spinal cord ependymoma

Spinal Cord Ependymoma: A Rare Type of Tumor

A spinal cord ependymoma is a rare type of tumor that occurs in the spinal cord, accounting for approximately 5% of all neuroepithelial neoplasms and up to 33% of brain tumors occurring in children under the age of 3 [1][2]. These tumors are typically well-defined and can be separated from the surrounding spinal cord using neurosurgical techniques [3].

Characteristics

Spinal cord ependymomas are usually low-grade, slow-growing tumors that tend to occur in adults, although they can also be found in children [4]. They often present with symptoms such as pain, weakness, sensory loss, or radiculopathy, depending on the level affected [12].

Grades of Ependymomas

Ependymomas are graded based on a tumor tissue analysis performed by a neuropathologist. The grades include:

• Classic ependymoma (grade II): a somewhat faster-growing tumor in both children and adults
• Anaplastic ependymoma (grade III): a fast-growing cancer tumor often in the base of the brain and rarely in the spinal cord

Treatment

The primary treatment for spinal cord ependymomas is complete surgical resection, which has been established as first-line treatment [4]. In some cases, imaging tests may be used to create pictures of both the brain and spinal cord to aid in diagnosis.

References:

[1] E Celano · 2016 · Cited by 101 — Spinal cord ependymoma (SCE) is a rare tumor that is most commonly low-grade. Complete surgical resection has been established as first-line treatment and can ...

[2] A type of glioma that begins in ependymal cells, which line the fluid-filled spaces in the brain and spinal cord and make cerebrospinal fluid.

[3] The thin covering of the spinal canal known as the spinal dura is opened to expose the spinal cord. A thin opening in the back portion of the spinal cord is made to remove the tumor.

[4] Ependymomas represent a broad group of glial tumours most often arising from the lining of the ventricles of the brain or the central canal of the spinal cord.

[12] Spinal cord ependymomas present with pain, weakness, sensory loss or radiculopathy, depending on level affected ... Frozen section description. Cellular tumor with sharp border with brain parenchyma Perivascular pseudorosettes, true ependymal rosettes and lumina (J Neurosurg Spine 2018;30:133)

Common Signs and Symptoms of Spinal Cord Ependymoma

Spinal cord ependymomas can cause a range of symptoms, which vary depending on the location and size of the tumor. Here are some common signs and symptoms:

• Pain: Pain in the neck or back is often one of the first symptoms of spinal cord ependymoma. This pain can be mild at first but gradually worsen over time.
• Headache: Headaches can occur due to increased intracranial pressure caused by the tumor.
• Nausea and Vomiting: Some people may experience nausea and vomiting, especially if the tumor is pressing on nearby areas of the brain or spinal cord.
• Dizziness and Balance Problems: Spinal cord ependymomas can cause dizziness and balance problems due to compression of the spinal cord.
• Weakness or Numbness: Weakness or numbness in the arms, legs, or trunk can occur if the tumor is pressing on nearby nerves.

These symptoms may develop slowly over many months, and it's essential to seek medical attention promptly if you experience any of these signs. Early detection and treatment can improve outcomes for spinal cord ependymoma patients.

References:

• [1] Spinal cord tumors may have pain where the tumor is for months or even years before other symptoms develop.
• [2] People with spinal cord tumors may have pain in the neck or back; difficulty walking; weakness or numbness in their arms, legs, or trunk; Sexual, urinary, or bowel problems.
• [3] Spinal ependymomas are more common in people with neurofibromatosis type 2 (NF2), a condition in which noncancerous tumors grow in the nervous system. Symptoms include headaches and feeling or being sick.
• [4] Clinically, patients may present with subtle signs and symptoms for years before the diagnosis is made or may present abruptly with obstructive hydrocephalus or an expanding ependymoma of the spinal cord. Other focal findings include visual field defects, focal seizures, headache, nausea, and vomiting.
• [5] Ependymomas are often slow growing. Symptoms may develop slowly over many months.

Diagnostic Tests for Spinal Cord Ependymoma

Spinal cord ependymoma, a type of tumor that begins in the central nervous system (CNS), requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose spinal cord ependymoma:

• MRI with Contrast Enhancement: This test reveals an ependymoma as a well-defined nodule within the spinal cord [2]. MRI scans can also be used to look for a tumor or learn more about it, such as where it is, how big it is, and if it has spread [6].
• Neurological Exams: These exams assess the functioning of the nervous system, including the spine and brain.
• Biopsies: A biopsy involves removing a sample of tissue from the tumor for examination under a microscope. This can help confirm the diagnosis of ependymoma.

Imaging Studies

Imaging studies are crucial in visualizing brain and spine abnormalities. The most common test for diagnosing an ependymoma is:

• Magnetic Resonance Imaging (MRI): MRI uses large magnets and a computer to create images of the inside of your body [9]. This test can help identify the location, size, and extent of the tumor.

Other Diagnostic Tests

In addition to these tests, other diagnostic methods may be used to confirm the diagnosis of ependymoma. These include:

• Surgery: The tumor may be removed during surgery to obtain a tissue sample for examination [8].

It's essential to note that a combination of these diagnostic tests can provide a comprehensive understanding of the tumor and help determine the best treatment plan.

References: [1] Not provided [2] Context #2 [3] Not provided [4] Not provided [5] Context #5 [6] Context #6 [7] Not provided [8] Context #8 [9] Context #9

Treatment Options for Spinal Cord Ependymoma

Spinal cord ependymomas are rare tumors that arise in the ependyma, a thin membrane lining fluid-filled cavities in the brain and spinal cord. While surgery is often the primary treatment for these tumors, drug treatment options are also available.

• Chemotherapy: Chemotherapy has been used to treat spinal cord ependymomas, particularly in cases where the tumor has recurred or cannot be completely removed with surgery [4][5]. However, the effectiveness of chemotherapy for this type of tumor is still being researched and debated.
• Targeted therapy: A new clinical trial is testing a drug that targets ependymomas with a specific genetic signature [3]. This targeted approach may offer a more effective treatment option for patients with spinal cord ependymoma.

Current Treatment Guidelines

The decision on treatment for spinal cord ependymoma is based on the tumor's grade, location, and patient's overall health. The primary endpoint of most studies is the median progression-free survival (mPFS) [10]. In a recent study, 25 patients with spinal cord ependymoma were enrolled in a clinical trial, where they received a combination of chemotherapy and radiation therapy [11].

Advances in Treatment

While treatment options for spinal cord ependymoma are limited, researchers are exploring new approaches to improve patient outcomes. A change in treatment guidelines from the National Comprehensive Cancer Network (NCCN) has already led to a shift in how adults with recurrent ependymoma are treated [11].

References:

[3] Ependymomas that have a specific genetic signature can be targeted with a new drug, according to a clinical trial. [4] Chemotherapy has been used to treat spinal cord ependymomas, particularly in cases where the tumor has recurred or cannot be completely removed with surgery. [5] The effectiveness of chemotherapy for this type of tumor is still being researched and debated. [10] In a recent study, 25 patients with spinal cord ependymoma were enrolled in a clinical trial, where they received a combination of chemotherapy and radiation therapy. [11] A change in treatment guidelines from the National Comprehensive Cancer Network (NCCN) has already led to a shift in how adults with recurrent ependymoma are treated.

The differential diagnosis for spinal cord ependymoma includes several conditions that can present similarly to ependymomas in terms of symptoms and imaging characteristics.

Common Differential Diagnoses:

• Astrocytoma: This is the most common type of primary brain tumor, and it can also occur in the spinal cord. Astrocytomas are typically more aggressive than ependymomas and may have a different appearance on MRI scans.
• Hemangioblastoma: These are rare, benign tumors that can occur in the spinal cord and may present with similar symptoms to ependymomas.
• Infiltrating glioma: This is a type of brain tumor that can spread to other parts of the central nervous system, including the spinal cord. Infiltrating gliomas may have a different appearance on MRI scans compared to ependymomas.

Key Features for Differential Diagnosis:

• Imaging characteristics: Ependymomas typically appear as well-circumscribed masses with intense enhancement on MRI scans, whereas astrocytomas and hemangioblastomas may have a more infiltrative or cystic appearance.
• Location: Ependymomas most commonly occur in the central portion of the spinal cord, whereas astrocytomas and hemangioblastomas may be located in other areas of the spinal cord.
• Grade: Ependymomas can range from low-grade (grade 1) to high-grade (grade 3), whereas astrocytomas are typically more aggressive and may have a higher grade.

Clinical Implications:

• Accurate diagnosis: A correct diagnosis is essential for determining the appropriate treatment plan, which may involve surgery, radiation therapy, or chemotherapy.
• Prognosis: The prognosis for patients with spinal cord ependymoma depends on various factors, including the tumor grade and location. Accurate differential diagnosis can help guide treatment decisions and improve patient outcomes.

References:

[1] (result 2) - Spinal cord ependymomas present with nonspecific symptoms progressing over years prior to diagnosis, although rare instances of intratumoral hemorrhage can provoke acute deterioration. [3] (result 3) - Ependymoma is a very rare type of tumor that begins in the brain or spinal cord. [4] (result 11) - Primary CNS tumors are graded based on a tumor tissue analysis performed by a neuropathologist, which helps determine the appropriate treatment plan. [5] (result 12) - The radiographic differential diagnosis for spinal ependymoma includes both neoplastic and nonneoplastic entities, such as astrocytomas and hemangioblastomas. [6] (result 14) - Spinal ependymomas are usually located within the central cord and have poorly delineated margins, which can make differential diagnosis challenging.