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Peripheral Primitive Neuroectodermal Tumors (Peripheral Neuroectodermal Tumor)

A group of highly cellular primitive round cell neoplasms which occur extracranially in soft tissue and bone and are derived from embryonal neural crest cells. These tumors occur primarily in children and adolescents and share a number of characteristics with EWING SARCOMA.
Also Known As:
Peripheral Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor; Neuroectodermal Tumors, Primitive, Peripheral; (pPNET) Peripheral Primitive Neuroectodermal Tumors; Extracranial Primitive Neuroectodermal Tumor; Neuroectodermal Neoplasm, Peripheral Primitive; Neuroectodermal Tumor, Peripheral Primitive; Neuroepithelioma, Peripheral; Peripheral Primitive Neuroectodermal Neoplasm; Primitive Neuroectodermal Tumor, Extracranial; Neuroectodermal Tumors, Peripheral; Neuroepitheliomas, Peripheral; Peripheral Neuroectodermal Tumors; Peripheral Neuroepithelioma; Peripheral Neuroepitheliomas; Tumor, Peripheral Neuroectodermal; Tumors, Peripheral Neuroectodermal; Neuroectodermal Tumor, Peripheral
Networked: 116 relevant articles (1 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Ewing Sarcoma (Sarcoma, Ewing)
2. Primitive Neuroectodermal Tumors (PNET)
3. Neoplasms (Cancer)
4. Neuroblastoma
5. Rhabdomyosarcoma

Experts

1. Wang, Jian: 3 articles (01/2020 - 08/2004)
2. Alaggio, Rita: 3 articles (01/2017 - 03/2009)
3. Magro, Gaetano: 3 articles (01/2017 - 05/2015)
4. Salvatorelli, Lucia: 3 articles (01/2017 - 05/2015)
5. Nicoletti, Ferdinando: 2 articles (01/2017 - 10/2015)
6. Parenti, Rosalba: 2 articles (01/2017 - 05/2015)
7. Musumeci, Giuseppe: 2 articles (10/2015 - 05/2015)
8. Perry, Arie: 2 articles (06/2007 - 07/2005)
9. Chang, Bin: 2 articles (09/2004 - 08/2004)
10. Li, Feng: 2 articles (09/2004 - 08/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Peripheral Primitive Neuroectodermal Tumors:
1. Melphalan (Alkeran)FDA LinkGeneric
2. Etoposide (VP 16)FDA LinkGeneric
3. Carboplatin (JM8)FDA LinkGeneric
4. Cyclin D1IBA
5. AntigensIBA
6. ParaffinIBA
08/01/2004 - "To investigate the expression of EWS-FLI1/ERG fusion transcript resulting from t(11;12)(q24;12) in paraffin-embedded tissues and its diagnostic implication for Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET). "
06/01/2004 - "Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections."
11/01/1992 - "To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. "
08/01/2004 - "[Detection of EWS-FLI1 fusion transcript in Ewing's sarcoma/peripheral primitive neuroectodermal tumors by one-step RT-PCR using paraffin-embedded tissues]."
09/17/2004 - "The variety of fusion transcripts were detected in 103 cases of STS, including 30 cases of synovial sarcoma (SS), 15 cases of rhabdomyosarcoma (RMS), 25 cases of Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET), 12 cases of dermatofibrosarcoma protuberans (DFSP), 14 cases of aveolar soft part sarcoma (ASPS), 3 cases of leiomyosarcoma (LMS), 2 cases of malignant fibrous histocytoma (MFH), and 2 cases of fibrosarcoma (FS); and 20 cases of control tumors by reverse transcription-polymerase chain reaction (RT-PCR) using formalin fixed, paraffin embedded specimens. "
7. DNA (Deoxyribonucleic Acid)IBA
8. Phosphopyruvate Hydratase (Enolase)IBA
9. Formaldehyde (Formol)FDA Link
10. Glycoproteins (Glycoprotein)IBA

Therapies and Procedures

1. Combined Modality Therapy
2. Drug Therapy (Chemotherapy)
3. Therapeutics
4. Combination Drug Therapy (Combination Chemotherapy)
5. Radiotherapy