21st Century Pediatric Cancer Sourcebook: Childhood Craniopharyngiomas - Clinical Data and Practical Information for Patients, Families, and Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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American Cancer Society (ACS) * http://www.cancer.org/
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PART ONE
Chapter 1A: Childhood Craniopharyngioma
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
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Chapter 1A: Childhood Craniopharyngioma
Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland. There are no known risk factors for childhood craniopharyngioma. Possible signs of childhood craniopharyngioma include vision changes and slow growth. Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas. Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery. Certain factors affect prognosis (chance of recovery) and treatment options.
Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland.
Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.
Craniopharyngiomas are usually part solid mass and part fluid -filled cyst. They are benign (not cancer) and do not spread to distant parts of the brain or to other parts of the body. However, they may grow and press on parts of the brain or other nearby areas, including the pituitary gland, the optic chiasm, optic nerves, and fluid-filled spaces in the brain. Many functions including hormone making, growth, vision, and normal working of the brain may be affected. Benign brain tumors need treatment.
This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary.
Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults.
There are no known risk factors for childhood craniopharyngioma.
Craniopharyngiomas are rare in children younger than 2 years of age and are most often diagnosed in children aged 5 to 14 years. It is not known what causes these tumors to form.
Possible signs of childhood craniopharyngioma include vision changes and slow growth.
These and other symptoms may be caused by craniopharyngiomas. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
Headaches including morning headache or headache that goes away after vomiting.
Vision changes.
Nausea and vomiting.
Loss of balance or trouble walking.
Increase in thirst or urination.
Increase in head size (in infants).
Unusual sleepiness or change in energy level.
Unusual changes in personality or behavior.
Short stature, slow growth, or delayed puberty.
Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas.
The following tests and procedures may be used:
Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
Visual field exam: An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. For example, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). TSH and ACTH are made by the pituitary gland in the brain.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help areas of the brain show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium. A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.
Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery.
Doctors may diagnose a craniopharyngioma based on where the tumor is in the brain and how it looks on a CT scan or MRI. Sometimes a biopsy is required to find out the type of tumor. The biopsy is done during surgery, and a tissue sample is removed, using one of the following procedures:
An open biopsy (a hollow needle is inserted through a hole in the skull into the brain).
A computer-guided needle biopsy (guided by a computer, a hollow needle is inserted through a small hole in the skull into the brain).
A transsphenoidal biopsy (instruments are inserted into part of the brain by going through the nose and the sphenoid bone, a butterfly-shaped bone at the base of the skull).
A pathologist views the tissue under a microscope to look for tumor cells. If tumor cells are found, the doctor may remove as much tumor as safely possible during the same surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
The size of the tumor.
Where the tumor is in the brain.
Whether the tumor is solid or a fluid-filled cyst.
Whether there are tumor cells left after surgery.
The child's age.
Whether the tumor has just been diagnosed or has recurred (come back).
Stages of Childhood Craniopharyngioma
The process used to find out if cancer has spread within the brain or to other parts of the body is called staging. There is no standard system for staging childhood craniopharyngioma. Craniopharyngioma is described as newly diagnosed disease or recurrent disease.
The results of the tests and procedures done to diagnose craniopharyngioma are used to help make decisions about treatment.
Recurrent Childhood Craniopharyngioma
Recurrent craniopharyngioma is a tumor that has recurred (come back) after it has been treated. The tumor may come back in the same area of the brain where it was first found.
Treatment Option Overview
There are different types of treatment for children with craniopharyngioma.
Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.
Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years.
Some treatments for tumors cause side effects months or years after treatment has ended.
Three types of treatment are used:
Surgery
Limited surgery and radiation therapy
Chemotherapy
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their treatment.
Follow-up tests may be needed.
There are different types of treatment for children with craniopharyngioma.
Different types of treatments are available for children with craniopharyngioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with tumors. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because tumors in children are rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team.
Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with tumors. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:
Neurosurgeon. * Radiation oncologist. * Neurologist. * Endocrinologist. * Ophthalmologist. * Rehabilitation specialist. * Psychologist. * Social Worker. * Nurse specialist.
Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years.
Symptoms caused by the tumor may begin before diagnosis. These symptoms may continue for months or years. It is important to talk with your child's doctors about symptoms caused by the tumor that may continue after treatment.
Some treatments for tumors cause side effects months or years after treatment has ended.
Side effects from tumor treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of tumor treatment may include the following:
Physical problems such as vision problems and obesity.
Changes in mood, feelings, thinking, learning, or memory.
Second cancers (new types of cancer).
For example, serious late effects of radiation therapy for craniopharyngioma may include the following:
Pituitary gland stops making hormones.
Stroke or blood vessel problems.
Blindness.
Second cancer in the area that received radiation.
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects tumor treatment can have on your child.
Three types of treatment are used:
Surgery
The way the surgery is done depends on the size of the tumor and where it is in the brain. It also depends on whether the tumor has grown into nearby tissue in a finger-like way. The types of surgery include the following:
Transsphenoidal surgery: A type of surgery in which the instruments are inserted into part of the brain by going through an incision (cut) made under the upper lip or at the bottom of the nose between the nostrils and then through the sphenoid bone (a butterfly-shaped bone at the base of the skull).
Craniotomy: Surgery to remove the tumor through an opening made in the skull.
Sometimes all of the tumor that can be seen is removed in surgery and no further treatment is needed. At other times, it is more difficult to remove the tumor because it is growing into or pressing on nearby organs. Serious problems may occur if the pituitary gland, hypothalamus, optic nerves, or carotid artery are affected during surgery.
If there is tumor remaining after the surgery, radiation therapy is usually given to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
After surgery, follow-up testing with MRI will be done for several years to check if the tumor has come back. Also, if part of the pituitary gland is removed in surgery, several types of pituitary hormone replacement pills will be given for life.
Limited surgery and radiation therapy
Limited surgery is used to treat some craniopharyngiomas. It is used to diagnose the tumor, remove fluid from a cyst, and relieve any pressure on the optic nerves. Limited surgery is followed by radiation therapy. This treatment option may have fewer serious side effects than surgery to remove tumor from the pituitary gland or hypothalamus.
Radiation therapy is a tumor treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the tumor. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor. The way the radiation therapy is given depends on the type of tumor being treated and whether it has spread.
Because radiation therapy to the brain can affect growth and development in young children, ways of giving radiation therapy that have fewer side effects are being studied. These include:
Stereotactic radiosurgery: For very small craniopharyngiomas at the base of the skull, stereotactic radiosurgery may be used. Stereotactic radiosurgery uses a head frame to aim radiation at the tumor only. This treatment is also called stereotaxic radiosurgery.
Intracavitary radiation therapy: Intracavitary radiation therapy is a type of internal radiation therapy that may be used in tumors that are part solid mass and part fluid-filled cyst. Radioactive material is sent directly into the tumor, and causes less damage to the nearby hypothalamus and optic nerves.
Chemotherapy
Chemotherapy is a treatment that uses anticancer drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid or an organ, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Intracavitary chemotherapy is a type of regional chemotherapy that places drugs directly into a cavity, such as a cyst. The way the chemotherapy is given depends on the type of tumor being treated.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients who have not improved. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the disease or decide how to treat it may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed. These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Craniopharyngioma
A link to a list of current clinical trials is included for this treatment section. Check with your doctor for clinical trials that are not listed here but may be right for you.
Newly Diagnosed Childhood Craniopharyngioma
Treatment of newly diagnosed childhood craniopharyngioma may include the following:
Surgery.
Limited surgery followed by external-beam radiation therapy.
Stereotactic radiosurgery.
Intracavitary radiation therapy with or without chemotherapy.
Recurrent Childhood Craniopharyngioma
Craniopharyngioma may recur (come back) no matter how it was treated the first time. Treatment options for recurrent childhood craniopharyngioma are based mainly on what type of treatment was given before and the needs of the individual child. Treatment options may include:
Repeat surgery.
External-beam radiation therapy.
Intracavitary radiation therapy with or without chemotherapy.
Intracavitary chemotherapy.
Glossary Terms
adrenocorticotropic hormone (uh-DREE-noh-KOR-tih-koh-TROH-pik HOR-mone)
A hormone made in the pituitary gland. Adrenocorticotropic hormone acts on the outer part of the adrenal gland to control its release of corticosteroid hormones. More adrenocorticotropic hormone is made during times of stress. Also called ACTH and corticotropin.
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood chemistry study (blud KEH-mih-stree STUH-dee)
A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
contrast material
A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
craniopharyngioma (KRAY-nee-oh-fuh-RIN-jee-OH-muh)
A benign brain tumor that may be considered malignant because it can damage the hypothalamus, the area of the brain that controls body temperature, hunger, and thirst.
CT scan
A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cyst (sist)
A sac or capsule in the body. It may be filled with fluid or other material.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
fluid (FLOO-id)
A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
gadolinium (GA-duh-LIH-nee-um)
A metal element that is used in magnetic resonance imaging (MRI) and other imaging methods. It is a contrast agent, which helps show abnormal tissue in the body during imaging with a special machine.
gland
An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.
hormone (HOR-mone)
One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.
hypothalamus (HY-poh-THAL-uh-mus)
The area of the brain that controls body temperature, hunger, and thirst.
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
metastatic (meh-tuh-STA-tik)
Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.
MRI
A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
nausea
A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.
needle biopsy (NEE-dul BY-op-see)
The removal of tissue or fluid with a needle for examination under a microscope. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
nerve
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.
open biopsy
A procedure in which a surgical incision (cut) is made through the skin to expose and remove tissues. The biopsy tissue is examined under a microscope by a pathologist. An open biopsy may be done in the doctor’s office or in the hospital, and may use local anesthesia or general anesthesia. A lumpectomy to remove a breast tumor is a type of open biopsy.
optic chiasm (OP-tik ky-AZ-um)
The place in the brain where some of the optic nerve fibers coming from one eye cross optic nerve fibers from the other eye. Also called optic chiasma.
optic nerve
The nerve that carries messages from the retina to the brain.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
pituitary gland (pih-TOO-ih-TAYR-ee...)
The main endocrine gland. It produces hormones that control other glands and many body functions, especially growth.
primary tumor (PRY-mayr-ee TOO-mer)
The original tumor.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
puberty (PYOO-ber-tee)
The time of life when a child experiences physical and hormonal changes that mark a transition into adulthood. The child develops secondary sexual characteristics and becomes able to have children. Secondary sexual characteristics include growth of pubic, armpit, and leg hair; breast enlargement; and increased hip width in girls. In boys, they include growth of pubic, face, chest and armpit hair; voice changes; penis and testicle growth, and increased shoulder width.
recur
To come back or to return.
spectroscopy (spek-TROS-koh-pee)
The study of the amount of light that is taken up, given off, or scattered (reflected) by an object. Spectroscopy breaks down light and measures different wavelengths of visible and non-visible light. In medicine, different types of spectroscopy are being used to study tissues and to help make a diagnosis.
sphenoid sinus (SFEE-noyd SY-nus)
A type of paranasal sinus (a hollow space in the bones around the nose). There are two large sphenoid sinuses in the sphenoid bone, which is behind the nose between the eyes. The sphenoid sinuses are lined with cells that make mucus to keep the nose from drying out.
spinal cord (SPY-nul kord)
A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.
stature (STA-cher)
The height of a person while standing.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
thyroid-stimulating hormone (THY-royd STIM-yoo-LAY-ting HOR-mone)
A hormone produced by the pituitary gland. Thyroid-stimulating hormone stimulates the release of thyroid hormone from thyroglobulin. It also stimulates the growth of thyroid follicular cells. An abnormal thyroid-stimulating hormone level may mean that the thyroid hormonal regulation system is out of control, usually as a result of a benign condition (hyperthyroidism or hypothyroidism). Also called TSH.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
urine (YOOR-in)
Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
vomit
To eject some or all of the contents of the stomach through the mouth.
x-ray
A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.
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Childhood Craniopharyngioma Treatment - Last Modified: 06/30/2010 - Health Professional Version
Background Information About Childhood Craniopharyngioma Incidence and Presentation * Prognosis * Histopathologic Classification of Childhood Craniopharyngioma * Diagnostic Evaluation of Childhood Craniopharyngioma * Stage Information * Treatment Options for Newly Diagnosed Childhood Craniopharyngiom Radical Surgery * Limited Surgery and Radiation Therapy * Intracavitary Radiation Therapy and/or Chemotherapy * Treatment Options for Recurrent Childhood Craniopharyngioma * Late Effects in Patients Treated for Childhood Craniopharyngioma
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%.[2] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment.
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.
References
Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.
Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [PUBMED Abstract]
Background Information About Childhood Craniopharyngioma
Incidence and Presentation
Craniopharyngiomas are relatively rare pediatric tumors, accounting for about 6% of all intracranial tumors in children.[1] They are believed to be congenital in origin, and may arise from embryonic remnants. No predisposing factors have been identified.
Because craniopharyngiomas occur in the region of the pituitary gland, endocrine function and growth may be affected. Additionally, the close proximity of the tumor to the optic nerves and chiasm may result in vision problems. Some patients present with obstructive hydrocephalus due to tumor obstruction of the third ventricle.
Prognosis
Long-term survival for children with craniopharyngioma is generally good. Regardless of the treatment modality, long-term survival is approximately 79%.[2]
References
Bunin GR, Surawicz TS, Witman PA, et al.: The descriptive epidemiology of craniopharyngioma. J Neurosurg 89 (4): 547-51, 1998. [PUBMED Abstract]
Sanford RA, Muhlbauer MS: Craniopharyngioma in children. Neurol Clin 9 (2): 453-65, 1991. [PUBMED Abstract]
Histopathologic Classification of Childhood Craniopharyngioma
Craniopharyngiomas are histologically benign and do not metastasize to remote brain locations or to areas outside the sellar region except by direct extension. They may be invasive, however, and may recur locally. They may be classified as adamantinomous or squamous papillary, with the former being the predominant form in children.[1] They are typically composed of both a solid portion with an abundance of calcification, and a cystic component which is filled with a dark, oily fluid.
References
Miller DC: Pathology of craniopharyngiomas: clinical import of pathological findings. Pediatr Neurosurg 21 (Suppl 1): 11-7, 1994. [PUBMED Abstract]
Diagnostic Evaluation of Childhood Craniopharyngioma
The results of imaging studies (computerized tomography scans and magnetic resonance imaging [MRI] scans) are often diagnostic for craniopharyngiomas, with most demonstrating intratumoral calcifications. Craniopharyngiomas without calcification may be confused with other tumor types, such as germinoma or hypothalamic/chiasmatic astrocytoma, and biopsy may be required.[1] Magnetic resonance spectroscopy may be diagnostically helpful in some cases.[2] MRI of the spinal axis is not routinely performed.
Apart from imaging, patients often undergo formal visual examination including visual field evaluation, and endocrine testing.
References
Harwood-Nash DC: Neuroimaging of childhood craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 2-10, 1994. [PUBMED Abstract]
Sutton LN, Wang ZJ, Wehrli SL, et al.: Proton spectroscopy of suprasellar tumors in pediatric patients. Neurosurgery 41 (2): 388-94; discussion 394-5, 1997. [PUBMED Abstract]
Stage Information
There is no generally applied staging system for childhood craniopharyngiomas. Patients are classified as having newly diagnosed or recurrent disease.
Treatment Options for Newly Diagnosed Childhood Craniopharyngioma
There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma. Little data exists to compare the different modalities in terms of recurrence rate or quality of life.[1] For this reason, treatment is individualized.
Radical Surgery
Because these tumors are histologically benign, it may be possible to remove all the visible tumor resulting in long-term disease control. Many surgical approaches have been described, and the route should be determined by the size, location, and extension of the tumor. A transsphenoidal approach may be possible in some small tumors located entirely within the sella, but this is not usually possible in children, in which case a craniotomy is usually required.
Gross total resection is technically challenging because the tumor is surrounded by vital structures, including the optic nerves and chiasm, the carotid artery and its branches, the hypothalamus, and the third cranial nerve. The tumor may be adherent to these structures, which may cause complications, and may limit the ability to remove all the tumor. The surgeon often has limited visibility in the region of the hypothalamus and in the sella, and portions of the mass may be left in these areas, accounting for some recurrences. Almost all craniopharyngiomas have an attachment to the pituitary stalk, and of the patients who undergo radical surgery, virtually all will require life-long pituitary hormone replacement with multiple medications.[2,3]