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Paul S Dickman - One of the best experts on this subject based on the ideXlab platform.
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ewing sarcoma peripheral Primitive Neuroectodermal Tumor and related Tumors
Pediatric and Developmental Pathology, 2012Co-Authors: Maria Tsokos, Rita Alaggio, Louis P Dehner, Paul S DickmanAbstract:Ewing sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) and other Tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell Tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell Tumor in the spectrum of Tumors with EWS gene rearrangements. Other Tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.
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ewing sarcoma Primitive Neuroectodermal Tumor of the chest wall impact of initial versus delayed resection on Tumor margins survival and use of radiation therapy
Annals of Surgery, 2003Co-Authors: Robert C Shamberger, Nancy J Tarbell, Mark C Gebhardt, Michael P Laquaglia, James R Neff, Karen J Marcus, Scott L Sailer, Richard B Womer, James S Miser, Paul S DickmanAbstract:Objective: To establish outcome and optimal timing of local control for patients with nonmetastatic Ewing sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) of the chest wall.
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addition of ifosfamide and etoposide to standard chemotherapy for ewing s sarcoma and Primitive Neuroectodermal Tumor of bone
The New England Journal of Medicine, 2003Co-Authors: Holcombe E Grier, Mark Krailo, Nancy J Tarbell, Michael P Link, Chris Fryer, Douglas J Pritchard, Mark C Gebhardt, Paul S Dickman, Elizabeth J Perlman, Paul A MeyersAbstract:Background Ewing's sarcoma and Primitive Neuroectodermal Tumor of bone are closely related, highly malignant Tumors of children, adolescents, and young adults. A new drug combination, ifosfamide and etoposide, was highly effective in patients with Ewing's sarcoma or Primitive Neuroectodermal Tumor of bone who had a relapse after standard therapy. We designed a study to test whether the addition of these drugs to a standard regimen would improve the survival of patients with newly diagnosed disease. Methods Patients 30 years old or younger with Ewing's sarcoma, Primitive Neuroectodermal Tumor of bone, or Primitive sarcoma of bone were eligible. The patients were randomly assigned to receive 49 weeks of standard chemotherapy with doxorubicin, vincristine, cyclophosphamide, and dactinomycin or experimental therapy with these four drugs alternating with courses of ifosfamide and etoposide. Results A total of 518 patients met the eligibility requirements. Of 120 patients with metastatic disease, 62 were random...
Qihui Jim Zhai - One of the best experts on this subject based on the ideXlab platform.
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primary ewing sarcoma peripheral Primitive Neuroectodermal Tumor of the vulva
Human Pathology, 2008Co-Authors: Erika Y Fong, Dolores Lopezterrada, Qihui Jim ZhaiAbstract:We report a case of Ewing sarcoma/peripheral Primitive Neuroectodermal Tumor (ES/pPNET) arising in the vulva in a 17-year-old adolescent girl. Primary extraskeletal ES/pPNET is uncommon and only rarely affects the female genital tract. Characteristic histologic features of ES/pPNET were present in this case including a monomorphic population of small round blue cells with cytoplasmic glycogen confirmed by periodic acid-Schiff and were immunohistochemically positive for CD99 in a membranous and Fli-1 in a nuclear pattern. The presence of an EWS/Fli-1 fusion transcript was demonstrated by reverse transcription polymerase chain reaction and confirmed by direct sequencing. This report described another very rare case of ES/pPNET arising in the vulva and confirmed by molecular analysis.
Louis P Dehner - One of the best experts on this subject based on the ideXlab platform.
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ewing sarcoma peripheral Primitive Neuroectodermal Tumor and related Tumors
Pediatric and Developmental Pathology, 2012Co-Authors: Maria Tsokos, Rita Alaggio, Louis P Dehner, Paul S DickmanAbstract:Ewing sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) and other Tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell Tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell Tumor in the spectrum of Tumors with EWS gene rearrangements. Other Tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.
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Primitive Neuroectodermal Tumor and ewing s sarcoma
The American Journal of Surgical Pathology, 1993Co-Authors: Louis P DehnerAbstract:Many of the major solid, malignant Tumors of childhood have histologic similarities that reflect their dysembryonic and Primitive features. One subset of these neoplasms, Ewing's sarcoma (ES) and Primitive Neuroectodermal Tumor (PNET), presents primarily in the bone and soft tissues. Both Tumor types were reported at a time and date well before the advent of electron microscopy and immunohistochemistry. Opposition to ES and PNET as distinctive entities developed and persisted because these Tumors were considered incompletely documented examples of metastatic neuroblastoma or malignant lymphoma. General acceptance of ES as a unique Tumor type occurred well before the PNET had been fully defined and characterized. Once these neoplasms had joined the other round cell neoplasms, the quest for the histogenesis was pursued, but the results were frustratingly inconclusive, especially for ES. Because of the resemblance of the PNET to classic neuroblastoma, the neural crest was regarded as the most likely progenitor. With the recognition of osseous PNET, extraosseous ES, and a shared cytogenetic abnormality between ES and PNET, more recent speculation has focused on the possibility that these presumably separate neoplasms are closely related histogenetically without directly answering the question of histogenesis. Despite the likely common progenitorship of ES and PNET, the latter neoplasm is seemingly the more aggressive. Although melanotic Neuroectodermal Tumor of infancy, intra-abdominal desmoplastic small cell Tumor, and polyphenotypic small cell Tumors have some overlapping microscopic and immunohistochemical features with PNET, their relationship to ES-PNET has otherwise not been resolved.
Joseph G Ouzounian - One of the best experts on this subject based on the ideXlab platform.
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peripheral Primitive Neuroectodermal Tumor of the vulva
Gynecologic Oncology, 1994Co-Authors: Gary R Scherr, Gerrit Dablaing, Joseph G OuzounianAbstract:A 10-year-old Hispanic female presented with a 3-month history of a vulvar mass. The histologic diagnosis was a peripheral Primitive Neuroectodermal Tumor (PNET). To our knowledge, this is the second reported case of vulvar peripheral PNET and the first such case reported in the English language.
Dolores Lopezterrada - One of the best experts on this subject based on the ideXlab platform.
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primary ewing sarcoma peripheral Primitive Neuroectodermal Tumor of the vulva
Human Pathology, 2008Co-Authors: Erika Y Fong, Dolores Lopezterrada, Qihui Jim ZhaiAbstract:We report a case of Ewing sarcoma/peripheral Primitive Neuroectodermal Tumor (ES/pPNET) arising in the vulva in a 17-year-old adolescent girl. Primary extraskeletal ES/pPNET is uncommon and only rarely affects the female genital tract. Characteristic histologic features of ES/pPNET were present in this case including a monomorphic population of small round blue cells with cytoplasmic glycogen confirmed by periodic acid-Schiff and were immunohistochemically positive for CD99 in a membranous and Fli-1 in a nuclear pattern. The presence of an EWS/Fli-1 fusion transcript was demonstrated by reverse transcription polymerase chain reaction and confirmed by direct sequencing. This report described another very rare case of ES/pPNET arising in the vulva and confirmed by molecular analysis.