study was to evaluate clinical outcomes and prognostic factors for children and adults with CNS PNET. The records of 26 patients (11 children and 15 adults) with CNS PNET from 1991 to 2011 were reviewed retrospectively. Disease-free survival (DFS) and overall survival (OS) were estimated with the Kaplan–Meier method, and relevant prognostic factors were analyzed. For the cohort, both the 5-year DFS and the OS were 46 %. For pediatric patients, the 5-year DFS was 78 %; for adult patients, it was 22 % (P = 0.004). Five-year OS for the pediatric and adult patients was 67 and 33 %, respectively (P = 0.07). With bivariate analysis including chemotherapy regimen (high dose vs. standard vs. nonstandard) or risk stratification (standard vs. high) and age, the increased risk of disease recurrence in adults persisted. A nonsignificant tendency toward poorer OS in adult patients relative to pediatric patients also persisted. High-dose chemotherapy with stem cell rescue was associated with a statistically significant improvement in OS and a tendency toward improved DFS, although the findings were mitigated when the effect of age was considered. Local recurrence was the primary pattern of treatment failure in both adults and children. Our results suggest that adult patients with CNS PNETs have inferior outcomes relative to the pediatric cohort. Further research is needed to improve outcomes for CNS PNET in populations of all ages.

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Abbreviations

CD:

Cluster of differentiation

CCG:

Children’s Cancer Group

CNS:

Central nervous system

DFS:

Disease-free survival

GTR:

Gross total resection

HDCT:

High-dose chemotherapy

HR:

Hazard ratio

OS:

Overall survival

PNET:

Primitive neuroectodermal tumor

NF:

Neurofilament

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Authors and Affiliations

  1. Department of Radiation Oncology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Lindsay C. Brown, Robert T. Foote & Nadia N. Laack

  2. Department of Radiology, Mayo Clinic, Rochester, MN, USA

    Laurence J. Eckel

  3. Division of Pediatric Hematology and Oncology, Mayo Clinic, Rochester, MN, USA

    Amulya A. NageswaraRao

  4. Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA

    Jan C. Buckner

  5. Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA

    Ian F. Parney & Nicholas M. Wetjen

  6. Mayo Medical School, Mayo Clinic College of Medicine, Rochester, MN, USA

    Rachael A. Lester

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  1. Rachael A. Lester
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  2. Lindsay C. Brown
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  3. Laurence J. Eckel
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Correspondence to Nadia N. Laack.

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Lester, R.A., Brown, L.C., Eckel, L.J. et al. Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol 120, 371–379 (2014). https://doi.org/10.1007/s11060-014-1561-8

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  • DOI: https://doi.org/10.1007/s11060-014-1561-8

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