Carcinoid Tumor-cancer

About the endocrine system and endocrine tumors

The endocrine system consists of cells that produce hormones. Hormones are chemical substances that are carried through the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body. Part of the endocrine system is the neuroendocrine system, which is made up of cells that are a cross between traditional hormone-producing cells and nerve cells.

A tumor begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign or malignant. A benign tumor is not cancerous and usually can be removed without it causing much harm. A malignant tumor is cancerous and can be harmful if not found early and treated. It can spread to and damage the body’s healthy tissues and organs.

An endocrine tumor is a mass that affects the parts of the body that secrete hormones. Because an endocrine tumor develops from cells that produce hormones, the tumor itself can produce hormones and cause serious illness.

About carcinoid tumors

A carcinoid tumor starts in the hormone-producing cells of various organs, mostly in the gastrointestinal tract (such as the stomach and intestines) and the lungs, but it can also start in the pancreas, testicles (in males), or ovaries (in females). More than one carcinoid tumor can occur within the same organ. The cause of carcinoid tumors is unknown.

A carcinoid tumor is a type of neuroendocrine tumor, which means it starts in cells of the neuroendocrine system that make hormones. A carcinoid tumor can make high levels of neuropeptides and amines (hormone-like substances); however, they may not be released in large enough amounts to cause symptoms, or they may be defective and not cause symptoms. A carcinoid tumor can grow slowly for many years without causing symptoms. Although a carcinoid tumor is cancerous, it is often described as "cancer in slow motion."

Here is a general overview of where carcinoid tumors begin:

• 39% occur in the small intestine.
• 15% occur in the rectum.
• 10% occur in the bronchial system of the lungs.
• 7% occur in the appendix.
• 5% to 7% occur in the colon.
• 2% to 4% occur in the stomach.
• 2% to 3% occur in the pancreas.
• About 1% occurs in the liver.
• They rarely occur in ovaries, testicles, and other organs.

There are two subtypes of lung carcinoid tumors: typical and atypical. The difference is based on how a tumor processes and makes serotonin (5-HT, a neurotransmitter involved in behavior and depression):

• A typical lung carcinoid tumor causes high levels of serotonin and chromogranin-A in the blood and high levels of 5-HIAA (a product of serotonin breakdown) in the urine.
• An atypical lung carcinoid tumor has normal levels of serotonin and chromogranin-A in the blood and normal levels of 5-HIAA in the urine but high levels of serotonin and 5-HTP (an amino acid) in the urine, and it can make 5-HTP.

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Breast Cancer - Metaplastic

Metaplastic breast cancer, also called metaplastic carcinoma of the breast, is a rare type of breast cancer. Cancer begins when normal cells in the breast begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

About the breast

The breast is mainly composed of fatty tissue. Within this tissue is a network of lobes, which are made up of tiny, tube-like structures called lobules that contain milk glands. Tiny ducts connect the glands, lobules, and lobes, carrying the milk from the lobes to the nipple, located in the middle of the areola (darker area that surrounds the nipple of the breast). Blood and lymph vessels run throughout the breast; blood nourishes the cells, and the lymph system drains bodily waste products. The lymph vessels connect to lymph nodes, the tiny, bean-shaped organs that ordinarily help fight infection.

About metaplastic breast cancer

Metaplastic breast cancer describes a cancer that begins in one type of cell (such as those from the glands of the breast) and changes into another type of cell. It is very different from the typical ductal or lobular breast cancer. Most cases of metaplastic breast cancer start in the epithelial cells, and then change into squamous (nonglandular) cells. Because the cells that give rise to metaplastic breast cancer are not part of the normal breast gland, this cancer does not have estrogen receptors (ERs), progesterone receptors (PRs), or HER2 (a protein found in 20% of ductal and lobular breast cancers).

Metaplastic breast cancer can spread to the lymph nodes and other areas of the body, especially the lungs. At the time of diagnosis, metaplastic breast cancer is considered an invasive cancer, meaning that it has already spread beyond the duct or lobe

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Breast Cancer - Male

Breast cancer in men is rare, accounting for less than 1% of all breast cancer cases. Although breast cancer in men occurs less frequently than breast cancer in women, the diseases are similar in many ways. Cancer begins when normal cells in the breast begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

The breast is mainly composed of fatty tissue. Within this tissue is a network of lobes, which are made up of tiny, tube-like structures called lobules that contain milk glands. Tiny ducts connect the glands, lobules, and lobes to the nipple, located in the middle of the areola (darker area that surrounds the nipple of the breast). Blood and lymph vessels run throughout the breast; blood nourishes the cells, and the lymph system drains bodily waste products. The lymph vessels connect to lymph nodes, which are tiny, bean-shaped organs that normally help fight infection.

The main types of breast cancer are the same for men and women. Most breast cancer cases start in the ducts or lobes. Almost 75% of all breast cancers begin in the cells lining the milk ducts and are called ductal carcinomas. Approximately 25% of male breast cancers are lobular carcinoma (cancer that begins in the lobules).

A type of breast cancer that has spread outside of the duct and into the surrounding tissue is called invasive or infiltrating carcinoma. The majority of male breast cancer cases are infiltrating ductal carcinomas (IDC).

Disease that has not spread is called in situ, meaning "in place." Ductal carcinoma in situ (DCIS) is the most common type of in situ breast cancer, but it is uncommon in men.

Inflammatory breast cancer makes up about 1% to 5% of all breast cancers. Paget's disease of the nipple begins in the ducts, but spreads to the skin of the nipple. Paget's disease is more common in men than in women. Other, less common subtypes of breast cancer include medullary, mucinous, tubular, or papillary.

Cancer may begin as a single, genetically abnormal cell. As this one cell divides, it eventually becomes a tumor and develops a blood supply to nourish its continued growth. At some point, cells may break off from the primary mass and move to other parts of the body in a process called metastasis.

Breast cancer spreads when breast cancer cells move to other sites in the body through the blood vessels and/or lymph vessels. A common site of spread is the regional lymph nodes. The lymph nodes can be axillary (located under the arm), mediastinal (under the sternum or breast bone), or supraclavicular (located just above the collarbone). The most common sites of distant metastasis are the bones, lungs, and liver. Less commonly, breast cancer may spread to the brain. The cancer can also recur (come back after treatment) locally in the skin, in the same breast (if it was not removed as part of treatment), other tissues of the chest, or elsewhere in the body.

Breast cancer in men is detected the same way as breast cancer in women is—through self-examination, clinical examination, or mammography (x-ray of the breast). Changes in the breast may be easier to detect because men have less breast tissue. However, the awareness of breast cancer in men is much lower than it is in women; therefore, men may not perform regular breast self-examinations or talk with their doctor about the disease.

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Breast Cancer - Inflammatory

Inflammatory breast cancer is a rare form of breast cancer. The cancer gets its name because the symptoms are like those of mastitis (inflammation of the breast) and include redness, tenderness, swelling, and pain in the breast. However, unlike mastitis, inflammatory breast cancer does not improve with antibiotic treatment.

Cancer begins when normal cells in the breast begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). At some point, cells may break off from the primary mass and move to other parts of the body in a process called metastasis.

The breast is mainly composed of fatty tissue. Within this tissue is a network of lobes, which are made up of tiny, tube-like structures called lobules that contain milk glands. Tiny ducts connect the glands, lobules, and lobes, carrying the milk from the lobes to the nipple, located in the middle of the areola (darker area that surrounds the nipple of the breast). Blood and lymph vessels run throughout the breast; blood nourishes the cells, and the lymph system collects fluid from tissues to return to the blood and carries cells that help fight infection and disease. The lymph vessels connect to lymph nodes, the tiny, bean-shaped organs that help fight infection.

In inflammatory breast cancer, the cancer cells block the lymph vessels within the breast, which causes fluid backup and swelling of the breast and overlying skin. Because this type of breast cancer can grow quickly, it is treated with a combination of surgery, radiation therapy, and chemotherapy; see Treatment for more information.

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Breast Cancer

In the United States, breast cancer is the most common cancer in women (excluding skin cancer). Men can also develop breast cancer, but breast cancer in men is rare, accounting for less than 1% of all breast cancers.

About the breast

The breast is mostly made up of fatty tissue. Within this tissue is a network of lobes, which are made up of tiny, tube-like structures called lobules that contain milk glands. Tiny ducts connect the glands, lobules, and lobes, carrying the milk from the lobes to the nipple, located in the middle of the areola (darker area that surrounds the nipple). Blood and lymph vessels also run throughout the breast; blood nourishes the cells, and the lymph system drains bodily waste products. The lymph vessels connect to lymph nodes, the tiny, bean-shaped organs that help fight infection.

About breast cancer

Cancer begins when normal cells in the breast change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

Breast cancer spreads when the cancer grows into other parts of the body or when breast cancer cells move to other parts of the body through the blood vessels and/or lymph vessels. This is called metastasis. Breast cancer most commonly spreads to the regional lymph nodes. The lymph nodes can be axillary (located under the arm), cervical (located in the neck), internal mammary (located under the chest bone), or supraclavicular (located just above the collarbone). When it spreads further through the body, it most commonly spreads to the bones, lungs, and liver. Less commonly, breast cancer may spread to the brain. The cancer can also recur (come back after treatment) locally in the skin, in the same breast (if it was not removed as part of treatment), other tissues of the chest, or elsewhere in the body.

Types of breast cancer

Most breast cancers start in the ducts or lobes. Almost 75% of all breast cancers begin in the cells lining the milk ducts and are called ductal carcinomas. Cancer that begins in the lobules is called lobular carcinoma. The difference between ductal and lobular cancer is determined by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease), who examines a tumor sample removed during a biopsy (see Diagnosis).

If the disease has spread outside of the duct or lobule and into the surrounding tissue, it is called invasive or infiltrating ductal or lobular carcinoma. Cancer that is located only in the duct or lobule is called in situ, meaning “in place.” How in situ disease grows and spreads, as well as how it is treated, depends on whether it is ductal carcinoma in situ (DCIS) or lobular carcinoma in situ (LCIS).

Most in situ breast cancers are DCIS. Currently, oncologists recommend surgery to remove DCIS to help prevent the cancer from becoming an invasive breast cancer and spreading to other parts of the breast or the body. Radiation therapy and hormonal therapy may also be recommended for DCIS (see Treatment for more information).

LCIS is not considered cancer and is usually monitored by the doctor. LCIS in one breast is a risk factor for developing invasive breast cancer in both breasts (see the Risk Factors section for more information).

Other, less common types of breast cancer include medullary, mucinous, tubular, metaplastic, and papillary breast cancer, as well as other even less common types. Inflammatory breast cancer is a faster-growing type of cancer that accounts for about 1% to 5% of all breast cancers. It may be misdiagnosed as a breast infection because there is often swelling of the breast and redness of the breast skin that starts suddenly. Paget’s disease is a type of cancer that begins in the ducts of the nipple. The skin often appears scaly and may be itchy. Although it is usually in situ, it can also be an invasive cancer.

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Brain Tumor-cancer

The brain and spinal column make up the central nervous system (CNS), where all vital functions, including thought, speech, and body movements are controlled. When a tumor occurs in the CNS, it is especially problematic because of the possible effect on a person's thought processes or movements.

A brain tumor begins when normal cells in the brain change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous). In general, primary CNS tumors do not spread outside of the CNS. Malignant brain tumors are further classified using a grade: low, intermediate, or high. More information can be found in Staging.

This section describes primary brain tumors, which are tumors that begin in the brain. Secondary brain tumors (also called brain metastases) are much more common than primary tumors. A secondary brain tumor is a cancerous tumor that started in another part of the body (such as the breast, lung, or colon) and then spread to the brain. Learn more about cancer that started elsewhere in the body and spread to the brain by reading about that specific type of cancer.

Anatomy of the brain

The brain is made up of four main parts: the cerebrum, the cerebellum, the brain stem, and the meninges.

The cerebrum. This is the largest part of the brain. It contains two cerebral hemispheres and is divided into four lobes where specific functions occur:

• The frontal lobe controls reasoning, emotions, problem-solving, expressive speech, and movement
• The parietal lobe controls the sensations of touch, such as pressure, pain, and temperature, and parts of speech, visual-spatial orientation, and calculation
• The temporal lobe controls memory, the special senses such as hearing, and the ability to understand spoken or written words
• The occipital lobe controls vision

The cerebellum. The cerebellum is located at the back part of the brain below the cerebrum. It is responsible for coordination and balance.

The brain stem. This is the portion of the brain that connects to the spinal cord, controls involuntary functions essential for life, such as the beating of the heart and breathing. In addition, messages for all the functions controlled by the cerebrum and cerebellum travel through the brain stem to the connections in the body.

The meninges. These are the membranes that surround and protect the brain and spinal cord. There are three meningeal layers, called the dura mater, arachnoid, and pia mater. The cerebrospinal fluid (CSF) is made near the center of the brain, in the lateral ventricles, and circulates around the brain and spinal cord between the arachnoid and pia layers.

View illustrations of the anatomy of the brain.

Types of brain tumors

There are more than 100 types of primary brain tumors, and about 5% of all brain tumors cannot be assigned an exact type. For a complete list of the types of brain tumors and how often they are diagnosed, please refer to the Central Brain Tumor Registry of the United States. This section covers brain tumors diagnosed in adults. (Learn about brain tumors in children.) For practical purposes, this section's coverage is divided into gliomas and non-glioma types of tumors in adults:

Gliomas

As a group, a glioma is one of the most common types of brain tumor. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain. There are two main types of supportive cells: astrocytes and oligodendrocytes. Most gliomas are called either astrocytoma or oligodendroglioma, or a mix of both. A glioma is given a grade (a measure of how much the tumor appears like normal brain tissue) from I to IV (one to four) based how likely they are to grow quickly. A grade I glioma is often considered a benign tumor, while grades II through IV are tumors with an increasing likelihood of growing and spreading quickly and are therefore considered possibly cancerous.

Types of gliomas include:

Astrocytoma. Astrocytoma is the most common type of glioma and begins in cells called astrocytes in the cerebrum or cerebellum. There are four grades of astrocytoma.

• Grade I or pilocytic astrocytoma is a slow-growing tumor that is most often benign and rarely spreads into nearby tissue. It accounts for about 2% of all brain tumors.
• Grade II or low-grade diffuse astrocytoma is a slow-growing tumor that can often spread into nearby tissue and can become a higher grade. It accounts for about 3% of all brain tumors.
• Grade III or anaplastic astrocytoma is a cancerous tumor that can quickly grow and spread to nearby tissues. It accounts for about 2% of all brain tumors.
• Grade IV or glioblastoma multiforme is a very aggressive form of astrocytoma that accounts for about 16% of all brain tumors.

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Brain Stem Glioma - Childhood-cancer

About the brain stem

The brain stem connects the brain to the spinal cord. It is the lowest portion of the brain, located above the back of the neck. The brain stem controls many of the body’s basic functions, such as motor skills, sensory activity, coordination and walking, the beating of the heart, and breathing. It has three parts:

• The midbrain, which develops from the middle of the brain
• The medulla oblongata, which connects to the spinal cord
• The pons, which is located between the medulla oblongata and the midbrain

About brain stem glioma

Brain stem glioma is a type of central nervous system (CNS; brain and spinal cord) tumor that begins when normal cells in the brain stem change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain.

Brain stem glioma is most often diffuse (spread freely) through the brain stem by the time it is found. This type of tumor is typically very aggressive, meaning that it grows and spreads quickly. A small percentage of brain stem tumors are very localized, called focal tumors. A focal tumor is often a low-grade tumor (the tumor cells look similar to normal cells) that is less likely to grow and spread quickly.

Brain stem glioma occurs most commonly in children between five and 10 years old. Most brain stem tumors develop in the pons and grow in a part of the brain stem where it can be difficult to perform surgery, making brain stem glioma challenging to treat (see the Treatment section).

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Bone Cancer

About bones

The human skeletal system is made up of more than 200 bones that protect the internal organs, allow people to stand upright, and attach to muscles that allow movement. Bones are connected to other bones by ligaments, which are bands of tough, fibrous tissue, while cartilage covers and protects the joints where bones come together. Bones are hollow and filled with bone marrow, which is the spongy, red tissue that produces blood cells. The cortex is the hard, outer portion of the bone.

Bone is a tissue that consists of collagen (a soft, fibrous tissue) and calcium phosphate (a mineral that helps harden and strengthen the bone). There are three types of bone cells:

• Osteoclasts break down and remove old bone.
• Osteoblasts build new bone.
• Osteocytes carry nutrients to the bone.

About bone cancer

Cancer can occur in any part of the bone. Cancer begins when normal cells in the bone change and grow uncontrollably, forming a mass called a tumor. A bone tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Even though a benign tumor does not spread outside the bone, it can grow large enough to press on surrounding tissue and weaken the bone. A malignant tumor can destroy the cortex and spread to nearby tissue. If bone tumor cells get into the bloodstream, they can spread to other parts of the body, especially the lungs.

There are different types of bone cancer, including:

• Osteosarcoma and Ewing sarcoma are two of the most common types of bone cancer and mostly occur in children and young adults.
• Chondrosarcoma is cancer of the cartilage and is more common in adults.
• Chordoma is a type of bone cancer that typically starts in the lower spinal cord.

Rarely, soft tissue sarcomas begin in the bone, including:

• Malignant fibrous histiocytoma (MFH), which makes up less than 1% of bone tumors and is usually found in adults. An arm or leg, especially around the knee joint, is the most common place for MFH to appear.
• Fibrosarcoma is also more common among adults, particularly during middle age, and most often begins in the thighbone.
• Paget’s disease of the bone generally occurs in older adults and involves the overgrowth of bony tissue.  

This section contains information about primary bone cancer (cancer that begins in the bone). It is much more common for bones to be the site of metastasis (spread) from other cancers, such as breast, lung, or prostate cancer. For example, lung cancer that has spread to the bone is called metastatic lung cancer, not bone cancer. For information about cancer that has started in another part of the body and spread to the bone, please see the information for that type of cancer or read the fact sheet on bone metastasis.

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Bladder Cancer

About the bladder and bladder cancer

The bladder is an expandable, hollow organ in the pelvis that stores urine (the body’s liquid waste) before it leaves the body during urination. The urinary tract is made up of the kidneys, ureters, bladder, and urethra and is lined with a layer of cells called the urothelium. This layer of cells is separated from the muscularis propria (bladder muscles) by the lamina propria (a thin, fibrous band).

Bladder cancer begins when normal cells in the bladder lining, most commonly urothelial cells, change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning is can spread to other parts of the body).

Types of bladder cancer

First, the type of bladder cancer depends on the type of cell where the cancer begins:

• Urothelial carcinoma. Urothelial carcinoma accounts for about 90% of all bladder cancers and begins in the urothelium. Urothelial carcinoma is the common term for this type of bladder cancer. It was previously called transitional cell carcinoma or TCC.

• Squamous cell carcinoma. This type accounts for about 4% of all bladder cancers and starts in squamous cells, which are thin, flat cells that form part of the bladder lining.

• Adenocarcinoma. This type accounts for about 2% of all bladder cancers and begins in glandular cells.

There are other, less common cell types that can develop into bladder cancer, including sarcoma (which begins in the fat or muscle layers of the bladder) and small cell anaplastic cancer (a rare type of bladder cancer that is likely to spread to other parts of the body).

In addition to its cell type, bladder cancer may be described as noninvasive, non-muscle-invasive, or muscle-invasive.

• Noninvasive. This type of bladder cancer usually does not extend through the lamina propria, while both types of invasive cancer can extend through the lamina propria. Noninvasive cancer may also be called superficial cancer, although that term is being used less often because it may incorrectly imply that this type of cancer is not serious. Noninvasive bladder cancer is less likely to spread and can often be managed with surgery to remove tumors and chemotherapy placed in the bladder (see Treatment).

• Non-muscle-invasive. Non-muscle-invasive bladder cancer typically grows only into the lamina propria. It is called invasive, but it is not the deeply invasive type that can spread to the muscle layer.

• Muscle-invasive. Muscle-invasive bladder cancer spreads into the bladder's muscularis propria and sometimes to the fatty layers or surrounding tissue outside the muscle.

It is important to note that both noninvasive and non-muscle-invasive bladder cancers have the possibility of spreading into the bladder muscle or to other parts of the body. Additionally, all cell types of bladder cancer can metastasize (spread) beyond the bladder. If the tumor has spread into the surrounding organs (the uterus and vagina in women, the prostate in men, and/or nearby muscles), it is called locally advanced disease. Bladder cancer also often spreads to the lymph nodes in the pelvis. If it has spread into the liver, bones, lungs, lymph nodes outside the pelvis, or other parts of the body, the cancer is called metastatic disease. This will be outlined more in Staging.

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Birt-Hogg-Dubé Syndrome-cancer

What is Birt-Hogg-Dubé syndrome?

Birt-Hogg-Dubé syndrome (BHD) is a hereditary condition associated with multiple noncancerous (benign) skin tumors, lung cysts, and an increased risk of both benign kidney tumors and kidney cancer. Symptoms of BHD generally do not appear until adulthood.

The most common skin tumors in BHD are called fibrofolliculomas. These are pale or flesh-colored tumors that occur in the hair follicles. Other skin tumors associated with BHD are called trichodiscomas and acrochordons. Acrochordons are also known as skin tags, and trichodiscomas are a growth of normal-appearing tissue that builds up into a noncancerous tumor. The lung cysts in BHD do not cause problems with breathing and do not increase the risk of lung cancer. There is an increased risk of spontaneous pneumothorax, which is air leaking out of the lungs and into the chest. Spontaneous pneumothorax may result in a collapsed lung. Many different types of kidney tumors have been seen in people with BHD. Multiple tumors may occur on both kidneys. Tumors tend to grow slowly, but they are likely to develop into kidney cancer.

What causes BHD?

BHD is a genetic condition. This means that the cancer risk and other features of BHD can be passed from generation to generation in a family. A specific gene called FLCN, which creates a protein called folliculin, is believed to cause most cases of BHD. FLCN is currently thought to be a tumor suppressor gene. A tumor suppressor gene's natural role in the body is to make proteins that prevent tumor formation by limiting cell growth. Research is ongoing to learn more about BHD.

How is BHD inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. BHD follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation.

Having the mutation in one copy of the gene is enough to cause BHD. However, current research shows that a person with BHD must have mutations in both copies of the FLCN gene in order for a tumor to appear. A person can be born with one mutated gene (the autosomal dominant inheritance pattern described above) and then acquire a mutation to the second copy of the FLCN gene at some point during his or her lifetime. Then, with both copies of the FLCN gene altered, the body loses its ability to suppress tumor growth.

How common is BHD?

BHD is considered to be rare. The number of people and families who have BHD is unknown. To date, about 60 families worldwide have been reported to have BHD.

How is BHD diagnosed?

BHD is suspected when a person has skin tumors associated with BHD, especially if that person or his or her family members have a history of lung cysts, spontaneous pneumothorax, or kidney cancer. BHD is also suspected in families with multiple cases of kidney cancer, particularly if family members have had different types of kidney cancer. Genetic testing to look for mutations in the FLCN gene is available for people suspected of having BHD.

What are the estimated cancer risks associated with BHD?

BHD is associated with an increased risk for kidney cancer, particularly the chromophobe renal cell carcinoma (RCC) type of kidney cancer. The estimated risk for kidney cancer in people with BHD is around 15%. The risk of kidney cancer is significantly increased in people who smoke. Individuals with BHD or at risk for BHD should avoid smoking. It is not known if people with BHD have an increased risk of other specific types of cancer.

What are the screening options for BHD?

There are no specific screening guidelines for BHD. Due to the risk of kidney cancer, some doctors suggest that individuals who have BHD or a family history of BHD have a yearly ultrasound (which uses sound waves to create a picture of the internal organs) of their kidneys, beginning at age 25. Other doctors suggest an abdominal computed tomography (CT or CAT; creates a three-dimensional picture of the inside of the body) scan or magnetic resonance imaging (MRI; uses magnetic fields, not x-rays, to produce detailed images of the body) every two years. Evaluation by a dermatologist (a doctor who specializes in diseases and conditions of the skin) and screening for lung cysts are also suggested.

Screening options may change over time as new technologies are developed and more is learned about BHD. It is important to talk with your doctor about appropriate screening tests.

Learn more about what to expect when having common tests, procedures, and scans.

Questions to ask the doctor

If you are concerned about your risk for kidney cancer, talk with your doctor. Consider asking the following questions:

• What is my risk of developing kidney cancer?
• What can I do to reduce my risk of cancer?
• What are my options for cancer screening?

If you are concerned about your family history and think your family may have BHD, consider asking the following questions:

• Does my family history increase my risk of developing kidney cancer?
• Are the skin tumors on me or on my family members consistent with a diagnosis of BHD?
• Should I meet with a genetic counselor?
• Should I consider genetic testing?

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Bile Duct Cancer

Bile duct cancer begins when normal cells in the bile duct change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

About the bile duct

The bile duct is a 4-inch to 5-inch long tube that connects the liver and gallbladder to the small intestine. The bile duct allows bile, which is made in the liver and stored in the gallbladder, to flow into the small intestine. Bile is a liquid that helps to break down fats found in foods and helps the body get rid of the waste material that is filtered out of the bloodstream by the liver.

The bile duct starts in the liver. Within the liver, smaller tubes (similar to small blood vessels) drain bile from the cells in the liver into larger and larger branches, ending in a tube called the common bile duct. Outside of the liver, the bile duct drains into the small intestine. The gallbladder is a reservoir that holds bile until food reaches the intestines. It is attached by a small duct, called the cystic duct, to the common bile duct about one-third of the way down the bile duct from the liver. The end of the bile duct empties into the small intestine.

See illustrations of the bile duct.

Types of bile duct cancer

Cancer can occur in any part of the bile duct. For bile duct cancer, doctors look at the exact location of the tumor:

Extrahepatic. The part of the bile duct that is outside of the liver is called extrahepatic. It is in this part of the bile duct where cancer usually begins. A common site for bile duct cancer is at the point where the right and left hepatic ducts join. A tumor that starts in this area is also sometimes called a Klatskin's tumor. The rest of the bile duct cancers that begin outside the liver occur between where the right and left hepatic ducts meet and where the bile duct empties into the small intestine.

Intrahepatic. About 5% to 10% of bile duct cancers are intrahepatic, or inside the liver.

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Beckwith-Wiedemann Syndrome-cancer

What is Beckwith-Wiedemann syndrome?

Beckwith-Wiedemann syndrome (BWS) is a growth regulation disorder. The most common features of BWS include macrosomia (large body size), macroglossia (large tongue), abdominal wall defects, an increased risk for childhood tumors, kidney abnormalities, hypoglycemia (low blood sugar) in the newborn period, and unusual ear creases or pits. Children with BWS may also have hemihyperplasia, in which some parts of the body are larger on one side than on the other.

The major features of BWS, macrosomia and macroglossia, are often present at birth. Abdominal wall defects such as omphalocele, which causes the inside of the abdomen to protrude through the navel, are also present at birth and may require surgery before an infant leaves the hospital. Mothers of children with BWS may have pregnancy complications, including premature delivery and polyhydramnios (excess amniotic fluid). An unusually large placenta and long umbilical cord may also be present.

The increased growth rate generally slows during childhood. Intellectual development is usually normal, and adults with BWS typically do not experience any medical problems related to their condition. 

What causes BWS?

BWS is a genetic condition related to changes in the genes (in an area called the short arm) of chromosome 11 (11p15.5). This is the area of the chromosome where two genes are located: insulin-like growth factor II (IGF-2) and cyclin-dependent kinase inhibitor (CDKN1C). In most cases (about 85%), the genetic changes that cause BWS happen sporadically (occurs by chance) in families where there is no history of the condition. In about 10% to 15 % of cases, the genetic changes may be inherited. This means that the risk for BWS can be passed from generation to generation in a family. The genetic mechanisms that cause gene mutations (alterations) resulting in BWS are complex.

How is BWS inherited?

The 10% to 15% of BWS that is inherited follows an autosomal dominant inheritance pattern. Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. In autosomal dominant inheritance, a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation. 

How common is BWS?

BWS has been found across different population groups. Approximately one in 13,700 people have BWS. Some researchers believe this number could be an underestimate.

How is BWS diagnosed?

The diagnosis of BWS is clinical, meaning that it is based primarily on physical features. BWS is suspected in children who are larger than expected for their age, especially if growth is not symmetrical (the same on both sides). An enlarged tongue and abdominal wall defect, primarily omphalocele, are also considered to be common features. There are many other features that may be seen in some children with BWS. However, not every child with BWS will have every feature. Features are listed as major (common) or minor (less common). It is generally agreed that at least one major feature and two minor features are required for a diagnosis of BWS:

Major Features

• Macrosomia (large body size)
• Macroglossia (large tongue)
• Omphalocele (abdomen protrudes through navel)
• Hemihyperplasia (some parts of the body are larger on one side)
• Ear lobe creases or pits
• Visceromegaly (enlargement of one or more abdominal organ)
• Embryonal tumor (Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma)
• Adrenocortical tumor (adrenal gland tumor)
• Kidney abnormalities
• Cleft palate (gap in the roof of the mouth)
• Family history of BWS

Minor Features

• Polyhydramnios (excessive amniotic fluid)
• Prematurity (low birth weight)
• Hypoglycemia (low blood sugar)
• Advanced bone age
• Heart problems
• Diastatsis recti (separation of the right and left sides of the main abdominal muscle)
• Hemangioma (noncancerous tumor made up of blood vessels)
• Facial nevus flammeus (hemangioma of the skin, also called a “port-wine stain”)
• Characteristic facial features
• Identical twins

Genetic testing for gene mutations associated with BWS is available, but it is complex. It is recommended that all families considering genetic testing for BWS meet with a clinical geneticist (a medical doctor who has training in genetics) and genetic counselor that can explain the tests and coordinate testing. The genetic testing methods that are currently available may be able to identify up to 80% of genetic mutations causing BWS.

What are the estimated cancer risks associated with BWS?

The estimated risk for a tumor in a child with BWS is about 7.5%.  Tumors are very rare after age 10, and the risk for an individual tumor decreases over time until the risk is similar to that of the general population. The cancer risk is highest in children with BWS who have hemihyperplasia and organomegaly (enlargement of organs), especially nephromegaly (enlargement of the kidneys) than in children with isolated hemihypertrophy. Several different tumor types, both cancerous and benign (noncancerous), have been reported in children with BWS. The most common tumor types are:

• Wilms tumor (kidney tumor; about 40% of cases)
• Hepatoblastoma (liver tumor)
• Adrenocortical carcinoma (about 20% of cases)
• Neuroblastoma
• Rhabdomyosarcoma

What are the screening options for BWS?

Screening recommendations for patients with BWS are aimed primarily at detecting hepatoblastoma and Wilms tumor. Current suggested screenings for people who are known or suspected to have BWS include:

• Baseline magnetic resonance imaging (MRI) or computed tomography (CT or CAT) scan of the abdomen at the time of diagnosis
• Abdominal ultrasound to screen for hepatoblastoma and Wilms tumor every three months, until age 8. After age 8, imaging may be limited to just a renal ultrasound
• Serum alpha-fetoprotein blood test every six weeks (every three months at the minimum), until age 4
• Regular physical examination, including abdominal exam; schedule determined by your doctor

If a child has an identical twin who doesn’t have signs of BWS, the twin should still be screened with ultrasounds and serum alpha-fetoprotein blood tests, as noted above.

Additionally, screening for hypoglycemia is important in infancy. Children with BWS may also need to be evaluated by a craniofacial team (doctors who specialize in treating head and face conditions) to determine if surgery may be required to decrease tongue size. Support may be needed to assist with feeding difficulties in infancy and speech development in childhood. Children with significant hemihyperplasia may need to be evaluated by an orthopedist (bone doctor).

Screening recommendations may change over time as new technologies are developed and more is learned about BWS. It is important to talk with your doctor about appropriate screening tests.

Learn more about what to expect when having common test, procedures, and scans.

Questions to ask the doctor

If you are concerned about the risk for cancer in your child, talk to your doctor. Consider asking the following questions of your doctor:

• What is my child’s risk of developing cancer?
• What can I do to reduce my child’s risk of cancer?
• What are my options for cancer screening? 

If you are concerned about your family history and think that you, your child, or other family members could have BWS, consider asking the following questions:

• Does our family history increase my child’s risk of developing cancer?
• Could my family have BWS?
• Should I meet with a genetic counselor?
• Should I consider genetic testing?

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Attenuated Familial Adenomatous Polyposis-cancer

What is attenuated familial adenomatous polyposis?

Attenuated familial adenomatous polyposis (AFAP) is a subtype of a condition known as familial adenomatous polyposis (called FAP or classic FAP). People with FAP or AFAP will have an increased number of adenomatous colon polyps during their lifetime and an increased risk of developing colorectal cancer. An adenomatous polyp is a lump filled with the cells that make mucous and line the inside of a person's colon. Normally these cells are in flat sheets, but in FAP and AFAP they build up into polyps inside the intestinal tract. In AFAP, the total number of colon polyps is less than 100, with 30 being average. In FAP, polyps are far more frequent.

Polyps in people with AFAP tend to develop later in life than in individuals with classic FAP, although polyps may develop as early as the late teens. Colorectal cancer can develop later in people with AFAP as well, with the average age at diagnosis being around 50. Polyps and cancer of the stomach and small intestines are also seen in families with AFAP.

AFAP is still being defined by doctors. It has not been determined if families with AFAP have the same risk for other types of cancer, in addition to colon cancer, as families with classic FAP. Risks for some of the other, noncancerous features associated with classic FAP have not been determined. For instance, people with AFAP appear to have a lesser chance of developing desmoid tumors (noncancerous fibrous tumors that can grow anywhere in the body) or congenital hypertrophy of the retinal pigment epithelium (CHRPE, which is an eye condition), than people diagnosed with FAP. Find out more about classic FAP.

What causes AFAP?

AFAP is a genetic condition. This means that the cancer risk can be passed from generation to generation in a family, but usually not all children of a person with AFAP will be affected. The gene associated with AFAP is APC; APC stands for adenomatous polyposis coli. A mutation (alteration) in the APC gene gives a person an increased lifetime risk of developing multiple adenomatous colon polyps, colorectal cancer, and other cancers of the digestive tract. People who are diagnosed with AFAP, and their family members, should talk with a genetics counselor or medical genetics specialist (a health professional with specialized training in medical genetics).

How is AFAP inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. AFAP follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation.

How common is AFAP?

AFAP is uncommon and possibly under-diagnosed. Most colorectal cancer and colon polyps are sporadic (occurs by chance), not related to AFAP, classic FAP, or other inherited syndromes. The number of colorectal cancer cases and the number of people with multiple adenomatous colon polyps that are related to AFAP are unknown.

How is AFAP diagnosed?

AFAP is suspected when a person has a history of more than 20, but less than 100, adenomatous colon polyps. People suspected of having AFAP can have a blood test to look for a mutation in the APC gene. If an APC gene mutation is found, a diagnosis of AFAP is confirmed. Other family members will be diagnosed with AFAP if they are tested and have the same gene mutation.

What are the estimated cancer risks associated with AFAP?

The cancer risks for AFAP are considered to be similar to the risks associated with classic FAP, but the overall cancer risks may be lower. As more information is learned about AFAP, more specific cancer risks may emerge.

• Colorectal cancer: considered to be high, but less than 100% (if not treated)

Estimated digestive tract cancer risks for classic FAP. (It is unknown if risks are the same in AFAP but screening is suggested due to the similarities in both syndromes.)

• Small bowel (intestines): 4% to 12%
• Pancreatic cancer: 2%
• Stomach: 0.5%

What are the screening options for AFAP?

It is important to discuss with your doctor the following screening options, as each individual is different:

• Colonoscopy: every two to three years, beginning at age 18
• Colectomy(the surgical removal of the entire colon). This may be considered if polyps cannot be managed with regular colonoscopies because there are too many or if a patient cannot have colonoscopies on the recommended schedule above.

There is an increased risk of polyps of the upper digestive tract in AFAP, and regular screening should be considered similar to classic FAP. The suggested screening for classic FAP is below.

• Upper endoscopy (EGD) every one to three years, beginning at age 25 or after polyps are detected
• X-ray or computed tomography (CT or CAT) scan of the small bowel if adenomas are found on the EGD or before a colectomy; repeat every one to three years depending on symptoms

It is unknown whether screening of the thyroid or screening for hepatoblastoma (a type of liver cancer) in young children is appropriate for families with AFAP as it is with classic FAP; see additional suggested screening for families with classic FAP.

Screening options may change over time as new technologies are developed and more is learned about AFAP and classic FAP. It is important to talk with your doctor about appropriate screening tests.

Learn more about what to expect when having common tests, procedures, and scans.

Questions to ask the doctor

If you are concerned about your risk of colorectal cancer, talk with your doctor. Consider asking the following questions of your doctor:

• What is my risk of developing colorectal cancer?
• How many colon polyps have I had in total?
• What type of colon polyps have I had? (The two most common kinds are hyperplastic and adenomatous.)
• What can I do to reduce my risk of cancer?
• What are my options for cancer screening?

If you are concerned about your family history and think your family may have AFAP, consider asking the following questions:

• Does my family history increase my risk of colorectal cancer?
• Should I meet with a genetic counselor?
• Should I consider genetic testing?

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Ataxia-Telangiectasia-cancer

Ataxia-Telangiectasia  

What is ataxia-telangiectasia?

Ataxia-telangiectasia (A-T) is a hereditary condition characterized by progressive neurologic problems that lead to difficulty walking. Signs of A-T often develop in childhood. Children with A-T may begin staggering and appear unsteady (ataxia) shortly after learning to walk. Most people with A-T will eventually need to use a wheelchair. People with A-T have normal intelligence, but over time, they will develop slurred speech and have difficulty with writing and other tasks. Red marks called telangiectasias are caused by dilated capillaries (tiny blood vessels) and appear on the skin and eyes as people get older. People with A-T also have a weakened immune system and are prone to infections. In addition, they appear to be particularly sensitive to ionizing radiation (such as x-rays) and have an increased risk of cancer.

What causes A-T?

A-T is a genetic condition. This means that the risk for A-T can be passed from generation to generation in a family. The gene associated with A-T is ATM (ataxia telangiectasia mutated). Mutations (changes) in the ATM gene cause A-T.

How is A-T inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. A-T follows an autosomal recessive inheritance pattern. In autosomal recessive inheritance, a mutation must be present in both copies of the gene in order for a person to be affected. This means that each parent must pass on a gene mutation for a child to be affected. A person who has only one copy of the gene mutation is called a carrier. When both parents are carriers of a recessive gene mutation, there is a 25% chance that a child will inherit two mutations and be affected.

How common is A-T?

A-T is rare. It is estimated that A-T affects one in 40,000 to one in 100,000 individuals. The chance that a person is a carrier of a single ATM gene mutation is about 1%, or one in 100.

How is A-T diagnosed?

A-T is suspected whenever a child develops signs of ataxia (unsteady walking). Testing of the ATM gene is available, and about 90% of mutations can be found. The following tests may be more helpful in determining if someone has A-T or another type of ataxia:

Immunoblotting (ATM protein testing). This is the best test to diagnose A-T. Nearly all individuals with A-T will have very low or no amounts of the protein made by the ATM gene.

Radiosensitivity assay. Since people with A-T have an increased sensitivity to radiation, removing some cells and treating the sample with radiation therapy can help make the diagnosis. It can take up to three months to get a result from this test.

ATM kinase activity. This test looks at the activity level of the protein made by the ATM gene. Little to no activity means that there is likely a mutation in the ATM gene.

What are the estimated cancer risks associated with A-T?

People with A-T also have about a 40% risk of developing cancer. The most common types of cancer seen in people with A-T are leukemia and lymphoma. These two types of cancers can appear in childhood and account for 85% of all cancers in people with A-T. As people with A-T live longer, there appears to be an increased risk of other cancer types, including breast cancer, ovarian cancer, stomach cancer, melanoma, and sarcoma.

Carriers (people with one ATM gene mutation) also seem to have an increased cancer risk. It is estimated that carriers have a 4% increase in cancer risk compared with the general population. Of particular concern is the potential breast cancer risk in women who carry an ATM gene mutation. Some studies have shown a large increase in breast cancer risk for women who are carriers, while other studies have shown no increased risk. Additional research is needed to clarify the cancer risk for ATM mutation carriers. Studies also show that carriers may have an increased risk of heart disease.

What are the screening options for A-T?

Children and adults with A-T should see their doctor regularly and be monitored for signs of cancer. Individuals with A-T who frequently develop infections are encouraged to have their immune status checked regularly.

There are no specific cancer screening or prevention recommendations for individuals with A-T or gene mutation carriers. However, women who are carriers are encouraged to talk with their doctor about breast cancer screening options.

Screening options may change over time as new technologies are developed and more is learned about A-T. It is important to talk with your doctor about appropriate screening tests.

Learn more about what to expect when having common tests, procedures, and scans.

Questions to ask the doctor

If you are concerned about your risk of cancer, talk with your doctor. Consider asking the following questions of your doctor:

• What is my risk of developing cancer?
• What can I do to reduce my risk of cancer?
• What are my options for cancer screening?

If you are concerned about your family history and think your family may have A-T, consider asking the following questions:

• Does my family history increase my risk of developing cancer?
• Could my family have A-T?
• Should I meet with a genetic counselor?

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Astrocytoma - Childhood

The brain and spinal column make up the central nervous system (CNS), where all vital functions of the body, including thought, speech, and strength, are controlled.

Astrocytoma is a type of CNS tumor that forms in cells called astrocytes. Normal astrocytes provide the connecting network of the brain and spinal cord and form scar tissue when the CNS is damaged. Astrocytoma begins when normal astrocytes change and grow uncontrollably, forming a mass called a tumor.

Astrocytoma can occur throughout the CNS, including in the following places:

• Cerebellum (the back part of the brain responsible for coordination and balance)
• Cerebrum (the top part of the brain that controls motor activities and talking)
• Diencephalon or central part of the brain (controls vision, hormone production, and arm and leg movement)
• Brain stem (controls eye and facial movements, arm and leg movement, and breathing)
• Spinal cord (controls sensation and arm and leg motor function)

A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Astrocytoma is more commonly referred to by its grade (a measure of how much the tumor cells appear like normal cells), as either high grade or low grade, depending on the number and shape of the tumor cells and how quickly they grow and spread. A low-grade tumor is less likely to grow quickly or spread. Once a tumor is found in the CNS, the doctor will usually perform a biopsy (see Diagnosis) to see if the tumor is an astrocytoma and to determine the grade of the tumor. A biopsy is the removal of a small amount of tissue for examination under a microscope.

This section covers astrocytoma diagnosed in children; learn more about brain tumors in adults

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Appendix Cancer

About the appendix

The appendix is a pouch-like tube that is attached to the cecum (the first section of the large intestine or colon). The appendix averages 10 centimeters (cm) in length and is considered part of the gastrointestinal (GI) tract. Generally thought to have no significant function in the body, the appendix may be a part of the lymphatic, exocrine, or endocrine systems.

Appendix cancer occurs when cells in the appendix become abnormal and multiply without control. These cells form a growth of tissue, called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Another name for this type of cancer is appendiceal cancer.

Types of appendix tumors

There are different types of tumors that can start in the appendix:

• Carcinoid tumor. A carcinoid tumor starts in the hormone-producing cells that are normally present in small amounts in almost every organ in the body. A carcinoid tumor starts primarily in either the GI tract or lungs, but it also may occur in the pancreas, a man’s testicles, or a woman’s ovaries. An appendix carcinoid tumor most often occurs at the tip of the appendix. Approximately 66% of all appendix tumors are carcinoid tumors. This type of cancer usually causes no symptoms until it has spread to other organs and often goes unnoticed until it is found during an examination or procedure performed for another reason. An appendix carcinoid tumor that remains confined to the area where it started has a high chance of successful treatment with surgery. Learn more about carcinoid tumors.

• Mucinous cystadenocarcinoma. Mucinous cystadenocarcinoma is the most common non-carcinoid appendix tumor and accounts for about 20% of appendix cancer cases. This type of tumor produces a jelly-like substance called mucin that can fill the abdominal cavity and can cause abdominal pain, bloating, and changes in bowel function if the tumor breaks through the appendix or grows in the abdomen.

• Colonic-type adenocarcinoma. Colonic-type adenocarcinoma accounts for about 10% of appendix tumors and usually occurs at the base of the appendix. This type of tumor looks and behaves like the most common type of colorectal cancer. It often goes unnoticed, and a diagnosis is frequently made during or after surgery for appendicitis (inflammation of the appendix that can cause abdominal pain or swelling, loss of appetite, nausea, vomiting, constipation or diarrhea, inability to pass gas, or a low fever that begins after other symptoms).

• Signet-ring cell adenocarcinoma. Signet-ring cell adenocarcinoma (so called because, under the microscope, the cell looks like it has a signet ring inside it) is very rare and considered to be more aggressive and more difficult to treat than other types of adenocarcinomas. This type of tumor usually occurs in the stomach or colon, and it can cause appendicitis when it develops in the appendix.

• Paraganglioma. Paraganglioma is a rare tumor that develops from cells of the paraganglia, a collection of cells that come from nerve tissue that persist in small deposits after fetal (pre-birth) development, and is found near the adrenal glands and some blood vessels and nerves. This type of tumor is usually considered benign and is often successfully treated with the complete surgical removal of the tumor. Paraganglioma is very rare outside of the head and neck region

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Amyloidosis

 

 

About amyloidosis

Amyloidosis is not cancer, but it is a serious, potentially life-threatening condition that can occur because of some cancers. The basis of amyloidosis is the amyloid, which is an abnormal protein that enters tissues or organs. When enough amyloid proteins are present in an organ to interfere with its functioning and cause symptoms, it becomes a condition called amyloidosis.
Only in the past few decades have doctors and researchers begun to understand the nature of amyloid proteins and how they can cause damage. Research is ongoing to learn more.

Types of amyloidosis

There are different types of amyloidosis, including the following:

• AL amyloidosis. This is the most common type of amyloidosis in the United States. The amyloid proteins that accumulate in the tissues in this condition are known as light (L) chains. AL amyloidosis is not usually associated with another underlying condition, although it is sometimes associated with multiple myeloma.
• AA amyloidosis. In this condition, the amyloid protein that accumulates in the tissues is known as the A protein. AA amyloidosis is associated with such chronic diseases as diabetes, tuberculosis, rheumatoid arthritis, or inflammatory bowel disease. It may also accompany aging. The spleen, liver, kidneys, adrenal glands, and lymph nodes may be involved.

• Hereditary amyloidosis (ATTR). Hereditary amyloidosis is rare. It is a specific type of amyloidosis that can be passed down from generation to generation in a family. It may cause peripheral sensory and motor neuropathy problems (issues relating to the central nervous system), carpal tunnel syndrome, and eye abnormalities. The most common subtypes involve the transthyretin (TTR) protein.

This section covers AL, AA, and hereditary amyloidosis. Other types of amyloidosis include ß2 microglobulin amyloidosis, which occurs in some patients with chronic renal (kidney) problems, and localized forms of amyloidosis

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Adrenal Gland Tumor

About the adrenal glands

Each person has two adrenal glands—one located on top of each of the body’s two kidneys. These glands are important to the body’s endocrine (hormonal) system. Each adrenal gland has two main parts that function separately:
Adrenal cortex. The outer part of the adrenal gland is called the cortex. The adrenal cortex makes three main hormones: cortisol, aldosterone, and dehydroepiandrosterone (DHEA). These hormones carefully control metabolism and body characteristics, such as hair growth and body shape.
Adrenal medulla. The gland’s inner part is called the medulla. The adrenal medulla makes other hormones: epinephrine, norepinephrine, and dopamine. These hormones control the body’s responses to stress, including the “fight or flight” adrenaline surge.

Types of adrenal gland tumors

A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
An adrenal gland tumor can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. An adrenal gland tumor that does not produce hormones is called a nonfunctioning tumor. A tumor can start in an adrenal gland (called a primary adrenal tumor), or it can begin in another organ, such as the lungs, and then metastasize (spread) to the adrenal glands. The symptoms and treatment of an adrenal gland tumor depend on whether the tumor is functioning or nonfunctioning, what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or a metastases from cancer of another organ.
This section focuses on primary adrenal gland tumors, which include the following:
Adenoma. Adenoma is the most common type of adrenal gland tumor, making up the majority of all adrenal gland tumors. It is a noncancerous, nonfunctioning tumor of the adrenal cortex. Also called an adrenocortical adenoma, this tumor usually does not cause symptoms and, if it is small, often does not need treatment.
Adrenocortical carcinoma. Although rare, the most common type of cancerous adrenal gland tumors begins in the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Approximately four to 12 out of one million people develop this type of tumor. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor; if the tumor is functioning, it may produce more than one hormone.
Neuroblastoma. This is a type of childhood cancer that can begin in the adrenal medulla. Learn more about childhood neuroblastoma.
Pheochromocytoma. This type of cancerous neuroendocrine tumor most often begins in the adrenal medulla.

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Adenoid Cystic Carcinoma

Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
Adenoid cystic carcinoma (AdCC) is a rare form of adenocarcinoma, which is a broad term describing any cancer arising from glandular tissues. AdCC is found mainly in the head and neck, but it can occasionally occur in a woman’s uterus or other sites in the body. It most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva scattered throughout the upper aerodig  estive tract (the organs and tissues of the upper respiratory tract, including the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus and windpipe). A tumor may begin in the:

Minor salivary glands

• Palate (roof of the mouth)
• Nasopharynx (air passageway at the upper part of the throat and behind the nose)
• Tongue base (the back third of the tongue)
• Mucosal lining of the mouth (inner lining of the mouth; glands located here produce mucus)
• Larynx (voicebox)
• Trachea (windpipe)

Major salivary glands

• Parotid (largest salivary gland found on either side of the face in front of the ears)
• Submandibular (found under the jawbone)
• Sublingual glands (in the bottom of the mouth under the tongue)

Regardless of where it starts, AdCC tends to spread along nerves (perineural invasion) or through the bloodstream. It spreads to the lymph nodes in only about 5% to 10% of cases. The most common place of metastases (spreading) is the lung. AdCC is known for having long periods of indolence (no growth) followed by growth spurts. However, AdCC can behave aggressively in some people, making the course of the AdCC unpredictable.
Besides being classified based on where the cancer begins, AdCC is also described based on the histologic (how cells look under a microscope) variations of the tumor, including cylindroma, cribiform, and solid AdCC. AdCC is sometimes classified as a disease of the minor salivary gland, even though it may begin at other sites.
This section covers AdCC. Learn more about head and neck cancers.
Find out more about basic cancer terms used in this section. Or, choose “Next” (below, right) to continue reading this detailed section. To select a specific topic within this section, use the icon panel located on the right side of your screen.

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