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Ependymoma is a cancer that occurs in the central nervous system. Its second name, ependymoma, comes from the name of the tissue it is derived from, the ependyms. It lines the ventricles of the brain and the lumen of the spinal cord canal. What are the causes and symptoms of ependymoma? How is his treatment going?

Ependymomasin the WHO grade classification were classified as neoplasms IIo . These tumors are characteristic primarily of young adults and children, in which they account for approximately 10 percent of all tumors in the central nervous system. There are two peaks of ependymoma, ages 5 and 35. In children and adolescents, ependymomas are most often found intracranially, and in adults - intramedullary. Sometimes ependymomas accompany type two neurofibromatosis.

Types of ependymomas

Ependymomas are classified into four main types:

  • sub-ependymomas and myxoma-papillomatous ependymomas, which grow slowly and are among the mildest (Grade I)
  • common ependymomas, the most common which have several subtypes (grade II)
  • anaplastic ependymomas (grade III), which are characterized by the fastest growth and the most aggressive course.

Ependymoma: etiology

The causes of ependymomas have not been described in detail so far. Many mutations have been found that could cause this type of cancer, but none of them are closely related to the occurrence of ependymomas. Also, it has not been proven that any environmental factors play a role in their etiology.

Location of ependymomas

Sub-ependymomas are most often located near the ventricular system. In some cases, they may appear in the cervical spinal canal. This location is typical for people around the age of 40 and is more likely to be of the male gender.

The location of ependymomas largely depends on the type.

Myxoma-papillomatous ependymomas tend to form at the bottom of the spinal canal. Ependymomas usually grow near, inside or along the ventricles of the brain on different levels. Anaplastic ependymomas are usually seen in children,most often in the posterior fossa of the skull.

Ependymomas: microscopic and macroscopic images

Ependymomas are soft, grayish or red bumps that can contain calcification. Their characteristic feature is the presence of real rosettes and pseudo-rosettes in the microscopic image, which are located mainly around small cavities, crevices or vessels.

Ependymomas: clinical symptoms

The clinical picture of ependymomas is very similar to the symptoms of all other tumors of the central nervous system. It largely depends on the patient's age, location and size of the tumor.

In children, an increase in cranial pressure will primarily cause severe headache, nausea and vomiting. When the cranial sutures are not yet fully fused, the first symptom may be hydrocephalus, caused by blockage of the cerebrospinal fluid flow in the ventricular system by the growing tumor. Disturbances in concentration, personality and mood changes may also appear. As the tumor grows, pressure on the brain will cause seizures, focal neurological symptoms, or symptoms of paralysis of the cranial nerves. If the tumor is located above the brain tent, it can cause loss of vision, sensation, cognitive dysfunction, or ataxia.

Ependymomas growing in the spinal canal will damage the function of the peripheral nervous system. This may result in spastic paralysis of the limbs and paraesthesia. The patient may also complain of pain located below the existing neoplastic lesion. Myxoma-papillomatous ependymoma is characterized by a possibly slightly different clinical picture. Their typical location in the lower section of the spinal canal will cause bladder dysfunction and impotence. You will also experience pain in the back, legs and anal area.

In addition to the symptoms listed above, you may complain of a loss of appetite, weight loss, temporary difficulty recognizing colors, uncontrolled tremors, temporary memory loss, or the sensation of seeing vertical or horizontal lines in high light.

Ependymoma diagnosis

When ependymoma is suspected, computed tomography is usually the first examination ordered by the attending physician. It reveals a well-defined mass that is located intraventricularly. This mass is hyperdense, it may contain cysts. Calcification is found in 50-80% of cases. Entering a contrast enhances the ependymoma image. Magnetic resonance imaging, both with and without contrast, is an even more precise examination that allows precise determinationthe size and nature of the lesion. The MRI signal may be heterogeneous when the tumor has cysts, calcification, bleeding and necrotic foci. Histological examination of the excised lesion makes it possible to accurately determine the type of ependymoma. It should be remembered that the collection of cerebrospinal fluid is contraindicated in suspected central nervous system neoplasm, as increased intracranial pressure after puncture may cause intussusception of the cerebellar tonsils.

Treatment of ependymomas

The method of treatment that allows for complete recovery is, of course, the excision of the entire tumor. Complementary radiotherapy is mandatory after resection. Due to the different location, such an operation is not always possible, then radiotherapy itself remains the treatment of choice. In young children, up to the age of three, chemotherapy with platinum compounds (cisplatin, carboplatin) is recommended.

Ependymomas: prognosis

Factors determining the prognosis include the radical nature of the operation, histological maturity, the type of neoplasm and its location, as well as the age of the patient. It is estimated that the probability of five-year survival is 40 to 60 percent, which is significantly greater in adults than in children. Unfortunately, patients with anaplastic ependymomas live much shorter lives - usually from 12 to 20 months.

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