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Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors

Langzeitüberleben nach additiver Radiotherapie bei Patienten mit atypischem teratoiden Rhabdoidtumoren

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Abstract

Background

Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive disease of embryonic origin accounting for <5% of all pediatric central nervous system (CNS) tumors.

Patients and methods

We describe a series of five cases of CNS ATRT. The first three patients underwent subtotal tumor resection. Gross total resection of the tumor was achieved in the fourth and fifth patients. Only 4 patients received chemotherapy, whereas all 5 patients received additive radiotherapy (RT). The latter included three dimensional (3D) conformal RT or intensity modulated RT (IMRT) with a median dose of 54 Gy (range 50.4–59.0 Gy) applied in daily fractions of 1.8 Gy. The median interval between surgery and RT was 5 months (range 2–11 months).

Results

Two months after completion of RT, 4 patients had achieved complete radiologic remission. The median event-free survival period was 46 months (range 10–90 months). However, the first patient died 17 months after developing an out-of-field recurrence. The third patient developed a recurrence 11 months after salvage RT. The other 3 patients (cases 2, 4, and 5) remain alive with no evidence of disease 59, 46 and 90 months after therapy, respectively.

Conclusion

Overall, the 5 patients survived for a median of 48 months (range 25–90 months) from the time of initial diagnosis and they tolerated the RT well, without severe acute or late onset toxicities. The results imply a potential survival gain after irradiation at acceptable toxicity level.

Zusammenfassung

Hintergrund

Atypische teratoide Rhabdoidtumore (ATRT) sind eine hoch aggressive Erkrankung embryonalen Ursprungs und repräsentieren <5% aller pädiatrischen Tumore des zentralen Nervensystems (ZNS).

Patienten und Methoden

Wir beschreiben eine Fallserie von 5 Patienten mit ATRT des ZNS. Bei den ersten 3 Patienten wurde eine subtotale Resektion des Tumors durchgeführt. Bei den anderen beiden Patienten wurden eine komplette Resektion des Tumors erreicht. Eine Chemotherapie erhielten 4 Patienten. Alle 5 Patienten bekamen eine additive Radiotherapie (RT). Eine dreidimensionale (3D) konformale RT oder intensitätmodulierte RT (IMRT) wurde mit einer mittleren Dosis von 54 Gy (Spanne 50,4–59,0 Gy) in einer täglichen Fraktionierung von 1,8 Gy durchgeführt. Das mediane Intervall zwischen Operation und RT betrug 5 Monate (Spanne 2–11 Monate).

Ergebnisse

Zwei Monate nach Abschluss der RT zeigten 4 Patienten eine komplette radiologische Remission. Die mediane ereignisfreie Überlebenszeit betrug 46 Monate (Spanne 10–90 Monate). Allerdings starb der erste Patient 17 Monate nach Entwicklung eines „Out-of-field“-Rezidivs. Der dritte Patient entwickelt ein Rezidiv 11 Monaten nach Salvage-RT. Die anderen 3 Patienten (Fall 2, 4 und 5) sind bisher 59, 46 und 90 Monate nach der Behandlung tumorfrei.

Zusammenfassung

Die mediane Gesamtüberlebenszeit seit Erstdiagnose beträgt 48 Monate (Spanne 25–90 Monate). Die RT wurde insgesamt ohne schwere akute oder späte Toxizitäten vertragen. Die Ergebnisse implizieren einen möglichen Überlebensgewinn nach der Bestrahlung mit einem akzeptablen Toxizitätsniveau.

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Authors and Affiliations

  1. Radiation oncology department, University Hospital Münster, Albert-Schweitzer-Campus 1, Building A1, 48149, Münster, Germany

    Khaled Elsayad MD, Jan Kriz MD, Laith Samhouri MD, Uwe Haverkamp MD PhD & Hans Theodor Eich MD PhD

  2. Pediatric oncology department, University Hospital Münster, Münster, Germany

    Ronald Straeter MD PhD

  3. Neurosurgery department, University Hospital Münster, Münster, Germany

    Walter Stummer MD PhD

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  1. Khaled Elsayad MD
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  2. Jan Kriz MD
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Correspondence to Khaled Elsayad MD.

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Conflict of interest

K. Elsayad, J. Kriz, L. Samhouri, U. Haverkamp, R. Straeter, W. Stummer, and H.T. Eich state that there are no conflicts of interest.

The accompanying manuscript does not include studies on humans or animals.

Additional information

These data were presented in part at the Annual Meeting of the German Society for Radiation Oncology, Hamburg, Germany, June 25–28, 2015.

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Elsayad, K., Kriz, J., Samhouri, L. et al. Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors. Strahlenther Onkol 192, 569–581 (2016). https://doi.org/10.1007/s00066-016-0978-8

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