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Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle: A Case of Relapse Treated with Proton Beam Therapy.

Authors
  • Cacchione, Antonella1
  • Mastronuzzi, Angela1
  • Carai, Andrea2
  • Colafati, Giovanna Stefania3
  • Diomedi-Camassei, Francesca4
  • Marrazzo, Antonio3
  • Carboni, Alessia3
  • Miele, Evelina1
  • Pedace, Lucia1
  • Tartaglia, Marco5
  • Amichetti, Maurizio6
  • Fellin, Francesco6
  • Lodi, Mariachiara1
  • Vennarini, Sabina6
  • 1 Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, 00165 Rome, Italy. , (Italy)
  • 2 Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, 00165 Rome, Italy. , (Italy)
  • 3 Oncological Neuroradiology Unit, Department of Imaging, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, 00165 Rome, Italy. , (Italy)
  • 4 Department of Laboratories, Pathology Unit, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, 00165 Rome, Italy. , (Italy)
  • 5 Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, 00165 Rome, Italy. , (Italy)
  • 6 Proton Therapy Center, Hospital of Trento, Azienda Provinciale per I Servizi Sanitari (APSS), 38123 Trento, Italy. , (Italy)
Type
Published Article
Journal
Diagnostics
Publisher
MDPI AG
Publication Date
May 19, 2021
Volume
11
Issue
5
Identifiers
DOI: 10.3390/diagnostics11050903
PMID: 34069450
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Rosette-forming glioneuronal tumors (RGNTs) are rare, grade I, central nervous system (CNS) tumors typically localized to the fourth ventricle. We describe a 9-year-old girl with dizziness and occipital headache. A magnetic resonance imaging (MRI) revealed a large hypodense posterior fossa mass lesion in relation to the vermis, with cystic component. Surgical resection of the tumor was performed. A RGNT diagnosis was made at the histopathological examination. During follow-up, the patient experienced a first relapse, which was again surgically removed. Eight months after, MRI documented a second recurrence at the local level. She was a candidate for the proton beam therapy (PBT) program. Three years after the end of PBT, the patient had no evidence of disease recurrence. This report underlines that, although RGNTs are commonly associated with an indolent course, they may have the potential for aggressive behavior, suggesting the need for treatment in addition to surgery. Controversy exists in the literature regarding effective management of RGNTs. Chemotherapy and radiation are used as adjuvant therapy, but their efficacy management has not been adequately described in the literature. This is the first case report published in which PBT was proposed for adjuvant therapy in place of chemotherapy in RGNT relapse.

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