Subependymoma

Alternate names

Subependymal astrocytoma (formerly)

Definition

Subependymomas are slow-growing brain tumors that are usually benign. They are most often found in the fourth or lateral ventricles in the brain, but may occur in the spine. It is composed of glial tumor cell clusters embedded in an abundant fibrillary matrix with frequent microcystic change. Some lesions have the histological features of both subependymoma and ependymoma. It is often detected incidentally and has a very favorable prognosis. (adapted from who.)

Cause

The cause of subependymomas is not well understood.

Inheritance

• In the vast majority of cases, subependymomas develop sporadically; this means that they usually are not inherited and occur in people with no family history of brain tumors.
• A few familial cases have been reported.
• However, to our knowledge, specific genes known to cause subependymomas have not been identified.

Signs and symptoms

• Symptoms depend on the tumor location.
• Some people do not have symptoms, while others may have headaches, changes in vision, and/or difficulty with balance.
• These symptoms are thought to be due to obstruction of cerebral spinal fluid (CSF) due to the tumor's location.
• Most subependymomas are benign with a low recurrence rate.
• However, a few progress to anaplastic ependymoma (a faster growing tumor).

Clinical presnetation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 100% of people have these symptoms

• Ependymoma

30%-79% of people have these symptoms

• Abnormal cell morphology
• Migraine(Intermittent migraine headaches)
• Pain

5%-29% of people have these symptoms

• Distal muscle weakness(Weakness of outermost muscles)
• Dysesthesia
• Gait disturbance(Abnormal gait)
• Seizure
• Spinal cord tumor(Tumor of the spinal cord)
• Supratentorial neoplasm
• Vomiting(Throwing up)

1%-4% of people have these symptoms

• Neoplasm of the breast(Breast tumor)
• Neoplasm of the liver(Liver cancer)
• Neoplasm of the lung(Lung tumor)
• Ovarian neoplasm(Ovarian tumor)

Diagnosis

• The diagnosis is based on tissue, e.g. a biopsy.
• Histologically subependymomas consistent of microcystic spaces and bland appearing cells without appreciable nuclear atypia or mitoses. The nuclei tend to form clusters.
• On a CT, it often shows a less dense to equalle dense mass.
• If it is big, it may have parts that are cystic or calcific.
• In 50-60% of cases, the tumor is in the fourth ventricle, while the second most common (30-40% of cases) location is the side ventricles.
• It is rare for it to be in the third ventricle or the central canal of the spinal cord.

Treatment

Surgery to remove the tumor is often curative.

NIH genetic and rare disease info

• NIH info on Subependymoma

Subependymoma is a rare disease.

Subependymoma Resources
Latest articles Most recent articles Ongoing trials	Wikipedia

Subependymoma Resources
Latest articles Most recent articles Ongoing trials	Wikipedia

Subependymoma Resources
Latest articles Most recent articles Ongoing trials	Wikipedia