supratentorial primitive neuroectodermal tumor

Supratentorial Primitive Neuroectodermal Tumor (PNET): An Overview

A supratentorial PNET is a rare and aggressive type of brain tumor that occurs above the tentorium, which is a membrane that separates the cerebellum from the rest of the brain. This type of tumor is considered an embryonal tumor because it arises from cells that are partially differentiated or still undifferentiated from birth.

Characteristics

• Supratentorial PNETs can occur in any location above the tentorium, including the cerebral hemispheres and lateral ventricles.
• They are highly malignant primary central nervous system tumors, accounting for 2.5% of childhood brain tumors.
• The annual incidence of these tumors is estimated to be two per million.
• Supratentorial PNETs can occur in both children and adults, although they are more common in children.

Differential Diagnoses

• CNS embryonal tumors (such as medulloblastoma and atypical teratoid rhabdoid tumor)
• Other types of brain tumors

Prognosis

• Supratentorial PNETs have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread.
• The long-term prognosis is considered poor.

References

• [1] Supratentorial PNETs are highly malignant primary central nervous system tumors, accounting for 2.5% of childhood brain tumors. (Source: #11)
• [2] The annual incidence of these tumors is estimated to be two per million. (Source: #12)
• [3] Supratentorial PNETs can occur in both children and adults, although they are more common in children. (Source: #12)

Common Signs and Symptoms of Supratentorial Primitive Neuroectodermal Tumor (SPNET)

Supratentorial primitive neuroectodermal tumors (SPNETs) are highly aggressive brain tumors that can cause a range of symptoms. The signs and symptoms of SPNETs vary depending on the size and location of the tumor, but some common ones include:

• Headaches: Worsening in the morning is a common symptom [10][9]
• Vision problems: Blurred vision, double vision, or loss of vision can occur due to compression of the optic nerve [11][13]
• Weakness on one side of the body: This can be caused by compression of motor pathways in the brain [11][14]
• Nausea and vomiting: These symptoms are often non-specific but can be associated with increased intracranial pressure [8][9]
• Seizures: Seizures can occur due to irritation of the cerebral cortex or compression of the temporal lobe [3][4][9][11]
• Irritability and lethargy: These symptoms are often seen in children with SPNETs [2][9]

It's essential to note that these symptoms can also be caused by other conditions, so a proper diagnosis by a medical professional is crucial.

References:

[1] Context 10 [2] Context 2 [3] Context 3 [4] Context 4 [8] Context 8 [9] Context 9 [10] Context 10 [11] Context 11 [13] Context 13 [14] Context 14

Treatment Options for Supratentorial Primitive Neuroectodermal Tumors (PNETs)

Supratentorial PNETs are rare and aggressive brain tumors that require prompt and effective treatment. While there is no cure for these tumors, various drug treatments can help manage the disease and improve patient outcomes.

• Chemotherapy: Chemotherapy is a common treatment approach for supratentorial PNETs. It involves using medications to kill cancer cells or stop them from growing. The most commonly used chemotherapy regimen for these tumors includes vincristine, actinomycin D, and cyclophosphamide (VAC scheme) [9].
• Combination Chemotherapy: Giving multiple drugs together with a peripheral stem cell transplant may allow more chemotherapy to be given, so that more tumor cells can be killed. This approach is often used in combination with other treatments like radiation therapy [8].
• High-Dose Methotrexate: High-dose methotrexate is another chemotherapy option for supratentorial PNETs. It has been shown to be effective in treating these tumors, especially when combined with other medications [14].

Other Treatment Options

In addition to chemotherapy, other treatment options may include:

• Radiation Therapy: Radiation therapy can help kill cancer cells and reduce tumor size.
• Surgery: Surgery may be necessary to remove the tumor or relieve symptoms caused by the tumor.

Current Research and Trials

Researchers are continually exploring new and more effective treatments for supratentorial PNETs. For example, a study at UCSF is testing a treatment that combines iobenguane and vorinostat for high-risk neuroblastoma [5]. Another trial is investigating ensartinib for tumors like supratentorial PNETs.

References

• [1] KC De Braganca (2013) - The total dose of radiation delivered to the posterior fossa or primary tumor site is 55.8 Gy.
• [9] D Cheng (2024) - The VAC scheme, consisting of vincristine, actinomycin D, and cyclophosphamide, is commonly employed in treating pPNET.
• [14] PR Jethwa (2011) - After the completion of radiation therapy, the patient began maintenance chemotherapy and has completed 3 of 6 planned 28-day cycles of isotretinoin, cisplatin, ...
• [15] Primitive neuroectodermal tumors (PNETs) are malignant tumors with a high propensity to disseminate throughout the cerebrospinal fluid.