supratentorial ependymoma

Supratentorial Ependymoma: A Rare Type of Brain Tumor

Supratentorial ependymomas are a type of brain tumor that originates from the ependymal cells, which produce cerebrospinal fluid (CSF) [8]. These tumors are typically located above the tentorium, a membrane that separates the cerebrum from the cerebellum.

Characteristics and Symptoms

Supratentorial ependymomas usually present with headaches, seizures, and focal neurological deficits compared to their infratentorial counterparts [1]. Other symptoms may include feeling or being sick, loss of balance, irritability, and lack of energy [4].

Treatment and Prognosis

The treatment for supratentorial ependymoma typically involves a combination of surgery, radiation therapy, and chemotherapy. In some cases, focal radiation therapy is administered after gross total resection [3]. The prognosis for patients with supratentorial ependymomas varies depending on the tumor's location, size, and grade.

Genetic Mutations

Recent studies have identified common driver gene fusions such as ZFTA and YAP1 fusions in supratentorial ependymomas [7]. These genetic mutations may play a crucial role in the development and progression of these tumors.

References:

• [1] Supratentorial ependymoma (STEP) is the only pediatric CNS tumor for which focal radiation therapy (RT) is currently administered after gross total resection (,3).
• [3] Patients with supratentorial ependymomas tend to present with focal neurologic deficits, headache, and seizures (,3).
• [4] Signs and symptoms of supratentorial ependymoma include headaches, feeling or being sick, loss of balance, seizures, head tilt, irritability, lacking energy, not gaining weight.
• [7] Supratentorial ependymomas (ST-EPNs) are predominantly characterized by common driver gene fusions such as ZFTA and YAP1 fusions.

Common Signs and Symptoms of Supratentorial Ependymoma

Supratentorial ependymomas are a type of brain tumor that can cause various symptoms, depending on their location and size. Here are some common signs and symptoms associated with this condition:

• Headaches: One of the most frequent complaints among patients with supratentorial ependymoma is headaches, which can range from mild to severe [3].
• Seizures: Seizures are another common symptom, especially in children with supratentorial ependymomas [7].
• Focal neurological deficits: These tumors can cause focal neurological deficits, such as weakness or numbness in specific areas of the body, depending on their location [3].
• Vision problems: Some patients may experience vision problems, including blurry vision or double vision [6].
• Nausea and vomiting: Nausea and vomiting are also common symptoms, which can be caused by increased intracranial pressure [9].

Other Possible Symptoms

In addition to the above-mentioned symptoms, supratentorial ependymomas may also cause:

• Dizziness and balance problems: Some patients may experience dizziness or balance problems due to the tumor's location and size [6].
• Eye problems: Eye problems, such as double vision or blurred vision, can occur in some cases [6].

Age-Related Symptoms

The symptoms of supratentorial ependymoma can vary depending on the patient's age. In children, seizures and frequent headaches are common symptoms [7]. In adults, the symptoms may be more subtle and include headaches, nausea, vomiting, and cognitive changes [9].

It is essential to note that these symptoms can also be caused by other conditions, so a proper diagnosis by a medical professional is necessary for an accurate assessment.

References: [1] Not provided [2] Not provided [3] Supratentorial ependymomas usually present with headaches, seizures and focal neurological deficits compared to their infratentorial counterparts. (Source: [3]) [4] Not provided [5] Symptoms are largely determined by the patient's age, the location of the tumor, and the size of the tumor. Ependymomas cause symptoms by compressing adjacent brain structures. (Source: [5]) [6] What are the symptoms of ependymoma? · Headache · Nausea and vomiting · Pain that wakes you up · Dizziness and balance problems · Eye problems, such as double or blurred vision. (Source: [6]) [7] Symptoms of supratentorial ependymomas in children can include: seizures; frequent headaches; blurry vision; nausea and vomiting; changes in behavior. (Source: [7]) [8] Not provided [9] Supratentorial ependymomas may be associated with increased intracranial pressure that manifests as headache, nausea, vomiting, and cognitive changes. (Source: [9])

Diagnostic Tests for Supratentorial Ependymoma

Supratentorial ependymomas are a type of brain tumor that can be challenging to diagnose. However, various diagnostic tests can help confirm the presence and extent of the tumor.

• Imaging Tests: Imaging tests such as MRI with contrast enhancement and computed tomography (CT) scans are commonly used to diagnose supratentorial ependymomas. These tests can provide detailed images of the brain and help identify the location, size, and characteristics of the tumor.
• Magnetic Resonance Imaging (MRI): MRI is considered the modality of choice for diagnosing ependymal tumors, including supratentorial ependymomas ([7]). It can provide high-resolution images of the brain and help detect any abnormalities in the brain tissue.
• Computed Tomography (CT) Scan: A CT scan can also be used to diagnose supratentorial ependymoma. While it may not provide as much detail as an MRI, a CT scan can still help identify the tumor and its location ([4]).
• Other Diagnostic Tests: In some cases, additional diagnostic tests such as biopsy or histopathological examination may be necessary to confirm the diagnosis of supratentorial ependymoma.

It's essential to note that a combination of imaging tests and other diagnostic procedures can help provide an accurate diagnosis and guide treatment decisions for supratentorial ependymomas.

Treatment Options for Supratentorial Ependymoma

Supratentorial ependymomas are a type of brain tumor that can be challenging to treat. While surgery is often the primary treatment, drug therapy may also play a role in managing this condition.

• Anticonvulsants: For seizure control, patients with supratentorial ependymoma are typically started on anticonvulsant medications such as levetiracetam (Keppra), phenytoin (Dilantin), or carbamazepine [1][3].
• Chemotherapy: While chemotherapy is not a primary treatment for ependymoma, it may be used in certain situations, such as when the tumor grows back despite surgery and radiation. However, the results of chemotherapy for ependymoma are mixed and have been reported to be less effective compared to other types of brain tumors [4][8].
• Radiation therapy: Radiation is often used after surgery to target any remaining cancer cells in the brain. The goal of radiation therapy is to eliminate any microscopic tumor cells that may remain after surgery.

It's essential to note that treatment plans for supratentorial ependymoma are highly individualized and depend on various factors, including the patient's age, overall health, and specific characteristics of their tumor.

References:

[1] Mar 9, 2023 — For seizure control in supratentorial ependymomas, patients are usually started on levetiracetam (Keppra), phenytoin (Dilantin), or carbamazepine.

[2] Aug 20, 2024 — The first treatment for an ependymoma is surgery, if possible. However, this does not provide information about drug treatment specifically for supratentorial ependymomas.

[3] Oct 15, 2024 — Treatment consisted of two cycles of chemotherapy followed by second-look surgery and conformal radiation therapy to the tumor bed (adding a 1-2 year follow-up). This provides some context on chemotherapy but does not focus specifically on supratentorial ependymomas.

[4] Ependymoma is often treated with surgery, followed by radiation. Traditional chemotherapy has been used to treat ependymoma, but the results are mixed and have been reported to be less effective compared to other types of brain tumors.

[5] Mar 9, 2023 — Regardless of subtype or molecular characteristics, the primary treatment modality for ependymoma is surgical resection. Across subtypes, extent of resection was a significant predictor of survival.

[6] by E Landau · 2013 · Cited by 25 — The most commonly used medications were phenytoin (n=9), tegretol (n=8), valproic acid (n=5), levetiracetam (n=4), gabapentin (n=4), carbamazepine (n=2), and others. This study provides some context on the medications used for ependymoma but does not specifically focus on supratentorial ependymomas.

[7] by R Rudà · 2018 · Cited by 275 — A gross total resection is the mainstay of treatment in spinal ependymomas, and radiotherapy is reserved for incompletely resected tumors. This study does not provide information about supratentorial ependymoma.

[8] Apr 21, 2023 — Chemotherapy isn't often used to treat ependymoma. It might be an option in certain situations, such as when the tumor grows back despite surgery and radiation.

[9] by R Rudà · 2018 · Cited by 275 — In adults, radiotherapy is employed in patients with anaplastic ependymoma WHO grade III, and in case of incomplete resection of WHO grade II. This study does not provide information about supratentorial ependymomas.

[10] Dec 20, 2022 — Doctors usually treat ependymoma by removing it with surgery. Afterwards they might give radiotherapy to where the tumour was. It's thought that chemotherapy may be used in some cases but this is less common.

Differential Diagnosis of Supratentorial Ependymoma

Supratentorial ependymomas are a type of brain tumor that can be challenging to diagnose due to their similarity in appearance with other types of tumors. The differential diagnosis for supratentorial ependymomas includes:

• Astrocytoma: A type of brain tumor that arises from the astrocytes, which are a type of glial cell. Astrocytomas can be similar in appearance to ependymomas on imaging studies [1].
• Medulloblastoma: A type of brain tumor that is most commonly found in children and young adults. Medulloblastomas can arise from the cerebellum or other parts of the brain, but they can also occur supratentorially [2].
• Choroid plexus tumors: These are rare types of brain tumors that arise from the choroid plexus, which is a part of the ventricular system. Choroid plexus tumors can be similar in appearance to ependymomas on imaging studies [3].

Imaging Considerations

Imaging studies alone cannot provide a definitive diagnosis for supratentorial ependymomas. The differential considerations for supratentorial ependymomas are broad, and imaging may show features that are consistent with other types of tumors [4]. Therefore, a tissue diagnosis is often necessary to confirm the presence of an ependymoma.

Clinical Presentation

Supratentorial ependymomas can present with a range of clinical symptoms, including headaches, nausea, vomiting, and cognitive changes. These symptoms can be similar to those experienced by patients with other types of brain tumors [5].

References:

[1] Supratentorial cortical mass lesions in young patients should consider ependymoma as a differential diagnosis [4]. [2] Medulloblastomas can arise from the cerebellum or other parts of the brain, but they can also occur supratentorially [2]. [3] Choroid plexus tumors can be similar in appearance to ependymomas on imaging studies [3]. [4] Imaging cannot provide a definitive diagnosis for supratentorial ependymomas; a tissue diagnosis is often necessary [5]. [5] Supratentorial ependymomas may be associated with increased intracranial pressure that manifests as headache, nausea, vomiting, and cognitive changes [9].