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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%.
The same holds true for the relationship between volume of tumor removed by surgery and survival. [2] Furthermore, factors such as tumor size, location of origin, race, and sex of individual don't show any influence on the outcome of the disease. [2] However, interactions of some factors such as tumor site, age, and treatment strategy can ...
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.
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]
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.
NETs include certain tumors of the gastrointestinal tract and of the pancreatic islet cells, [1] certain thymus and lung tumors, and medullary carcinoma of the parafollicular cells of the thyroid. [1] Tumors with similar cellular characteristics in the pituitary, parathyroid, and adrenomedullary glands are sometimes included [9] or excluded. [1]
Ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that occurs mainly in children, although cases have been reported in patients up to age 60. [1] Ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, or limbs. Also called malignant ectomesenchymoma.
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]