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Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR)

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A Correction to this article was published on 27 July 2023

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Abstract

Purpose

Atypical teratoid/rhabdoid tumors (ATRT) of the central nervous system (CNS) are rare tumors with a poor prognosis and variable use of either focal or craniospinal (CSI) radiotherapy (RT). Outcomes on the prospective Pediatric Proton/Photon Consortium Registry (PPCR) were evaluated according to RT delivered.

Methods

Pediatric patients receiving RT were prospectively enrolled on PPCR to collect initial patient, disease, and treatment factors as well as provide follow-up for patient outcomes. All ATRT patients with evaluable data were included. Kaplan–Meier analyses with log-rank p-values and cox proportional hazards regression were performed.

Results

The PPCR ATRT cohort includes 68 evaluable ATRT patients (median age 2.6 years, range 0.71–15.40) from 2012 to 2021. Median follow-up was 40.8 months (range 3.4–107.7). Treatment included surgery (65% initial gross total resection or GTR), chemotherapy (60% with myeloablative therapy including stem cell rescue) and RT. For patients with M0 stage (n = 60), 50 (83%) had focal RT and 10 (17%) had CSI. Among patients with M + stage (n = 8), 3 had focal RT and 5 had CSI. Four-year overall survival (OS, n = 68) was 56% with no differences observed between M0 and M + stage patients (p = 0.848). Local Control (LC) at 4 years did not show a difference for lower primary dose (50–53.9 Gy) compared to ≥ 54 Gy (73.3% vs 74.7%, p = 0.83). For patients with M0 disease, four-year OS for focal RT was 54.6% and for CSI was 60% (Hazard Ratio 1.04, p = 0.95. Four-year event free survival (EFS) among M0 patients for focal RT was 45.6% and for CSI was 60% (Hazard Ratio 0.71, p = 0.519). For all patients, the 4-year OS comparing focal RT with CSI was 54.4% vs 60% respectively (p = 0.944), and the 4-year EFS for focal RT or CSI was 42.8% vs 51.4% respectively (p = 0.610).

Conclusion

The PPCR ATRT cohort found no differences in outcomes according to receipt of either higher primary dose or larger RT field (CSI). However, most patients were M0 and received focal RT. A lower primary dose (50.4 Gy), regardless of patient age, is appealing for further study as part of multi-modality therapy.

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Funding

Data for this project was obtained from the multi-center Pediatric Proton and Photon Consortium Registry (PPCR) sponsored by the NIH/MGH. The PPCR is supported by the Federal Share of program income earned by Massachusetts General Hospital on C06 CA059267, Proton Therapy Research and Treatment Center.

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Authors and Affiliations

  1. SUNY Upstate Medical University, 750 E Adams St, Syracuse, NY, 13210, USA

    Andrew Roehrig & Paul D. Aridgides

  2. University of Florida, Gainesville, USA

    Daniel J. Indelicato

  3. MD Anderson Cancer Center, Houston, USA

    Arnold C. Paulino

  4. University of Washington, Seattle, USA

    Ralph Ermoian

  5. Northwestern Medicine Chicago Proton Center, Warrenville, USA

    William Hartsell

  6. Cincinnati Children’s Hospital Medical Center, Cincinnati, USA

    John Perentesis

  7. University of Pennsylvania, Philadelphia, USA

    Christine Hill-Kayser

  8. ProCure Proton Therapy Center, Princeton Radiation Oncology, Somerset NJ, USA

    Jae Y. Lee

  9. Mayo Clinic Rochester, Rochester, USA

    Nadia N. Laack

  10. Texas Center for Proton Therapy, Irving, USA

    Victor Mangona

  11. University of California San Diego, San Diego, USA

    Iain MacEwan

  12. Emory University Proton Center, Atlanta, USA

    Bree R. Eaton

  13. Massachusetts General Hospital, Boston, USA

    Sara Gallotto, Benjamin V. M. Bajaj & Torunn I. Yock

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  1. Andrew Roehrig
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Andrew Roehrig, Paul Aridgides, Torunn Yock, Daniel Indelicato, Sarah Gallotto, and Benjamin Bajaj. The first draft of the manuscript was written by Andrew Roehrig and Paul Aridgides and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Paul D. Aridgides.

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Paul Aridgides and Torunn I. Yock contributed equally as co-senior authors.

The original version of this article has been revised: Figure 4 has been corrected

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Roehrig, A., Indelicato, D.J., Paulino, A.C. et al. Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR). J Neurooncol 162, 353–362 (2023). https://doi.org/10.1007/s11060-023-04296-5

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