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's Sarcoma (SARCOMA, EWING'S). They may arise from the chest wall, skin, orbit, kidney, and other structures and tend to be locally invasive or metastasize, although relatively benign forms may occur. Characteristic histologic features include a tendency to form Homer-Wright rosettes and to stain positively with neuron-specific enolase and vimentin. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2113; J Clin Oncol 1998 Mar;16(3):1150-7)
Also Known As:
Peripheral Neuroectodermal Tumor; Neuroepithelioma; Neuroectodermal Tumors, Primitive, Peripheral; (pPNET) Peripheral Primitive Neuroectodermal Tumors; Extracranial Primitive Neuroectodermal Tumor; Neuroectodermal Neoplasm, Peripheral Primitive; Neuroectodermal Tumor, Peripheral Primitive; Peripheral Primitive Neuroectodermal Neoplasm; Primitive Neuroectodermal Tumor, Extracranial; Neuroectodermal Tumors, Peripheral; Neuroepitheliomas; Peripheral Neuroectodermal Tumors; Tumor, Peripheral Neuroectodermal; Tumors, Peripheral Neuroectodermal; Neuroectodermal Tumor, Peripheral
Networked: 177 relevant articles (0 outcomes, 10 trials/studies)

Disease Context: Research Results

Related Diseases

1. Primitive Neuroectodermal Tumors (PNET)
2. Ewing's Sarcoma (Sarcoma, Ewing)
3. Neuroblastoma
4. Neoplasms (Cancer)
5. Sarcoma (Soft Tissue Sarcoma)


1. Richardson, Des R: 6 articles (12/2013 - 07/2002)
2. Spinedi, Angelo: 5 articles (03/2015 - 12/2002)
3. Di Bartolomeo, Sabrina: 5 articles (03/2015 - 12/2002)
4. Kalinowski, Danuta S: 4 articles (12/2013 - 10/2010)
5. Lovejoy, David B: 4 articles (01/2013 - 07/2002)
6. Pantarat, Namfon: 3 articles (12/2013 - 10/2010)
7. Spinedi, A: 3 articles (06/2002 - 08/2000)
8. Di Bartolomeo, S: 3 articles (06/2002 - 08/2000)
9. Merlot, Angelica M: 2 articles (12/2013 - 10/2010)
10. Alaggio, Rita: 2 articles (09/2012 - 03/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Peripheral Primitive Neuroectodermal Tumors:
1. DNA (Deoxyribonucleic Acid)IBA
2. AntibodiesIBA
3. 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). "
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]."
11/01/1988 - "A new cell line (SIM-1) was derived from a peripheral neuroectodermal tumor (PNET) and used for the production of a monoclonal antibody (HBA-71), which recognizes a novel cell surface antigen of ES- and PNET-derived cells and paraffin-embedded tumor sections. "
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. "
4. Phosphopyruvate Hydratase (Enolase)IBA
5. Nerve Growth Factor (NGF)IBA
6. Melphalan (Alkeran)FDA LinkGeneric
7. Formaldehyde (Formol)FDA Link
8. Epidermal Growth Factor (EGF)IBA
9. Integrin alpha6beta4IBA
10. Intercellular Signaling Peptides and Proteins (Growth Factors)IBA

Therapies and Procedures

1. Combined Modality Therapy
2. Drug Therapy (Chemotherapy)
3. Combination Drug Therapy (Combination Chemotherapy)
4. Heterologous Transplantation (Xenotransplantation)
5. Radiotherapy