Other brain and spinal cord tumours

There are many less common types of brain and spinal cord tumours that occur in adults.

Acoustic neuromas (also called vestibular schwannomas) are low-grade tumours. They develop in Schwann cells. These cells make up a protective covering around nerves. Acoustic neuroma begins in the vestibular nerve in the middle ear that helps control balance.

Acoustic neuromas are found most often in people between the ages of 30 and 60 and occur twice as often in women. People with neurofibromatosis have an increased risk of developing acoustic neuroma.

The most common symptom of acoustic neuroma is hearing loss in 1 ear.

The following are treatments for acoustic neuroma:

• Active surveillance with regular imaging tests may be done to watch the growth of the tumour. This may be an option for older people with a small tumour or people with multiple health problems. Treatment may begin when symptoms appear.
• Surgery is used to remove the tumour.
• Stereotactic radiosurgery may be used instead of surgery for small tumours.

Brain stem gliomas are astrocytomas that start in the brain stem. They can be low or high grade, ranging from grades 1–3. These tumours are rare in adults. When they do occur in adults, it is most often in adults who are between 30 and 40 years of age.

The following are treatments for brain stem glioma:

• Radiation therapy is the main treatment for brain stem glioma in adults.
• Surgery may be done if the tumour has not spread. But most tumours spread to different areas of the brain stem and can’t be completely removed.

Chondrosarcomas develop in the bones. These tumours are made up mostly of cartilage. Chondrosarcomas are most often found in the bony ridge running along the back of the eyes or the bony area at the base of the skull. These tumours tend to be low grade.

Treatment for chondrosarcoma is surgery to remove as much of the tumour as possible. Radiation therapy may be given after surgery.

Chordomas are low-grade tumours. They are most often found in the lower end of the spine, the upper back or neck or the base of the skull. Chordomas can grow to a large size and may occur as a single tumour or as multiple tumours. They can spread to nearby tissue and often come back after treatment (recur).

The following are treatments for chordoma:

• Surgery is done to remove as much of the tumour as possible while maintaining normal function of the spine (called a laminectomy).
• Radiation therapy may be given after surgery if the tumour can’t be completely removed. It may also be used to treat tumours that are in an area where surgery can’t be done.

Choroid plexus carcinomas are high-grade tumours. They start in the tissue of the choroid plexus, which is located in each of the ventricles. Choroid plexus carcinomas are most common in children, but may occur in adults. The tumour often grows into nearby tissue and is spread by the cerebrospinal fluid.

Treatments for choroid plexus carcinoma may include surgery, radiation therapy and chemotherapy.

Craniopharyngiomas are low-grade tumours that grow at the base of the brain near the pituitary gland. Most craniopharyngiomas are made up of cysts. Craniopharyngiomas are more common in children, but can be found in adults of any age. These tumours can affect the normal functions of the pituitary gland causing hormone problems. They can also damage nerves in the area, mainly the optic nerve, which is responsible for sight.

Treatment for craniopharyngiomas is surgery to remove as much of the tumour as possible. Radiation therapy may be given after surgery if the tumour can’t be completely removed.

Germ cells normally develop into eggs in women and sperm in men. During normal development, germ cells travel to either the ovaries, where they mature into eggs, or to the testicles, where they mature into sperm. Sometimes a few germ cells do not move to the ovaries or testicles as they should and end up in abnormal places, such as the brain. In some cases, these cells develop into a tumour, usually near the pineal gland in the centre of the brain.

Germ cell tumours most often occur in children, but may be found in adults under the age of 30. The tumours can be low grade or high grade and can spread to other parts of the brain and spinal cord.

Treatment for germ cell tumours is radiation therapy, with or without chemotherapy.

Gliomatosis cerebri is a widespread, low-grade glioma. It typically involves 2 or more lobes of the brain. Because the tumour is widespread, surgery usually can’t be done.

Treatments for gliomatosis cerebri may include radiation therapy and chemotherapy.

A glomus jugulare tumour, also called paraganglioma, is a low-grade tumour. These tumours start in the nerve that runs through the temporal bone of the skull, near the middle ear. They are most often found in people around the age of 50.

The following are treatments for glomus jugulare tumours:

• Surgery may be done to remove all of the tumour or as much of the tumour as possible.
• Radiation therapy may be given as the main treatment instead of surgery. It may also be used after surgery if the tumour can’t be completely removed.

Hemangiomas are a non-cancerous, abnormal buildup of blood vessels. They are not related to hemangioblastomas or hemangiopericytomas.

The treatment for hemangioma is active surveillance or surgery.

Hemangioblastomas are low-grade tumours that start in the linings of blood vessels and may contain cysts. They are most often found in the cerebellum or brain stem but can occur in the cerebrum and spinal cord. Hemangioblastoma occurs most often in people around the age of 40. People with von Hippel-Lindau (VHL) syndrome have an increased risk of developing hemangioblastomas.

The following are treatments for hemangioblastomas:

• Surgery is done to remove all of the tumour or as much of the tumour as possible.
• Radiation therapy may be given after surgery if the tumour can’t be completely removed or if the tumour is in an area where surgery can’t be done.

Hemangiopericytomas (solitary fibrous tumours) are grade 2 or grade 3 tumours that develop in the membranes that cover and protect the brain and spinal cord (meninges). Although these tumours do not spread to the brain, they can spread to other parts of the body such as to the bone, lung or liver. Hemangiopericytomas often come back after treatment near the original location of the tumour.

The following are treatments for hemangiopericytomas:

• Surgery is done to remove all of the tumour or as much of the tumour as possible.
• Radiation therapy may be given after surgery if the tumour can’t be completely removed or if the tumour is in an area where surgery can’t be done.

Medulloblastomas are high-grade tumours that develop from neuroectodermal cells (primitive nerve cells) in the cerebellum. They are part of a class of tumours called primitive neuroectodermal tumours (PNETs). Medulloblastomas occur more often in children than in adults. They frequently spread through the cerebrospinal fluid to other parts of the central nervous system.

The following are treatments for medulloblastomas:

• Surgery is done to remove as much of the tumour as possible.
• Radiation therapy may be given to the area around the primary tumour. A lower dose of radiation may be given to the whole brain and spinal cord after surgery if there is no evidence that the tumour has spread.
• Chemotherapy may be given if the tumour can’t be completely removed or there is evidence that the tumour has spread to the cerebrospinal fluid or brain stem.

Pituitary gland tumours develop in the pituitary gland and are almost always non-cancerous (benign). The pituitary gland produces hormones that control growth and physical and sexual development. These hormones also affect other glands in the body, such as the thyroid and adrenal glands.

Find out more about pituitary gland tumours.

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma. It starts in cells of the lymphatic system. PCNSL starts most often in the brain but may also involve the cerebrospinal fluid, the spinal cord or the eyes. This type of lymphoma is more common in people who have problems with their immune system.

Schwannomas (neurilemomas) are usually low-grade tumours. They start in Schwann cells, which insulate cranial and other nerves. They can start on any cranial nerve. When they start on the cranial nerve responsible for hearing and balance they are called acoustic neuromas.

They can also start on spinal nerves. When this happens, the tumour can press on the spinal cord causing weakness and bladder problems.

The following are treatments for schwannomas:

• Surgery is done to remove as much of the tumour as possible.
• Radiation therapy is given after surgery if the tumour can’t be completely removed or if the tumour is in an area where surgery can’t be done.

The following are rare low-grade (grade 1 or grade 2) brain tumours.

• central neurocytomas
• cerebellar liponeurocytomas
• choroid plexus papillomas
• gangliocytomas
• gangliogliomas
• melanocytomas
• neurofibromas
• pineocytomas
• subependymal giant cell astrocytomas
• pleomorphic xanthoastrocytomas