Papiloma de plexos coroideos / Choroid plexuses papilloma

Introducción: el papiloma de plexos coroideos es un tipo de tumor cerebral o neoplasia papilar intraventricular de origen neuroectodérmico (se deriva del epitelio de los plexos coroideos). Junto con el carcinoma de plexos coroideos, de similar origen, constituye 3-5 por ciento del total de tumores intracraneales de la infancia.Objetivo: presentar el caso de un niño con diagnóstico de papiloma de plexos coroideos con antecedente prenatal de ventriculomegalia. Presentación del caso: neonato masculino nacido en el Hospital Roberto Galindo Terán, en Cobija, Departamento de Pando (Bolivia). Nació producto de un parto distócico por cesárea a las 39 semanas, debido al antecedente prenatal de ventriculomegalia, diagnosticada en el ultrasonido transfontanelar a las 32 semanas. Se le realizó un ultrasonido craneal con transductor neonatal, en el que se observó, al nivel de los cuernos occipitales, una masa hiperecogénica lobulada en los plexos coroideos con hidrocefalia comunicante. Se le diagnosticó papiloma de plexos coroideos. Discusión: la intervención quirúrgica es el tratamiento de elección para los papilomas de plexos coroideos, puesto que, además de extirpar el tumor, en la mayoría de los casos se elimina la hidrocefalia. Los avances de las técnicas quirúrgicas aseguran la retirada o disminución de la aferencia vascular tumoral y, consecuentemente, que la tasa de mortalidad sea baja.Conclusiones: ante un neonato con antecedente prenatal de ventriculomegalia o signos y síntomas relacionados con hidrocefalia, se debe sospechar la presencia de papiloma de plexos coroideos. El diagnóstico debe ser confirmado por estudios imagenológicos(AU)

Introduction: the choroid plexus papilloma is a type of brain tumor or intraventricular papillary neoplasm of neuroectodermal origin (derived from the epithelium of the choroid plexus). Together with choroidal plexus carcinoma, of similar origin, it constitutes 3-5 percent of all childhood intracranial tumors.Objective: to present the case of a child with a diagnosis of choroid plexus papilloma with a prenatal history of ventriculomegaly.Case presentation: male newborn born in the Hospital Roberto Galindo Terán, in Cobija, Pando Department (Bolivia). He was born due to a cesarean birth at the 39th week, due to a prenatal history of ventriculomegaly, diagnosed in the transfontanellar ultrasound at 32 weeks. A cranial ultrasound was carried out with a neonatal transducer, in which a hyperechoic mass lobulated in the choroid plexuses with communicating hydrocephalus was observed at the level of the occipital horns. He was diagnosed with choroid plexus papilloma.Discussion: surgical intervention is the treatment of choice for choroid plexus papillomas, since, in addition to removing the tumor, in most cases hydrocephalus is eliminated. Advances in surgical techniques ensure the removal or reduction of tumor vascular aference and, consequently, that the mortality rate is low.Conclusions: in the presence of a neonate with a prenatal history of ventriculomegaly or signs and symptoms related to hydrocephalus, the presence of choroid plexus papilloma should be suspected. The diagnosis must be confirmed by imaging studies(AU)

Tratamiento de los tumores de plexos coroideos en el Hospital Infantil Niño Jesús de Madrid: nuestra experiencia en las últimas tres décadas / Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades

Objetivo: Realizar una revisión de los pacientes en edad infantil con tumores de plexos coroideos (TPC) intervenidos en el Hospital Infantil Niño Jesús de Madrid desde enero de 1981 hasta septiembre de 2014. Material y método: Los casos registrados fueron analizados en base a la epidemiología, el grado tumoral, las características clínicas, la localización, las características de la diseminación, la actitud terapéutica, el pronóstico y las complicaciones. Resultados: Se registraron 17 pacientes con TPC en edad infantil. Los casos se distribuyeron de modo que 9 casos fueron diagnosticados como papiloma de plexos coroideos (PPC) (52,9%), 2 casos como PPC atípicos (11,7%) y 6 casos como carcinoma de plexos coroideos (CPC) (35,2%). La edad al diagnóstico fue menor de 2 años en 14 de los 17 pacientes (82,3%) y la incidencia fue mayor en los varones (82,3% de los casos). Se realizó resección completa en 16 pacientes (94,1%). Recibieron tratamiento complementario 6 pacientes (todos diagnosticados de CPC) (35,2%). Dos de los 17 pacientes fallecieron (11,7%), mostrando una densidad de incidencia de 0,01 muertes/año. Conclusiones : Nuestra serie de casos es concordante con series previas publicadas en la literatura científica en cuanto a epidemiología, grado tumoral, presentación clínica, características radiológicas y actitud terapéutica. El patrón oro en el tratamiento de los TPC es la resección quirúrgica completa. La quimioterapia y radioterapia deben reservarse para el tratamiento complementario de CPC y recidiva o resto tumoral de PPC atípico

Objective: To review childhood patients with choroid plexus tumors (CPT) who underwent surgery at Hospital Infantil Niño Jesús of Madrid since January 1981 to September 2014. Material and methods: Registered charts were analyzed based on the epidemiology, tumor grade, clinical profile, location, dissemination characteristics, therapy, prognosis and complications. Results: Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year. Conclusions: Our case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP

An unusual location for a choroid plexus papilloma: the pineal region.

PURPOSE: Choroid plexus papillomas (CPP) are rare benign neoplasms of the central nervous system that occur most often in children during the first decade of life. They occur most often in the lateral ventricle. It is extremely rare for a CPP to occur in the pineal region. We describe the case of a child with a CPP located in the pineal region, who was initially diagnosed with obstructive hydrocephalus by cranial computed tomography (CT). METHODS: A 9-year-old female patient presented with complaints of visual disturbance, nausea, and vomiting. Magnetic resonance imaging (MRI) showed a poor contrast-enhanced pineal-localized lesion. Anatomical variations within the patient caused her surgery to proceed using a supratentorial-occipital interhemispheric approach. RESULTS: The tumor was totally removed, and a histological examination revealed the tumor to be a typical CPP. The patient received follow-up neurological and ophthalmologic examinations at 3, 6, 9, 12, 24, and 36 months postoperatively, which demonstrated her progressive improvement. CONCLUSIONS: CPPs may have a wide range of locations and resulting symptoms. However, the pineal region is a rarely encountered location, particularly for pediatric patients. It is of great value to correctly differentiate neoplasms such as germ cell tumors, pineocytomas, meningiomas, and astrocytomas, so that patients receive the correct diagnosis and treatment approach.

Choroid plexus tumors in children: a population-based study.

BACKGROUND: Choroid plexus tumors are rare neoplasms that primarily occur in children. The use of the SEER (Surveillance, Epidemiology and End Results) database allows for the analysis of the relationship between prognostic factors and survival. METHODS: We analyzed the SEER database to select pediatric patients (<18 years old) with histologically confirmed diagnoses of choroid plexus papillomas (CPP; WHO Grade 0), atypical CPP (WHO Grade I) and choroid plexus carcinomas (CPC; WHO grade III). In univariate and multivariate analysis, we analyzed the relationship between demographic (age, gender, race, date of diagnosis) and treatment factors (extent of surgical resection, use of adjuvant radiation) on survival. RESULTS: Overall, 168 pediatric subjects with choroid plexus tumors were identified as follows: 75 cases of CPP, 12 cases of atypical CPP and 81 cases of CPC. The median follow-up time was 3.5 years for CPP and 7.7 years for CPC. The median age at diagnosis was 4 years for CPP (10-90th percentile 0-16 years) and 1 year for CPC (10-90th percentile 0-10 years). In univariate regression analysis, CPC histology (ß = -3.2, 95% confidence interval, CI -4.8 to -1.5, p < 0.001) was significantly associated with younger age at diagnosis in comparison to CPP. The mean tumor size was 3.7 cm for CPP and 6.0 cm for CPC (p < 0.001). A higher-grade tumor was associated with significantly increased mortality (hazard ratio, HR = 28.90, 95% CI 3.94-211.83, p = 0.001). Overall survival at 5 years was 98.7% for CPP and 58.5% for CPC (p < 0.001). Among those patients with CPC, gross total resection (GTR) was associated with a significantly lower mortality (HR = 0.21, 95% CI 0.07-0.66, p = 0.007). Overall survival at 5 years was 70.9% after GTR, significantly better than 35.9% after subtotal resection (p = 0.012) and 30% after no surgery (p = 0.003). Radiation treatment was not found to confer a survival benefit in CPC. No demographic characteristics (age, sex, race, date of diagnosis) were significantly associated with mortality. CONCLUSIONS: Analysis of a pediatric cohort of choroid plexus tumors in children in the SEER database shows that tumor grade is predictive of survival. In cases of CPC, the extent of surgical resection, especially GTR, is significantly associated with increased survival. Radiation did not confer survival benefit.

Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience.

BACKGROUND: Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors. METHODS: The CPT population from the Canadian databank of CNS tumor in children ≤ 36 months diagnosed between 1990 and 2005 was reviewed RESULTS: Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children < 3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC. CONCLUSION: By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy.

Clinicopathologic and immunohistochemical study of choroid plexus tumors: single-institution experience in Mexican population.

In recent years, few studies have specifically focused on only histological features in choroid plexus tumors. We retrospectively reviewed the clinicopathologic and histological features in 37 patients with choroid plexus tumors and correlated these with glial fibrillary acidic protein (GFAP) expression and proliferation cell nuclear antigen (PCNA), p53, p21, and Rb labeling indexes, with special attention to tumor recurrence/regrowth. The study included 24 choroid plexus papillomas (CPPs), 4 atypical choroid plexus papillomas (ACPPs), and 9 choroid plexus carcinomas (CPCs). Patient age ranged from 15 to 70 years (mean 44 years). Most of the choroid plexus tumors were located in the IV ventricle. Recurrence was observed in 21 (52%) cases, 14 of which were CPP and 7 of which were CPC (P = 0.032). Histologic findings included major necrosis, fibrosis and psammoma bodies, amyloid deposits, inflammation, and thick vessels in recurrent tumors. The PCNA labeling index was 52.04 + or - 13.92 in CPPs, 76.50 + or - 17 in ACPPs, and 95.22 + or - 21.34 in CPCs (P = 0.009), and 67.43 + or - 28 in recurrent tumors. Similar values were found for p53, p21, and Rb. Furthermore, we observed that these presented more histological changes, adding, than nonrecurrent tumors, as well as a higher proliferation index of cell-cycle markers, and these were dependent predictor factors of survival. Recurrent tumors showed a different biological behavior than nonrecurrent tumors, but histological observations showed no mitotic features in order to consider them as grade II.

Fetal brain tumors: a review of 154 cases.

The purpose of this literature review is to describe the various types of brain tumors that occur in the fetus, their locations, initial findings, pathology, and outcome. Data are presented that show which patients are likely to survive or benefit from treatment compared with those who are unlikely to respond. An analysis is performed on patients with fetal brain tumors that were detected prenatally by imaging studies or discovered directly after birth. The review revealed 154 fetuses presenting with brain tumors. Teratoma was the leading neoplasm. Next was astrocytoma followed by craniopharyngioma, primitive neuroectodermal tumor, choroid plexus papilloma, meningeal tumors, and ependymoma. The most common presenting findings were macrocephaly and an intracranial mass. Overall hydrocephalus was next, followed by stillbirth. Most tumors were detected during the third trimester of pregnancy. Over a third of the affected fetuses were stillborn. Many tumors were inoperable as they occupied much of the intracranial cavity and involved large areas of the brain. Overall survival rate was 23/154, or 15%. Generally fetal brain tumors are associated with a poor prognosis.

Choroid plexus tumors: a review of 28-year experience.

The aims of the study were to review the patients with choroid plexus tumor (CPT) treated in Slovenia between 1972-1999, to calculate the incidence of CPTs, and to evaluate treatment results in respect to tumor histology and mode of therapy. Cancer Registry of Slovenia database was used for patients identification. Histological specimens and medical records were reviewed. Twelve patients (7 females, 5 males), 0.8-43 years old (median 6.1 years; <15 years: 10/12,83%) with CPT, representing 0.36% of all intracranial tumors registered during the period under study, were identified. Considering children less than 15 years old only, the corresponding percentage was 3.69. There were eight papillomas (CPPs) and four carcinomas (CPCs) with no difference in age distribution between the groups. Of seven patients with gross tumor resection in CPP group, one patient died of postoperative meningitis and one had local recurrence 1.6 years after surgery; the latter is disease-free 17.9 years after re-operation. One patient with macroscopic residue after surgery is alive and asymptomatic after 16.5 years. In the CPC-group, only the patient who received adjuvant BEP chemotherapy and craniospinal irradiation following incomplete surgery is alive with no signs of disease after 6.5 years. Ten-year disease-specific survival for all CPTs and for CPP subgroup was 73% and 100%, respectively. In Slovenia, CPTs represent 0.36% of intracranial tumors. In children, the incidence is 3.69%. CPPs are twice as frequent as CPCs. In CPPs, the treatment of choice is surgery alone. After incomplete surgery, wait-and-see policy seems to be justified. For CPCs, adjuvant multiagent chemotherapy and craniospinal radiotherapy following surgery should be considered.