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Neuroectodermal Tumors, PrimitiveNeuroectodermal TumorsNeuroectodermal Tumors, Primitive, PeripheralNeoplasms, Multiple PrimaryNeoplasms, Second PrimaryNeuroectodermal Tumor, MelanoticBrain NeoplasmsSarcoma, EwingMedulloblastomaCerebellar NeoplasmsSupratentorial NeoplasmsProto-Oncogene Protein c-fli-1RNA-Binding Protein EWSNeoplasms, Unknown PrimaryPinealomaChromosomes, Human, Pair 22EpendymomaSpinal Cord NeoplasmsBone NeoplasmsMaxillary NeoplasmsSarcoma, Small CellNeuroblastomaRhabdoid TumorTumor Markers, BiologicalNeoplasms, Germ Cell and EmbryonalThoracic NeoplasmsCentral Nervous System NeoplasmsImmunohistochemistryChromosomes, Human, Pair 11AstrocytomaOrbital NeoplasmsNeural PlateOncogene Proteins, FusionTomography, X-Ray ComputedFatal OutcomeVincristineNeoplasmsSkull NeoplasmsIfosfamideCell Line, TumorTranslocation, GeneticDNA, NeoplasmGanglioneuromaAntigens, NeoplasmPeripheral Nervous System NeoplasmsParaparesisThoracic WallNeoplasms, Nerve TissueTeratomaAbdominal NeoplasmsSoft Tissue NeoplasmsPancreatic NeoplasmsCombined Modality TherapyMagnetic Resonance ImagingRhabdomyosarcomaEtoposideGliomaTumor Necrosis Factor-alphaTumor BurdenChromosomes, Human, Pair 17Pineal GlandRetinoblastomaTumor Cells, CulturedNeurofilament ProteinsNeoplasm ProteinsCranial IrradiationThoracic Surgical ProceduresNeoplasms, ExperimentalCyclophosphamideIn Situ Hybridization, FluorescenceNeoplasm Recurrence, LocalSynaptophysinPhosphopyruvate HydrataseGangliosidesTreatment OutcomeGenes, mycLomustineAntineoplastic Combined Chemotherapy ProtocolsReverse Transcriptase Polymerase Chain ReactionCell Transformation, NeoplasticPolymerase Chain ReactionWilms TumorImmunoenzyme TechniquesCell DivisionRNA, MessengerTranscription FactorsSkin NeoplasmsGene Expression Regulation, NeoplasticAntibodies, MonoclonalCell DifferentiationThiotepaEctodermBiopsy, NeedleGene AmplificationGenes, Tumor SuppressorPrognosisBiopsyCarcinoid TumorDisease ProgressionKidney Neoplasms
InfancyMalignantBenignNeuroblastomaCarcinoidMetastaticEpithelialAdenomaGermEmbryonalMalignancyMNTIPediatricMedulloblastomaPathologicalHumansFibrousMutationsImmunohistochemistryTissuesChildhoodDiagnosisAdultFluidsRareAbnormalitiesBrainDiameterPatientsEvaluationCancerSectionsIncludeChildrenCellsComprehensiveRateExact
Infancy12
  • Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants. (medscape.com)
  • Some of the terms applied to this lesion included pigmented ameloblastoma, retinal anlage tumor, melanotic progonoma, melanotic adamantinoma, and pigmented epulis of infancy. (medscape.com)
  • In 1966, Borello and Gorlin reported a case with high urinary excretion of vanillylmandelic acid (VMA), suggesting a neural crest origin, and they proposed the term melanotic neuroectodermal tumor of infancy. (medscape.com)
  • [ 6 ] Since then, numerous histochemical, immunohistochemical, electron microscopic, and tissue culture studies have supported the neural crest origin and confirmed the preferred term of melanotic neuroectodermal tumor of infancy. (medscape.com)
  • Several patients with melanotic neuroectodermal tumor of infancy (MNTI) have demonstrated a high urinary excretion of VMA. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm and approximately 485 cases have been reported in the literature to date worldwide. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy (MNTI) has a slight male predilection, with a male-to-female ratio of 1.3:1. (medscape.com)
  • [ 14 ] A few cases of melanotic neuroectodermal tumor of infancy (MNTI) have been reported in adults, notably, a 23-year-old man, a 39-year-old woman, and a 67-year-old woman. (medscape.com)
  • Most melanotic neuroectodermal tumors of infancy (MNTIs) are benign and effectively managed by aggressive surgical excision. (medscape.com)
  • A germline mutation of CDKN2A and a novel RPLP1-C19MC fusion detected in a rare melanotic neuroectodermal tumor of infancy: a case report. (ox.ac.uk)
  • BACKGROUND: Melanotic neuroectodermal tumor of infancy (MNTI) is exceptionally rare and occurs predominantly in the head and neck (92.8 % cases). (ox.ac.uk)
  • CONCLUSIONS: In the absence of somatic copy number variations or mutations, the fully transformed phenotype of the MNTI may have arisen in infancy because of the combined effects of a germline CDKN2A mutation, tumor promoting somatic fusion genes and epigenetic deregulation. (ox.ac.uk)
Malignant7
  • While most tumors metastatic to the serous membranes are of epithelial origin, cytologists should be aware that non-epithelial neoplasms can also cause malignant effusions including sarcomas, melanomas, germ cell tumors, and, more rarely, brain tumors. (cytojournal.com)
  • The differential diagnosis of a malignant effusion is accordingly broad, especially for the small round blue cell tumors that includes not only mesenchymal tumors, but also non-mesenchymal tumors, such as neuroblastoma and Wilms tumor. (cytojournal.com)
  • As malignant cells have a tendency to round up in body fluids these non-epithelial neoplasms can therefore mimic reactive mesothelial cells and metastatic adenocarcinoma. (cytojournal.com)
  • Common non-epithelial neoplasms that may cause malignant effusions include malignant melanoma, sarcomas, and other neoplasms including germ cell tumors [ Figure 1 ]. (cytojournal.com)
  • 4 Malignant effusions caused by non-epithelial neoplasms are more frequently encountered in children than in adults. (cytojournal.com)
  • A benign or malignant neoplasm that arises from the brain or the spinal cord. (beds.ac.uk)
  • Infratentorial primitive neuroectodermal tumor that is, by definition, malignant and invasive and thus classified as Grave IV by the WHO. (uchicago.edu)
Benign1
  • more precisely, a benign epithelial neoplasm consisting of villous or arborescent outgrowths of fibrovascular stroma covered by neoplastic cells. (lookformedical.com)
Neuroblastoma1
Carcinoid1
  • These include some fibrosarcomas and neurosarcomas and occasional carcinoid tumors. (medscape.com)
Metastatic2
  • Most tumors metastatic to the serous membranes are of epithelial origin. (cytojournal.com)
  • 1.5 cm diameter) residual tumor by postoperatively imaging, and no evidence of metastatic spread within the neuraxis. (uchicago.edu)
Epithelial3
  • Diagnosing non-epithelial malignancies in effusion specimens based entirely upon their cytomorphologic features is difficult because these neoplasms often exhibit considerable morphological overlap and their cytomorphology can differ from the original tumor. (cytojournal.com)
  • The classification of epithelial tumors of the kidney has in particular undergone substantial progress in the last two decades with major contributions from genetic typing of renal cell carcinomas (RCCs). (abdominalkey.com)
  • This is followed by coverage of neoplasms in the more traditional categories of epithelial, mesenchymal, and other categories. (abdominalkey.com)
Adenoma1
Germ1
  • The peak incidence is in the third decade of life for non-seminomatous germ cell tumour (NSGCT) and mixed GCT patients, and in the fourth decade for seminoma testis (ST) patients. (uroweb.org)
Embryonal1
  • Multiple chromosomal translocations were identified by RNA-Seq, and fusion genes included RPLP1-C19MC, potentially deregulating the C19MC cluster, an imprinted locus containing microRNA genes reactivated by gene fusion in embryonal tumors with multilayered rosettes. (ox.ac.uk)
Malignancy1
  • Note: In children, brain tumors are the most common solid tumor and the second most common malignancy (after leukemia). (uchicago.edu)
MNTI2
  • A comprehensive systematic review of 472 cases of MNTI found that age at diagnosis is an important prognostic indicator in these tumors. (medscape.com)
  • Very little is known about the etiology of MNTI and this report advances knowledge of these rare tumors by providing the first comprehensive genomic, transcriptomic and epigenetic characterization of a case. (ox.ac.uk)
Pediatric2
  • The first section will cover those tumors that characteristically are associated with the pediatric population. (abdominalkey.com)
  • Medulloblatoma is the most common posterior fossa (i.e. infratentorial) tumor in children (although some sources will state that it is the second most common behind cerebellar astrocytoma), making up approximately 18% of all primary pediatric brain tumors. (uchicago.edu)
Medulloblastoma2
  • 1 - 3 Intracranial brain tumors, most commonly medulloblastoma [ Figure 2 ], can metastasize to the peritoneal cavity via ventriculo-peritoneal shunts used to divert excess cerebrospinal fluid. (cytojournal.com)
  • Medulloblastoma is a highly radiosensitive tumor. (uchicago.edu)
Pathological1
  • Exhaustive genomic, transcriptomic, epigenetic and pathological characterization was performed on the excised primary tumor and a derived cell line. (ox.ac.uk)
Humans1
Fibrous1
  • brown t. a mass of fibrous tissue containing hemosiderin-pigmented macrophages and multinucleated giant cells, replacing and expanding part of a bone in primary hyperparathyroidism . (en-academic.com)
Mutations2
  • Whole-exome analysis of genomic DNA from both the tumor and blood indicated no somatic, non-synonymous coding mutations within the tumor, but a heterozygous, unique germline, loss of function mutation in CDKN2A (p16(INK4A), D74A). (ox.ac.uk)
  • The development of Wilms tumor has been linked to mutations of the WT1 and WT2 genes located on chromosome 11 at 11p13 and 11p15.5, respectively ( 8 , 9 ). (abdominalkey.com)
Immunohistochemistry1
  • The role of genotyping and ancillary tools, in particular immunohistochemistry, in correctly classifying these tumors is highlighted in the section on Renal Cell Carcinoma. (abdominalkey.com)
Tissues2
  • Wilms tumor is believed to arise from embryonic tissues called nephrogenic rests that fail to undergo normal involution ( 7 ). (abdominalkey.com)
  • The tumor is usually circumscribed by a pseudocapsule formed of compressed renal and perirenal tissues. (abdominalkey.com)
Childhood4
  • More than 80% of renal tumors of childhood are Wilms tumor (nephroblastoma) ( 1 , 2 ). (abdominalkey.com)
  • Although the mortality rate for children with brain tumors has decreased significantly over the last 40 years, deaths from childhood brain tumors are the highest among all childhood cancer deaths. (uchicago.edu)
  • Long-term sequelae of childhood brain tumors are often from the effects of chemotherapy and/or radiation therapy. (uchicago.edu)
  • 1. Crawford, J. Childhood Brain Tumors. (uchicago.edu)
Diagnosis1
  • Depends on the size, subtype, and dissemination of the tumor at the time of diagnosis. (uchicago.edu)
Adult1
  • Testicular cancer represents 1% of adult neoplasms and 5% of urological tumours, with three to ten new cases per 100,000 males/per year in Western societies [ 6 ]. (uroweb.org)
Fluids1
  • Examination of cells whether from a primary or secondary site, including fluids aspirated using endoscopes or needles. (who.int)
Rare2
Abnormalities1
  • Wiedemann-Beckwith syndrome is related to abnormalities on chromosome 11p15 and characterized by multiple craniofacial anomalies, abdominal wall defects, and tumors of the genitourinary tract, liver, adrenal gland, and central nervous system among other abnormalities. (abdominalkey.com)
Brain3
  • The Utility of Whole Body Imaging in the Evaluation of Solitary Brain Tumors. (beds.ac.uk)
  • Approximately 60% of brain tumors are infratentorial, 25% are supratentorial, and 15% arise in the midline. (uchicago.edu)
  • There is also significant neurological morbidity associated with brain tumors. (uchicago.edu)
Diameter1
  • Wilms tumor often is greater than 5 cm in diameter, with an average size of 10 cm ( 3 ). (abdominalkey.com)
Patients4
  • Most patients, by some estimates more than 90%, present with the tumor in the first year of life, usually from age 1-6 months, with a peak between the second and sixth month of life. (medscape.com)
  • Patients with Beckwith-Wiedemann syndrome and Denys-Drash syndrome have an increased risk of developing Wilms tumor ( 5 , 6 ). (abdominalkey.com)
  • In 5% of GCT patients, the primary site is at an extragonadal location [ 9 ]. (uroweb.org)
  • This number is even higher for patients who have tumors with favorable molecular phenotypes. (uchicago.edu)
Evaluation1
  • CT scan is a routine part of the diagnostic evaluation of mediastinal tumors, cysts, and other masses. (medscape.com)
Cancer1
Sections1
  • Specific discussions of etiology and pathogenesis are dealt with in each of the sections on individual tumors, rather than as a freestanding section, as is used elsewhere in this text. (abdominalkey.com)
Include2
  • To include biochemical and/or immunological markers which are specific for a tumour site (Table 2). (who.int)
  • Additional risk factors include a family history of TC among first-degree relatives and the presence of a contralateral testicular tumour or GCNIS [ 15-22 ]. (uroweb.org)
Children1
Cells1
  • A deoxyribonucleotide polymer that is the primary genetic material of all cells. (lookformedical.com)
Comprehensive1
  • The purpose of this chapter is to familiarize the reader with the tumor types encountered in the human rather than to provide a comprehensive diagnostic reference, which is better handled in more comprehensive textbooks and monographs. (abdominalkey.com)
Rate1
  • Agents that reduce the frequency or rate of spontaneous or induced tumors independently of the mechanism involved. (lookformedical.com)
Exact1
  • CT scan images can greatly assist in determining the exact location of the mediastinal tumor and in determining its relationship to adjacent structures. (medscape.com)