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Neuroectodermal Tumors, PrimitiveNeuroectodermal TumorsNeuroectodermal Tumors, Primitive, PeripheralNeuroectodermal Tumor, MelanoticSarcoma, EwingMedulloblastomaCerebellar NeoplasmsBrain NeoplasmsSupratentorial NeoplasmsProto-Oncogene Protein c-fli-1RNA-Binding Protein EWSTumor Markers, BiologicalPinealomaEpendymomaChromosomes, Human, Pair 22Bone NeoplasmsNeuroblastomaCell Line, TumorRhabdoid TumorSpinal Cord NeoplasmsSarcoma, Small CellMaxillary NeoplasmsTumor BurdenNeoplasms, Germ Cell and EmbryonalTumor Necrosis Factor-alphaCentral Nervous System NeoplasmsThoracic NeoplasmsImmunohistochemistryAstrocytomaChromosomes, Human, Pair 11Wilms TumorNeoplasms, ExperimentalGenes, Tumor SuppressorTomography, X-Ray ComputedNeoplasmsOncogene Proteins, FusionAntigens, NeoplasmNeural PlateGliomaDNA, NeoplasmOrbital NeoplasmsTumor Suppressor Protein p53Fatal OutcomeCarcinoid TumorVincristineIfosfamideTranslocation, GeneticNeoplasm ProteinsCombined Modality TherapySkull NeoplasmsTeratomaNeuroendocrine TumorsCell Transformation, NeoplasticRhabdomyosarcomaTumor Suppressor ProteinsMagnetic Resonance ImagingReverse Transcriptase Polymerase Chain ReactionCell DivisionThoracic WallGene Expression Regulation, NeoplasticRNA, MessengerGanglioneuromaParaparesisNeoplasms, Nerve TissueChromosomes, Human, Pair 17Treatment OutcomeRetinoblastomaTumor MicroenvironmentEtoposideAntibodies, MonoclonalTumor Cells, CulturedSoft Tissue NeoplasmsMammary Neoplasms, ExperimentalNeoplasm MetastasisIn Situ Hybridization, FluorescenceNeoplasm Recurrence, LocalAdenocarcinomaNeovascularization, PathologicAntineoplastic Combined Chemotherapy ProtocolsPolymerase Chain ReactionImmunoenzyme TechniquesAbdominal NeoplasmsPeripheral Nervous System NeoplasmsPineal GlandCyclophosphamideDisease ProgressionCell ProliferationTransplantation, HeterologousTranscription FactorsCranial IrradiationNeurofilament ProteinsGenes, mycMolecular Sequence DataThoracic Surgical ProceduresGene AmplificationBase SequenceSurvival AnalysisPrognosisXenograft Model Antitumor AssaysCell Line
MalignantInfancyPrimitiveNeuroblastomaEpithelialBenignMetastaticEmbryonalSchwannomaNeuroendocrine tumorsEwingMorphologicRetinalPrognosisPrimary tumorNeuralMelanocyticSolitaryPediatricImmunohistochemistryMedulloblastomaCraniospinalResidualOccursCarcinomaMutationsLeukemiaChildhoodClinicalTissuesCancerDiagnosisAriseConsistentCellAbnormalitiesPatientsExtremelyCellsRareDiameterBrainCase report
Malignant9
  • Moreover, an estimated malignant transformation rate of 6.5% highlights the possibly serious nature of this tumor and the need for careful clinical evaluation and close follow-up of affected patients, especially for tumors located in the brain and skull. (medscape.com)
  • Additionally, a malignancy rate of 6.5% has been reported for these tumors, with most malignant cases occurring in the skull and brain. (medscape.com)
  • Malignant melanotic neuroectodermal tumor of infancy: a clinical, pathologic, ultrastructural and tissue culture study. (medscape.com)
  • Malignant melanotic neuroectodermal tumor: light and electron microscopic study. (medscape.com)
  • Of note, the benign nerve sheath tumors are classified as World Health Organization (WHO) grade I on the basis of their benign cytologic features, in contrast to the malignant counterparts, which are WHO grade III or IV. (medscape.com)
  • 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)
  • Common non-epithelial neoplasms that may cause malignant effusions include malignant melanoma, sarcomas, and other neoplasms including germ cell tumors [ Figure 1 ]. (cytojournal.com)
  • Infratentorial primitive neuroectodermal tumor that is, by definition, malignant and invasive and thus classified as Grave IV by the WHO. (uchicago.edu)
Infancy11
  • Melanotic neuroectodermal tumor of infancy is a very rare oral cavity tumor that is seen in patients usually at or around birth. (wikipedia.org)
  • 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)
  • Address the documented 20% recurrence rate of melanotic neuroectodermal tumor of infancy (MNTI) in the postoperative care by monitoring the patient with physical and radiographic examination at monthly intervals for the first two postoperative years. (medscape.com)
  • Most melanotic neuroectodermal tumors of infancy (MNTIs) are benign and effectively managed by aggressive surgical excision. (medscape.com)
  • Neville B, Damm D, Allen C. Melanotic Neuroectodermal Tumor of Infancy. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy--a neoplasm of neural crese origin. (medscape.com)
  • Cutler LS, Chaudhry AP, Topazian R. Melanotic neuroectodermal tumor of infancy: an ultrastructural study, literature review, and reevaluation. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy: 2 decades of clinical experience with 18 patients. (medscape.com)
  • Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J. Melanotic neuroectodermal tumor of infancy: systematic review of the literature and presentation of a case. (medscape.com)
Primitive1
  • It is important in this age group to exclude other tumors that can have a similar appearance, such as rhabdomyosarcoma, lymphoma, Ewing sarcoma (primitive neuroectodermal tumor), or even a melanoma (although they are very very rare in infants). (wikipedia.org)
Neuroblastoma4
  • Other rare non-epithelial malignancies that may be encountered in effusion cytology include neuroblastoma, Wilms tumor, and metastatic brain tumors. (cytojournal.com)
  • Vanillylmandelic acid (VMA), a metabolic by-product of norepinephrine and epinephrine, can be used to detect neuroblastoma and other tumors of neural crest origin. (medscape.com)
  • Elevated metabolic by-products of catecholamines-dihydroxyphenylalanine (DOPA), dopamine, norepinephrine, and epinephrine-can be detected in the urine of patients with neuroblastoma and other tumors of neural crest origin. (medscape.com)
  • Ninety percent of neuroblastoma tumors secrete these by-products. (medscape.com)
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)
  • Most tumors metastatic to the serous membranes are of epithelial origin. (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)
Benign2
  • Even though there is often a very dramatic and rapidly enlarging, destructive tumor, there is usually a benign clinical course after complete local excision (usually by partial maxillectomy) with clear or free surgical margins. (wikipedia.org)
  • Along with neurofibroma, schwannoma constitutes one of the two most common benign peripheral nerve sheath tumors. (medscape.com)
Metastatic2
  • Systemic Therapy for Tumor Control in Metastatic Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline. (harvard.edu)
  • 1.5 cm diameter) residual tumor by postoperatively imaging, and no evidence of metastatic spread within the neuraxis. (uchicago.edu)
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)
Schwannoma1
  • [ 12 ] There have also been reports of nerve sheath tumors exhibiting histologic hybrid features of schwannoma and soft-tissue perineurioma. (medscape.com)
Neuroendocrine tumors6
  • Neuroendocrine Tumors" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
  • Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition. (harvard.edu)
  • This graph shows the total number of publications written about "Neuroendocrine Tumors" by people in Harvard Catalyst Profiles by year, and whether "Neuroendocrine Tumors" was a major or minor topic of these publication. (harvard.edu)
  • Below are the most recent publications written about "Neuroendocrine Tumors" by people in Profiles. (harvard.edu)
  • Intertumoral lineage diversity and immunosuppressive transcriptional programs in well-differentiated gastroenteropancreatic neuroendocrine tumors. (harvard.edu)
  • Genome-Wide Association Study Identifies 4 Novel Risk Loci for Small Intestinal Neuroendocrine Tumors Including a Missense Mutation in LGR5. (harvard.edu)
Ewing3
Morphologic1
  • Conclusions: The morphologic subtypes of EBV-related LELC have different tumor immune characteristics. (bvsalud.org)
Retinal2
Prognosis1
  • A low ratio of vanillylmandelic acid to HVA is consistent with a poorly differentiated tumor and indicates a poor prognosis. (medscape.com)
Primary tumor1
  • Exhaustive genomic, transcriptomic, epigenetic and pathological characterization was performed on the excised primary tumor and a derived cell line. (ox.ac.uk)
Neural2
  • It is thought to be derived from neural crest, which is one of the embryologic tissue types. (wikipedia.org)
  • It is generally accepted that in embryogenesis, the Schwann cells are derived from the neural crest and are of neuroectodermal origin. (medscape.com)
Melanocytic1
  • These melanocytic cells are of neuroectodermal origin, and melanocytic tumors may arise from these cells. (medscape.com)
Solitary1
  • The Utility of Whole Body Imaging in the Evaluation of Solitary Brain Tumors. (beds.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)
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)
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)
Craniospinal1
  • The majority of craniospinal tumors are of neuroepithelial origin. (nih.gov)
Residual1
  • In rare cases, chemotherapy may be used for recurrent or residual tumors. (wikipedia.org)
Occurs1
Carcinoma1
  • Objective: To investigate the association between the distribution of tumor infiltrating lymphocytes (TIL) in EBV associated lymphoepitheliomatoid carcinoma (LELC) and the pathological subtypes of LELC, as well as the clinical significance of TIL distribution. (bvsalud.org)
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)
Leukemia1
  • Note: In children, brain tumors are the most common solid tumor and the second most common malignancy (after leukemia). (uchicago.edu)
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)
Clinical1
  • Methods: The LELC patients with sufficient tumor tissues, complete clinical data and positive EBER, who visited Zhejiang Cancer Hospital, Hangzhou, China from January 2006 to October 2018, were selected. (bvsalud.org)
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)
Cancer2
Diagnosis2
  • The presence of a noninvasive tumor next to a peripheral nerve suggests the diagnosis of neurilemmoma. (medscape.com)
  • Depends on the size, subtype, and dissemination of the tumor at the time of diagnosis. (uchicago.edu)
Arise2
  • A diverse array of tumors can arise in the human kidney. (abdominalkey.com)
  • Approximately 60% of brain tumors are infratentorial, 25% are supratentorial, and 15% arise in the midline. (uchicago.edu)
Consistent1
  • Consistent with the melanotic phenotype of the tumor, elevated expression of tyrosinase was observed. (ox.ac.uk)
Cell1
  • There are two cell types present (biphasic), arranged in alveolar or tubular configurations. (wikipedia.org)
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)
Patients2
  • Patients with Beckwith-Wiedemann syndrome and Denys-Drash syndrome have an increased risk of developing Wilms tumor ( 5 , 6 ). (abdominalkey.com)
  • This number is even higher for patients who have tumors with favorable molecular phenotypes. (uchicago.edu)
Extremely1
  • This tumor is extremely rare, with fewer than 500 cases reported world wide. (wikipedia.org)
Cells1
  • Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. (harvard.edu)
Rare3
  • 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)
  • Neonatal Wilms tumor is rare. (abdominalkey.com)
  • Wilms tumor is rare in adults ( 3 ). (abdominalkey.com)
Diameter1
  • Wilms tumor often is greater than 5 cm in diameter, with an average size of 10 cm ( 3 ). (abdominalkey.com)
Brain1
  • There is also significant neurological morbidity associated with brain tumors. (uchicago.edu)
Case report1
  • Atwal D, Joshi KP, Jeffus S, Ntambi J, Mahmoud F. Well-Differentiated Neuroendocrine Tumor-A Low b-Grade Tumor's Aggressive Course and Dismal Outcome: A Case Report. (uams.edu)