RESUMO
OBJECTIVE: To study the prevalence of OTX1 and OTX2 gene expression in 60 medulloblastoma specimen samples and to establish correlations between gene expression and clinical and histopathological aspects. METHODS: We performed a retrospective analysis of 60 patients with a diagnosis of medulloblastoma at the Clinicas Hospital of the School of Medicine, University of São Paulo, and the Cancer Hospital of Barretos. We created a database of the 60 patients containing information on the gene expression of OTX1 and OTX2 (obtained using real-time polymerase chain reaction) and clinical and epidemiological data. Statistical tests were performed to verify potential correlations of clinicopathological data and follow-up aspects with gene expression. RESULTS: The OTX1 gene was expressed in 52% of the study population. Expression varied with age (higher in adults), location (predominantly by hemisphere), and histological type (desmoplastic). The OTX2 gene was expressed in 62% of the study population. Expression varied with age (higher in younger age groups), location (predominantly vermis), and histological type (classic and anaplastic). A statistical correlation between OTX2 gene expression and the development of leptomeningeal metastases was observed. CONCLUSIONS: The relative expression of OTX1 and OTX2 was dependent on patient age, tumor location, and histological variant. In addition, OTX2 expression might be a predictive factor for leptomeningeal metastases of medulloblastoma. The OTX pathway should be consider as an important venue for medulloblastomas development.
Assuntos
Neoplasias Cerebelares/genética , Meduloblastoma/genética , Proteínas de Neoplasias/genética , Fatores de Transcrição Otx/genética , Adolescente , Adulto , Fatores Etários , Brasil , Neoplasias Cerebelares/epidemiologia , Neoplasias Cerebelares/terapia , Criança , Pré-Escolar , Feminino , Regulação Neoplásica da Expressão Gênica , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Meduloblastoma/epidemiologia , Meduloblastoma/secundário , Meduloblastoma/terapia , Neoplasias Meníngeas/secundário , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase em Tempo Real , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE: To review the published data to create a more comprehensive natural history of multiple meningiomas (MM). METHODS: A review of MM published until now was carried out through a Medline search up to August 2018. The use of the "multiple meningiomas" keyword returned 278 articles, and the characteristics analyzed in our present cohort were searched on those publications. Articles without detailed description of clinical findings, neuroimaging confirmation of tumor multiplicity, follow-up at least of 5 years, and clear description of clinical findings were excluded. We added series to this review. RESULTS: 293 patients with MM were analyzed: 220 women and 73 men, with a total of 932 tumors (3.1 tumors per patient). The majority of tumors were located in the convexity (653% to 74.5%). The total number of tumors treated was 429 (43.9%): 338 (78.8%) by surgical resection and 91 (21.2%) by radiotherapy. Histopathologic description was available in 303 of 429 cases, being grade I in 272 (90.3%) cases, with a predominance of the meningothelial subtype (30.7%). Tumor recurrence was described in 32 (8.07%) among 397 and only 10 deaths (3.4%) of 281 reported cases, where this characteristic was evaluated. CONCLUSIONS: World Health Organization grade I predominance was observed among multiple meningiomas in similarity to single meningiomas. Only a fraction of MM patients (43.89%) needed treatment. A benign tumor behavior was corroborated by the observed low frequency of recurrence and mortality.