Tumors of the choroid plexus in children

Ellenbogen, Richard G.; Winston, Ken R.; Kupsky, William J.

doi:10.1097/00006123-198909000-00001

Cited by 91 publications

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“…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,…”

unclassified

Carcinoma de plexo coróide: relato de quinze casos

Bleggi-Torres

Urban²,

Антонюк

et al. 2000

Arq. Neuro-Psiquiatr.

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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,

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unclassified

Carcinoma de plexo coróide: relato de quinze casos

Bleggi-Torres

Urban²,

Антонюк

et al. 2000

Arq. Neuro-Psiquiatr.

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“…9,12,13,16,23 Their benign nature and intraventricular, noninfiltrative nature renders them surgically curable. 15,20,27 Gross-total resection has been associated with excellent prognosis, 9,18,20 with 5-year survival being close to 100% after complete resection, but survival is only 56% after subtotal resection.…”

Section: Discussionmentioning

confidence: 99%

“…1,3,7,10,11,20 One recent series documented a mean intraoperative blood loss of 182% of each patient's blood volume for unembolized CPPs. 11 In published series, the perioperative mortality rate has been reported to range from 0% to 25%, 3,7,[9][10][11][12]16,20,23 and the mortality rate attributed to blood loss has been reported to be as high as 12%. 12 In response to this difficulty, endovascular embolization has been advocated as a means to reduce intraoperative blood loss for these tumors.…”

Section: B C Kennedy Et Almentioning

confidence: 99%

Superior parietal lobule approach for choroid plexus papillomas without preoperative embolization in very young children

Kennedy¹,

Cloney²,

Anderson

et al. 2015

PED

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OBJECT Choroid plexus papillomas (CPPs) are rare neoplasms, often found in the atrium of the lateral ventricle of infants, and cause overproduction hydrocephalus. The extensive vascularity and medially located blood supply of these tumors, coupled with the young age of the patients, can make prevention of blood loss challenging. Preoperative embolization has been advocated to reduce blood loss and prevent the need for transfusion, but this mandates radiation exposure and the additional risks of vessel injury and stroke. For these reasons, the authors present their experience using the superior parietal lobule approach to CPPs of the atrium without adjunct therapy. METHODS A retrospective review was conducted of all children who presented to Columbia University/Morgan Stanley Children's Hospital of New York with a CPP in the atrium of the lateral ventricle and who underwent surgery using a superior parietal lobule approach without preoperative embolization. RESULTS Nine children were included, with a median age of 7 months. There were no perioperative complications or new neurological deficits. All patients had intraoperative blood loss of less than 100 ml, with a mean minimum hematocrit of 26.9% (range 19.6%–36.2%). No patients required a blood transfusion. The median follow-up was 39 months, during which time no patient demonstrated residual or recurrent tumor on MRI, nor did any have an increase in ventricular size or require CSF diversion. CONCLUSIONS The superior parietal lobule approach is safe and effective for very young children with CPPs in the atrium of the lateral ventricle. The results suggest that preoperative embolization is not essential to avoid transfusion or achieve overall good outcomes in these patients. This management strategy avoids radiation exposure and the additional risks associated with embolization.

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“…4,8,10) The updated World Health Organization (WHO) classification divides this entity into three types: choroid plexus papilloma (CPP) (grade I), atypical choroid plexus papilloma (ACPP) (grade II), and choroid plexus carcinoma (CPC) (grade III). 8) ACPP was newly defined, and the clinical course and effective treatment are not well known.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Multi-staged Surgery and Adjuvant Chemotherapy for Successful Treatment of Atypical Choroid Plexus Papilloma in an Infant -Case Report-

Takahashi

Yamamoto

Aoyama

et al. 2009

Neurol. Med. Chir.(Tokyo)

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A 12-month-old girl presented with a rare atypical choroid plexus papilloma manifesting as conscious disturbance and vomiting. Magnetic resonance (MR) imaging revealed a well-delineated 5 × 6-cm lobulate mass in the right lateral ventricle. Only partial removal of the tumor was performed because of excessive intraoperative hemorrhage at the first surgery. The histological diagnosis was atypical choroid plexus papilloma. To control the intraoperative hemorrhage, embolization of the feeding artery was performed before the second surgery, and the tumor was macroscopically totally removed. MR imaging disclosed a small residual tumor which showed relatively rapid growth. The patient underwent the third removal 5 months after the second surgery. Two months later, MR imaging showed a cystic lesion with a small nodule adjacent to the midbrain, indicating dissemination of the tumor. The lesion was successfully treated with chemotherapy. Atypical choroid plexus papilloma was recently defined in the classification of the World Health Organization, so clinical data based on these criteria are lacking to establish the therapeutic strategy. Total resection of atypical choroid plexus papilloma is the most reliable treatment at present. However, postoperative chemotherapy should be considered for recurrence or dissemination.

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Tumors of the choroid plexus in children

Cited by 91 publications

References 0 publications

Carcinoma de plexo coróide: relato de quinze casos

Carcinoma de plexo coróide: relato de quinze casos

Superior parietal lobule approach for choroid plexus papillomas without preoperative embolization in very young children

Efficacy of Multi-staged Surgery and Adjuvant Chemotherapy for Successful Treatment of Atypical Choroid Plexus Papilloma in an Infant -Case Report-

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