Central nervous system primitive neuroectodermal tumor

Central nervous system primitive neuroectodermal tumor (CNS PNET) is a rare and highly aggressive cancer that primarily affects children and young adults. It is a type of neuroectodermal tumor that originates from the neural crest cells in the central nervous system.

Classification

CNS PNETs are classified as embryonal tumors, a category of neoplasms that also includes medulloblastoma and atypical teratoid rhabdoid tumor (ATRT). They are further classified based on their location in the brain, with the most common types being cerebral, pineal, and spinal.

Symptoms

The symptoms of CNS PNETs vary depending on the location of the tumor. Common symptoms include headache, nausea, vomiting, and changes in behavior or personality. In some cases, the tumor may cause seizures or neurological deficits.

Diagnosis

Diagnosis of CNS PNETs typically involves a combination of neuroimaging techniques, such as magnetic resonance imaging (MRI) and computed tomography (CT) scans, and biopsy to confirm the diagnosis. Histopathology and immunohistochemistry are also used to differentiate CNS PNETs from other types of brain tumors.

Treatment

Treatment for CNS PNETs usually involves a combination of surgery, radiation therapy, and chemotherapy. The goal of treatment is to remove as much of the tumor as possible and to kill any remaining cancer cells. Despite aggressive treatment, the prognosis for CNS PNETs is generally poor, with a five-year survival rate of less than 50%.

Research

Research into CNS PNETs is ongoing, with a focus on understanding the genetic and molecular mechanisms that drive these tumors. This research may lead to the development of new treatments and improve the prognosis for individuals with this disease.