Carcinoma of the choroid plexus: A pediatric experience

Abstract

Carcinoma of the choroid plexus (CCP) is a rare tumor of the central nervous system which occurs more frequently in patients under 3 years of age. We report 10 cases of CCP in children. There were 5 males and 5 females, aged 5 to 84 months at diagnosis (median 32 months). Eight CCP were located in the lateral ventricle and 2 in the fourth ventricle. All patients underwent initial surgery, with complete resection in 5 cases. Postoperative treatment included radiotherapy alone in 4 patients, chemotherapy alone in 4, both in 1 patient, and no further treatment in one patient. Six patients remain in first continuous complete remission with a follow-up of 12 to 156 months (mean 34 months). Five of these six patients had a complete surgical resection of their tumor and 4 of them received chemotherapy alone. A comparison with the series in the literature suggests that total resection of the tumors is the major prognostic factor for survival. Recourse to additional treatments to prevent local relapses and CNS dissemination seems necessary. In our opinion, radiotherapy should be avoided as far as possible because of its deleterious effect in very young children. As some of the long term survivors did not receive radiation treatment, we conclude that CCP can be treated post operatively using chemotherapy protocols designed for infants with malignant brain tumors.

Carcinoma de plexo coróide: relato de quinze casos

“…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,…”

Prolonged Survival for Choroid Plexus Carcinoma with Oncocytic Changes: A Case Report

“…Radiation therapy alone was not suggested for CPCs in the pediatric patient population due to the intolerable and undesirable side effects [1,15,17]. Several articles support the use of post-operative treatments such as combined therapies or adjuvant chemotherapy alone [12,15,17,18].…”

“…The study concluded that combined therapy versus chemotherapy alone yielded increased survival [13]. However, radiation therapy alone was found not to contribute to survival [17]. Due to this tumor's tendency to disseminate throughout the cerebral spinal fluid space, it is believed that treatment of the entire neuraxis is necessary [14].…”

Tumores de plexo coróide: estudo epidemiológico comparativo de 24 casos

“…Alguns dados de neuroimagem podem ser sugestivos de malignidade: grande volume tumoral, invasão do parênquima encefálico, edema, hidrocefalia e por vezes herniação ou desvio de linha média 6 . A ressonância nuclear magnética (RM), permite melhor visualização da extensão, limites do tumor e suas relações anatômicas, facilitando desta forma o tratamento cirúrgico e o controle de lesões residuais no pós-operatório 5 .…”

“…A quimioterapia apresenta-se como instrumento valioso a ser associado ao tratamento cirúrgico, sendo válida associação de várias drogas para tumores parcialmente ressecáveis ou para controle da doença residual no pós-operatório. 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 .…”