What are primitive neuroectodermal tumors (PNETs)?

Updated: Feb 07, 2019
  • Author: Carlo P Honrado, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Answer

Answer

Peripheral primitive neuroectodermal tumors (pPNETs) are a group of aggressive malignancies that most commonly present in the thoracopulmonary region (Askin tumor), abdomen, pelvis, and, rarely, in the head and neck. The most significant prognostic factor is the presence or absence of metastatic disease, with the former group of patients having a dismal long-term prognosis. Significant advances in the neoadjuvant and adjuvant chemotherapeutic regimens, as well as improved facility in diagnosing these tumors through cytogenetic and immunohistochemical analysis, should improve long-term disease-free survival.

A study by de Blank et al found that in the United States in 2009, among children and adolescents who died of cancer, PNET was the form of cancer responsible for the highest mean years of life lived with disease (4.6 years lived). [29]


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