Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol

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• 作者：Carsten Friedrich (1)
  Klaus Müller (2)
  Katja von Hoff (1)
  Robert Kwiecien (3)
  Torsten Pietsch (4)
  Monika Warmuth-Metz (5)
  Nicolas U. Gerber (6)
  Peter Hau (7)
  Joachim Kuehl (8)
  Rolf D. Kortmann (2)
  André O. von Bueren (1) (9)
  Stefan Rutkowski (1)
  
• 关键词：Brain tumor ; Primitive neuroectodermal tumor (PNET) ; Pineoblastoma ; Adults ; Radiotherapy ; Chemotherapy
• 刊名：Journal of Neuro-Oncology
• 出版年：2014
• 出版时间：February 2014
• 年：2014
• 卷：116
• 期：3
• 页码：567-575
• 全文大小：377 KB
• 作者单位：Carsten Friedrich (1)
  Klaus Müller (2)
  Katja von Hoff (1)
  Robert Kwiecien (3)
  Torsten Pietsch (4)
  Monika Warmuth-Metz (5)
  Nicolas U. Gerber (6)
  Peter Hau (7)
  Joachim Kuehl (8)
  Rolf D. Kortmann (2)
  André O. von Bueren (1) (9)
  Stefan Rutkowski (1)
  
  1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany
  2. Department of Radiation Oncology, University of Leipzig, Leipzig, Germany
  3. Institute of Biostatistics and Clinical Research, University of Muenster, Muenster, Germany
  4. Department of Neuropathology, University of Bonn, Bonn, Germany
  5. Department of Neuroradiology, University of Wuerzburg, Wuerzburg, Germany
  6. Department of Oncology, University Children’s Hospital, Zurich, Switzerland
  7. Department of Neurology and Wilhelm Sander-NeuroOncology Unit, University Hospital Regensburg, Regensburg, Germany
  8. Department of Pediatrics, University of Wuerzburg, Wuerzburg, Germany
  9. Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University Medical Center Goettingen, Goettingen, Germany
  
• ISSN：1573-7373

文摘

Central nervous system primitive neuroectodermal tumors (CNS-PNET) and pineoblastomas (PBL) are rare in adulthood. Knowledge on clinical outcome and the efficacy and toxicities of chemotherapy in addition to radiotherapy is limited. Patients older than 21?years at diagnosis were followed in the observational arm of the prospective pediatric multicenter trial HIT 2000. After surgery, craniospinal irradiation and maintenance or sandwich chemotherapy were recommended. Radiotherapy was normo- (35.2?Gy; tumor region, 55.0?Gy; metastasis, 49.6?Gy) or hyperfractionated (40.0?Gy; tumor bed, 68.0?Gy; metastasis, 50-0?Gy). Maintenance chemotherapy consisted of eight courses (vincristine, lomustine, cisplatin). Sandwich chemotherapy included two cycles of postoperative chemotherapy followed by radiotherapy, and four courses of maintenance chemotherapy. Seventeen patients (CNS-PNET, n?=?7; PBL, n?=?10), median age 30?years, were included. Eight patients had a postoperative residual tumor and four patients metastatic disease. The median follow-up of ten surviving patients was 41?months. The estimated rates for 3-year progression-free survival (PFS) and overall survival were 68?±?12 and 66?±?13?%, respectively. PBL compared to CNS-PNET tended towards a better PFS, although the difference was not clear (p?=?0.101). Both chemotherapeutic (maintenance, n?=?6; sandwich, n?=?8) protocols did not differ in their PFS and were feasible with acceptable toxicities. Intensified regimens of combined chemo- and radiotherapy are generally feasible in adults with CNS-PNET/PBL. The impact of intensified chemotherapy on survival should be further assessed.