ABSTRACT
RESUMEN: La clasificación de los Tumores Primarios del Sistema Nervioso Central (SNC) tiene su origen en la descripción morfológica, cuyo análisis histopatológico ha permitido identificar la línea celular involucrada en estos tumores y obtener el reconocimiento de ciertas características de estas lesiones y su evolución clínica. El estudio molecular ha venido a complementar el diagnóstico inicial permitiendo reconocer entidades que no son distinguibles de otra manera y que han variado los conceptos y definiciones de varias entidades patológicas que modifican el horizonte visible de estas enfermedades. El papel de las imágenes de Resonancia Magnética (RM) en el manejo de los tumores intraaxiales se puede dividir ampliamente en el diagnóstico y la clasificación de los tumores, la planificación del tratamiento y el tratamiento posterior. El presente artículo resume la evidencia epidemiológica relacionada en la clasificación de los tumores primarios del SNC con marcadores moleculares y biomarcadores de imágenes de RM, apuntando a la importancia del uso de la investigación clínica con el manejo terapéutico.
SUMMARY: The classification of primary tumors of the Central Nervous System (CNS) has its origin in the morphological description whose histopathological analysis has allowed to identify the cell line involved in these tumors and obtain the recognition of certain characteristics of these lesions and their clinical evolution. The molecular study has come to complement the initial diagnosis allowing to recognize entities that are not distinguishable in another way and that have varied the concepts and definitions of various pathological entities modifying the visible horizon of these diseases. The role of Magnetic Resonance (MR) images in the management of intraaxial tumors can be broadly divided into the diagnosis and classification of tumors, treatment planning and subsequent treatment. The present article summarizes the epidemiologic evidence related to the classification of primary tumors of the CNS with molecular markers and MR imaging biomarkers.
Subject(s)
Humans , Magnetic Resonance Imaging , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/diagnostic imaging , Brain Neoplasms/diagnostic imaging , BiomarkersSubject(s)
Humans , Child, Preschool , Child , Adolescent , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/diagnosis , Retinoblastoma/diagnosis , Retinoblastoma/epidemiology , Retinoblastoma/genetics , Retinoblastoma/therapy , Choroid Plexus Neoplasms , Craniopharyngioma , Ependymoma , Glioma , Medulloblastoma , Neoplasms, Germ Cell and Embryonal , Neuroectodermal Tumors, Primitive , PinealomaABSTRACT
Los tumores (Tu) del SNC constituyen la segunda enfermedad oncológica en edad pediátrica, con una incidencia referida aproximada que oscila entre el 10 y 15%. En 309 pacientes con tumores selares y supraselares, seguidos durante 15 años, se evaluó en función de los distintos oncotipos tumorales, síntomas iniciales y alteraciones endocrinológicas previas al inicio del tratamiento. De ellos, 227 pacientes presentaron el tumor a edad prepuberal. Los oncotipos tumorales más frecuentes fueron craneofaringioma (CRA), glioma (GLIA) y tumor de células germinales (GERM). También, se encontró una mayor incidencia de presentación en varones. En edad puberal (n:92), el oncotipo tumoral más frecuente fue adenoma hipofisario (ADENO), seguido de GLIA y CRA. En este ultimo oncotipo tumoral, y, a diferencia del grupo prepuberal, su incidencia fue significativamente mayor en niñas. Aproximadamente 90% de los pacientes tuvieron anormalidades neuro-oftalmológicas (hipertensión craneal, dolores de cabeza, vómitos y pérdida progresiva de la visión) como uno de los signos y/o síntomas iniciales. Alteraciones clínicas endocrinológicas como baja talla, velocidad de crecimiento anormal, diabetes insípida y alteraciones del tempo puberal son frecuentes en estos pacientes y están habitualmente asociadas con las alteraciones clínico-neuro-oftalmológicas como las ya mencionadas. No obstante, la mayoría de los tumores del SNC localizados en la línea media suelen ser diagnosticados por manifestaciones neuro-oftalmológicas. Los resultados del estudio muestran alteración de la función endócrina al diagnóstico del Tu. Se concluye que en todo paciente con crecimiento lento o baja talla, así como también signos clínicos que orienten a un diagnóstico de pubertad precoz y/o retardada, el pediatra debe incluir dentro de los diagnósticos diferenciales, el diagnóstico del tumor selar o supraselar. La morbilidad aumenta frecuentemente luego de la cirugía (AU)
During the last 15 years, 309 patients with tumors of the sellar and suprasellar areas of CNS were followed in our Hospital (Endocrine Service). Tumor oncotype, initial symptoms and endocrine disturbances before any treatment was started are presented. In 227 patients, the tumor was diagnosed at prepubertal age. In this group, the most frequent tumoral oncotypes were craniopharyngioma (CRA), glial tumors (GLIA) and germ cells tumors (GERM). The incidence was higher in boys. At pubertal age (n:92), the most frequent tumoral oncotype was pituitary adenoma (ADENO), followed by GLIA and CRA. In the latter, and different from the prepubertal group, the incidence was significantly higher in girls. Approximately 90% of patients had neuro-ophtalmological abnormalities (cranial hypertension, headaches, vomits, and progressive loss of vision) as one of the initial signs and/or symptoms. Clinical endocrine disorders, such as short stature, low growth velocity, diabetes insipidus, and alterations in pubertal "tempo" are frequent in these patients and are often associated with the neuro-ophtalmological abnormalities mentioned above. This clinical symptomatology has to alert the medical team to discard the presence of a CNS tumor at the sellar and/or suprasellar level. We conclude that tumors of the SNC localized in the midline, have potential capacity to provoke abnormalities in endocrine function. Morbidity is often increased after surgery (AU)
Subject(s)
Humans , Child , Adolescent , Vision Disorders/etiology , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/complications , Central Nervous System Neoplasms/diagnosis , Diabetes Insipidus/etiology , Sella Turcica , Retrospective Studies , Growth Disorders/etiologyABSTRACT
The World Health Organization (WHO) classification of central nervous system (CNS) tumors incorporates morphology, cytogenetics, molecular genetics, and immunologic markers. Despite the relatively large number of CNS tumors with clonal chromosome abnormalities, only few studies have investigated cytogenetic abnormalities for CNS tumors in Korea. Thus, we investigated 119 CNS tumors by conventional G-banded karyotypes to characterize patterns of chromosomal abnormalities involving various CNS tumors, and 92.4% of them were cultured and karyotyped successfully. Totally, 51.8% of karyotypable CNS tumors showed abnormal cytogenetic results, including neuroepithelial tumors (75.0%), meningeal tumors (71.1%), pituitary adenomas (4.2%), schwannomas (44.4%), and metastatic tumors (100.0%). Glioblastomas had hyperdiploid, complex karyotypes, mainly involving chromosomes Y, 1, 2, 6, 7, 10, 12, 13, and 14. Monosomy 22 was observed in 56.4% of meningiomas. There was a significant increase in the frequencies of karyotypic complexity according to the increase of WHO grade between grades I and II (P=0.0422) or IV (P=0.0101). Abnormal karyotypes were more complex at high-grade tumors, suggesting that the karyotype reflects the biologic nature of the tumor. More detailed cytogenetic and molecular characterizations of CNS tumors contribute to better diagnostic criteria and deeper insights of tumorigenesis, eventually resulting in development of novel therapeutic strategies.
Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Asian People/genetics , Central Nervous System Neoplasms/classification , Chromosome Aberrations , Glioblastoma/genetics , Karyotyping , Korea , Meningeal Neoplasms/genetics , Neurilemmoma/genetics , Pituitary Neoplasms/geneticsABSTRACT
The World Health Organization (WHO) classification of central nervous system (CNS) tumors incorporates morphology, cytogenetics, molecular genetics, and immunologic markers. Despite the relatively large number of CNS tumors with clonal chromosome abnormalities, only few studies have investigated cytogenetic abnormalities for CNS tumors in Korea. Thus, we investigated 119 CNS tumors by conventional G-banded karyotypes to characterize patterns of chromosomal abnormalities involving various CNS tumors, and 92.4% of them were cultured and karyotyped successfully. Totally, 51.8% of karyotypable CNS tumors showed abnormal cytogenetic results, including neuroepithelial tumors (75.0%), meningeal tumors (71.1%), pituitary adenomas (4.2%), schwannomas (44.4%), and metastatic tumors (100.0%). Glioblastomas had hyperdiploid, complex karyotypes, mainly involving chromosomes Y, 1, 2, 6, 7, 10, 12, 13, and 14. Monosomy 22 was observed in 56.4% of meningiomas. There was a significant increase in the frequencies of karyotypic complexity according to the increase of WHO grade between grades I and II (P=0.0422) or IV (P=0.0101). Abnormal karyotypes were more complex at high-grade tumors, suggesting that the karyotype reflects the biologic nature of the tumor. More detailed cytogenetic and molecular characterizations of CNS tumors contribute to better diagnostic criteria and deeper insights of tumorigenesis, eventually resulting in development of novel therapeutic strategies.
Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Asian People/genetics , Central Nervous System Neoplasms/classification , Chromosome Aberrations , Glioblastoma/genetics , Karyotyping , Korea , Meningeal Neoplasms/genetics , Neurilemmoma/genetics , Pituitary Neoplasms/geneticsABSTRACT
Tumors of the central nervous system constitute the largest group of solid neoplasms in children and are second only to leukemia in their overall frequency during childhood. The main purpose of the present study is to determine the incidence, age, sex, location and histological diagnosis of CNS tumors in children, less than 15 years of age, in the Mofid Children's Hospital, in the past 10 years. In this descriptive retrospective study we reviewed the medical records of 143 children with diagnosis of CNS tumors admitted during the past 10 years in neurology and surgery departments of Mofid Children's Hospital between the years 1996 and 2006. During the 10 year study period, CNS tumor was diagnosed in 143 patients; of these tumors, 119 were intracranial and 58 were intraspinal; 51.3% of brain tumors were located in the supratentorial and 48.7% in the infratentorial regions. The most common intracranial neoplasms were astrocytic tumors [36.8%], embryonal tumors [31.1%] and ependymal tumors [13.4%]. Of the intraspinal neoplasms the most frequently noted were embryonal tumors [37.5%], mesenchymal meningothelial tumors [20.8%], followed by astrocytic tumors [16.7%]. The median age at diagnosis was 8.9 +/- 4.1 years with a male to female ratio of 1.4:1 [P<0.5]. The most common intracranial astrocytic and embryonal neoplasms were pilocytic astrocytoma and medulloblastoma/ PNET respectively. Brain tumors in children constitute a diverse group in terms of incidence, distribution and histopathological diagnosis
Subject(s)
Humans , Male , Female , Central Nervous System Neoplasms/pathology , Child , Retrospective Studies , Central Nervous System Neoplasms/classification , Data CollectionABSTRACT
True cysts of the central nervous system (CNS) are rare lesions. A retrospective study of patients with symptomatic non-neoplastic cystic lesions of CNS operated in the Department of Neurosurgery, G.B. Pant Hospital, New Delhi between Jan 1994 and Feb 2001 was conducted. Parasitic cysts, cystic transformation of hemmorhages, vascular malformations and cystic tumours were excluded from the study. A total of 109 cases were reviewed. There were 34, 27, 17, 16, 8, 3 and 2 cases of epidermoid, arachnoid, dermoid, colloid, neurenteric, Rathke's and ependymal cysts and 1 case each of choroid plexus and glial cysts. The clinical presentations, locations, incidence and pathogenesis of these cysts is discussed.
Subject(s)
Central Nervous System Neoplasms/classification , Cysts/classification , Humans , Immunohistochemistry , Retrospective StudiesABSTRACT
Estudio clínico patológico de 187 casos de tumores del sistema nervioso central en niños atendidos en el INEN en los niños 1987 - 1997 de un total de 250 niños, que representan el 27.5 por ciento de número total de 924 pacientes con tumores cerebrales. 84 tumores supratentoriales representan el 44.9 por ciento y 101 tumores infratentoriales representan el 54.1 por ciento. 68 tumores fueron astrocitomas, 56 meduloblastomas, 25 ependimomas, 18 craniofaringiomas, 9 sarcomas, 3 oligodendrogliomas, 2 gliomas mixtos, 2 tumores neuroectodermales primitivos, 2 neuroblastomas, 2 tumores del plexo coroideo, 1 ganglioglioma, 1 teratoma benigno, 1 germinoma, 1 pineoblastoma. Los tumores supratentoriales más frecuentes en niños son los tumores astrociticos, predominando los astrocitomas pilocíticos, seguido de los craniofaringiomas. Los tumores infratentoriales más frecuentes son los meduloblastomas seguido de los astrocitomas fundamentalmente la variedad pilocitica.
Subject(s)
Humans , Male , Female , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/pathology , Hospitals, StateABSTRACT
El OBJETIVO de nuestro trabajo es comparar 2 métodos diagnósticos, y demostrar las semejanzas de las distintas Clasificaciones de los Tumores Gliales del Sistema Nervioso Central. Se realizaron técnicas de inmunomarcación para proteína gliofibrilar ácida (GFAP), e impregnaciones argénticas especiales para confrontar los hallazgos según las últimas interpretaciones histogenéticas. Estudiamos 95 Gliomas con hematoxilina-eosina y técnicas argénticas, y en 58 casos tomados al azar realizamos además la técnica para GFAP. Con ambos métodos obtuvimos resultados iguales en el diagnóstico de tumores gliales cuyas células poseen gliofibrillas: Glioepiteliomas (Ependimonas), Glioblastomas, Astroblastomas y Astrocitomas. En los Oligodendrogliomas cuyas células no poseen gliofibrillas pero sí microtúbulos, las técnicas argénticas marcaron estas células y sus prolongaciones, no así la técnica para GFAP. Hay semejanzas al comparar la Clasificación de Del Río Horttega-Polak con la de la OMS y la de otros autores, en lo referente a los Gliomas. Sólo hay diferencias en la nomenclatura de algunos tumores o en su interpretación histogenética, que no son sustanciales y están limitados, que no son sustanciles y están limitados al "Espongioblastoma Polar" y al "Gliosarcoma". Por lo tanto, creemos que la revalorización de la Clasificación de Del Río Hortega-Polak está justificada.
Subject(s)
Humans , Central Nervous System Neoplasms/pathology , Glioma/pathology , Brain Neoplasms/classification , Brain Neoplasms/pathology , Central Nervous System Neoplasms/classification , Glioma/classification , Silver Staining , Spinal Cord Neoplasms/classification , Spinal Cord Neoplasms/pathologyABSTRACT
Analizamos retrospectivamente la frecuencia de tumores neuroepiteliales en el Servicio de Neurocirugía del Hospital Carlos Andrade Marín en el período comprendido entre 1981 y 1990. Identificamos 69 gliomas (21por ciento) entre 327 tumores. la incidencia correspondió a 7 nuevos casos anuales. La edad media fue 40 años, con un rango entre 2 y 73 años. la relación hombre mujer estuvo en 1,76/1. Los tumores más frecuentes fueron los astrocíticos (42.7 por ciento, glioblastomas (26 por ciento) y los oligodendrogliomas y meduloblastomas (14,5 por ciento cada uno). La localizacíon supratentorial fue la más común (62,3 por ciento) representado principalmente por astrocitomas, y en la localización infratentorial, el meduloblastoma fue el tumor más frecuente.