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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%. [2]
Magnetic resonance image of PNET. Most children that develop primitive neuroectodermal tumors are diagnosed early in life, usually at around 3–6.8 years of age. [2] Symptoms patients present at time of diagnosis include irritable mood, visual difficulties, lethargy, and ataxia. [2]
Medulloblastoma is the second-most frequent brain tumor in children after pilocytic astrocytoma [37] and the most common malignant brain tumor in children, comprising 14.5% of newly diagnosed brain tumors. [38] In adults, medulloblastoma is rare, comprising fewer than 2% of CNS malignancies. [39]
[8] [9] Treatment of PNETs is the same as extra-osseous Ewing’s sarcoma, with resection of the whole tumor alongside chemotherapy and radiation. Outcomes however are poor as PNET remains an aggressive cancer as a member of the Ewing Family of Tumors. [9]
The tumor histopathology is jumbled small and large cells. The tissue of this tumor contains many different types of cells including the rhabdoid cells, large spindled cells, epithelial and mesenchymal cells, and areas resembling primitive neuroectodermal tumor (PNET). As much as 70% of the tumor may be made up of PNET-like cells.
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]
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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.