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Because most PNETs are indolent, a "wait-and-see" approach has historically predominated.
FDG-PET is not usually indicated because most PNETs are negative; however, FDG-PET can ascertain the overall tumor burden in high-grade PNETs [59], [60].
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Most PNETs are indolent but have malignant potential.
Most PNETs are indolent but have malignant potential [1]– [3].
Functioning PNETs, such as gastrinoma and insulinoma, and non-functioning PNETs are most common.
Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumours (PNETs) are the most common highly aggressive paediatric brain tumours.
Pancreatic neuroendocrine tumors (pNETs) are the second most common pancreatic neoplasms, exhibiting a complex spectrum of clinical behaviors.
In most academic centers in North America and Europe, non-functioning PNETs are more common than functioning PNETs [142]– [142].
The knowledge of and experience with PNETs are sufficient so that most patients with PNETs today should expect a satisfactory diagnosis and treatment at academic centers with a multidisciplinary team experienced in PNETs.
Many pNETs are diagnosed at late stages because of the lack of symptoms in the case of non-functional tumors.
Ewing's sarcoma family tumors (ESFTs), which include peripheral primitive neuroectodermal tumors (PNETs), are characterized by chromosomal translocations that generate fusions between the EWS gene and ETS-family transcription factors, most commonly FLI1.
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