Carcinoma de plexo coróide: relato de quinze casos

Abstract

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,

The Impact of Radiotherapy Fields in the Treatment of Patients With Choroid Plexus Carcinoma

“…Our objective for this study was to determine the optimal RT field for the treatment of CPC patients. Patients in our review had a better 5-year OS (59.5%) compared with other series reported in the literature (26%-51%) in which RT was not routinely given (4,19,33,44,48,49). This confirms earlier analyses, showing better survival among patients who received radiation.…”

Choroid plexus carcinoma: Case report and review of literature

“…[1] Due to their rarity, reports on CPC most often focus on single cases or single-institution experiences with a limited number of patients. [2] Recently, we also encountered a case of left lateral ventricle CPC in a young child who was operated in poor neurological grade.…”

“…reported 15 cases of CPC and pointed out that the main symptoms of this tumor are hydrocephalus (62.5%), intracranial hypertension (25%), and convulsion (12.5%). [2] Neuroradiological features are nonspecific in CPC. Some features may suggest the diagnosis, such as when the tumor invades the parenchyma or presents with metastatic nodules in the third, fourth, or lateral ventricles.…”

“…All the tumors in the differential diagnosis, including ependymoma, primitive neuroectodermal tumor, astrocytoma, teratoma, and meningioma, can have similar imaging characteristics and modern imaging cannot yet accurately define the pathologic diagnosis. [23]…”

Tumores de plexo coróide: estudo epidemiológico comparativo de 24 casos

“…Não existe, entretanto, consenso acerca do quimioterápico de escolha, devido à dificuldade na elaboração de protocolos tendo em vista a raridade do tumor 6 . O comportamento biológico agressivo do tumor justifica, portanto, o seu prognóstico reservado, com taxas de sobrevida média em torno de 9 meses em crianças e 3,5 anos em adultos 14 . A ressecção cirúrgica completa ainda constitui o principal fator a contribuir para o aumento das taxas de sobrevida a longo prazo 15 .…”