Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. 2003

Alper I Dai, and James W Backstrom, and Peter C Burger, and Patricia K Duffner
West Virginia University Children's Hospital, Department of Pediatrics, Morgantown, West Virginia, USA.

One hundred ninety-eight children were entered on POG 8633, "Prolonged Postoperative Chemotherapy and Delayed Radiation for Children <3 years of age with Malignant Brain Tumors" (1986-1990). Thirteen manifested supratentorial nonpineoblastoma primitive neuroectodermal tumors, making this the second most common supratentorial tumor in the study. Symptoms and signs included seizures, nausea, vomiting, lethargy, irritability, headache, focal motor weakness, and increased head circumference. Twelve of 13 had symptoms for less than 1 month before diagnosis. The average tumor size was 5.96 cm (+/- 0.37) x 5 cm (+/- 0.28) x 5.15 cm (+/- 0.31). Eight tumors were predominantly hemispheral, and five were midline. Computed tomographic scans on nine patients revealed tumor hyperdensity (nine), midline shift (eight), hydrocephalus (seven), cysts (six), well-defined borders (five), and calcification (four). No patients exhibited peritumoral edema. Contrast enhancement, primarily heterogeneous, was present in all patients. Magnetic resonance imaging appearance of the tumor (six patients) demonstrated midline shift (four), well-defined margins (four), necrosis (two), cysts (three), and hemorrhage (two). No peritumoral edema was present. Most enhanced heterogeneously. The diagnosis of supratentorial nonpineoblastoma primitive neuroectodermal tumors should be suspected when a large, sharply marginated, hyperdense supratentorial mass is observed in a young child, particularly when no peritumoral edema is present.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D015173 Supratentorial Neoplasms Primary and metastatic (secondary) tumors of the brain located above the tentorium cerebelli, a fold of dura mater separating the CEREBELLUM and BRAIN STEM from the cerebral hemispheres and DIENCEPHALON (i.e., THALAMUS and HYPOTHALAMUS and related structures). In adults, primary neoplasms tend to arise in the supratentorial compartment, whereas in children they occur more frequently in the infratentorial space. Clinical manifestations vary with the location of the lesion, but SEIZURES; APHASIA; HEMIANOPSIA; hemiparesis; and sensory deficits are relatively common features. Metastatic supratentorial neoplasms are frequently multiple at the time of presentation. Cancer, Supratentorial,Benign Supratentorial Neoplasms,Malignant Supratentorial Neoplasms,Neoplasms, Supratentorial,Primary Supratentorial Neoplasms,Supratentorial Neoplasms, Benign,Supratentorial Neoplasms, Malignant,Supratentorial Neoplasms, Primary,Supratentorial Tumors,Benign Supratentorial Neoplasm,Cancers, Supratentorial,Malignant Supratentorial Neoplasm,Neoplasm, Benign Supratentorial,Neoplasm, Malignant Supratentorial,Neoplasm, Primary Supratentorial,Neoplasm, Supratentorial,Neoplasms, Benign Supratentorial,Neoplasms, Malignant Supratentorial,Neoplasms, Primary Supratentorial,Primary Supratentorial Neoplasm,Supratentorial Cancer,Supratentorial Cancers,Supratentorial Neoplasm,Supratentorial Neoplasm, Benign,Supratentorial Neoplasm, Malignant,Supratentorial Neoplasm, Primary,Supratentorial Tumor,Tumor, Supratentorial,Tumors, Supratentorial
D018242 Neuroectodermal Tumors, Primitive A group of malignant tumors of the nervous system that feature primitive cells with elements of neuronal and/or glial differentiation. Use of this term is limited by some authors to central nervous system tumors and others include neoplasms of similar origin which arise extracranially (i.e., NEUROECTODERMAL TUMORS, PRIMITIVE, PERIPHERAL). This term is also occasionally used as a synonym for MEDULLOBLASTOMA. In general, these tumors arise in the first decade of life and tend to be highly malignant. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2059) Ependymoblastoma,Medulloepithelioma,Neuroepithelial Tumors, Primitive,PNET,Spongioblastoma,Cerebral Primitive Neuroectodermal Tumor,Neoplasms, Primitive Neuroepithelial,Neuroectodermal Tumor, Primitive,Neuroepithelial Neoplasms, Primitive,Primitive Neuroepithelial Neoplasms,Ependymoblastomas,Medulloepitheliomas,Neoplasm, Primitive Neuroepithelial,Neuroepithelial Neoplasm, Primitive,Neuroepithelial Tumor, Primitive,PNETs,Primitive Neuroectodermal Tumor,Primitive Neuroectodermal Tumors,Primitive Neuroepithelial Neoplasm,Primitive Neuroepithelial Tumor,Primitive Neuroepithelial Tumors,Spongioblastomas,Tumor, Primitive Neuroectodermal,Tumor, Primitive Neuroepithelial,Tumors, Primitive Neuroectodermal,Tumors, Primitive Neuroepithelial

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