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Outcome of patients with intracranial non-germinomatous germ cell tumors-lessons from the SIOP-CNS-GCT-96 trial.

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Publication Date
2017-11
ICR Author
Marsden,
Author
Calaminus, G
Frappaz, D
Kortmann, RD
Krefeld, B
Saran, F
Pietsch, T
Vasiljevic, A
Garre, ML
Ricardi, U
Mann, JR
Göbel, U
Alapetite, C
Murray, MJ
Nicholson, JC
Type
Journal Article
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Abstract
<h4>Background</h4>Following promising results to increase survival and reduce treatment burden in intracranial non-germinomatous germ cell tumors (NGGCTs), we conducted a European study using dose-intense chemotherapy followed by risk-adapted radiotherapy.<h4>Methods</h4>All patients received 4 courses of cisplatin/etoposide/ifosfamide. Non-metastatic patients then received focal radiotherapy only (54 Gy); metastatic patients received 30 Gy craniospinal radiotherapy with 24 Gy boost to primary tumor and macroscopic metastatic sites.<h4>Results</h4>Patients with localized malignant NGGCT (n = 116) demonstrated 5-year progression-free survival (PFS) and overall survival (OS) of 0.72 ± 0.04 and 0.82 ± 0.04, respectively. Primary tumor sites were: 67 pineal, 35 suprasellar, 5 bifocal, 9 others. One patient died postsurgery in clinical remission; 3 patients progressed during treatment and 27 (23%) relapsed afterward. Fourteen were local, 6 combined, and 7 distant relapses (outside radiation field). Seventeen of the 27 relapsed patients died of disease. Patients with metastatic disease (n = 33) demonstrated 5-year PFS and OS of 0.68 ± 0.09 and 0.75 ± 0.08, respectively; 1 patient died following progression on treatment and 9 (27%) relapsed afterward (5 local, 1 combined, 3 distant). Only one metastatic patient with recurrence was salvaged. Multivariate analysis identified diagnostic alpha-fetoprotein level (serum and/or cerebrospinal fluid level >1000 ng/mL, 19/149 patients, of whom 11 relapsed; P < 0.0003) and residual disease following treatment, including after second-look surgery (n = 52/145 evaluable patients, 26 relapsed; P = 0.0002) as significant prognostic indicators in this cohort.<h4>Conclusion</h4>In localized malignant NGGCT, craniospinal radiotherapy could be avoided without increased relapses outside the radiotherapy field. Chemotherapy and craniospinal radiotherapy remain the gold standard for metastatic disease.
URL
https://repository.icr.ac.uk/handle/internal/1482
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  • Other ICR Research
Version of record
10.1093/neuonc/nox122
Subject
Humans
Neoplasms, Germ Cell and Embryonal
Testicular Neoplasms
Brain Neoplasms
Lymphatic Metastasis
Neoplasm Recurrence, Local
Cisplatin
Ifosfamide
Etoposide
Antineoplastic Combined Chemotherapy Protocols
Prognosis
Cranial Irradiation
Survival Rate
Follow-Up Studies
Prospective Studies
Adolescent
Adult
Child
Child, Preschool
International Agencies
Female
Male
Young Adult
Chemoradiotherapy
Biomarkers, Tumor
Language
eng
License start date
2017-11
Citation
Neuro-oncology, 2017, 19 (12), pp. 1661 - 1672

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