Primitive neuroepithelial neoplasm (PNET) refers to a group of rare and aggressive tumors that originate from immature or undifferentiated cells within the central nervous system (CNS). These tumors primarily affect children and young adults, predominantly occurring in the brain but can also arise in the spinal cord and other areas of the CNS.
Characterized by rapid growth and invasive behavior, PNETs display histopathologic features that resemble the primitive neuroepithelium, a tissue composed of undifferentiated neural cells observed during early embryonic development. Although the exact causes of PNETs are yet to be fully understood, certain genetic mutations and chromosomal abnormalities have been associated with the development of these tumors.
Histologically, PNETs exhibit small, round blue cells that lack the distinctive features of mature neural cells. They are often highly malignant and have a tendency to metastasize to other parts of the CNS or even distant sites. Symptoms of PNETs depend on the specific location of the tumor within the CNS and may include headaches, seizures, behavioral changes, motor dysfunction, and visual disturbances.
The treatment of PNETs usually involves a multidisciplinary approach, combining surgical intervention, chemotherapy, and radiation therapy, depending on the stage, location, and extent of the tumor. Prognosis for patients with PNETs varies depending on various factors, including tumor size, location, age of the patient, and response to treatment. Survival rates can range widely, with some individuals achieving long-term remission, while others experience recurrence or metastasis despite treatment.
