RESUMEN
BACKGROUND: Supratentorial ependymomas (STEs) are an aggressive group of ependymomas, topographically distinct from their posterior fossa and spinal counterparts. Zinc finger translocation associated (ZFTA) fusion-positive cases have been reported to account for the majority of STEs, although data on its association with poorer outcomes are inconsistent. MATERIALS AND METHODS: We assessed the prevalence of the ZFTA fusion by reverse-transcription polymerase chain reaction and fluorescence in situ hybridization in a cohort of 61 patients (68 samples) with STE. Our primary outcome was to determine the role of the ZFTA fusion on progression-free and overall survival of patients with STE. Our secondary objectives were to assess the impact of ZFTA fusion on nuclear factor (NF)-kB pathway signaling via surrogate markers of this pathway, namely COX-2, CCND1, and L1 cell adhesion molecule. RESULTS: ZFTA fusion was noted in 21.3% of STEs in our cohort. The presence of this rearrangement did not significantly impact the progression-free or overall survival of patients with STEs and was not associated with upregulation of markers of the NF-kB pathway. Only gross total resection was significantly associated with better progression-free survival. CONCLUSIONS: In contradiction to previous reports from across the world, the ZFTA fusion is far less prevalent among our population. It does not appear to drive NF-kB signaling or significantly affect outcomes. Gross total resection must be attempted in all cases of STE and adjuvant radiation and/or chemotherapy employed when gross total resection is not achieved.
Asunto(s)
Ependimoma , Neoplasias Supratentoriales , Ependimoma/genética , Ependimoma/metabolismo , Ependimoma/cirugía , Humanos , Hibridación Fluorescente in Situ , FN-kappa B/metabolismo , Prevalencia , Neoplasias Supratentoriales/genética , Neoplasias Supratentoriales/metabolismo , Neoplasias Supratentoriales/cirugía , Factor de Transcripción ReIA/genética , Factor de Transcripción ReIA/metabolismo , Translocación Genética/genética , Dedos de ZincRESUMEN
BACKGROUND: (1)H-MR spectroscopy (MRS) and (18)F-dihydroxyphenylalanine (DOPA) PET are noninvasive imaging techniques able to assess metabolic features of brain tumors. The aim of this study was to compare diagnostic and prognostic information gathered by (18)F-DOPA PET and (1)H-MRS in children with supratentorial infiltrative gliomas or nonneoplastic brain lesions suspected to be gliomas. METHODS: We retrospectively analyzed 27 pediatric patients with supratentorial infiltrative brain lesions on conventional MRI (21 gliomas and 6 nonneoplastic lesions) who underwent (18)F-DOPA PET and (1)H-MRS within 2 weeks of each other. (1)H-MRS data (choline/N-acetylaspartate, choline-to-creatine ratios, and presence of lactate) and (18)F-DOPA uptake parameters (lesion-to-normal tissue and lesion-to-striatum ratios) were compared and correlated with histology, WHO tumor grade, and patient outcome. RESULTS: (1)H-MRS and (18)F-DOPA PET data were positively correlated. Sensitivity, specificity, and accuracy in distinguishing gliomas from nonneoplastic lesions were 95%, 83%, and 93% for (1)H-MRS and 76%, 83%, and 78% for (18)F-DOPA PET, respectively. No statistically significant differences were found between the 2 techniques (P > .05). Significant differences regarding (18)F-DOPA uptake and (1)H-MRS ratios were found between low-grade and high-grade gliomas (P≤.001 and P≤.04, respectively). On multivariate analysis, (18)F-DOPA uptake independently correlated with progression-free survival (P≤.05) and overall survival (P = .04), whereas (1)H-MRS did not show significant association with outcome. CONCLUSIONS: (1)H-MRS and (18)F-DOPA PET provide useful complementary information for evaluating the metabolism of pediatric brain lesions. (1)H-MRS represents the method of first choice for differentiating brain gliomas from nonneoplastic lesions.(18)F-DOPA uptake better discriminates low-grade from high-grade gliomas and is an independent predictor of outcome.
Asunto(s)
Glioma/diagnóstico por imagen , Glioma/metabolismo , Tomografía de Emisión de Positrones/métodos , Espectroscopía de Protones por Resonancia Magnética/métodos , Neoplasias Supratentoriales/diagnóstico por imagen , Neoplasias Supratentoriales/metabolismo , Adolescente , Encéfalo/diagnóstico por imagen , Encéfalo/metabolismo , Encéfalo/patología , Niño , Preescolar , Dihidroxifenilalanina/farmacocinética , Femenino , Fluorodesoxiglucosa F18/farmacocinética , Glioma/patología , Humanos , Estimación de Kaplan-Meier , Imagen por Resonancia Magnética , Masculino , Pronóstico , Estudios Retrospectivos , Sensibilidad y Especificidad , Neoplasias Supratentoriales/patologíaRESUMEN
Metabolic imaging with positron emission tomography (PET) provides, in neuro-oncology, information complementary to that provided by anatomic imaging obtained with CT-scanner or MRI. Only a few publications have yet reported its use in oligodendroglial tumors. These findings and partial results obtained in ongoing work, suggest some preliminary conclusions: 11C-MET (L-methyl-methionine) is a more appropriate tracer than 18F-FDG (fluoro-deoxy-glucose), in terms of both specificity and sensitivity, for the assessment of patients with this category of tumor. PET/MET allows differentiation between grade II and grade III oligodendrogliomas; better targeting for stereotactic biopsy; more accurate assessment of the post-operative residual tumor; identification of progression from low-grade to anaplastic grade during the disease course; differentiation between recurrence and a post-radiation processes. PET/MET allows, to some extent, prediction of response to radiotherapy; and, probably, to chemotherapy.