Removing a Myopapillary Ependymoma requires a skilled surgeon like those at the Southwest Scoliosis and Spine Institute
Myxopapillary Ependymoma
A different variety of spinal cord ependymomas with a preference for the lumbosacral area refers to a Myxopapillary Ependymoma tumor. Only 15% of ependymomas identify as Myxopapillary Ependymomas (MPEs), which doctors rarely see. Myxopapillary and subependymoma are the two varieties of Grades 1 ependymoma. These tumors classify as extremely rare in adulthood.
Doctors identify MPEs as low-grade tumors and the World Health Organization (WHO) classifies them as grade 1 tumors. The typical presentation age ranges from 30 to 50. Nearly all intradural MPEs happen at the conus medullaris or filum terminale. The sacrococcygeal area is where extraspinal MPEs most frequently manifest as subcutaneous soft tissues. Rarely, it may also appear in the brain. The microscopic appearance of MPE reveals the creation of pseudopapillaries, symmetrical zones of mucoid matrix around branching tumescent arteries, and mucin buildup inside and between tumor cells.
The coccygeal medullary vestige or the extradural vestiges of the filum terminale are the origin of extradural Myxopapillary Ependymomas. Myxopapillary Ependymoma tumors retain a sausage form and are frequently encapsulated. They maintain a delicate consistency and appear crimson to purple in hue. These tumors can occasionally exhibit mucin degradation or bleeding.
Questions and Answers
What is a Myxopapillary Ependymoma?
Myxopapillary ependymoma refers to a type of ependymoma, a rare tumor that arises from the ependymal cells lining the central canal of the spinal cord. Doctors find the tumor in the lower part of the spinal cord, particularly the lumbar region. Myxopapillary ependymomas grow slowly and doctors consider them as low-grade tumors, meaning their removal brings positive results.
What are the Symptoms of Myxopapillary Ependymoma?
The symptoms of Myxopapillary Ependymoma relate to its location in the spinal cord. Common symptoms may include back pain, leg pain or weakness, difficulty walking, changes in bowel or bladder function, and sensory abnormalities in the lower extremities. The specific symptoms can vary depending on the size and location of the tumor and the pressure it exerts on surrounding structures.
How is Myxopapillary Ependymoma Treated?
Doctors treat Myxopapillary Ependymomas by surgically removing them. The goal of surgery is to achieve complete resection while preserving neurological function. In some cases, if complete resection cannot occur due to the tumor’s location or involvement with critical structures, a surgeon will remove as much as possible — followed by other treatment options such as radiation therapy. Surgeons and their multidisciplinary team of specialists will make treatment decisions based on the extent of surgery conducted and the need for adjuvant treatments.
Treatment for Ependymoma
Each patient’s course of treatment for Myxopapillary Ependymal tumors will depend on the individual and the location of the tumor. Surgery and radiation treatment are the two primary treatments utilized to treat Myxopapillary Ependymoma tumors. Adjuvant treatment for MPE is crucial since the majority of Myxopapillary Ependymomas are situated in areas where total resection after surgery becomes less possible. The nature and location of the tumor will provide information about the possibility of any postoperative complications.
After complete tumor excision, the long-term survival rate in adjuvant therapies, which combine radiation and surgery, was about 40 percent. Surgeons use the conventional treatment to remove Myxopapillary Ependymomas by surgery combined with irradiation to the affected region because the probability of recurrence with only surgical treatment occurs too often. It goes without saying that assessing the state of adjuvant therapies can lead to the development of success by increasing positive results.
The Categories of Ependymomas
Healthcare providers rate ependymoma on a scale of 1 to 3. Grade 1 tumors are the slowest growing and grade 3 tumors are the fastest growing. There are different types of ependymoma, including:
Grade 1:
A grade 1 tumor called a subependymoma develops close to the ventricles, which house the cerebrospinal fluid. Adults are more likely than kids to experience it. Adult men appear more likely than women to develop grade 1 Myxopapillary Ependymoma in their lower spinal cord.
Grade 2:
The most prevalent ependymoma is the classic kind (grade 2), which can affect both adults and children.
Grade 3:
The grade 3 tumor anaplastic ependymoma frequently develops close to the base of the brain and spreads to different regions of the brain. After treatment, these cancers frequently recur.
Symptoms of Myxopapillary Ependymoma
Myxopapillary Ependymomas have a non-specific clinical appearance that compares to other intradural tumors in the area of the filum terminale, which lies underneath the spinal cord. The tumor’s size, location of presentation, and local expansion all have an impact on the symptoms associated with it. Clinically, Myxopapillary Ependymomas progress slowly. The majority of patients come with the following vague symptoms and wait months to years for a diagnosis.
- The most common patient complaints consist of back pain that worsens at night and when resting.
- The most common patient complaints consist of Motor, sensory, urogenital, and gait problems
- Other signs include scalloping of the vertebral body, expansion of the neural foramina, and scoliosis.
- Hemorrhage can cause the symptoms to suddenly worsen, including leg weakness and sphincter abnormalities.
Initial Treatment of Myxopapillary Ependymomas
Radiotherapy and surgery provide two major methods of treating Myxopapillary Ependymoma tumors. In order to get a histologic diagnosis and remove most of the tumor, surgery becomes an essential step in treating the primary spinal ependymoma. In circumstances where a tumor does not spread, studies reveal that surgery produces the best results. Radiotherapy provides an adjuvant to surgery, and when doctors believe surgery should not be performed, this therapy becomes the primary form of treatment.
Surgery
Surgically removing a myxopapillary ependymoma from the spine can be a complex procedure due to the tumor’s location and potential involvement with nearby structures. However, with appropriate surgical planning and the expertise of a skilled surgeon, the procedure provides positive outcomes. Here are some important points to consider regarding the surgical removal of myxopapillary ependymomas:
- Tumor Location: Myxopapillary ependymomas typically occur in the lower part of the spine, most commonly in the lumbar region. The tumor may arise within the spinal cord itself or extend into the surrounding structures, such as the nerve roots or spinal canal. The precise location and involvement of adjacent structures will influence the complexity of the surgery.
- Surgical Approach: The choice of surgical approach depends on factors such as tumor size, location, and the patient’s individual characteristics. The two main approaches used for spinal tumor removal are the traditional open approach and minimally invasive techniques. The surgeon will assess the situation and determine the most appropriate approach to maximize tumor removal while minimizing potential risks to the surrounding structures.
- Surgical Considerations: During the surgery, the primary goal is to achieve complete resection of the tumor while preserving neurological function. However, the surgeon must exercise caution due to the tumor’s proximity to critical structures, such as the spinal cord and nerve roots. In cases where complete resection is not feasible without risking neurological deficits, surgeons sometimes perform a partial resection, followed by additional treatment options such as radiation therapy.
- Postoperative Care: After the surgery, patients typically require close monitoring and postoperative care to ensure proper healing and recovery. This may involve pain management, physical therapy, and close follow-up with the surgical team to monitor for any potential complications or tumor recurrence.
Myxopapillary Ependymoma Treated with Adjuvant Radiotherapy
Surgical resection is considered the treatment for MPE, but adjuvant radiation therapy (RT) has helped prolong progression-free survival in pediatric and adult patients. RT also provides patients with avoiding the tumor’s recurrence after surgery. Also, the use of 3D conformal radiation or IMRT is appropriate and is under investigation. In addition, a dose of 5400 cGy is suitable for spinal Myxopapillary Ependymoma. A number of studies have examined the effects of RT on the treatment of MPE in adults and children.
They showed that RT was highly effective in controlling residual, metastatic, and/or recurrent disease and that routine adjuvant RT could improve outcomes in pediatric MPE. On the other hand, the degree of resection correlates with outcomes following MPE resection, but the impact of the capsular breach remains uncertain. However, Abdulaziz and his team looked at the significance of capsule integrity after Myxopapillary Ependymoma excision. They discovered a strong association between Myxopapillary Ependymoma recurrence and capsular violation.
Pediatric Myxopapillary Ependymoma Treated with Adjuvant Radiotherapy
Successful treatments for pediatric patients continue as a difficult challenge as they typically have lower outcomes. Even after GTR of the tumor, several publications recommend using adjuvant radiation in pediatric patients. 100% of patients survived when GTR was combined with adjuvant RT. Therefore, in children with Myxopapillary Ependymoma, RT becomes necessary as the next step after GTR.
In a related investigation, Doctors studies the impact of adjuvant RT in 16 juvenile MPE patients. The first form of treatment for all patients was either GTR or a subtotal resection (STR) operation. As a result, doctors concluded that pediatric patients with MPE should undergo adjuvant RT following surgical resection since it improved significantly improved survival rates. Furthermore, the survival percentage for GTR patients was close to 100%. However, the probability of survival with STR plus radiation therapy was 91%.
Other Adjuvant Therapy
Temozolomide, a drug used to treat glioblastoma, recently reported scientific information about it being effective with low-grade spinal cord gliomas, including spinal cord intramedullary ependymoma. However, at this time, scientists and doctors do know if TMZ is effective for Myxopapillary Ependymoma. To this end, TMZ has been presented as a possible option for adjuvant therapy in multiple recurrent Myxopapillary Ependymomas. TMZ showed significant efficacy in recurrent spinal cord Myxopapillary Ependymoma.
Conclusion
As previously stated, treating Myxopapillary Ependymoma tumors that are located in or on the spine are complex medical problems that require skill and expertise. The surgeons at the Southwest Scoliosis and Spine Institute have the knowledge, skills, and abilities to treat these tumors. With offices in Dallas, Plano, and Frisco, Texas, the doctors at the Southwest Scoliosis and Spine Institute specialize in the diagnosis and treatment of spinal conditions, including scoliosis, kyphosis, and other spinal deformities.
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National Institute of Health: Myxopapillary Ependymoma
If you or a loved one suffers from spinal pain, you owe it to yourself to call Southwest Scoliosis and Spine Institute at 214-556-0555 to make an appointment.