Primary dissemination of high-grade gliomas in children: experiences from four studies of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH)
- 161 Downloads
Purpose. Clinical data on central nervous system (CNS) dissemination of high-grade gliomas (HGG) at initial presentation in children are rare.
Patients and methods. We conducted a retrospective data analysis of all patients enrolled into four consecutive HGG protocols of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH) to determine the incidence of primary CNS dissemination of HGG and to describe clinical characteristics and outcome of children with HGG who were diagnosed with CNS dissemination at initial presentation. 546 patients with newly diagnosed HGG (n=348) or diffuse intrinsic pontine gliomas (n=198) were enrolled in these four studies. Data concerning tumor dissemination are available from 324 patients.
Results. A total of 10 patients (3.1%) (anaplastic astrocytoma: n=3, glioblastoma multiforme: n=6, diffuse intrinsic pontine glioma: n=1) had primary tumor dissemination. Median age at diagnosis was 9.3 years (range: 0.3–21.3 years). The most frequent primary tumor sites were the cortex (n=4), followed by the ventricles (n=2), cerebellum (n=1), spinal cord (n=1), and pons (n=1). One patient had diffuse gliomatosis cerebri. Following surgery eight patients received local radiotherapy and eight additional chemotherapy. At a median follow-up of 10 months (range: 0.05–3 years) four patients are alive. None is disease-free. Median progression-free and overall survival was 0.8 years (95% CI 0.2–1.4) and 1.5 years (95% CI 0.67–2.29) for patients with primary tumor dissemination, respectively, with no statistically significant differences between the group with and the group without primary tumor dissemination.
Conclusions. Initial diagnostic evaluation should include complete CNS imaging as well as cerebrospinal fluid examination in all patients with HGG. As prognosis of children with HGG and primary CNS dissemination was not inferior to patients without dissemination in our population, these patients should be treated in the same way as patients without primary CNS dissemination.
Keywordschildren high-grade gliomas primary dissemination prognosis treatment
Unable to display preview. Download preview PDF.
- 5.Sposto, R, Ertel, IJ, Jenkin, RDT, Boesel, CP, Venes, JL, Ortega, JA, Evans, AE, Wara, W, Hammond, D 1989The effectiveness of chemotherapy for treatment of high grade astrocytoma in children: results of a randomized trial. A report from the Children’s Cancer Study GroupJ Neurooncol7165177CrossRefPubMedGoogle Scholar
- 6.Duffner, PK, Horowitz, ME, Krischer, JP, Friedman, HS, Burger, PC, Cohen, ME, Sanford, RA, Mulhern, RK, James, HE, Freeman, CR, Seidel, FG, Kun, LE 1993Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumorsNew Engl J Med32817251731CrossRefPubMedGoogle Scholar
- 7.Finlay, JL, Boyett, JM, Yates, AJ, Wisoff, JH, Milstein, JM, Geyer, JR, Bertolone, SJ, McGuire, P, Cherlow, JM, Tefft, M, Turski, PA, Wara, WM, Edwards, M, Sutton, LN, Berger, MS, Epstein, F, Ayers, G, Allen, JC, Packer, RJ 1995Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimenJ Clin Oncol13112123PubMedGoogle Scholar
- 11.Kleihues, PBurger, PCScheithauer, BW eds. 1993Histological Typing of Tumours of the Central Nervous SystemSpringer-VerlagBerlinGoogle Scholar
- 12.Kühl, J, Bode, U, Deinlein, F, Ottensmeier, H, Warmuth-Metz, M, Graf, N, Henze, G, Kaatsch, P, Kortmann, RD, Müller, H, Pietsch, T, Rating, D, Scheurlen, W, Sörensen, N, Wolff, JEA, Riehm, H 1999Cure of infants with medulloblastoma (M0/M1-stage) by postoperative chemotherapy onlyMed Pediatr Oncol33149(Abstract #O-107)Google Scholar
- 24.Slavc, I, Schuller, E, Falger, J, Günes, M, Pillwein, K, Czech, T, Dietrich, W, Rössler, K, Dieckmann, K, Prayer, D, Hainfellner, J 2003Feasibility of long-term intraventricular therapy with mafosfamide (n=26) and etoposide (n=11): experience in 26 children with disseminated malignant brain tumorsJ Neurooncol64239247CrossRefPubMedGoogle Scholar