Treatment of Medulloepithelioma

About the disease

Medulloepithelioma is a rare tumour that develops when neoplasm forms in the intracranial area. It usually develops in the ciliary body, which is the part of the eye that is responsible for controlling the shape of the lens. It is also part of the process that delivers oxygen and essential nutrients to the eye. Medulloepithelioma can also develop in the brain. It is mostly a congenital or infantile condition, meaning that it is usually present from birth or develops during infancy. The average age of children diagnosed with medulloepithelioma is 5 years. It is rare for anyone over the age of 7 to develop this condition, but there have been very rare cases reported in adults. Age, sex and race seem to have no bearing on who is more at risk of developing medulloepithelioma.

Medulloepithelioma is often mistaken for retinoblastoma at first, as these two conditions manifestation themselves in a similar way. However, it is important to make the distinction, since medulloepithelioma is considered to be highly malignant and can metastasize to other tissue and even the bones. If not treated in time, medulloepithelioma can also result in glaucoma. Without treatment, the average survival time for the patient is approximately 5 months, because the tumor is so aggressive. With timely treatment, however, the prognosis is favorable.

Symptoms

for medulloepithelioma of the eye:

• Redness in the eye
• Iris changes color
• Vision problems
• Mass in the iris

for medulloepithelioma of the brain:

• Headaches
• Dizziness
• Weakness
• Fatigue
• Moodswings
• Vision problems in some cases

Diagnosis

• During a general examination, the doctor will examine the patient’s eyes to determine whether there is any redness.
• The doctor will also check the patient’s acuity of vision.
• Imaging tests, such as MRI and CT scans, are useful in establishing whether or not the patient has a tumour and if so, where it is located and how big it is.
• A biopsy is performed so that a tissue sample can be examined under the microscope. This will determine whether or not the tumour is malignant.

Treatment

• A total resection to completely remove the tumor is usually necessary to prevent it from spreading further.
• Radiotherapy is used may also be necessary in some cases, in order to shrink the tumor and prevent it from growing.

Authors: Dr. Vadim Zhiliuk, Dr. Sergey Pashchenko