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Primary Brain tumors

General

A primary brain tumor refers to a group of abnormal, immature cells that form a mass originating in the brain or spinal cord. Primary brain tumors can be cancerous or benign. The etiology of primary brain tumors is unknown.

Risks

Many of the risk factors of brain cancer are known. A few of the risk factors that have been proven are:

  • Radiation therapy used to treat brain cancer increases the risk of developing subsequent brain tumors for up to 30 years post treatment.

  • Genetics: neurofibromatosis, Von Hippel-Lindau syndrome, Li-Fraumeni syndrome, and Turcot syndrome.

  • Individuals infected with Epstein-Barr virus are known to have an increased risk of brain tumors.

There are other risk factors which show a high probability of causing brain tumors:

  • Radiation exposure. This may include work such as a dental hygienist or a radiology technician, living under power lines, or frequent use of cell phones and other wireless devices.

  • Hormone therapy

  • Smoking

  • Head injuries

Types

Brain tumors are classified and staged by location of the tumor, extent of tissue involvement, and if they are benign or malignant.

Gliomas, meningiomas, and schwannomas are the most common types in adults.

  • Glioblastomas are the most aggressive type of brain tumors and occur most often in young adults 20-40 years of age.

  • Meningiomas can be either malignant or benign, some are aggressive, most occur between the ages 40 and 70.

  • Schwannomas can also be malignant or benign, mostly slow growing but can be aggressive and occur most often at a later age.

There are many other primary brain tumors that occur less frequently:

  • Primary CNS (central nervous system) lymphoma

  • Pituitary tumors

  • Primary germ cell tumors of the brain

  • Ependymomas

  • Craniopharyngiomas

Signs and Symptoms

Primary brain tumors often do not cause symptoms until they have grown large enough to put pressure on other parts of the brain. The symptoms felt by the patient are directly related to where in the brain the tumor is located. The most common signs and symptoms are:

  • Headaches that occur during sleep and get worse with coughing, exercise, or change of position.

  • Seizures

  • Weakness on one side or the other

  • Confusion or any other changes in mental acuity

  • Vomiting with headache

  • Eye problems, droop or double vision

  • Difficulty swallowing

  • Difficulty reading and/or writing

  • Pupils of different size, uncontrollable eye movement

  • Changes in taste and/or smell

  • Hand tremors

  • Loss of balance, vertigo

  • Loss of bladder and /or bowel control

  • Muscle weakness, trouble walking, clumsiness

Staging

When brain cancer is diagnosed it is given a stage. The WHO has given CNS tumors a histologic grading system comprised of four grades:

  • Grade I- Lesion with low proliferation potential and high possibility for cure with surgical resection.

  • Grade II – Lesions that are infiltrating but low in mitotic activity.

  • Grade III- Lesions with evidence of malignancy generally in the form of high mitotic activity.

  • Grade IV- Lesions that are mitotically active, necrosis-prone and display rapid evolution of disease preoperatively and postoperatively.

Treatment

Early treatment is critical if the goal is achieving a cure. To do this, it is important for the patient to get their team of physicians in place. The team usually will consist of the the following physicians and specialists:

  • Neuro-oncologist

  • Neurosurgeon

  • Medical oncologist

  • Radiologist

  • Oncology certified RN-cancer coordinator

A patient is more successful in transition through diagnosis, treatment and follow-up when they are assigned a cancer coordinator early in the process. The cancer coordinator will be the one person who will be there for them through the entire process. He or she will help with such things as:

  • Organizing financial burden (copays, deductibles, no insurance)

  • Appointment times (missed or conflicting)

  • Education (drugs and procedures)

  • Complimentary treatments (acupuncture, massage, yoga, wigs)

  • Transportation.

Once a team has been assembled, treatment can be provided. Surgery is generally the first choice. Resection of the tumor before it has spread gives the patient the best possible outcome and chance of full recovery.

If the goal of treatment is not for a cure but rather for palliative treatment, radiation or debulking of the tumor may be used. Debulking is a procedure to remove as much of the tumor as possible to relieve symptoms and improve brain function. This is used when the cancer has metastasized or is deep in the brain. With this procedure the patient may live longer with reduced symptoms, but is still unlikely to make a full recovery.

Chemotherapy may be used in conjunction with radiation and surgery but is very seldom used on its own. With brain cancer there is a unique obstacle: the blood brain barrier. Very few chemotherapy drugs can pass through this barrier.  If the drug can’t get to the tumor it can’t kill the cancer. This makes chemo the least effective treatment for brain cancer, and is often not used at all.

Prognosis

Unfortunately, recurrence is high with brain cancer. There is no standard therapy for recurrent glioma; however, there are numerous clinical trials that are ongoing. Patients that have failed primary treatment should be encouraged to participate. Many chemotherapies, cancer vaccines, and stereotactic radiosurgery techniques are being researched. These clinical trials are our best hope of someday eradicating this terrible disease.

References:

Brain tumor – primary – adults. Retrieved from https://medlineplus.gov/ency/article/007222.htm.

Bruce, Jeffrey N. (2016). Brain Cancer Treatment Protocols. Medscape. Retrieved from https://emedicine.medscape.com/article/2005182-overview.

Bruce, Jeffrey N. (2015). Brain Cancer Staging. Medscape. Retrieved from https://emedicine.medscape.com/article/2006770-overview.

Lo, Bruce M. (2015). Brain Neoplasmas. Medscape. Retrieved from https://emedicine.medscape.com/article/779664-overview.