Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical, and deoxyribonucleic acid analyses in a pediatric series.
Overtreatment by radiotherapy and/or chemotherapy for central nervous system tumors in infancy and childhood may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. The dysembryoplastic neuroepithelial tumor is a complex multinodular lesion consisting of glial nodules, associated with a specific glioneuronal element and/or with focal cortical dysplasia, and occurring in young patients presenting with intractable, mostly complex partial, seizures without neurological deterioration. We report on 14 patients; 9 were from a series of 600 pediatric patients with intracranial central nervous system tumors studied at a single institution from 1988 to 1993, and 5 were referred from other pediatric hospitals. Six tumors were frontal, six were temporal, one was parietal, and one was occipitoparietal. Computed tomographic scans disclosed hypodense lesions with cystic appearances in 4 patients and slight focal postcontrast enhancements in only 2 patients, whereas magnetic resonance imaging, available for 7 of 14 patients, showed hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images. Deformities of the overlying cranium were also observed in five patients. The age range at the time of surgery (excluding a 20-year-old male patient who underwent surgery at the main pediatric hospital) was 2.6 to 13 years, with a mean of 6.68 years. The male to female patient ratio was 10:4, and the duration of symptoms was 0.2 to 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)
Taratuto
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AL
Instituto de Investigaciones Neurológicas Raúl Carrea, Fundacion para la Lucha contra las Enfermedades Neurológicas de la Infancia, Buenos Aires, Argentina.
Pomata
H
H
Sevlever
G
G
Gallo
G
G
Monges
J
J
D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004273:DNA, Neoplasm / Q000032:analysis*; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008279:Magnetic Resonance Imaging; D008297:Male; D009419:Nerve Tissue Proteins / Q000032:analysis*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000737:chemistry / Q000175:diagnosis* / Q000601:surgery; D011003:Ploidies; D014057:Tomography, X-Ray Computed
D004273:DNA, Neoplasm; D009419:Nerve Tissue Proteins