Primitive neuroectodermal tumor

Primitive neuroectodermal tumor (PNET) is a term used to describe a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissues and bone. They are part of the Ewing family of tumors.

Classification

PNETs are classified into two types: central PNETs (cPNETs) and peripheral PNETs (pPNETs). Central PNETs are intracranial and arise from the cells lining the ventricles or from the medulla. Peripheral PNETs are found outside the central nervous system and are most commonly found in the chest, abdomen, or pelvis.

Symptoms

The symptoms of PNETs depend on the location of the tumor. Symptoms may include pain, swelling, or a noticeable lump. If the tumor is in the brain, symptoms may include headache, nausea, vomiting, or changes in vision.

Diagnosis

Diagnosis of PNETs is based on the histological examination of the tumor. Immunohistochemistry is used to confirm the diagnosis. Other diagnostic tests may include CT scan, MRI, and PET scan.

Treatment

Treatment for PNETs typically involves a combination of surgery, chemotherapy, and radiation therapy. The choice of treatment depends on the size and location of the tumor, as well as the patient's overall health.

Prognosis

The prognosis for patients with PNETs is generally poor, with a five-year survival rate of less than 50%. Factors that may affect the prognosis include the size and location of the tumor, the patient's age and overall health, and the response to treatment.

See also

• Ewing's sarcoma
• Neuroblastoma
• Medulloblastoma

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