About P.N.E.T Tumors:
The final diagnosis assigned to Spencer's tumor is: primitive neuroectodermal tumor with features of ependymoblastoma.
Primitive neuroectodermal tumors (or P.N.E.T.'s as they are called) are a poorly understood group of pediatric brain tumors that are all thought to be distantly related to one or more type of brain cell. Based on which cell they seem to most closely resemble and where they are in the brain, the specific name of the tumor changes. The different types of PNETs include tumors like medulloblastoma, neuroblastoma and ependymoblastoma (to name only a few). Based on the tumor specimens that we gave to the pathologists, they feel that Spencer's tumor most closely resembled an ependymoblastoma, a very rare and very malignant tumor. Incidentally, the ependyma, or ependymal cells, are the cells that line the fluid filled cavities in the brain and although the names sound similar, the tumor Spencer had is very different and much, much rarer than the tumor we call "ependymoma".
To give you an idea how rare they are, only seven examples of this tumor type have been found over the last 12 years at Duke and Johns Hopkins. All occurred in children (1-3 years old) 6 of the 7 children had died within an average of 9 months of surgery in spite of chemotherapy (and in some cases radiation therapy as well).
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