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Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. [1] It is a rare tumor , usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%.
Patients diagnosed with a medulloblastoma or PNET are 50 times more likely to die than a matched member of the general population. A population-based (SEER) 5-year relative survival rates indicated 69% overall: 72% in children (1–9 years) and 67% in adults (20+ years). The 20-year survival rate is 51% in children.
A phase III study of sunitinib treatment in well differentiated pNET that had worsened within the past 12 months (either advanced or metastatic disease) showed that sunitinib treatment improved progression-free survival (11.4 months vs. 5.5 months), overall survival, and the objective response rate (9.3% vs. 0.0%) when compared with placebo.
A central nervous system primitive neuroectodermal tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET, [1] is one of the 3 types of embryonal central nervous system tumors (medulloblastoma, atypical teratoid rhabdoid tumor, and PNET). [2]
Brain tumors are the most common type of solid tumors to affect the pediatric population. [3] In particular, medulloblastoma is the most common of them, and constitutes about 20% of all the malignant pediatric brain tumors, classified as a primitive neuroectodermal tumor (PNET) of the cerebellum. [4]
The Ewing family of tumors (EFTs) is a group of small cell sarcomas including Ewing sarcoma of the bone, extra osseous Ewing tumors, and primitive neuroectodermal tumors. They are rare cancers, usually diagnosed in peoples' twenties.
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In the United States there has been an increase in the 5-year relative survival rate between people diagnosed with cancer in 1975-1977 (48.9%) and people diagnosed with cancer in 2007-2013 (69.2%); these figures coincide with a 20% decrease in cancer mortality from 1950 to 2014. [8]