RESUMEN
Choroid plexus tumors (CPT) are rare and highly vascularized neoplasms that have three histologically confirmed diagnoses, including choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma (CPC). This study aimed to determine the epidemiology and survival of patients with CPTs and develop a nomogram to quantify the prognosis of the patients with CPT. Data of 808 patients who were diagnosed as CPT between 2000 and 2020 was obtained from the surveillance, epidemiology, and end results database. Descriptive analysis was used to assess the distribution and tumor-related characteristics of the patients with CPT. Independent prognostic factors for patients with CPT were identified by univariate and multivariate Cox regression analysis. The nomogram was established and evaluated by receiver operating characteristic curve, and decision curve analysis (DCA), calibration curves. The independent prognostic factors for patients with CPT are age, tumor size, surgery, chemotherapy, tumor number, pathologies, and race. For the prognostic nomogram, the area under the curve (AUC) of 60-, 120-, and 180-months were 0.855, 0.869 and 0.857 in the training set and 0.836, 0.864 and 0.922 in the test set. The DCA and calibration curve indicated the good performance of the nomogram. Patients with CPTs can be diagnosed at any age. Among the three histopathological tumors, patients with CPC had the worst prognosis. The nomogram was established to predict the prognosis of patients with CPT, which had satisfactory accuracy, and clinical utility may benefit for clinical decision-making.
Asunto(s)
Neoplasias del Plexo Coroideo , Nomogramas , Programa de VERF , Humanos , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/diagnóstico , Neoplasias del Plexo Coroideo/mortalidad , Femenino , Masculino , Pronóstico , Persona de Mediana Edad , Adulto , Adolescente , Anciano , Niño , Curva ROC , Adulto Joven , Preescolar , Lactante , CarcinomaRESUMEN
TP53 p.R337H germline mutation is highly prevalent in the Southern region of Brazil. We sought to investigate TP53 p.R337H mutation in pediatric tumor samples from a population settled in a geographic area of high prevalence for this variant. Mutation assessment and genetic counseling for carriers/relatives were provided. 6/57 tumor samples were heterozygous for TP53 p.R337H. As expected, a high frequency was observed within adrenocortical tumors (3/3) and choroid plexus carcinomas (2/2). Interestingly, the TP53 R337H mutation was found in one case of pediatric rhabdomyosarcoma with Li-Fraumeni pedigree. Our finding expands the spectrum of childhood cancer associated with this germline mutation.
Asunto(s)
Mutación de Línea Germinal , Neoplasias/genética , Proteína p53 Supresora de Tumor/genética , Neoplasias de la Corteza Suprarrenal/epidemiología , Neoplasias de la Corteza Suprarrenal/genética , Brasil/epidemiología , Carcinoma/epidemiología , Carcinoma/genética , Preescolar , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/genética , Estudios de Cohortes , Femenino , Humanos , Masculino , Tasa de Mutación , Neoplasias/epidemiología , Mutación Puntual , Rabdomiosarcoma/epidemiología , Rabdomiosarcoma/genéticaAsunto(s)
Neoplasias del Plexo Coroideo/patología , Glioma/patología , Biomarcadores de Tumor/análisis , Neoplasias del Plexo Coroideo/química , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/cirugía , Glioma/química , Glioma/epidemiología , Glioma/cirugía , Humanos , Masculino , Persona de Mediana Edad , Proteínas de Neoplasias/análisisRESUMEN
Choroid plexus tumors (CPTs) are rare neoplasms of the central nervous system whose optimal management is not well defined. The Surveillance Epidemiology and End Results (SEER) Database from 1978 to 2009 was queried to define population-based outcomes for all patients with CPTs. Patient demographic data, histological classification (choroid plexus papilloma [CPP], atypical CPP [aCPP], and choroid plexus carcinoma [CPC]), extent of surgery, and use of radiation therapy (RT) as part of an initial course of therapy were analyzed for impact on overall survival (OS) and cause-specific survival (CSS). Chemotherapy data were not available within the SEER database. A total of 349 patients with CPTs were identified (120 CPCs, 26 aCPPs, and 203 CPPs). Patients with CPC presented at a younger age (median 3, mean 14.8 years) relative to CPP (median 25, mean 28.4 years; p < 0.0001). Histology was a significant predictor of OS, with 5-year OS rates of 90, 77, and 58 % for CPP, aCPP, and CPC, respectively. Older age and male sex were prognostic for worse OS and CSS for CPP. Only extent of surgery had a significant impact on survival for CPC. The use of adjuvant RT in patients with CPC undergoing surgery was not associated with a significantly improved OS (p = 0.17). For patients undergoing GTR without RT as part of an initial course of therapy, estimated 5- and 10-year OS were 70 % (±7 %) and 67 % (±8 %), respectively. Prospective data are required to define the optimal combination of surgery with adjuvant therapies, including chemotherapy.
Asunto(s)
Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/terapia , Adolescente , Adulto , Anciano , Niño , Preescolar , Terapia Combinada , Bases de Datos Factuales , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Análisis de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
Choroid plexus papillomas (CPPs) and carcinomas (CPCs) are rare neoplasms that affect mostly children. Due to their rarity, their epidemiology and outcomes are incompletely understood. The National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) Program is a well-established population-based group of registries that collects and publishes cancer incidence and survival data representing approximately 28 % of the US population. SEER-STAT v8.1.2 was used to identify patients with ICD-O-3 codes for choroid plexus tumors in patients aged 0-19. Demographics, initial treatment, and follow-up data were collected. Statistical methods including Kaplan-Meier curves, log rank tests, and Cox proportional hazards regression were used to estimate associations between independent variables and survival. The SEER registries contained 107 CPPs (2004-2010) and 95 CPCs (1978-2010). Median follow-up was 38 and 40 months, respectively. More than 75 % of CPCs were diagnosed before the age of 5 years, versus 48 % for CPPs. Sixty-five percent of CPCs and 57 % of CPPs occurred in males. In both groups at least 90 % of children underwent surgical resection. Gross total resection (GTR) was achieved in 67.0 % of CPCs and 63.6 % of CPPs. Almost 17 % of CPCs were treated with radiation versus only 0.9 % of CPPs. More than 98 % of patients with CPP were alive at the last follow-up, versus 62 % of CPC patients. For CPC, surgery was significantly associated with increased overall survival, but contrary to previous reports, extent of surgical resection was not associated with survival. Age, sex, race, and radiation treatment also had no effect on survival. This report, using the SEER datasets, corroborates many findings of previous smaller studies on CPTs. CPC occurs in younger children, with a male predominance, and a much worse prognosis than CPP. As such, these tumors have been treated aggressively with high rates of GTR and radiation treatment. Despite these treatments, overall survival for CPC remains poor.
Asunto(s)
Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/terapia , Adolescente , Carcinoma/epidemiología , Carcinoma/terapia , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Análisis Multivariante , Análisis de Supervivencia , Resultado del Tratamiento , Estados Unidos/epidemiología , Adulto JovenRESUMEN
Choroid plexus carcinoma (CPC) is a World Health Organization (WHO) grade III brain tumor with a poor prognosis that occurs mainly in children. Gross total resection of CPC is highly recommended and is associated with improved overall survival, although it is often associated with increased morbidity. The use of adjuvant therapies has yet to be standardized, although evidence suggests that for patients with incompletely resected CPCs, a combination of chemotherapy and radiation therapy may be beneficial. The use of radiation therapy for younger children (<3 years old) with CPC, however, is not recommended, due to the potential negative neurological sequelae associated with radiation to the developing brain. Given that the majority of CPC patients are young children, questions regarding optimal radiation dose, chemotherapy agents, and how to combine these two adjuvant treatment modalities to achieve the best outcomes remain unanswered. In this paper we summarize the current management of CPC in the literature. Further studies are needed to standardize the treatment paradigm for this malignant brain tumor.
Asunto(s)
Neoplasias Encefálicas/patología , Neoplasias Encefálicas/terapia , Carcinoma/patología , Carcinoma/terapia , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/terapia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/epidemiología , Carcinoma/diagnóstico , Carcinoma/epidemiología , Neoplasias del Plexo Coroideo/diagnóstico , Neoplasias del Plexo Coroideo/epidemiología , Terapia Combinada/métodos , Humanos , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Carcinoma/patología , Carcinoma/terapia , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/terapia , Papiloma del Plexo Coroideo/patología , Papiloma del Plexo Coroideo/terapia , Sistema de Registros , Adolescente , Carcinoma/epidemiología , Carcinoma/mortalidad , Niño , Preescolar , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/mortalidad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Papiloma del Plexo Coroideo/epidemiología , Papiloma del Plexo Coroideo/mortalidad , Estados Unidos/epidemiologíaRESUMEN
SUMMARY: PT promises to reduce side effects in children with brain tumors by sparing normal tissue compared with 3D conformal or intensity-modulated radiation therapy. Information is lacking about the combined effects of PT and chemotherapy in young children. We describe imaging changes in 8 very young children with localized brain tumors who received PT after chemotherapy. Mostly transient signal abnormalities and enhancement in brain parenchyma were observed by serial MR imaging, which were consistent with radiation-induced effects on normal-appearing tissue. Correlation with PT planning data revealed that the areas of imaging abnormality were located within or adjacent to the volume that received the highest radiation dose. Radiologists should be aware of these findings in children who receive PT after chemotherapy. In this report, we describe the time course of these PT-related imaging findings and correlate them with treatment and clinical outcomes.
Asunto(s)
Neoplasias Encefálicas/patología , Neoplasias Encefálicas/terapia , Quimioradioterapia/métodos , Terapia de Protones/métodos , Tumor Rabdoide/patología , Tumor Rabdoide/terapia , Teratoma/patología , Teratoma/terapia , Neoplasias Encefálicas/epidemiología , Carcinoma/epidemiología , Carcinoma/patología , Carcinoma/terapia , Neoplasias Cerebelosas/epidemiología , Neoplasias Cerebelosas/patología , Neoplasias Cerebelosas/terapia , Quimioradioterapia/efectos adversos , Preescolar , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/terapia , Imagen de Difusión por Resonancia Magnética , Ependimoma/epidemiología , Ependimoma/patología , Ependimoma/terapia , Femenino , Estudios de Seguimiento , Gadolinio , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Meduloblastoma/epidemiología , Meduloblastoma/patología , Meduloblastoma/terapia , Tumores Neuroectodérmicos Primitivos/epidemiología , Tumores Neuroectodérmicos Primitivos/patología , Tumores Neuroectodérmicos Primitivos/terapia , Terapia de Protones/efectos adversos , Dosis de Radiación , Tumor Rabdoide/epidemiología , Factores de Riesgo , Teratoma/epidemiologíaRESUMEN
PURPOSE: Whether childhood adrenocortical tumors (ACTs), choroid plexus tumors (CPTs), and rhabdomyosarcoma (RMS) are early manifestation of Li-Fraumeni syndrome (LFS) is uncertain. In this study, we evaluated the frequency of germline TP53 mutations and family history in a population-based series of patients. PATIENTS AND METHODS: We identified children (≤18 years) diagnosed between 1958 and 2008 with ACT (n = 3) or CPT (n = 7), or children ≤5 years with RMS (n = 29). Registry-based pedigree expansion was performed. RESULTS: No patients had a family history of classic LFS but 17 fulfilled Chompret or Eeles criteria. TP53 mutations were found in 1/3 ACT patients and 1/18 RMS patients; both were novel mutations. Of five tested CPT patients none had a detectable mutation. No excess of LFS associated tumors was observed, except for breast cancer in families of CPT patients. An overall increased cancer incidence was observed in families of patients with CPT [standardized incidence ratio (SIR) = 2.0; 95% CI: 1.1-3.5] due to excess of breast and female kidney cancer and in families of patients with RMS (SIR = 1.2; 95% CI: 0.9-1.7), due to excess of early-onset melanoma and male stomach cancer. CONCLUSION: Relatives of patients with childhood ACTs, CPTs, and RMSs showed no increased risk of LFS associated tumors. However, TP53 mutations could be found in these children irrespective of family history. Absence of LFS associated tumors may suggest the presence of other cancer syndromes. Improved knowledge about relatives' cancer risks could be helpful in counseling family members of children with cancer.
Asunto(s)
Neoplasias de la Corteza Suprarrenal , Neoplasias del Plexo Coroideo , Mutación de Línea Germinal , Síndrome de Li-Fraumeni , Sistema de Registros , Proteína p53 Supresora de Tumor/genética , Adolescente , Neoplasias de la Corteza Suprarrenal/epidemiología , Neoplasias de la Corteza Suprarrenal/genética , Niño , Preescolar , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/genética , Femenino , Humanos , Incidencia , Síndrome de Li-Fraumeni/epidemiología , Síndrome de Li-Fraumeni/genética , Masculino , Linaje , Estudios Retrospectivos , Factores de Riesgo , Suecia/epidemiologíaRESUMEN
PURPOSE: To evaluate the incidence of infant brain tumors and survival outcomes by disease and treatment variables. METHODS AND MATERIALS: The Surveillance, Epidemiology, and End Results (SEER) Program November 2008 submission database provided age-adjusted incidence rates and individual case information for primary brain tumors diagnosed between 1973 and 2006 in infants less than 12 months of age. RESULTS: Between 1973 and 1986, the incidence of infant brain tumors increased from 16 to 40 cases per million (CPM), and from 1986 to 2006, the annual incidence rate averaged 35 CPM. Leading histologies by annual incidence in CPM were gliomas (13.8), medulloblastoma and primitive neuroectodermal tumors (6.6), and ependymomas (3.6). The annual incidence was higher in whites than in blacks (35.0 vs. 21.3 CPM). Infants with low-grade gliomas had the highest observed survival, and those with atypical teratoid rhabdoid tumors (ATRTs) or primary rhabdoid tumors of the brain had the lowest. Between 1979 and 1993, the annual rate of cases treated with radiation within the first 4 months from diagnosis declined from 20.5 CPM to <2 CPM. For infants with medulloblastoma, desmoplastic histology and treatment with both surgery and upfront radiation were associated with improved survival, but on multivariate regression, only combined surgery and radiation remained associated with improved survival, with a hazard ratio for death of 0.17 compared with surgery alone (p = 0.005). For ATRTs, those treated with surgery and upfront radiation had a 12-month survival of 100% compared with 24.4% for those treated with surgery alone (p = 0.016). For ependymomas survival was higher in patients treated in more recent decades (p = 0.001). CONCLUSION: The incidence of infant brain tumors has been stable since 1986. Survival outcomes varied markedly by histology. For infants with medulloblastoma and ATRTs, improved survival was observed in patients treated with both surgery and early radiation compared with those treated with surgery alone.
Asunto(s)
Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/radioterapia , Análisis de Varianza , Población Negra/estadística & datos numéricos , Neoplasias Encefálicas/etnología , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/etnología , Neoplasias del Plexo Coroideo/mortalidad , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/radioterapia , Ependimoma/epidemiología , Ependimoma/etnología , Ependimoma/mortalidad , Ependimoma/patología , Ependimoma/radioterapia , Femenino , Glioma/epidemiología , Glioma/etnología , Glioma/mortalidad , Glioma/patología , Glioma/radioterapia , Humanos , Incidencia , Lactante , Masculino , Meduloblastoma/epidemiología , Meduloblastoma/etnología , Meduloblastoma/mortalidad , Meduloblastoma/patología , Meduloblastoma/radioterapia , Neoplasias de Células Germinales y Embrionarias/epidemiología , Neoplasias de Células Germinales y Embrionarias/etnología , Neoplasias de Células Germinales y Embrionarias/mortalidad , Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias de Células Germinales y Embrionarias/radioterapia , Tumores Neuroectodérmicos Primitivos/epidemiología , Tumores Neuroectodérmicos Primitivos/etnología , Tumores Neuroectodérmicos Primitivos/mortalidad , Tumores Neuroectodérmicos Primitivos/patología , Tumores Neuroectodérmicos Primitivos/radioterapia , Tumor Rabdoide/epidemiología , Tumor Rabdoide/etnología , Tumor Rabdoide/mortalidad , Tumor Rabdoide/patología , Tumor Rabdoide/radioterapia , Programa de VERF , Estados Unidos/epidemiología , Población Blanca/estadística & datos numéricosRESUMEN
OBJECTIVE: To report the prenatal findings and postnatal outcome of fetal ventriculomegaly associated with isolated large choroid plexus cysts (CPCs). METHOD: Cases of isolated fetal ventriculomegaly and large CPCs (>10 mm) were identified through a search of patient records from 2003 to 2006. Ultrasound (US) findings were reviewed: unilateral or bilateral ventriculomegaly, ventricular size, size of CPCs, and changes on serial scans. Correlation was made with fetal magnetic resonance imaging (MRI), pregnancy outcome, and long-term follow-up. RESULTS: Six cases of isolated large CPCs (12-30 mm) with ventriculomegaly (11-17 mm) were detected on US at 18 to 26 weeks of gestation. Serial prenatal US showed the CPCs resolved (one case) or decreased in size (five cases). Ventricular size became normal during pregnancy in five cases and decreased in size in one case. Fetal MRI performed in three cases showed no additional findings. Five patients had amniocentesis which showed normal karyotype. There was one termination of pregnancy (the fetus showed no abnormality on external examination). There were five healthy newborns, with follow-up to 4.5 years of age (one), 5.5 years (one), and 6 years (three). All had normal physical and developmental outcome. CONCLUSION: Large isolated CPCs may transiently dilate the fetal cerebral ventricles. Follow-up to 6 years has shown normal growth and development.
Asunto(s)
Quistes del Sistema Nervioso Central/diagnóstico por imagen , Neoplasias del Plexo Coroideo/diagnóstico por imagen , Hidrocefalia/diagnóstico por imagen , Resultado del Embarazo/epidemiología , Ultrasonografía Prenatal , Quistes del Sistema Nervioso Central/complicaciones , Quistes del Sistema Nervioso Central/epidemiología , Quistes del Sistema Nervioso Central/patología , Niño , Preescolar , Plexo Coroideo/anomalías , Plexo Coroideo/diagnóstico por imagen , Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/complicaciones , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/patología , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Edad Gestacional , Humanos , Hidrocefalia/complicaciones , Hidrocefalia/epidemiología , Hidrocefalia/genética , Lactante , Recién Nacido , Enfermedades del Recién Nacido/epidemiología , Enfermedades del Recién Nacido/etiología , Embarazo , Estudios Retrospectivos , Carga Tumoral , Ultrasonografía Prenatal/métodos , Ultrasonografía Prenatal/estadística & datos numéricosRESUMEN
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.
Asunto(s)
Carcinoma/epidemiología , Carcinoma/terapia , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/terapia , Papiloma del Plexo Coroideo/epidemiología , Papiloma del Plexo Coroideo/terapia , Edad de Inicio , Antineoplásicos/uso terapéutico , Canadá/epidemiología , Quimioterapia Adyuvante , Preescolar , Neoplasias del Plexo Coroideo/patología , Supervivencia sin Enfermedad , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Masculino , Procedimientos Neuroquirúrgicos , Papiloma del Plexo Coroideo/patología , PronósticoRESUMEN
OBJECTIVE: The characteristics of 451 Egyptian children (aged 0-14 years) with primary intracranial neoplasms were investigated for demographic, clinical, topographical and pathological features using the most recent 2007 Classification of Central Nervous System Tumors. PATIENTS AND METHODS: This was a retrospective study performed in the Departments of Pediatric Neurosurgery of the Cairo University Hospitals from 2005 to 2008. RESULTS: There was a slight male predominance (51.4%) observed in our study, and the most affected age group was 5-9 years old (43.2%). Most of the tumors were confined to a single compartment (infratentorial in 49.7%, supratentorial in 46.6%), while 3.8% of the tumors involved multiple compartments. The most common intracranial tumors were astrocytomas (35%), medulloblastomas (18.8%), craniopharyngiomas (11.3%) and ependymomas (10%). Pilocytic astrocytomas constituted 55% of all astrocytomas and 19.3% of all brain tumors, only slightly ahead of medulloblastomas. Less common types were primitive neuroectodermal tumors (2.7%), followed by meningiomas, germ cell tumors and choroid plexus tumors (2.4% each). According to the International Classification of Diseases for Oncology Coding (ICD-O-4), benign, borderline and malignant tumors constituted 7.54, 36.14 and 56.32%, respectively. CONCLUSION: The characteristics of pediatric intracranial tumors in Egypt are generally similar to those reported in the literature, with only minor differences.
Asunto(s)
Astrocitoma/epidemiología , Neoplasias Encefálicas/epidemiología , Hospitales Universitarios/estadística & datos numéricos , Adolescente , Distribución por Edad , Neoplasias Cerebelosas/epidemiología , Niño , Preescolar , Neoplasias del Plexo Coroideo/epidemiología , Craneofaringioma/epidemiología , Egipto/epidemiología , Ependimoma/epidemiología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Meduloblastoma/epidemiología , Neoplasias de Células Germinales y Embrionarias/epidemiología , Neurilemoma/epidemiología , Oligodendroglioma/epidemiología , Estudios Retrospectivos , Distribución por Sexo , Neoplasias Supratentoriales/epidemiologíaRESUMEN
OBJECTIVES: Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. METHODS: A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 % confidence intervals. Standardised morbidity rates were also calculated. RESULTS: There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 % of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. CONCLUSIONS: There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.
Asunto(s)
Neoplasias del Sistema Nervioso Central/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias del Plexo Coroideo/epidemiología , Colombia/epidemiología , Femenino , Germinoma/epidemiología , Glioma/epidemiología , Humanos , Incidencia , Masculino , Meningioma/epidemiología , Persona de Mediana Edad , Pinealoma/epidemiología , Vigilancia de la Población , Población Urbana/estadística & datos numéricos , Adulto JovenRESUMEN
Objetivos Determinar la frecuencia de los tumores primarios del sistema nervioso central, diagnosticados en Cartagena de Indias durante el periodo de 2001-2006 y, determinar las características demográficas, epidemiológicas y clínicas de los pacientes con tumores del sistema nervioso central de una institución de la ciudad, en el mismo periodo. Métodos Se realizó un estudio descriptivo de vigilancia epidemiológica pasiva. Se tomaron los reportes de patología con diagnóstico nuevo de tumor primario del sistema nervioso central de todos laboratorios de Cartagena y se analizaron las historias clínicas disponibles de estos casos. Se estimaron las tasas de incidencia general, por año, género, grupos de edad y tipo histológico con intervalos de confianza al 95 por ciento. Además se calcularon razones estandarizadas de morbilidad. Resultados Durante los años 2001 y 2006 se encontraron 390 casos. La tasa incidencia general fue de 6,91/100 000 personas-año. El tipo histológico más frecuente fue meningioma (3,46/100 000 personas-año). Sólo se determinó la procedencia en el 43,1 por ciento de los casos. Las razones estandarizadas de morbilidad fueron más altas en Cartagena con respecto a las de los Estados Unidos, el Instituto Nacional de Cancerología de Colombia y el Registro Poblacional de Cáncer de Cali. Conclusiones Hubo una frecuencia de tumores primarios del sistema nervioso central más elevada en Cartagena que en el resto del país. Se recomienda mejorar los sistemas de registro y vigilancia para determinar la magnitud real del problema y fomentar investigaciones en busca de factores de riesgo.
Objectives Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. Methods A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 percent confidence intervals. Standardised morbidity rates were also calculated. Results There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 percent of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. Conclusions There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.
Asunto(s)
Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Neoplasias del Sistema Nervioso Central/epidemiología , Neoplasias del Plexo Coroideo/epidemiología , Colombia/epidemiología , Germinoma/epidemiología , Glioma/epidemiología , Incidencia , Meningioma/epidemiología , Pinealoma/epidemiología , Vigilancia de la Población , Población Urbana/estadística & datos numéricos , Adulto JovenRESUMEN
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.
Asunto(s)
Carcinoma/metabolismo , Carcinoma/patología , Neoplasias del Plexo Coroideo/metabolismo , Neoplasias del Plexo Coroideo/patología , Papiloma del Plexo Coroideo/metabolismo , Papiloma del Plexo Coroideo/patología , Adolescente , Adulto , Carcinoma/epidemiología , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/mortalidad , Inhibidor p21 de las Quinasas Dependientes de la Ciclina/metabolismo , Femenino , Estudios de Seguimiento , Proteína Ácida Fibrilar de la Glía/metabolismo , Humanos , Estimación de Kaplan-Meier , Masculino , México/epidemiología , Persona de Mediana Edad , Papiloma del Plexo Coroideo/epidemiología , Papiloma del Plexo Coroideo/mortalidad , Antígeno Nuclear de Célula en Proliferación/metabolismo , Proteína de Retinoblastoma/metabolismo , Estudios Retrospectivos , Proteína p53 Supresora de Tumor/metabolismo , Adulto JovenRESUMEN
This study investigates the clinical and pathologic findings associated with 173 primary brain tumors in our hospital population of dogs that presented between the years 1986 and 2002. Of the 173 primary brain tumors, 78 (45%) were meningiomas, 29 (17%) were astrocytomas, 25 (14%) were oligodendrogliomas, 12 (7%) were choroid plexus tumors, and 7 (4%) were primary central nervous system lymphomas. Smaller numbers of glioblastomas (n = 5), primitive neuroectodermal tumors (n = 5), histiocytic sarcomas (n = 5), vascular hamartomas (n = 4), and unclassified gliomas (n = 3) were identified. One dog had both a meningioma and an astrocytoma. Most tumors were located within the telencephalon, and seizures were the most common clinical presenting complaint. Of 168 tumors for which a location in the brain was recorded at postmortem examination, 79 were found to involve more than 1 brain division. Other neoplasms unrelated to the primary brain tumor were identified on postmortem examination in 39 dogs (23%). Intrathoracic and intraabdominal neoplasms were present at necropsy in 13 and 24 cases, respectively. Based on the results of this study, thoracic radiographs and abdominal ultrasonography may be indicated to look for extracranial neoplasia prior to advanced imaging of the brain or intracranial surgery.
Asunto(s)
Neoplasias Encefálicas/veterinaria , Enfermedades de los Perros/epidemiología , Animales , Astrocitoma/epidemiología , Astrocitoma/veterinaria , Autopsia/veterinaria , Neoplasias Encefálicas/epidemiología , Neoplasias del Plexo Coroideo/epidemiología , Neoplasias del Plexo Coroideo/veterinaria , Enfermedades de los Perros/etiología , Enfermedades de los Perros/patología , Perros , Linfoma/epidemiología , Linfoma/veterinaria , Imagen por Resonancia Magnética/veterinaria , Meningioma/epidemiología , Meningioma/veterinaria , Oligodendroglioma/epidemiología , Oligodendroglioma/veterinaria , Linaje , Philadelphia/epidemiología , Registros/veterinaria , Estudios RetrospectivosRESUMEN
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.
Asunto(s)
Neoplasias del Plexo Coroideo/epidemiología , Adolescente , Adulto , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/terapia , Carcinoma/diagnóstico , Carcinoma/epidemiología , Carcinoma/terapia , Niño , Preescolar , Neoplasias del Plexo Coroideo/patología , Neoplasias del Plexo Coroideo/terapia , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Papiloma del Plexo Coroideo/diagnóstico , Papiloma del Plexo Coroideo/epidemiología , Papiloma del Plexo Coroideo/terapia , Recurrencia , Factores de Tiempo , Resultado del TratamientoRESUMEN
Tumours derived from choroid plexus are rare central nervous system neoplasms affecting mainly children. This study presents a series of 38 patients with neuroectodermal tumours. Twenty four of them had the histological and/or immunohistochemical diagnosis of choroid plexus carcinoma (CPC). Sixteen of these patients were male. The left lateral ventricle was affected in most cases. Main clinical features were: hydrocephalus, intracranial hypertension and convulsion. All the patients were treated by surgery. There were 2 deaths due to surgical complications. Nine patients had recurrence, dying in a intermediate time of 12.3 months and 7 patients remain alive. The other cases did not present available data. CPC is very prevalent in Curitiba when compared to other choroid plexus tumours and it might be related to some pathogenic agent.
Asunto(s)
Carcinoma/epidemiología , Neoplasias del Plexo Coroideo/epidemiología , Brasil/epidemiología , Carcinoma/diagnóstico , Carcinoma/cirugía , Niño , Neoplasias del Plexo Coroideo/diagnóstico , Neoplasias del Plexo Coroideo/cirugía , Femenino , Humanos , Masculino , Recurrencia Local de Neoplasia , Prevalencia , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
INTRODUCTION AND OBJECTIVE: Tumors of the choroid plexus are rare tumors of neuro ectodermal origin, accounting for less 1% of intracranial tumors in all ages. Most cases present in children less than 2 years of age. These tumors have been classified according to histopathological criteria into papilloma and carcinoma. DEVELOPMENT: We review the epidemiological, clinical, neuropathological details, neuroradiological aspects and treatment of choroid plexus tumors. CONCLUSIONS: Choroid plexus tumors may present with overt intracranial hypertension with or without focal neurological signs. In the adult population, headaches are the most commonly encountered symptom. The CT characteristics of CPT are well characterized. On non enhanced studies the tumor appears as a smooth or lobulated mass, hyperdense in relation to surrounding brain parenchyma. With intravenous contrast, there is marked, homogeneous enhancement. With MRI these tumors showed an iso intensity in T1 weighted images and iso hypo intensity in T2 weighted images, with marked enhancement after gadolinium. The treatment of choice is total surgical excision with minimal damage to the surrounding neural elements. For carcinomas, adjuvant treatment in the form of chemotherapy supplemented by radiation therapy in older children.