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Primitive Neuroectodermal Tumors (PNET)

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A group of malignant tumors of the nervous system that feature primitive cells with elements of neuronal and/or glial differentiation. Use of this term is limited by some authors to central nervous system tumors and others include neoplasms of similar origin which arise extracranially (i.e., NEUROECTODERMAL TUMORS, PRIMITIVE, PERIPHERAL). This term is also occasionally used as a synonym for MEDULLOBLASTOMA. In general, these tumors arise in the first decade of life and tend to be highly malignant. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2059)

Also Known As:

PNET; Primitive Neuroectodermal Tumor; Ependymoblastoma; Tumor, Primitive Neuroectodermal; Spongioblastoma; Neuroectodermal Tumors, Primitive; Cerebral Primitive Neuroectodermal Tumor; Neoplasms, Primitive Neuroepithelial; Neuroectodermal Tumor, Primitive; Neuroepithelial Neoplasms, Primitive; Primitive Neuroepithelial Neoplasms; Ependymoblastomas; Medulloepitheliomas; Neoplasm, Primitive Neuroepithelial; Neuroepithelial Neoplasm, Primitive; Neuroepithelial Tumor, Primitive; PNETs; Primitive Neuroepithelial Neoplasm; Primitive Neuroepithelial Tumor; Primitive Neuroepithelial Tumors; Spongioblastomas; Tumor, Primitive Neuroepithelial; Tumors, Primitive Neuroectodermal; Tumors, Primitive Neuroepithelial; Medulloepithelioma; Neuroepithelial Tumors, Primitive

Networked: 1219 relevant articles (61 outcomes, 179 trials/studies)

Relationship Network

Disease Context: Research Results

Neoplasms: 649836
- Neoplasms by Histologic Type: 1

Related Diseases

1.	Medulloblastoma
2.	Primitive Neuroectodermal Tumors (PNET)
3.	Neoplasms (Cancer)
4.	Neuroendocrine Tumors (Neuroendocrine Tumor)
5.	Ewing's Sarcoma (Sarcoma, Ewing)

Experts

1.	Rutkowski, Stefan: 14 articles (03/2015 - 04/2006)
2.	Phillips, P C: 14 articles (01/2002 - 03/2000)
3.	Pietsch, Torsten: 13 articles (03/2015 - 02/2002)
4.	Grotzer, M A: 11 articles (01/2002 - 03/2000)
5.	Saif, Muhammad Wasif: 10 articles (03/2014 - 03/2011)
6.	Janss, A J: 10 articles (11/2001 - 03/2000)
7.	Warmuth-Metz, Monika: 9 articles (03/2015 - 05/2004)
8.	Gajjar, Amar: 9 articles (08/2014 - 08/2004)
9.	von Hoff, Katja: 8 articles (03/2015 - 09/2007)
10.	von Bueren, André O: 8 articles (03/2015 - 05/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primitive Neuroectodermal Tumors:

1.	everolimus FDA Link 03/01/2013 - "Everolimus significantly improved progression-free survival among patients with pNET in the phase III RADIANT-3 study. " 03/01/2011 - "Since last year, there has been a significant advance in the management of PNET, after reported data confirmed the efficacy of everolimus, an mTOR inhibitor, in patients with advanced disease. " 11/01/2014 - "Although everolimus-based phase II/III trials have improved progression-free survival for pNET, its use has not impacted on prolonging overall survival. " 09/01/2012 - "Based on review of the available literature, everolimus is a safe and effective treatment option for patients with low- to intermediate-grade, unresectable or metastatic pNET that have progressed on prior therapies. " 01/01/2012 - "Everolimus was again associated with improvement in PFS compared to placebo and it has been approved by the FDA for patients with progressive pNET. "
2.	sunitinib (Sutent)FDA Link 06/01/2013 - "A recent Phase III clinical trial of sunitinib demonstrated significant improvement of progression-free survival in patients with advanced or metastatic well-differentiated PNET that led to its approval in several countries, including Europe and United States. " 06/01/2013 - "Emerging data from a Phase III trial demonstrates improved progression-free survival of patients with advanced PNET on treatment with sunitinib . " 01/01/2014 - "This review will detail the preclinical data leading up to the results of the pivotal phase III clinical trials that have led to the widespread use of sunitinib in advanced RCC, GIST, and PNET. " 07/01/2012 - "Finally, Vinik et al. (Abstract #4118) provided updated survival data form the seminal phase III trial that led to approval of sunitinib in the treatment of pNET. " 01/01/2012 - "The Oncologic Drugs Advisory Committee voted eight to two that, despite residual uncertainty about the magnitude of the PFS effect because of early trial termination, sunitinib demonstrated a favorable benefit-risk profile in pNET patients. "
3.	Etoposide (VP 16)FDA LinkGeneric 01/01/2014 - "Acute transient parotitis in a child with primitive neuroectodermal tumor after a standard dose etoposide." 01/01/2000 - "Nude mice transplanted with a human tumor (ependymoblastoma) were treated with etoposide (5-40 mg/kg) or 1-2 Gy X-ray irradiation or both. " 01/01/1999 - "Intravenous etoposide has a low level of activity in the treatment of recurrent low grade astrocytomas and medulloblastoma or PNET. " 07/01/1997 - "Daily oral etoposide is active in recurrent PNET/medulloblastoma and ependymoma. " 01/01/2008 - "Eleven patients with recurrent or treatment-induced malignant CNS tumors, including treatment-induced PNET (in 1 patient), brainstem glioma (in 3 patients; 1 with treatment-induced, 2 with recurrence), recurrent anaplastic astrocytoma (in 3 patients), and recurrent glioblastoma (in 4 patients) were evaluated in a pilot study of TMZ and oral VP-16 chemotherapy. "
4.	Carboplatin (JM8)FDA LinkGeneric 03/01/1995 - "Sixteen patients with high risk MB/PNET at diagnosis were included in a pilot study employing carboplatin (CBDCA) as a single drug prior to conventional therapy. " 05/01/2015 - "Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children's Oncology Group." 10/01/1995 - "In this paper, the authors report 3 cases of children under three years of age, with a presumed diagnosis of PNET/MB, who were preoperatively treated with chemotherapy consisting of high doses of carboplatin alone. " 03/01/1995 - "Carboplatin in childhood medulloblastoma/PNET: feasibility of an in vivo sensitivity test in an "up-front" study." 07/20/2012 - "We evaluated the feasibility of administering carboplatin as a radiosensitizer during craniospinal radiation therapy (CSRT) to patients with high-risk medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumors, and we report the outcome in the subset with metastatic (M+) MB. "
5.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 04/01/1991 - "The treatment results favor: (1) early surgical removal, (2) dose-intensive use of drugs active against PNET (especially cyclophosphamide), and (3) radiation therapy to ablate residual microscopic disease." 11/01/2012 - "Ten consecutive children (aged: 3.5-15.5 years, median: 10.2 years, six male) with high-risk embryonal tumors, including: large cell/anaplastic medulloblastoma (6), atypical teratoid rhabdoid tumor (1), and leptomeningeal primitive neuroectodermal tumor (3), were treated with IV cyclophosphamide 1 g/M(2) on days 1 and 2 of CSI. " 07/01/1995 - "However, such therapy is associated with unexpected high-frequency hearing loss; even so, on the basis of the favorable responses in patients with primitive neuroectodermal tumors, germ cell tumors, and lymphomas, the study of this regimen for those tumors is being extended in a multiinstitutional trial that also includes cytoxan to further evaluate the potential enhanced drug delivery." 01/01/1999 - "Whereas it was confirmed that 2 courses of high dose carboplatin is effective in high risk MB/PNET children, we observed an unacceptable number of PD during the subsequent high dose cyclophosphamide therapy. " 08/01/2015 - "•Central type primitive neuroectodermal tumor (cPNET) of the ovary is difficult to treat.•Etoposide, cisplatin and cyclophosphamide is a pediatric medulloblastoma regimen.•Our case with cPNET responded to this combination chemotherapy. "
6.	Vincristine (Oncovin)FDA LinkGeneric 08/01/2015 - "We report the results of treatment of patients with malignant transformation to PNET with cyclophosphamide+doxorubicin+vincristine (CAV) alternating with ifosfamide+etoposide (IE). " 02/01/1990 - "To increase the rate and duration of response in children with recurrent PNET/MB, the authors treated seven patients (ages 2-18 years; median, 10 years) with lomustine (CCNU) (100 mg/m2), cisplatin (CPDD) (90 mg/m2) and vincristine (VCR) (1.5 mg/m2; maximum, 2 mg) in a 6-week cycle for a maximum of eight cycles. " 03/01/1991 - "Beginning in 1983, children with MB/PNET were prospectively assigned to risk groups; those with "standard-risk" MB/PNET were treated with radiation therapy alone, while those in the "poor-risk" group received similar radiation therapy plus adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatin. " 09/01/1989 - "A computerized axial tomography scan identified a mass within the right psoas muscle, and a needle biopsy showed a primitive neuroectodermal tumor, which did not respond to two courses of chemotherapy (Adriamycin, vincristine, and cytoxan). " 07/01/1995 - "After surgery and staging, 55 patients aged 1.5 to 19.3 years with S-PNETs were randomized to receive craniospinal radiotherapy (RT) followed by eight cycles of 1-(2-chloro-ethyl)-3-cyclohexylnitrosourea (CCNU), vincristine (VCR), and prednisone (standard treatment) or two cycles of 8-in-1 chemotherapy followed by RT and then eight additional cycles of 8-in-1. "
7.	Octreotide (Sandostatin)FDA LinkGeneric 02/28/2015 - "In patients with uncontrolled secretory symptoms, octreotide LAR doses can be titrated up to 60 mg every 4 wk or up to 40 mg every 3 or 4 wk. Using the Delphi process allowed physician experts to systematically obtain a consensus on the appropriateness of a variety of medical therapies in patients with PNETs." 08/01/2015 - "On the other hand, results from the PROMID study demonstrated a tumor-stabilizing effect when octreotide LAR (long acting repeatable) was used to treat patients with advanced midgut NET; however, there has been no consensus regarding its antitumor effect for PNET. " 11/01/2014 - "Although only 2 cases were evaluated in this study, multimodal treatment consisting of TACE with DSM and octreotide effectively controlled liver metastasis of pNET in both cases." 09/01/2013 - "We reviewed the medical records of 7 patients with metastatic pNETs who had had progressive cancer prior to treatment despite therapy, including long-acting release octreotide (60 mg/month), chemotherapy and hepatic chemoembolization. " 05/01/2008 - "In liver metastasis of PNET, mass reduction surgery with hepatectomy improves the prognosis of patients, and octreotide LAR has been shown to be useful for reducing complications and inhibiting the growth of tumors."
8.	Doxorubicin (Adriamycin)FDA LinkGeneric 09/01/1994 - "ES and PNET are sensitive to doxorubicin, but may escape treatment by expression of the multidrug-resistant phenotype and/or other mechanisms. " 05/01/2000 - "Administration of high dose Doxorubicin (75 mg/m2) seems feasible in association with irradiation treatment in patients with extraosseal Ewing sarcoma/PNET but the place of high dose chemotherapy within this setting remains to be determined." 07/01/2015 - "Chemotherapy regimens, including doxorubicin used in primitive neuroectodermal tumor's (PNET) treatment can cause life-threatening disorders in cardiac functions. " 03/01/2000 - "Human ependymoblastoma (NNE), primitive neuroectodermal tumor (YKP), glioblastoma (KYG) and small cell lung carcinoma (GLS) that are all transplantable to nude mice were treated with doxorubicin (8 mg/kg) or radiation (1 Gy). " 03/01/2012 - "Prior to 2011, the only approved agent for unresectable pNETs was streptozocin (often used in combination with doxorubicin) but the efficacy of this drug was questionable. "
9.	Ifosfamide FDA LinkGeneric 06/01/2004 - "One patient with a pancreatic primitive neuroectodermal tumor received concurrent etoposide and ifosfamide. " 01/01/2014 - "Adjuvant chemotherapy with adriamycin and ifosfamide was initiated as ES/PNET often exhibits aggressive biological behavior. " 02/20/2003 - "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. " 01/01/2013 - "She received radiotherapy and chemotherapy with ifosfamide and etoposide, following surgery for the right parietal PNET. " 02/20/2003 - "Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone."
10.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 07/01/2012 - "In cases of unresectable pNET, a somatostatin analog has been shown to demonstrate antitumor effects and is considered to be a promising treatment. " 09/01/2014 - "On the other hand, there is increasing evidence supporting the use of PET with somatostatin analogues (DOTA-TOC, DOTA-NOC, or DOTA-TATE) labeled with gallium-68 (Ga-68) in pNETs. " 09/01/2013 - "Somatostatin analogues control symptoms in some types of pNETs, but have not yet demonstrated antitumor activity. " 01/01/2012 - "Patients with "carcinoid" syndrome and syndromes associated with functional pancreatic NET (PNET) can be effectively treated with somatostatin analogs. " 08/01/2015 - "Somatostatin analogs are recommended to treat the endocrine symptoms of functional PNET; however, in case they can cause hypoglycemia in patients with insulinoma. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 06/01/2004 - "We also demonstrate the importance of including PNET as a differential diagnosis because combined chemotherapy is thought to be the best first-line treatment in most cases. " 05/01/2005 - "The purpose of this study was to evaluate the efficacy of high-dose, marrow-ablative chemotherapy with autologous hemopoietic stem cell rescue in the treatment of recurrent central nervous system medulloepithelioma. " 03/01/2012 - "Advanced PNETs remain a difficult therapeutic challenge because of their high malignant potential and their resistance to conventional chemotherapy although there have been recent developments with promising results with the use of novel agents for the treatment of this disease. " 10/01/1999 - "With multimodality therapy, including maximal surgical resection, craniospinal radiation therapy and chemotherapy, the survival for patients with pineal PNETs has significantly improved. " 06/01/1995 - "(2) For children more than 18 months of age at diagnosis treated with craniospinal RT and chemotherapy, the PFS is superior to that of children with other supratentorial PNETs. "
2.	Radiotherapy 01/01/2012 - "The purpose of this study was to assess the frequency of the low-grade bone lesion development in the radiotherapy field in pediatric MB/PNET survivors and describe the imaging characteristics of these lesions. " 07/01/2009 - "Impact of radiotherapy parameters on outcome for patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study." 04/01/2006 - "Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials." 08/01/2015 - "To the best of our knowledge, this is the first reported case of long-term survival of CNS medulloepithelioma treated by HDCTX/auto-PBSCT without radiotherapy. " 09/01/2014 - "All PNETs have survival of less than 1 year even with adjuvant chemo and radiotherapy."
3.	Adjuvant Chemotherapy 02/01/1990 - "Further investigation into the efficacy of this combination as adjuvant chemotherapy in newly diagnosed high-risk PNET/MB is now being performed." 10/01/1988 - "The results of this study, although preliminary, suggest that adjuvant chemotherapy is at least transiently effective in improving the rate of disease-free survival for children with poor-risk MB/PNET." 08/01/2001 - "This study represents the largest series of patients with average-risk MB/PNETs treated with a combination of reduced-dose RT and adjuvant chemotherapy whose intellectual development has been followed prospectively. " 07/01/2012 - "We present the case of a congenital localised sacrococcygeal primitive neuroectodermal tumor treated aggressively with surgical resection and modified age-appropriate adjuvant chemotherapy. " 02/01/2012 - "This is a rare reported case of resection of a cardiac primitive neuroectodermal tumor without adjuvant chemotherapy. "
4.	Combination Drug Therapy (Combination Chemotherapy) 06/01/1997 - "In the present case a complete remission was obtained by a PNET oriented chemotherapy combination followed by 2 courses of classical BEP." 03/01/2010 - "The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy." 01/01/2013 - "Herein, we report the case of a gastric PNET with PVTT, which resolved after 3 cycles of polychemotherapy except for a residual tumor focus in the gastric corpus." 08/01/2006 - "Renal and bladder PNET are both often diagnosed at an advanced stage and, therefore, the prognosis is poor, despite aggressive multimodal treatment (surgery, polychemotherapy, radiotherapy). " 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. "
5.	Heterologous Transplantation (Xenotransplantation) 03/01/2001 - "The potential applications of this xenograft model in the biologic as well as therapeutic study of MB/PNET deserve continuing investigation." 02/01/2000 - "All embryonal tumours, MBs and PNET xenografts consistently showed GLUT-1 membrane staining. " 11/01/1998 - "In this study we evaluated the anti-tumor effects of ATRA and IFNá in PNET cells in vitro and in a human PNET xenograft model, using CHP100 cells. " 06/01/2014 - "A patient tumor-derived orthotopic xenograft mouse model replicating the group 3 supratentorial primitive neuroectodermal tumor in children." 11/01/1998 - "Taken together these data show an in vitro and in vivo anti-tumor activity of ATRA in human PNET cells, although no synergism of ATRA and IFNá was observed in our xenograft model."