MYXOPAPILLARY EPENDYMOMA

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 are Myxopapillary Ependymomas (MPEs), which are rare. Myxopapillary and subependymoma are the two varieties of Grades 1 ependymoma. These tumors are extremely rare in adulthood. 

MPEs are 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 creation of pseudopapillaries, symmetrical zones of mucoid matrix around branching tumescent arteries, and mucin buildup inside and between tumor cells are features of the microscopic appearance of MPE.

The coccygeal medullary vestige or the extradural vestiges of the filum terminale are the origin of extradural Myxopapillary Ependymomas. Myxopapillary Ependymoma tumors have a sausage form and are frequently encapsulated. They have a delicate consistency and are crimson to purple in hue. These tumors can occasionally exhibit mucin degradation or bleeding.

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.

What are the Categories of Ependymomas?

Health care 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 are 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.

• Radicular and back pain that worsens at night and when they are resting are the most common complaints of patients. 
• Motor, sensory, urogenital, and gait problems are common complaints among patients.
• 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 are the 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 the tumor has not spread, studies reveal that surgery produces the best results.  Radiotherapy provides an adjuvant to surgery, and when surgery cannot be performed it becomes the primary form of treatment.  

Surgeons know that removing as much tumor tissue as feasible during surgery becomes crucial for patient survival, lowering the need for further treatment, and improving radiotherapy’s efficacy. GTR has shown better therapeutic outcomes compared to subtotal resection (STR) in stopping the tumor from growing or spreading. Moreover, the largest possible surgical tumor resection can play an important factor in determining prognosis. In addition, a spinal Myxopapillary Ependymoma can invade the spinal cord, medullary pyramids, and nerve roots. 

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 tumors 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, the impact of 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 continues 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, has recently been reported as effective with low-grade spinal cord gliomas, including spinal cord intramedullary ependymoma. However, there are no reports on the effectiveness of TMZ 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.