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Strahlentherapie und Onkologie

, Volume 194, Issue 3, pp 225–234 | Cite as

Medulloblastoma in adults

A retrospective single institution analysis
  • Indrawati Hadi
  • Olarn Roengvoraphoj
  • Maximilian Niyazi
  • Falk Roeder
  • Ulrich Schüller
  • Claus Belka
  • Silke Birgit Nachbichler
Original Article

Abstract

Purpose

Adult medulloblastoma is a rare disease treated according to the current pediatric treatment guidelines. This retrospective analysis investigated the clinical outcomes and prognostic factors of adult medulloblastoma patients, who received multimodal therapy at our institution.

Methods

Treatment charts of all patients over the age of 15 years of age with de novo medulloblastoma, who had been treated at our institution between 2001 and 2014, were retrospectively analyzed. Patients’ demographic parameters, initial symptoms, treatment modalities, toxicities, and survival outcomes were investigated.

Results

In all, 21 patients with a median age of 30.2 years were identified. The most frequent histologies were desmoplastic and classic, and the most common molecular subtype was sonic hedgehog (SHH). After tumor resection, all patients received craniospinal irradiation (median dose 35.2 Gy) and a boost to the posterior fossa (median dose 19.8 Gy). Simultaneous chemotherapy with vincristine was given to 20 patients and sequential chemotherapy to 15 patients. The most common side effects were hematological toxicities. Median overall survival (OS) has not been reached after a median follow-up of 92 months. Estimated 5‑ and 10-year OS was 89 and 80%, respectively. Estimated 5‑ and 10-year progression-free survival (PFS) was 89 and 81%, respectively. In univariate analysis, a shorter interval between tumor resection and end of irradiation was significantly associated with improved OS and PFS, anaplastic histology with worse OS and PFS.

Conclusions

The combined modality treatment showed a good outcome in adults with medulloblastoma. Treatment time was revealed to be prognostic and should be kept as short as possible.

Keywords

Craniospinal irradiation Radiotherapy Combined modality treatment Prognostic factors Survival 

Medulloblastom beim Erwachsenen

Eine retrospektive Analyse einer Einzelinstitution

Zusammenfassung

Zielsetzung

Das Medulloblastom des Erwachsenen ist eine seltene Erkrankung, die analog pädiatrischer Behandlungsprotokolle therapiert wird. Diese retrospektive Analyse untersuchte die klinischen Ergebnisse und prognostischen Faktoren von erwachsenen Medulloblastompatienten, die eine multimodale Therapie in unserer Einrichtung erhalten haben.

Methoden

Die Akten aller Patienten mit De-novo-Medulloblastomen, die älter als 15 Jahre waren und in unserer Einrichtung zwischen 2001 und 2014 behandelt wurden, wurden retrospektiv ausgewertet. Demographische Parameter, initiale Symptome, Behandlungsmodalitäten, Toxizitäten und Überlebensresultate wurden untersucht.

Ergebnisse

Insgesamt wurden 21 Patienten mit einem medianen Alter von 30,2 Jahren identifiziert. Die häufigsten Histologien waren desmoplastisch und klassisch, der vorherrschende molekulare Subtyp war „sonic hedgehog“ (SHH). Nach der Tumorresektion erhielten alle Patienten eine kraniospinale Bestrahlung (mediane Dosis 35,2 Gy) und einen Boost auf die hintere Schädelgrube (mediane Dosis 19,8 Gy). Eine simultane Chemotherapie mit Vincristin erhielten 20 Patienten, eine sequentielle Chemotherapie 15 Patienten. Die häufigsten Nebenwirkungen waren hämatologische Toxizitäten. Das mediane Gesamtüberleben (OS) wurde nach einer medianen Nachbeobachtungszeit von 92 Monaten nicht erreicht. Das geschätzte 5‑ und 10-Jahres-OS war 89 und 80 %. Das geschätzte progressionsfreie 5‑ und 10-Jahres-Überleben (PFS) betrug 89 und 81 %. In der univariaten Analyse war ein kürzeres Intervall zwischen Tumorresektion und Ende der Bestrahlung signifikant assoziiert mit besserem OS und PFS, eine anaplastische Histologie mit einem schlechteren OS und PFS.

Schlussfolgerung

Die multimodale Behandlung zeigte ein gutes Ergebnis bei Erwachsenen mit Medulloblastomen. Nachdem sich die Behandlungsdauer als prognostisch relevant herausstellte, sollte sie so kurz als möglich gehalten werden.

Schlüsselwörter

Kraniospinale Bestrahlung Strahlentherapie Multimodale Behandlung Prognostische Faktoren Überleben 

Notes

Compliance with ethical guidelines

Conflict of interest

I. Hadi, O. Roengvoraphoj, M. Niyazi, F. Roeder, U. Schüller, C. Belka and S.B. Nachbichler declare that they have no competing interests.

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This retrospective analysis of anonymized patient data was approved by the institutional ethics board (LMU Munich).

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Copyright information

© Springer-Verlag GmbH Deutschland 2017

Authors and Affiliations

  • Indrawati Hadi
    • 1
  • Olarn Roengvoraphoj
    • 1
  • Maximilian Niyazi
    • 1
  • Falk Roeder
    • 1
    • 2
  • Ulrich Schüller
    • 3
    • 4
    • 5
  • Claus Belka
    • 1
    • 6
  • Silke Birgit Nachbichler
    • 1
  1. 1.Department of Radiation Oncology, University HospitalLMU MunichMunichGermany
  2. 2.Molecular Radiation OncologyGerman Cancer Research Center (DKFZ)HeidelbergGermany
  3. 3.Institute of NeuropathologyUniversity Medical CenterHamburg-EppendorfGermany
  4. 4.Research Institute Children’s Cancer CenterHamburgGermany
  5. 5.Department of Pediatric Hematology and OncologyUniversity Medical CenterHamburg-EppendorfGermany
  6. 6.German Cancer Consortium (DKTK)MunichGermany

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