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:

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)

Experts

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 08/15/1989 - "In order to study whether cell fusion would modify the DNA copy number of an amplified oncogene, somatic cell hybrids were made between the human neuroepithelioma cell line MCIXC and HeLaCOT human adenocarcinoma cells. " 06/01/1994 - "Significantly, the DNA binding portion of FLI-1 is the 3' part of an oncogenic fusion transcript (termed EWS-FLI) in human Ewing's sarcoma and neuroepithelioma." 11/01/1992 - "Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system." 11/01/1992 - "Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. " 11/01/1990 - "Procedures that enabled more consistent DNA insert size selection and enhanced yeast transformation frequency were employed to generate a human YAC library from a neuroepithelioma cell line containing a characteristic t(11;22) chromosomal translocation. "
2.	AntibodiesIBA 01/01/1991 - "These antibodies were used to screen six neuroblastoma cell lines and one neuroepithelioma cell line. " 10/01/2010 - "HCVpp infection of the neuroepithelioma cells was neutralized by antibodies to CD81, SR-BI, CLDN1, and HCV E2. " 11/01/1987 - "Thus, antibodies to cytokeratin are useful to distinguish neuroepitheliomas from ependymomas." 04/01/2001 - "In the present study, DNA flow cytometry (FCM) and immunocytochemistry (ICC) with a selected panel of antibodies were performed on 51 cases of malignant tumors which were referred for fine-needle aspiration biopsy (FNAB) to our Department of Cytology for the last 2 yr. Twelve cases were diagnosed as neuroblastoma, 16 as Ewing's sarcoma, 2 as retinoblastoma, 5 as non-Hodgkin's lymphoma (NHL), 5 as rhabdomyosarcoma, 2 as peripheral neuroectodermal tumors (PNETs), and 8 as Wilms' tumor. " 06/15/1989 - "The antibodies recognize a Mr 180,000 plasma membrane phosphoglycoprotein produced in increased amounts in SH-SY5Y/VCR as well as in vincristine-resistant human neuroepithelioma (MC-IXC/VCR), vinblastine-resistant human leukemia (CEM/VLB100), and actinomycin D- or vincristine-resistant Chinese hamster (DC-3F/AD X and DC-3F/VCRd-5L) cells, as compared to control cells. "
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 03/01/1987 - "[Immunohistochemical study of neuron specific enolase in peripheral neuroectodermal tumors]." 03/01/2009 - "Immunohistochemistry in Wilms tumor areas showed positivity for markers also indicative of peripheral primitive neuroectodermal tumors such as neuron-specific enolase, CD99, and CD56. " 11/01/1987 - "Morphological features and immunoreactivity for cytokeratin (CK), glial fibrillary acidic protein (GFAP) and neuron-specific enolase (NSE) of three canine neuroepitheliomas and three canine ependymomas were investigated. "
5.	Nerve Growth Factor (NGF)IBA 02/01/1990 - "All three neuroepithelioma cell lines and all nine neuroblastoma cell lines with amplified N-myc oncogene did not show any apparent NGF-induced differentiation. " 02/01/1990 - "A series of neuroepithelioma and neuroblastoma cell lines were screened for nerve growth factor (NGF)-induced differentiation. " 02/01/1990 - "Nerve growth factor-induced differentiation of human neuroblastoma and neuroepithelioma cell lines." 04/15/1995 - "Using two automated colorimetric assays for determinations of cell numbers, the present study demonstrates that a) different NB and neuroepithelioma cell lines show distinct responses, both qualitatively and quantitatively, to basic FGF (bFGF), NGF, and EGF; b) even closely related NB cell lines (e.g., SK-N-SH, SH-SY5Y, and SHEP) do not respond uniformly to these factors; c) responses of the two neuroepithelioma cell lines employed (SK-N-MC and CHP-100) differ, but match those of certain NB cell lines; and d) two growth factors, bFGF and EGF, may both stimulate or inhibit proliferation, depending on the cell line studied. "
6.	Melphalan (Alkeran)FDA LinkGeneric 08/01/1996 - "From November 1985 to September 1994, 13 patients aged 16-30 years (median 20.5) received high-dose melphalan (HDM) 140-200 mg/m2 +/- 500 cGy TBI followed by ASCT for relapsed/refractory (n = 4), metastatic (n = 2), or non-metastatic (n = 6) EWS, or for peripheral neuroectodermal tumor (PNET) (n = 1). " 08/01/1996 - "High-dose melphalan +/- total body irradiation and autologous hematopoietic stem cell rescue for adult patients with Ewing's sarcoma or peripheral neuroectodermal tumor." 09/01/2002 - "The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4-89) and the role of high-dose melphalan (MMT4-91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET). "
7.	Formaldehyde (Formol)FDA Link 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. " 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. "
8.	Epidermal Growth Factor (EGF)IBA 04/15/1995 - "Using two automated colorimetric assays for determinations of cell numbers, the present study demonstrates that a) different NB and neuroepithelioma cell lines show distinct responses, both qualitatively and quantitatively, to basic FGF (bFGF), NGF, and EGF; b) even closely related NB cell lines (e.g., SK-N-SH, SH-SY5Y, and SHEP) do not respond uniformly to these factors; c) responses of the two neuroepithelioma cell lines employed (SK-N-MC and CHP-100) differ, but match those of certain NB cell lines; and d) two growth factors, bFGF and EGF, may both stimulate or inhibit proliferation, depending on the cell line studied. " 10/09/1998 - "Potentiation of G-protein-coupled receptor-induced MAP kinase activation by exogenous EGF receptors in SK-N-MC neuroepithelioma cells."
9.	Integrin alpha6beta4IBA 01/01/2009 - "The objective of this study was to determine whether the combined expression of alpha6beta4 integrin and neuroepithelioma transforming gene 1 (Net1), a guanine nucleotide exchange factor specific for Ras homolog gene family member A, is associated with adverse clinical outcome in breast cancer patients. "
10.	Intercellular Signaling Peptides and Proteins (Growth Factors)IBA 04/15/1995 - "Using two automated colorimetric assays for determinations of cell numbers, the present study demonstrates that a) different NB and neuroepithelioma cell lines show distinct responses, both qualitatively and quantitatively, to basic FGF (bFGF), NGF, and EGF; b) even closely related NB cell lines (e.g., SK-N-SH, SH-SY5Y, and SHEP) do not respond uniformly to these factors; c) responses of the two neuroepithelioma cell lines employed (SK-N-MC and CHP-100) differ, but match those of certain NB cell lines; and d) two growth factors, bFGF and EGF, may both stimulate or inhibit proliferation, depending on the cell line studied. "

Therapies and Procedures

1.	Combined Modality Therapy 08/01/1993 - "The purpose of this study was to determine the clinical outcome for pediatric patients with peripheral neuroepithelioma treated with combined modality therapy and followed long enough to account for late relapses. " 10/15/1996 - "The disappointingly low survival rate observed in Ewing's sarcoma (ES)/peripheral neuroectodermal tumor (PNET) despite the adoption of aggressive multimodal treatments prompted us to study the existence of autocrine circuits to be used as innovative therapeutic targets. " 12/01/1987 - "Twenty-three consecutive patients with Ewing's sarcoma, alveolar rhabdomyosarcoma, undifferentiated sarcoma, or malignant peripheral neuroepithelioma originating in the pelvis were treated with short, intensive combined modality therapy. "
2.	Drug Therapy (Chemotherapy) 06/01/2007 - "A 16-yr-old female was admitted on 8 September 2005 with neutropenic fever during chemotherapy following surgical removal of peripheral neuroectodermal tumor. " 02/01/2000 - "The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported. " 08/01/1996 - "Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. " 11/01/1995 - "Very-high-dose short-term chemotherapy for poor-risk peripheral primitive neuroectodermal tumors, including Ewing's sarcoma, in children and young adults." 03/06/1991 - "The tumor biopsy of one patient showed a t(11;22) (q24;q12), and this patient had chemotherapy directed to neuroepithelioma. "
3.	Combination Drug Therapy (Combination Chemotherapy) 06/15/2000 - "The aim of the current study was to determine the maximum tolerated dose of a combination chemotherapy regimen in the treatment of patients with recurrent neuroblastoma and peripheral neuroepithelioma (primitive neuroectodermal tumor [PNET]) and whether the use of growth factor would allow increased dose intensity. " 02/01/1999 - "Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. "
4.	Heterologous Transplantation (Xenotransplantation) 08/01/1992 - "Chondrocytic differentiation of peripheral neuroectodermal tumor cell line in nude mouse xenograft." 08/01/1992 - "Chondrocytic differentiation was not observed in xenografts of Ewing's sarcoma cell lines SK-ES or RD-ES or the peripheral neuroectodermal tumor cell line SK-N-MC. " 11/01/1998 - "Effects of all-trans retinoic acid and interferon alpha in peripheral neuroectodermal tumor cell cultures and xenografts."
5.	Radiotherapy 08/01/2003 - "Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy." 08/01/2003 - "A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis. " 05/01/1995 - "In this report we describe a case of a 22-year-old male who developed a peripheral neuroectodermal tumor of the chest wall four years after completing successful treatment with combined chemotherapy and radiotherapy for Hodgkin's disease."