RESUMO
<p><b>OBJECTIVE</b>To investigate the expression of TSC1, TSC2, p-mTOR, p-4E-BP1, p-p70S6K and p-S6 in refractory epilepsy associated malformation of cortical development (MCD) tissues.</p><p><b>METHODS</b>A total of 43 cases of refractory epilepsy were involved in the study, and all the patients were treated in Xuanwu Hospital during 2005 - 2008, including focal cortical dysplasia type IIa (11 cases) and type IIb (11 cases), tuberous sclerosis complex (10 cases) and ganalioglioma (11 cases), and other 12 cases were used as control. These cases were divided into 7 study groups and immunohistochemical EnVision method was used. To detect the location and intensity of TSC1, TSC2, p-mTOR, p-4E-BP1, p-p70S6K and p-S6 expression in every group. Then the Image-Pro Plus 6.0 image processing and analysis software were used to measure the number, area, integrating absorbance (IA) of positive cells in every samples. The statistical software SPSS 16.0 was used to analyze the data.</p><p><b>RESULTS</b>The immunolocalization of TSC1 and TSC2 was similar. It could be observed the expression of various levels in the cytoplasm of dysmorphic neurons, balloon cells, giant cells, ganglioglioma cells and normal neurons. TSC1 staining in normal neurons was more notably than others but TSC2 staining in giant cells was weaker than other samples. p-mTOR mainly presented in giant cells, which could also be observed in astrocyte. P-4E-BP1 presented in the cytoplasm and nuclear membrane of balloon cells, giant cells and ganglioglioma cells, the staining of giant cells was stronger than balloon cells, but their staining were weaker than ganglioglioma cells. P-p70S6K mainly expressed in giant cells and less commonly presented in balloon cells. P-S6 typically presented in all abnormal glioneuronal cells and it nearly did not present in the normal neurons of N-CTX group.</p><p><b>CONCLUSIONS</b>PI3K pathway, at least in part, involves in the occurrence of MCD, and may play an important role in the pathogenesis.</p>
Assuntos
Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Proteínas Adaptadoras de Transdução de Sinal , Metabolismo , Epilepsia , Metabolismo , Patologia , Ganglioglioma , Metabolismo , Patologia , Malformações do Desenvolvimento Cortical , Metabolismo , Patologia , Fosfatidilinositol 3-Quinases , Metabolismo , Fosfoproteínas , Metabolismo , Proteínas Quinases S6 Ribossômicas , Metabolismo , Proteínas Quinases S6 Ribossômicas 70-kDa , Metabolismo , Transdução de Sinais , Serina-Treonina Quinases TOR , Metabolismo , Esclerose Tuberosa , Metabolismo , Patologia , Proteínas Supressoras de Tumor , MetabolismoRESUMO
<p><b>OBJECTIVE</b>To study the clinicopathologic features of brain tumors occurring in patients with medically intractable epilepsy.</p><p><b>METHODS</b>The clinical, radiologic and pathologic features of brain tumors occurring in 35 patients with intractable epilepsy encountered during the period from January, 2005 to April, 2008 in Xuanwu Hospital were retrospectively reviewed.</p><p><b>RESULTS</b>The mean age of seizure onset and duration of disease were 14.3-year-old and 8.6 years, respectively. Abnormal signals were observed in 94.3% of cases (33/35) by magnetic resonance imaging. The histologic types of brain tumors included ganglioglioma (13/35, WHO grade I and 6/35, WHO grade II), dysembryoplastic neuroepithelial tumor (3/35, WHO grade I), pleomorphic xanthoastrocytoma (3/35, WHO grade II), diffuse astrocytoma (1/35, WHO grade II), oligoastrocytoma (1/35, WHO grade II), angiocentric glioma (1/35, WHO grade I) and meningioangiomatosis (1/35). The 6 remaining cases showed features seen in between glioneuronal hamartoma and mixed neuronal-glial tumor. Most of these tumors were located in the temporal lobe (27/35) and associated with focal cortical dysplasia. Immunohistochemical study showed a remarkable expression of CD34 in gangliogliomas.</p><p><b>CONCLUSIONS</b>Brain tumors in patients with medically intractable epilepsy are almost always benign and located in the temporal lobe. Most of them represent mixed neuronal-glial tumors and some show transitional features in-between glioneuronal hamartoma and mixed neuronal-glial neoplasm. The similar morphologic pattern and biological behavior of glioneuronal hamartoma and mixed neuronal-glial tumor may suggest a common pathogenetic mechanism.</p>