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However, a positive correlation between individuals with Li-Fraumeni syndrome with a mutation in the gene p53 and PNET has been reported. [2] A significant number of individuals with mutations on the rb tumor suppressor gene have also developed the tumor. [2] Such gene encodes for the protein Rb responsible for stopping the cell cycle at the G1 ...
PNET of the CNS generally refer to supratentorial PNETs. In the past medulloblastomas were considered PNETs; however, they are genetically, transcriptionally and clinically distinct. As such, "infratentorial" PNETs are now referred to as medulloblastoma [ citation needed ] .
Medulloblastoma is a common type of primary brain cancer in children. It originates in the part of the brain that is towards the back and the bottom, on the floor of the skull, in the cerebellum, or posterior fossa.
One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal. [3] In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS. [4]
The supratentorial region contains the cerebrum, while the infratentorial region contains the cerebellum. Although the Roman era anatomist Galen commented upon it, the functional significance of this neuroanatomical division was first described using ‘modern’ terminology by John Hughlings Jackson , founding editor of the medical journal Brain .
The original source for this list was the US National Cancer Institute's public domain Dictionary ... – PNET – pneumonectomy ... – supratentorial ...
The free border of the tentorium is U-shaped; it forms an aperture - the tentorial notch (tentorial incisure) - which gives passage to the midbrain.The free border of each side extends anteriorly beyond the medial end of the superior petrosal sinus (i.e. the apex of the petrous part of the temporal bone [citation needed]) to overlap the attached margin, thenceforth forming a ridge of dura ...
To be specific, primative neuroectodermal tumor (PNET) is a type of brain tumor that has nothing to do with Ewing's sarcoma. This is sometimes called a "central PNET" or a "supratentorial PNET." Peripheral PNET (pPNET), on the other hand, is a tumor type that is essentially identical to Ewing's sarcoma, and is not found in the CNS.