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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%.
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]
Gastrinoma is the second most common functional pancreatic neuroendocrine tumor (pNET), with a yearly incidence of approximately 0.5 to 21.5 cases per a million of people worldwide. [5] Gastrinomas are located predominantly in the duodenum (70%) and pancreas (25%). [ 20 ]
Tumors with similar cellular characteristics in the pituitary, parathyroid, and adrenomedullary glands are sometimes included [9] or excluded. [1] Within the broad category of neuroendocrine tumors there are many different tumor types, [10] representing only a small proportion of the tumors or cancers in most of these tissues [citation needed]:
Malignant ectomesenchymoma (MEM) is a rare tumor of soft tissues or the CNS, which is composed of both neuroectodermal elements [represented by ganglion cells and/or well-differentiated or poorly differentiated neuroblastic cells such as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, peripheral primitive neuroectodermal tumors – PNET ...
Due to presence of these tumors, DIPNECH is classified as a pre-malignant condition. [1] Although there have been reports of atypical carcinoids with local lymph node involvement, there are no reports of more aggressive neuroendocrine tumors, such as large cell neuroendocrine or small cell lung cancer, associated with DIPNECH. [ 3 ]
A central nervous system tumor (CNS tumor) is an abnormal growth of cells from the tissues of the brain or spinal cord. [1] CNS tumor is a generic term encompassing over 120 distinct tumor types. [2] Common symptoms of CNS tumors include vomiting, headache, changes in vision, nausea, and seizures. [3]
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.