RESUMEN
Pediatric brain tumors currently show the highest incidence among solid childhood malignancies and, together with leukemia, are the leading cause of death from cancer in childhood. Embryonal brain tumors are the most common and frequent type of childhood brain cancer and are usually characterized by an extremely aggressive course of the disease with the worst outcomes in most cases. There is an urgent need for specific refined molecular diagnostics, which would help to develop personalized treatment. In the present review paper, the latest molecular characteristics of various classified forms of embryonal brain tumors were analyzed in detail. Overexpression of the MYC and MYCN genes is characteristic of many embryonal brain tumors, leading to enhanced cell proliferation and disturbances in the cell cycle. The functioning of the SWI2/SNF2 chromatin remodeling complex are distorted in such malignancies as well. Noteworthy, LIN28 and MYC discussed here are involved in the induction of pluripotency. We have to mention that molecular mechanisms underlying the development of embryonal brain tumors of the central nervous system (CNS) are still not well understood. Thus, it is important to uncover such mechanisms with the aim to provide a better prognosis of the course of disease and to create personalized therapy.
Asunto(s)
Neoplasias Encefálicas , Neoplasias de Células Germinales y Embrionarias , Niño , Humanos , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias de Células Germinales y Embrionarias/genética , Neoplasias de Células Germinales y Embrionarias/terapia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Pronóstico , Encéfalo , Patología MolecularRESUMEN
BACKGROUND/AIM: Hypo-fractionated radiotherapy (HF-RT) is increasingly used for elderly and frail glioblastoma patients. In countries with limited radiotherapy capacities, HF-RT is more widely applied. This allowed us to compare conventional fractionation (CF-RT) vs. HF-RT in patients of any age and performance status. PATIENTS AND METHODS: We retrospectively analysed 277 patients [110 HF-RT (52.5 Gy in 15 fractions) vs. 167 CF-RT (54.0-60.0 Gy in 27-33 fractions)] for local control (LC) and overall survival (OS) including subgroups considering specific patient characteristics. RESULTS: On univariable comparisons, CF-RT was associated with significantly better LC and OS in patients with KPS ≤70 and unifocal glioblastoma, and with OS in the entire cohort. Trends were found for LC and OS in patients aged <60 years, and for OS in additional four subgroups. On multivariable analyses, improved LC and OS were significantly associated with CF-RT, KPS 80-100, unifocal glioblastoma, resection, and receipt of chemotherapy. Maximum diameter <45 mm was associated with improved OS. CONCLUSION: Given the limitations of this study, CF-RT appeared associated with better outcomes. Selected patients may benefit from HF-RT.