RESUMO -O carcinoma de plexo coróide (CPC) é um raro tumor do sistema nervoso central (SNC), derivado do epitélio de revestimento dos plexos coróides, que acomete pacientes abaixo dos 3 anos de idade. Apresentamos um estudo clínico, epidemiológico e histopatológico de 15 casos de CPC. Destes, 10 eram do gênero masculino. As idades variaram de 4 meses a 21 anos. Quanto a localização, acometeram predominante o ventrículo lateral (73,3%). Os sinais e sintomas predominantes foram hidrocefalia (62,5%), hipertensão intracrania (25%) e crise convulsiva (12,5%). Os pacientes foram submetidos a tratamento cirúrgico com ressecção parcial em 75% dos casos e completa em 25%. Houve um óbito per-operatório. Em 85,7% dos pacientes houve recidiva tumoral, com evolução a óbito em média 13,6 meses após o diagnóstico. Apenas um paciente encontra-se vivo num seguimento de 5 anos. Esses dados suportam o prognóstico sombrio e a alta mortalidade do CPC. PALAVRAS -CHAVE: tumores cerebrais, tumor de plexo coróide, carcinoma de plexo coróide, imunohistoquímica. Choroid plexus carcinoma: report of fifteen casesABSTRACT -Choroid plexus carcinoma (CPC) are rare central nervous system tumours derived from choroid plexus epithelium, affecting mainly children under 3 years of age. We present a clinical, epidemiological and histopathological study of 15 cases of CPC. Ten patients were male. Age ranged from 4 months to 21 years (mean=3,4). The lateral ventricles were affected in 73,3% of cases. Main symptom were: hydrocephalus (62.5%), intracranial hypertension (25%) and convulsion (12.5%). The patients were treated by surgery with partial resection in 75% of cases and total resection in 25%. There was one death due to surgical complication, 85.7% of patients had recurrence of tumours with mean survival rate of 13.6% months after diagnosis. Only one patient remain alive 5 years after initial treatment. These results support the poor prognosis and high mortality rate of CPC.KEY WORDS: brain neoplasm, choroid plexus tumours, choroid plexus carcinoma, immunohistochemistry.Os tumores do plexo coróide são neoplasias raras do sistema nervoso central (SNC), originados do epitélio de revestimento dos plexos coróides localizado nos ventrículos cerebrais e que correspondem a 0,4 a 0,6% de todos os tumores cerebrais [1][2][3][4][5][6] . Em sua maioria são tumores benignos,

RESUMO -Ependimomas são neoplasias compostas por células ependimárias neoplásicas, manifestando-se predominantemente em crianças e adultos jovens. Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 22 casos de ependimomas. Dos 22 casos analisados, 14 ocorreram em pacientes do sexo masculino e 8 em pacientes femininos. As idades variaram de 1 a 58 anos, com média de 24,63 anos. Quanto à localização, 10 casos acometeram medula, 7 ocorreram em hemisférios cerebrais, 2 intraventriculares e 1 no tronco cerebral. Os sinais e sintomas observados incluiram redução da força muscular (59,1% dos pacientes), alterações da marcha (36,3%), alterações da sensibilidade(36,3%), hiperreflexia e sindrome de hipertensão endocraniana, com período de evolução variando de 15 dias a 8 anos, com média de 17,58 meses. Sete pacientes foram submetidos a ressecção cirúrgica total , 7 a exérese parcial, 4 a exérese parcial associada a radioterapia adjuvante, 3 a ressecção parcial seguida de quimioterapia e 1 a tumorectomia total associada a radioterapia. Evidenciou-se taxa de recidiva tumoral total de 18,2%. Estes achados aproximam-se dos encontrados na literatura internacional, corroborando para a compreensão do comportamento biológico deste tumor. PALAVRAS-CHAVE: ependimomas, neoplasias do sistema nervoso central, tumores cerebrais. Ependymonas: clinical, epidemiological and pathological findings of 22 casesABSTRACT -Ependymomas are composed of neoplastic ependymal cells, affecting mainly children and young adults. We report the clinical and pathological findings of 22 cases of ependymomas. Fourteen patients were males and 8 were females. The ages ranged between 1 and 58 years, with a mean of 24.63 years. The symptoms reflected the growth and topography of the tumours; muscle weakness (59.1%), gait disorders (36.3%), sensitive disorders (36.3%), hyperreflexia and intracranial hypertension syndrome were the most frequent symptoms. Ten tumours affected the medulla, 7 the cerebral hemispheres, 2 the cerebral ventricles and 1 brain stem. Seven patients were submitted total resection of the tumor, from which one received adjuvant radiotherapy. 15 other patients were submitted to partial resection; from which 4 received adjuvant radiotherapy, 3 adjuvant chemotherapy and 1 chemotherapy and radiotherapy. The recurrence rate was 18.2%. These results are similar with the literature and may contribute to further understanding the biological behavior of these tumours.KEY WORDS: ependymomas, central nervous system tumours, brain tumours.Os ependimomas se originam da camada ependimária que recobre os ventrículos cerebrais e o canal central da medula espinhal, manifestando-se em crianças e adultos jovens, com características morfológicas e comportamento biológico extremamente variáveis [1][2][3][4] . Relatamos os achados

RESUMO -Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 25 casos de craniofaringiomas. Dos 25 pacientes analisados, 14 ocorreram em pacientes do sexo masculino e 11 em pacientes femininos. As idades variaram de 3 a 64 anos, com média de 30,52 anos. Quanto à localização, 12 casos foram supra-selares, 10 ocorreram em região selar, sendo que 8 apresentaram extensão supra-selar, dois localizaram-se em lobo frontal e 1 em região ponto-cerebelar. Os sinais e sintomas observados incluiram alterações visuais (72% dos pacientes), cefaléia (68%), vômitos (40%) e edema de papila (24%), com período de evolução variando de 18 dias a 60 meses, com média de 11,66 meses. Treze pacientes foram submetidos a ressecção cirúrgica parcial, 11 a exérese total e 1 a ressecção parcial associada a quimioterapia adjuvante. Evidenciou-se taxa de recidiva tumoral total de 48%. Sete recidivas tumorais ocorreram nos pacientes submetidos apenas a exérese parcial, quatro nos pacientes submetidos a tumorectomia total e 1 no submetido a tratamento cirúrgico associado a quimioterapia. Estes achados aproximam-se dos encontrados na literatura, corroborando para a compreensão do comportamento biológico deste tumor e apontando a extensão do tumor residual como único fator prognóstico para recidiva desta neoplasia. PALAVRAS-CHAVE: craniofaringiomas, tumores do sistema nervoso central, neoplasias cerebrais. Craniopharyngiomas: clinical, epidemiological and pathological findings of 25 casesABSTRACT -We report the clinical and pathological findings of 25 cases of craniopharyngiomas. Fourteen patients were males and 11 were females. The ages ranged between 3 and 64 years, with a mean of 30.52 years. The symptoms reflected the growth and topography of the tumours; visual disorders (72%), headache (68%), vomits (40%) and papilledema (24%) were the most frequent symptoms. Twelve cases were suprasellar; 10 tumours arose from sellar region, from which 8 presented suprasellar extension; frontal lobe (n=2) and ponto cererebellar angle (n=1) were also affected. Eleven patients undergone total resection of the tumor and 14 were submitted to partial resection, from which 1 received adjuvant chemotherapy. The overall recurrence rate was 48%. Eight recurrences were observed in the patients submitted to partial resection and 4 in which total resection were performed. These results are similar with the literature, corroborating to the extension of residual tumour after the surgical resection as the main prognostic factor for this neoplasm.KEY WORDS: craniopharyngiomas, central nervous system tumours, brain neoplasms.Os craniofaringiomas são tumores encapsulados, de origem epitelial, derivados do epitélio da bolsa de Rathke 1-4 , caracterizados por acometerem crianças e adultos jovens, representando a neoplasia intra-craniana não neuroepitelial mais frequente neste grupo etário 1 . Relatamos os achados anatomopatológicos e clínico prognósticos de 25 casos de craniofaringiomas diagnosticados em paci-

Oligodendrogliomas account for 4-5% of primary central nervous system tumours with a slow and infiltrative growth. We report the clinical and pathological findings of 15 cases of oligodendrogliomas. Eight patients were males and 7 were females. The ages ranged between 17 and 66 years, with a mean of 39.73 years. The symptoms reflected the growth and topography of the tumours; migraine (60%) and seizures (60%) were the most frequent symptoms. Frontal (n = 6), parietal (n = 2), temporal (n = 1) and occipital (n = 1) lobes were affected. Five patients undergone total resection of the tumor and 10 were submitted to partial resection, from which 3 received adjuvant radiotherapy, 1 adjuvant chemotherapy and 1 chemotherapy and radiotherapy. The overall recurrence rate was 60% for a 32 month follow up. Five recurrences were observed in patients submitted only to the surgical treatment and 4 in which adjuvant radio or chemotherapy were performed. These results are similar with the literature and may contribute to further understanding the biological behavior of these rare tumours.

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