Adult Patient Resource Handbook - 5th Edition
BRAIN TUMOUR TYPES: A-C
This section provides the names of many different types of brain tumours that exist. General information about these tumours has been provided to assist you in learning more about your diagnosis. However, this list is not exhaustive and some rare types of brain tumours may not be listed as well as some mixed tumour types. If you are unable to locate the name of your tumour in this section, contact the Brain Tumour Foundation of Canada. It is advised that you also contact your doctor for further information. This section has been listed in alphabetical order to ease your search.
ACOUSTIC NEUROMA - SCHWANNOMA, NEURINOMA, NEUROFIBROMA These tumours arise from the Schwann cells, which insulate the 8th cranial nerve. These tumours can vary in size from very small to very large. Since these tumours grow slowly, symptoms may be slow to develop and the patient may delay seeking medical help.
Symptoms can include hearing loss, visual disturbances, dizziness, difficulty swallowing, or infrequently, facial numbness or weakness. In most cases, these tumours can be completely removed by surgery. Specialized radiation therapy may also be indicated. After treatment, the patient may have permanent or temporary damage to his/her facial nerve. Some centers have specialized programs to deal with facial weakness. Inquire about this. Traveling to another centre for rehabilitation may be a consideration. Hearing loss may be permanent.
The Acoustic Neuroma Association of Canada can be contacted for further in-depth information as well as support. They can be reached at 1-800-561-ANAC (2622) or their website is www.anac.ca
ASTROCYTOMA - (Also See Glioma) These tumours arise from cells called astrocytes. This is the supportive tissue of the brain. Astrocytomas are graded from I to IV (1 to 4) depending on how the tumour looks to the pathologist. Grade I tumours are slightly abnormal whereas Grade IV tumours are very abnormal in appearance.
Anaplastic Astrocytoma (Grade III) The cells of these tumours are moderately fast growing and less well defined than an astrocytoma (Grade I or II). These higher-grade tumours are malignant and treatment is usually more aggressive than that for lower grade tumours. Patients are often diagnosed in adulthood, with diagnoses peaking in their forties. These tumours are diffuse and infiltrating to the surrounding tissue, and the tentacle-like projections of these astrocytomas make them harder to remove. Treatment involves removal of as much tumour as safely possible plus radiation and chemotherapy.
Cerebellar Pilocytic Astrocytoma (Grade I) These tumours are astrocytomas found in the cerebellum. This is the part of the brain that controls balance. Most of these tumours are considered benign. First symptoms of these tumours are usually headache, vomiting or an unsteady gait. They occur equally between the sexes and at all ages in children, although they are unusual in infants. Approximately two-thirds of these tumours are cystic (have a fluid cyst associated with a solid mass) in nature. These tumours are very rare in adults.
The management of astrocytoma may involve treating hydrocephalus. This is usually accomplished pre-operatively with steroids (dexamethasone, or the more common trade name of Decadron), which frequently relieve symptoms in 24-48 hours. A second approach to hydrocephalus infrequently requires the placement of a shunt.
The goal of treatment of a cerebellar pilocytic astrocytoma is complete surgical removal. This is achieved in a large majority of cases and no further treatment is necessary. If total removal is not possible (for example it may have grown into the brain stem) radiation therapy may be necessary. The role of chemotherapy is changing in these tumours. Some response has now been found to chemotherapeutic agents. Pilocytic astrocytomas can occur in other parts of the brain and their treatment is usually surgery, and radiation therapy if necessary.
Glioblastoma Multiforme (Grade IV) These tumours may contain various cell types, hence the name "multiforme". The most common cell types are astrocytes. Most of these tumours occur in the cerebral hemispheres and often involve the corpus callosum. The cells of these tumours grow quickly, are not well defined, and they may frequently spread throughout the brain. The most common symptoms are due to increased pressure in the brain and can include headache, vomiting, drowsiness and nausea. Presentation of this tumour is often within the 4th to the 7th decade of life. This tumour type represents 12-15% of all intracranial tumours.
Surgery may be performed to remove as much tumour as possible. Radiation and chemotherapy are often then used to control growth of the tumour. Recent studies have shown promise in the use of radiation and chemotherapy (specifically Temozolomide®) at the same time to improve quality of life and increase survival.
Astrocytoma (Grade II) These are astrocytomas that are usually located in the hemispheres of the brain. First symptoms of these tumours usually include seizures, nausea, vomiting, headache and difficulties with vision. This type of tumour is most commonly diagnosed in patients who are 20 to 45 years of age. Grade 2 astrocytomas make up about 25% of all gliomas.
Surgical removal is the method of treatment for these astrocytomas. Radiation may also be necessary if the tumour is in an area of the brain responsible for such functions as speech, understanding or movement and if it cannot be easily surgically removed. Chemotherapy may be a consideration. A significant proportion of astrocytomas, over time, may alter their cell structure and become more aggressive (eg. anaplastic astrocytoma and glioblastoma multiforme).
Pilocytic Astrocytoma (Grade I) These tumours tend to be classified as a low-grade astrocytoma because they do not spread from their original site. They are generally considered to be benign tumours and can often be removed by surgery depending on their location. Pilocytic astrocytomas are often found in the optic chiasm, optic nerves, thalamus as well as the cerebellum.
Pleomorphic Xanthoastrocytoma (Grade II) This tumour usually occurs in adolescents and presents with seizures. It has been important to recognize this tumour type since the prognosis of this type of astrocytoma is very good. Pleomorphic Xanthoastrocytomas are a rare form of astrocytomas and represents less than 1% of all intracranial tumours. This tumour is usually treated with surgical removal and the role for radiation therapy and chemotherapy has not been defined.
CHONDROMA These tumours are slow growing and rarely metastasize. They may be present a long time before showing any symptoms. They are made up of cartilage. The malignant form is called a chondrosarcoma. A common location is in the base of the skull, which makes complete surgical removal difficult.
CHONDROSARCOMA Chondrosarcomas are bone tumours composed of cartilage and are the malignant form of chondromas. Although these tumours are still relatively slow growing, they often develop at the base of the skull or in the sphenoid bone and are invasive to the surrounding tissue, making surgical removal difficult.
CHORDOMA These tumours develop from remnants of embryonic tissue. They are slow growing and usually do not produce symptoms until middle age. These tumours can arise in the skull bones at the base of the brain, or in the vertebral bones in the neck or sacrum. Treatment is surgery and radiation therapy. A special type of radiation called Proton Beam Radiotherapy can be beneficial.
CHOROID PLEXUS TUMOUR The choroid plexus is an infolding of the lining of the ventricular surface. This surface has many blood vessels. The choroid plexus is involved in the production of most of the cerebrospinal fluid. A person with a choroid plexus papilloma usually has marked hydrocephalus due to the obstruction of flow and sometimes there is an overproduction of cerebrospinal fluid.
The most common location for this tumour is in the lateral ventricles (the ventricles on either side of the brain). These tumours account for one to three percent (1%-3%) of pediatric brain tumours and are most common in children under the age of 2 years. They make up eight percent (8%) of brain tumours diagnosed in newborn babies.
These tumours are separated into 2 categories:
Treatment for these tumours involves complete surgical resection (removal) for papillomas, whereas carcinomas require surgery, radiation and chemotherapy. On rare occasions, carcinomas may spread to other areas.
CRANIOPHARYNGIOMA These slow growing tumours account for approximately nine percent (9%) of all pediatric brain tumours and usually occur in children between 5 and 10 years of age. Rarely, they occur in adults. These tumours arise from cells along the pituitary stalk and may grow upwards and involve the hypothalamus, optic nerve pathways and the third ventricle. These tumours are cystic in nature and usually benign. At diagnosis, there may be signs of increased pressure in the brain (third ventricle blocked), visual loss (either decreased vision or visual field problems) and hormone difficulties (growth delay, thyroid deficiency, sexual delay).
Complete removal of this tumour is possible if it is in a favorable location. Otherwise, radiation therapy may be used. Long-term follow-up will be necessary by an endocrinologist (a physician who studies the hormone systems of the body). Lifelong hormone replacement may be necessary. Neuropsychological testing and follow-up are advisable. Problems with emotions and behaviour may need to be monitored.
CYST Cysts are abnormal accumulations of fluid enclosed in a lining. They may contain blood, tissue or tumour cells. There are specific cysts that are named for their type of tissue and their content.
Arachnoid Cyst Arachnoid cysts are congenital in origin and may occur anywhere in the brain. In children, they most commonly arise in the back of the brain and in the region of the third ventricle. They are cerebrospinal fluid (CSF)-filled cysts that are lined with the arachnoid membrane (one of the three meningeal coverings). Some arachnoid cysts are self-contained, while others may be connected by a passageway with the ventricles or subarachnoid space. The entrapped fluid may block the CSF pathways, producing hydrocephalus.
Colloid Cyst These cysts usually occur in the third ventricle and can cause hydrocephalus. They contain embryonic tissue (tissue formed before birth). Treatment includes surgery and sometimes shunting.
Dermoid and Epidermoid Cyst These cysts develop from congenital tissue (formed before birth). Epidermoid cysts contain keratin, cellular debris and cholesterol. Dermoid cysts contain hair and sebaceous (sweat) glands. These masses occur in central areas of the brain such as the hypothalamic region, the vermis of the cerebellum and the pineal region. The bones of the skull (not involving the brain itself) and the spine may also be involved. These cysts are treated with surgery and complete removal is usually possible.
Hamartoma This is an aggregation (or clumping) of a particular normal tissue or cell types in areas in which they are not typically found. It is not considered to be fast-growing or malignant and is presumed to be congenital (existing before birth).
The tissues included in the mass are normal but may be highly disorganized. Many of these tumours present with seizures and hydrocephalus. Treatment is usually surgical, although in the majority of cases, the tumour cannot be completely removed. These tumours have a tendency to grow during childhood and infrequently grow after puberty.
Rathke Cleft Cyst (Rathke Pouch Cyst) During development, the pituitary gland develops from two components. The anterior portion develops from the roof of the mouth, and the posterior portion from the brain itself.
Occasionally, non-malignant cysts develop from the fragments of tissue that initially had lined the mouth. These cysts have no solid component and are fluid filled sacs with a very specialized lining that secretes fluid. These cysts are always in the area of the pituitary gland and can be large. Treatment is surgical removal. At times a shunting procedure may be necessary.
Teratoma This is a congenital tumour, which is made up of elements from the three primary cell layers: Ectoderm (skin and nervous system), Mesoderm (muscle, bone and cartilage) and Endoderm (gut lining). These are the earliest cell types in development. It is most commonly a tumour of the pineal gland or lower spinal cord in the central nervous system, but may be found near the base of the brain near the pituitary gland, or the base of the third ventricle. Treatment is usually surgery.
Brain Tumour Types A-C
Brain Tumour Types
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