Search results for "Peripheral Primitive Neuroectodermal Tumor"

showing 3 items of 3 documents

Atypical Pleomorphic Extraosseous Ewing Tumor/Peripheral Primitive Neuroectodermal Tumor with Unusual Phenotypic/Genotypic Profile

2002

A pleomorphic undifferentiated tumor primarily located in the retroperitoneum with a phenotype compatible with an extraosseous Ewing tumor/peripheral primitive neuroectodermal tumor (ET/pPNET) pattern and unusual molecular features is described. Immunohistochemically, HBA-71 (CD99/mic2) and several neural markers were intensively expressed together with scattered cells expressing carcinoembryonic antigen (CEA). Short-term culture showed biphasic neuroblastic and epithelioid cell populations, with the latter expressing germ cell markers (CEA, alpha-fetoprotein, and the beta-subunit of chorionic gonadotrophin). Conventional cytogenetics displayed several chromosomic rearrangements, especially…

AdultMalePathologymedicine.medical_specialtyOncogene Proteins FusionChromosomes Human Pair 22CD99Soft Tissue NeoplasmsChromosomal translocationSarcoma EwingBiologyTranslocation GeneticPathology and Forensic MedicineExonFatal OutcomeCarcinoembryonic antigenBiomarkers TumorTumor Cells CulturedmedicineHumansNeuroectodermal Tumors PrimitiveRetroperitoneal NeoplasmsMolecular BiologyGene Rearrangementmedicine.diagnostic_testReverse Transcriptase Polymerase Chain ReactionPeripheral Primitive Neuroectodermal TumorChromosomes Human Pair 11Neoplasms Second PrimaryDNA NeoplasmCell BiologyGenes p53Chromosome Bandingmedicine.anatomical_structureKaryotypingMutationbiology.proteinEpithelioid cellGerm cellFluorescence in situ hybridizationDiagnostic Molecular Pathology
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Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.

2000

This study describes a new case of Ewing sarcoma (ES)-peripheral primitive neuroectodermal tumor (pPNET) with unusual phenotype and fusion gene structure. The tumor located in the inguinal area of a 15-year-old boy showed a highly aggressive behavior with hematogenous metastases after intensive chemotherapy and bone marrow transplant, causing death 28 months after diagnosis. The tumor displayed a clear cell pattern, and several neuroectodermal markers proved positive both in the original tumor and in xenografts. This neuroectodermal character was confirmed by electron microscopy. Moreover, cytogenetically the tumor has an unusual chromosomal rearrangement, t(2;22)(q13;q22,t(3;18)(p21;q23); …

MaleAdolescentOncogene Proteins FusionChromosomes Human Pair 22Transplantation HeterologousMice NudeSoft Tissue NeoplasmsChromosomal rearrangementSarcoma EwingBiologyGroinTranslocation GeneticPathology and Forensic MedicineFusion geneExonMiceFatal OutcomemedicineTumor Cells CulturedAnimalsHumansNeuroectodermal Tumors PrimitiveNeoplasm MetastasisMolecular BiologyPeripheral Primitive Neuroectodermal TumorReverse Transcriptase Polymerase Chain ReactionCell BiologyExonsmedicine.diseasePrognosisCombined Modality TherapyNeoplasm ProteinsFusion transcriptPrimitive neuroectodermal tumorChromosomes Human Pair 2KaryotypingCancer researchDisease ProgressionSarcomaClear cellDiagnostic molecular pathology : the American journal of surgical pathology, part B
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Ewing's Sarcoma and Peripheral Primitive Neuroectodermal Tumor of Bone and Soft Tissue

1999

The histological diagnosis of Ewing's sarcoma (Es) continues to be a difficult task for pathologists. A number of new Es varieties has been described, leading to further complexity. Conventional Es, atypical Es, and peripheral neuroectodermal tumor (pPNET), including peripheral neuroepithelioma, belong genetically to the same family of neoplasms, displaying common chromosomal rearrangements and analogous gene reorganizations. The main translocations are t(11;22) and t(21;22), with genes EWS, FLI-1 and ERG being involved, as well as other members of the ETS family of transcription factors. The prevalence of morphology should be maintained with the use of conventional histological techniques…

0301 basic medicinePathologymedicine.medical_specialtyPeripheral Primitive Neuroectodermal TumorSoft tissueEwing's sarcomaBiologymedicine.diseasePathology and Forensic Medicine03 medical and health sciences030104 developmental biology0302 clinical medicine030220 oncology & carcinogenesisHistological diagnosismedicineSurgerySarcomaAnatomyInternational Journal of Surgical Pathology
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