Neuroectodermal tumors primitive
Neuroectodermal Tumors, Primitive Neuroectodermal tumors, primitive (PNETs), are a group of rare, aggressive tumors that originate from primitive nerve cells. These tumors are part of the larger family of small round blue cell tumors and are most commonly found in children and young adults. PNETs can occur in various parts of the body, including the central nervous system and peripheral tissues.
Classification[edit | edit source]
PNETs are classified based on their location and histological characteristics. The main types include:
- Central Nervous System Primitive Neuroectodermal Tumors (CNS PNETs)
- Peripheral Primitive Neuroectodermal Tumors (pPNETs)
Central Nervous System Primitive Neuroectodermal Tumors (CNS PNETs)[edit | edit source]
CNS PNETs are found in the brain and spinal cord. They are highly malignant and can spread throughout the central nervous system. CNS PNETs are often treated with a combination of surgery, radiation, and chemotherapy.
Peripheral Primitive Neuroectodermal Tumors (pPNETs)[edit | edit source]
Peripheral PNETs occur outside the central nervous system, often in the soft tissues or bones. They are closely related to Ewing's Sarcoma and share similar genetic and histological features.
Symptoms[edit | edit source]
The symptoms of PNETs vary depending on the tumor's location. Common symptoms include:
- Headaches and nausea (for CNS PNETs)
- Seizures
- Weakness or paralysis
- Pain or swelling in the affected area (for pPNETs)
Diagnosis[edit | edit source]
Diagnosis of PNETs typically involves imaging studies such as MRI or CT scans, followed by a biopsy to confirm the histological type. Genetic testing may also be performed to identify specific chromosomal abnormalities associated with these tumors.
Treatment[edit | edit source]
Treatment strategies for PNETs depend on the tumor's location and stage. Common approaches include:
- Surgical resection to remove as much of the tumor as possible
- Radiation therapy to target residual tumor cells
- Chemotherapy to address systemic disease
Prognosis[edit | edit source]
The prognosis for patients with PNETs varies widely based on factors such as tumor location, size, and response to treatment. CNS PNETs generally have a poorer prognosis compared to pPNETs due to their aggressive nature and potential for widespread dissemination.
Research and Future Directions[edit | edit source]
Ongoing research is focused on understanding the genetic and molecular basis of PNETs to develop targeted therapies. Clinical trials are exploring new chemotherapy agents and immunotherapies to improve outcomes for patients with these challenging tumors.
See Also[edit | edit source]
External Links[edit | edit source]
- [National Cancer Institute - PNETs](https://www.cancer.gov/types/pnet)
- [American Brain Tumor Association - PNETs](https://www.abta.org/brain-tumor-information/types-of-tumors/pnet/)
NIH genetic and rare disease info[edit source]
Neuroectodermal tumors primitive is a rare disease.
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Contributors: Prab R. Tumpati, MD
