Primitive Neuroectodermal Tumors (PNET)

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

Related Diseases

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


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. everolimusFDA Link
2. sunitinib (Sutent)FDA Link
3. Etoposide (VP 16)FDA LinkGeneric
4. Carboplatin (JM8)FDA LinkGeneric
5. Cyclophosphamide (Cytoxan)FDA LinkGeneric
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
8. Doxorubicin (Adriamycin)FDA LinkGeneric
9. IfosfamideFDA LinkGeneric
10. Somatostatin (Somatotropin Release-Inhibiting Factor)IBA

Therapies and Procedures

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