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Pediatric low-grade glioma and neurofibromatosis type 1: A single-institution experience

1 Pediatric Oncology Unit, Department of Women and Child Health, Fondazione Policlinico Universitario A. Gemelli Hospital Foundation IRCCS, Universita' Cattolica del Sacro Cuore, Rome, Italy
2 Pediatric Neurosurgery Unit, Department of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Universita' Cattolica del Sacro Cuore, Rome, Italy
3 Radiology and Neuroradiology Unit, Department of Radiological Diagnostics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Universita' Cattolica del Sacro Cuore, Rome, Italy

Correspondence Address:
Antonio Ruggiero,
Paediatric Oncology Unit,. Gemelli Hospital Foundation IRCCS, Catholic University of Sacred Hearth, Largo A. Gemelli 8, 00168 Rome
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_1677_21

Background: Neurofibromatosis type 1 (NF1)-related gliomas appear to have a clinical behavior different from that of sporadic cases. The purpose of the study was to investigate the role of different factors in influencing the tumor response rate of children receiving chemotherapy for their symptomatic glioma. Methods: Between 1995 and 2015, 60 patients with low-grade glioma (42 sporadic cases and 18 cases with NF1) were treated. Patients with brainstem gliomas were excluded. Thirty-nine patients underwent exclusive or postsurgical chemotherapy (vincristine/carboplatin-based regimen). Results: Disease reduction was achieved in 12 of the 28 patients (42.8%) with sporadic low-grade glioma and in 9 of the 11 patients (81.8%) with NF1, with a significant difference between the 2 groups (P < 0.05). The response to chemotherapy in both the patient groups was not significantly influenced by sex, age, tumor site, and histopathology, although disease reduction occurred more frequently in children aged under 3 years. Conclusions: Our study showed that pediatric patients with low-grade glioma and NF1 are more likely to respond to chemotherapy than those with non-NF1.

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    -  Ruggiero A
    -  Attinà G
    -  Campanelli A
    -  Maurizi P
    -  Triarico S
    -  Romano A
    -  Massimi L
    -  Tamburrini G
    -  Verdolotti T
    -  Mastrangelo S
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