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Peripheral Primitive Neuroectodermal Tumors (Peripheral Neuroectodermal Tumor) Summary

Description: 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 Show All >>

Networked: 177 relevant articles (0 outcomes, 10 trials/studies) for this Disease

Key Drugs and Agents for Peripheral Primitive Neuroectodermal Tumors

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. DNA (Deoxyribonucleic Acid) : 2 studies in 6 results : IBA
  2. Antibodies : 1 study in 5 results : IBA
  3. Paraffin : 1 study in 4 results : IBA
  4. Phosphopyruvate Hydratase (Enolase) : 1 study in 3 results : IBA
  5. Nerve Growth Factor (NGF) : 1 study in 2 results : IBA
  6. Melphalan (Alkeran) : 1 study in 2 results : FDA 2 Generic
  7. Formaldehyde (Formol) : 1 study in 2 results : FDA 1
  8. Epidermal Growth Factor (EGF) : 1 study in 2 results : IBA
  9. Integrin alpha6beta4 : 1 study in 1 result : IBA
  10. Intercellular Signaling Peptides and Proteins (Growth Factors) : 1 study in 1 result : IBA
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Diseases Related to Peripheral Primitive Neuroectodermal Tumors

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

Efficacy Chart >>
  1. Combined Modality Therapy : 2 studies in 3 results
  2. Drug Therapy (Chemotherapy) : 1 study in 13 results
  3. Combination Drug Therapy (Combination Chemotherapy) : 1 study in 2 results
  4. Heterologous Transplantation (Xenotransplantation) : 2 results
  5. Radiotherapy : 2 results

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