Carcinoid Tumor Cancer http://carcinoidtumorsite.com Just another WordPress weblog Fri, 17 Aug 2007 09:11:32 +0000 http://wordpress.org/?v=2.1.2 en Ancestral Occurrence of Carcinoid http://carcinoidtumorsite.com/2007/08/17/ancestral-occurrence-of-carcinoid/ http://carcinoidtumorsite.com/2007/08/17/ancestral-occurrence-of-carcinoid/#comments Fri, 17 Aug 2007 09:01:08 +0000 admin http://carcinoidtumorsite.com/2007/08/17/ancestral-occurrence-of-carcinoid/ It explains the presence of carcinoid if someone have any familial occurrence. Carcinoid tumors are generally thought to be sporadic, except for a small proportion that occur as a part of multiple endocrine neoplasia syndromes. Data regarding the familial occurrence of carcinoid as well as its potential association with other neoplasms are limited. A chart review was conducted on patients indexed for malignant carcinoid tumor of the gastrointestinal tract seen at the Mayo Clinic between 1988 and 1996. A survey of family history of malignancies and personal history of other tumors was mailed to all eligible patients. Data for 245 patients were analyzed. Observed rates of carcinoids and other malignancies were compared with Surveillance, Epidemiology, and End Results data. Estimates of the cumulative probability for first-degree relatives developing a carcinoid tumor were calculated. Nine (3.7%) patients with carcinoid tumor had at least one first-degree relative with the same malignancy. The rate of carcinoid tumor in first-degree relatives of probands was higher (P < 0.0001) than expected based on the Surveillance, Epidemiology, and End Results population data. Cumulative probability in a first-degree relative for developing a carcinoid was calculated to be 1.5% at age 80. There was an increased risk for developing a carcinoid tumor among first-degree relatives of patients with carcinoid. Neither patients with carcinoid nor their first-degree relatives had an increased incidence of other malignancies.

Carcinoid crisis

Carcinoid crisis occurs when all of the symptoms of carcinoid syndrome occur at the same time. Carcinoid crisis is the most serious and life-threatening complication of carcinoid syndrome and is generally found in people who already have carcinoid syndrome. Carcinoid crisis may occur suddenly, or it can be associated with stress, chemotherapy, or anesthesia. A carcinoid crisis may be prevented and successfully treated with octreotide (Sandostatin), a therapy that can help to raise low blood pressure and control the production of hormones.

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]]> http://carcinoidtumorsite.com/2007/08/17/ancestral-occurrence-of-carcinoid/feed/ Midgut Carcinoids http://carcinoidtumorsite.com/2007/08/17/midgut-carcinoids/ http://carcinoidtumorsite.com/2007/08/17/midgut-carcinoids/#comments Fri, 17 Aug 2007 08:58:10 +0000 admin http://carcinoidtumorsite.com/2007/08/17/midgut-carcinoids/ Midgut Carcinoid is the type of Carcinoid tumor. General introduction Carcinoids are rare slowly growing, neuroendocrine tumors. In 1907 Obendorfer was the first to use the term carcinoid (Karzinoide). He described an ileal tumor with a much slower progression than expected from denocarcinomas.
The traditional classification of the carcinoids according to their embryonal site of origin was introduced in 1963. It comprises foregut-(in the lung, thymus, stomach, pancreas and proximal duodenum) midgut- (from the distal duodenum to proximal colon) and hindgut carcinoids (origin in the distal colon enrectum). This classification corresponds consequently to their vascular supply, namely the celiac axis, superior mesenteric artery and inferior mesenteric artery.
Carcinoids can develop in almost all organs arising from the primitive entoder as well as the ovary and retroperitoneum.
In this thesis we will especially focus on the midgut carcinoids. The midgut carcinoids are usually referred to as the “classical” carcinoids. They arise from cells of Kulchitsky in the intestinal crypts and display, when producing serotonin, both an argentaffin and argyrophilic staining reaction. Midgut carcinoids can produce several biogenic amines such as serotonin, radykinin, prostaglandin, catecholamines and substance P. These products only exert their influence once they have passed the liver into the systemic circulation, as the liver is able to metabolize these amines. An ovarian carcinoid can therefore cause early symptoms because the ovary drains directly into the caval vein and not into the portal vein. In case of liver metastases, the liver itself becomes a production site of these amines which then enter the systemic circulation causing several symptoms we refer to as the carcinoid syndrome, i.e.
hot flushes, diarrhea and circulatory imbalance. The carcinoid patient with the classical symptoms will often be diagnosed as having widespread hepatic metastases. This delay is partly explained by the relatively small mass effect of the primary tumor that causes only mild symptoms. At laparotomy a small
primary tumor and massive metastatic disease is suggestive for carcinoid disease.
From the surgeon’s point of view there are two major groups of carcinoid patients, those who are accidentally diagnosed during appendectomy for a suspected appendicitis and those who are referred by the gastroenterologist or medical oncologist, and are often metastasized. The first group offers no problems and abdominal spread or liver metastases are rare in these cases.5-7 In case of metastasized patients however the medical oncologist, surgical oncologist and the patient have to weigh several treatment options and the timing of interventions. Although the treatment is usually palliative in case of etastases, patients can survive many years.
Aim of this thesis
The aim of this thesis is to get insight in surgical aspects of the treatment of carcinoid patients and the role of vasoactive substances produced by the tumor and their vascular effects.

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]]> http://carcinoidtumorsite.com/2007/08/17/midgut-carcinoids/feed/ Drugs to Treat Carcinoid Syndrome http://carcinoidtumorsite.com/2007/08/17/drugs-to-treat-carcinoid-syndrome/ http://carcinoidtumorsite.com/2007/08/17/drugs-to-treat-carcinoid-syndrome/#comments Fri, 17 Aug 2007 08:53:20 +0000 admin http://carcinoidtumorsite.com/2007/08/17/drugs-to-treat-carcinoid-syndrome/ The most important treatment modality for the carcinoid syndrome is octreotide, a synthetic hormone similar in structure to the naturally-occurring hormone, somatostatin. Somatostatin is widely distributed in the body where it can inhibit the secretion of many other hormones including growth hormone, insulin, and gastrin. It exerts its action by binding to specific receptors on the membranes of cells that produce and release hormones and chemical substances.

Octreotide is an agent chemically related to a natural hormone, somatostatin. It is very helpful in treating the flushing, diarrhea, and wheezing from carcinoid syndrome. While this drug rarely shrinks carcinoid tumors, it may slow or stop their growth. Although this is not curative, it can prolong life. The main side effects are pain at the site of the injection, and rarely, stomach cramps, nausea, vomiting, headaches, dizziness, and fatigue. Octreotide causes sludging of bile and may cause gallstones in the gallbladder (cholelithiasis). It can also result in insulin resistance that can make pre-existing diabetes more difficult to control.

This drug has become available as a long-acting injection that needs to be given only once a month, which may help patients more than the short-acting version. A similar drug, lanreotide, is also being studied, as is a newer agent called pasireotide.

 Interferons are natural substances that normally activate the body’s immune system. They also slow the growth of tumor cells. Interferon-alfa is sometimes helpful in shrinking or slowing the growth of metastatic neuroendocrine cancers and improving symptoms of carcinoid syndrome. Its usefulness is sometimes limited by its flu-like side effects, which may be severe. The drug is given by injection.

An antihistamine called cyproheptadine can help relieve some symptoms. Other medicines are also available to control specific symptoms. Please ask your doctor about these, or describe your symptoms to your doctor and ask about medicines to control them.

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]]> http://carcinoidtumorsite.com/2007/08/17/drugs-to-treat-carcinoid-syndrome/feed/ Diagnostic Challenge of Carcinoid Tumor http://carcinoidtumorsite.com/2007/08/17/diagnostic-challenge-of-carcinoid-tumor/ http://carcinoidtumorsite.com/2007/08/17/diagnostic-challenge-of-carcinoid-tumor/#comments Fri, 17 Aug 2007 08:48:36 +0000 admin http://carcinoidtumorsite.com/2007/08/17/diagnostic-challenge-of-carcinoid-tumor/ Carcinoid tumor presents many diagnostic challenges. As rare cancers go, carcinoid tumors are among the more unusual. These slow-growing tumors can occur in almost any part of the body. If there are any symptoms at all, they tend to be misdiagnosed as other diseases, and the tumors are often discovered during unrelated surgeries or medical procedures. These baffling characteristics make it difficult to prevent and diagnose carcinoid tumors, which bring a large number of patients to M. D. Anderson.

“From 1999 to 2000, we saw about 140 new carcinoid tumor patients, and the numbers have been going up steadily,” says Dr. James Yao, a neuroendocrine tumor expert who handles virtually all carcinoid patients at M. D. Anderson. “Although it’s a fairly uncommon tumor, patients are becoming more educated and tend to seek out large cancer centers like ours where the experts are.”

Carcinoid tumors can develop wherever there are neuroendocrine (hormone-producing) cells in the body. “About 75% of carcinoids arise in the gastrointestinal tract, particularly the small intestine,” Dr. Yao says. “Another 24% occur in the lung and the remaining 1% occur everywhere else.” They are rare, occurring in roughly two out of every 100,000 people, Dr. Yao says, although accurate statistics for carcinoid tumors are not readily available.

Many carcinoids exist without symptoms for years, which makes early diagnosis difficult at best. “Because it’s such a slow-growing cancer, there are a lot of patients with incidental carcinoids that may never be detected,” Dr. Yao says. They are generally diagnosed only when they grow large enough to cause a bowel obstruction or other discomfort. In fact, most early-stage carcinoids are discovered incidentally during colonoscopies, regular physical exams and appendectomies or other surgeries.

A CT scan also can detect carcinoids, but it is not an effective screening tool.   

Larger, more advanced carcinoid tumors cause symptoms that include diarrhea, skin flushing and asthma – a combination of conditions known as carcinoid syndrome. “Patients with a long history of these symptoms were often previously thought to have sprue, irritable bowel syndrome or other diseases, and their carcinoids weren’t diagnosed until much later,” Dr. Yao says. “I have a patient who had skin flushing for 10 years, and is just now being diagnosed with a carcinoid tumor.”

The cause of carcinoid tumors is still not well understood. “There have been some studies linking carcinoids to multiple endocrine neoplasia (MEN), but that accounts for only a very small set of patients,” Dr. Yao says. “Chronic gastritis or other conditions that cause excess production of gastrin may also play a role, but there are too few studies at this point to determine what causes these tumors.”

Predicting the survival rate of carcinoid tumor patients isn’t easy, either. “There is an often-quoted retrospective review that shows a five-year survival rate of about 67% in patients with carcinoid syndrome, but good data isn’t easy to come by,” Dr. Yao says. “There are efforts under way to better define the prognosis of carcinoids and to create a uniform staging system to help predict tumor progression and patient survival.

Treatment of carcinoids depends largely on size and metastasis (spread). For small tumors that have not metastasized, surgery is the best option and often has a high success rate. “The success of shrinking carcinoids with chemotherapy is not as high as we’d like it to be; only about 10% to 15% at best,” Dr. Yao says. “Conventional radiation therapy is often difficult because carcinoids tend occur in the small bowel and other sensitive areas of the body.” Targeted internal radiation therapies are being tested at some cancer centers, and some new biologic therapies may soon be studied in clinical trials.

Dr. Yao is lead investigator on an M. D. Anderson clinical trial of the drug Gleevec for patients with metastatic or inoperable carcinoid tumors. Gleevec has been used successfully to treat certain leukemias and is being tested on other cancers. “It’s too early to tell if Gleevec will work for carcinoids, since it’s a new study,” he says. “We’re still actively recruiting patients.”

What’s the outlook for carcinoid patients? The lack of effective screening tools for the disease means that many, if not most, carcinoids won’t be diagnosed until they’re advanced, a situation that’s not likely to change soon. However, the outlook is far from grim. “We have to be careful how we define successful treatment,” Dr. Yao says. “Certainly, these patients can have good quality of life and live a long time even with their disease. While we may not have a cure for a majority of metastatic carcinoid tumors, we can still help these patients.” 

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]]> http://carcinoidtumorsite.com/2007/08/17/diagnostic-challenge-of-carcinoid-tumor/feed/ Carcinoid Tumor Metastatic to the Breast http://carcinoidtumorsite.com/2007/08/17/carcinoid-tumor-metastatic-to-the-breast/ http://carcinoidtumorsite.com/2007/08/17/carcinoid-tumor-metastatic-to-the-breast/#comments Fri, 17 Aug 2007 08:41:02 +0000 admin http://carcinoidtumorsite.com/2007/08/17/carcinoid-tumor-metastatic-to-the-breast/ Breast metastases from nonmammary malignant neoplasms are uncommon, accounting for approximately 2% of breast tumors. There are 13 cases reported in the literature of carcinoid tumor metastatic to the breast, and more than half of these cases were misdiagnosed pathologically and treated as primary breast carcinoma, even in cases with a medical record of carcinoid tumor. We describe a patient with a history of asthma and diarrhea who presented to the University of Arkansas for Medical Sciences,

Little Rock, with an exacerbation of the asthma. The results of routine physical examination revealed a mass in the left breast. A diagnosis of carcinoid tumor metastatic to the breast was made after a partial mastectomy was performed.The differential diagnosis between primary carcinoid tumor of the breast and carcinoid tumor metastatic to the breast is often controversial in surgical pathology. Diagnoses need to be made correlating clinical and histological examination in difficult cases in which there is not a diagnosis of carcinoid tumor elsewhere. Accurate diagnosis of breast metastases is important to avoid unnecessary treatment.

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]]> http://carcinoidtumorsite.com/2007/08/17/carcinoid-tumor-metastatic-to-the-breast/feed/ How to Cope up with Carcinoid Tumor? http://carcinoidtumorsite.com/2007/08/17/how-to-cope-up-with-carcinoid-tumor/ http://carcinoidtumorsite.com/2007/08/17/how-to-cope-up-with-carcinoid-tumor/#comments Fri, 17 Aug 2007 08:38:36 +0000 admin http://carcinoidtumorsite.com/2007/08/17/how-to-cope-up-with-carcinoid-tumor/ With treatment, it is also necessary to find other ways and means to handle this problem. Everyone has his or her own way of coping with cancer. But, you don’t have to do it alone. If you have questions or would like guidance, talk with a member of your health care team. Also consider the following steps to help you deal with your diagnosis:

  • Find out all you can about carcinoid tumors. Ask your doctor questions about your condition. Ask members of your health care team to recommend resources where you can get more information. The more you know about your condition, the better you’re able to participate in decisions about your care.
  • Talk to others. Support groups for people with carcinoid tumors put you in touch with others who have faced the same challenges you’re facing. Ask your doctor about groups in your area. Carcinoid tumors are rare, though, so you may need to connect with people outside your immediate area.
  • Take care of yourself. Do what you can to maintain a healthy lifestyle. Choose healthy meals with plenty of fruits and vegetables. When you feel up to it, work light exercise into your daily routine. Cut extra stress out of your life when possible. Get plenty of sleep so that you feel rested when you wake up. Take care of your body and mind so that you’re better able to stick to your cancer treatment plan.

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]]> http://carcinoidtumorsite.com/2007/08/17/how-to-cope-up-with-carcinoid-tumor/feed/ Complications Associated with Carcinoid Tumor http://carcinoidtumorsite.com/2007/08/17/complications-associated-with-carcinoid-tumor/ http://carcinoidtumorsite.com/2007/08/17/complications-associated-with-carcinoid-tumor/#comments Fri, 17 Aug 2007 08:36:33 +0000 admin http://carcinoidtumorsite.com/2007/08/17/complications-associated-with-carcinoid-tumor/ Different problems are associated with this disease. Like the neuroendocrine cells they come from, carcinoid tumors can secrete hormones and other chemicals into your body. Carcinoid tumors may produce serotonin, a chemical that helps regulate your mood, appetite and sensory perception. The chemicals from carcinoids can cause a number of medical problems. These and other complications of carcinoid tumors include:

·        Carcinoid syndrome. Carcinoid syndrome is a result of the hormones made by carcinoid tumors. The condition produces redness or a feeling of warmth in your face and neck (skin flushing), diarrhea and difficulty breathing, among other signs and symptoms.

·        When carcinoid tumors secrete chemicals, your liver usually eliminates them before they have a chance to circulate throughout your system. Most people who experience carcinoid syndrome have an advanced, metastatic carcinoid tumor that has spread to the liver, where the chemicals released have a better chance of getting into the bloodstream. Exceptions to this are carcinoids in the ovaries and the lungs, which can produce signs and symptoms of carcinoid syndrome before the disease has spread.

  • Heart problems. The hormones from carcinoids may cause thickening of the lining of your heart’s chambers, valves and blood vessels. It’s more common for the right side of the heart to be affected than the left. This condition often causes the valve between the upper and lower chambers on the right side of your heart to leak (tricuspid regurgitation), allowing blood to flow backward. It also leads to narrowing of the pulmonary valve — the valve that keeps blood from flowing back from your pulmonary artery to the lower right chamber of your heart (right ventricle) when the right ventricle relaxes between contractions. (Your pulmonary artery is the blood vessel that carries oxygen-poor blood from the right ventricle to your lungs.) If left untreated, these conditions may result in enlargement of your heart (cardiomegaly) and heart failure.
  • Cushing’s syndrome. A carcinoid tumor in your lung may produce an excess of adrenocorticotropic hormone (ACTH), which can lead to overproduction of the hormone cortisol. This is known as Cushing’s syndrome, a condition that occurs when your body is exposed to high levels of cortisol for a prolonged period of time.
  • Acromegaly. Carcinoid tumors in the lungs can cause your body to produce too much growth hormone. This may result in the rare hormonal disorder acromegaly, which causes your bones to increase in size, including those of your hands, feet and face.
  • Intestinal obstruction. Large carcinoid tumors in the intestines can block food and fluid from passing through your small intestine or colon. If left untreated, intestinal obstruction can cause parts of your intestine to die. This tissue death can lead to perforation of the intestine, severe infection and shock.

Peptic ulcers. Some carcinoid tumors (often those in the stomach) produce large       amounts of the hormone gastrin that causes your stomach to produce excess acid. This can lead to severe stomach ulcers, bleeding and even perforation of the stomach — a condition known as Zollinger-Ellison syndrome.

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]]> http://carcinoidtumorsite.com/2007/08/17/complications-associated-with-carcinoid-tumor/feed/ Incidence http://carcinoidtumorsite.com/2007/08/17/incidence/ http://carcinoidtumorsite.com/2007/08/17/incidence/#comments Fri, 17 Aug 2007 08:35:03 +0000 admin http://carcinoidtumorsite.com/2007/08/17/incidence/ Frequency of Carcinoid tumor is different in different people.

  • In the US: The frequency of carcinoid tumor is 1 case per 300 individuals. For carcinoid syndrome, it is 1 case per 300,000 individuals.

Mortality/Morbidity: Survival is dependent on the primary site and the size of the primary tumor. Patients with metastatic disease arising from midgut (ie, distal small intestine/cecal) primary tumors generally live longer than those with the foregut (ie, bronchial, gastric, pancreatic) and hindgut (ie, rectal) as primary sites. The 5-year survival rate from the time of diagnosis of metastatic disease is 67%. No therapy to date has been shown in any randomized clinical trial to prolong survival for patients with metastatic carcinoid tumors, and therapy remains palliative.

Race: No racial differences in the incidence of carcinoid tumors are known.

Sex: The incidence of carcinoid is similar between males and females

Age: The occurrence rate of carcinoid tumors peaks at approximately age 62 years. Carcinoid tumors are rare in young persons, particularly because the argentaffin cells from which this tumor develops are only identified in children older than 4 years (Deans, 1995).

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]]> http://carcinoidtumorsite.com/2007/08/17/incidence/feed/ Staging/ Spreading of Carcinoid Tumor http://carcinoidtumorsite.com/2007/08/17/staging-spreading-of-carcinoid-tumor/ http://carcinoidtumorsite.com/2007/08/17/staging-spreading-of-carcinoid-tumor/#comments Fri, 17 Aug 2007 08:33:42 +0000 admin http://carcinoidtumorsite.com/2007/08/17/staging-spreading-of-carcinoid-tumor/ Staging is a way of describing a cancer, such as where it is located, where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

Gastrointestinal carcinoid tumors

There is no standard system for staging gastrointestinal carcinoid tumors. Some doctors use the same system that is used for other cancers of the same organ. For example, stomach carcinoid tumors may be treated similar to stomach cancer.

Because there is no standard system for describing the spread of gastrointestinal carcinoid tumors, many doctors divide all gastrointestinal carcinoid tumors into three general stages:

Localized spread. The carcinoid tumor has not spread beyond the wall of the primary organ, such as the stomach, colon, or intestine.

Regional spread. The carcinoid tumor has spread through the wall of the primary organ and involves nearby tissues, such as fat, muscle, or lymph nodes.

Distant spread. The carcinoid tumor has spread to tissues or organs far away from the primary organ, such as the liver, bones, or lungs.

Lung carcinoid tumors

The staging of lung carcinoid tumors is the same as the staging of non-small cell lung cancer.

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]]> http://carcinoidtumorsite.com/2007/08/17/staging-spreading-of-carcinoid-tumor/feed/ What are Risk Factors Associated? http://carcinoidtumorsite.com/2007/08/17/what-are-risk-factors-associated/ http://carcinoidtumorsite.com/2007/08/17/what-are-risk-factors-associated/#comments Fri, 17 Aug 2007 08:05:26 +0000 admin http://carcinoidtumorsite.com/2007/08/17/what-are-risk-factors-associated/ Although carcinoid tumors can occur at any age, they’re more common in older people. Children rarely develop these types of tumors. A risk factor is anything that increases a person’s chance of developing a disease, including cancer. There are risk factors that can be controlled, such as smoking, and risk factors that cannot be controlled, such as age and family history. Although risk factors can influence disease, they do not cause cancer. Some people with several risk factors never develop the disease, while others with no known risk factors do. Knowing your risk factors and communicating with your doctor can help guide you in making wise lifestyle and health-care choices.

The cause of carcinoid tumors is unknown, and no avoidable risk factors have been identified. Lung carcinoid tumors are not caused by, nor related to, smoking.

The following factors can raise a person’s risk of developing carcinoid tumors:

Family history of multiple endocrine neoplasia, type 1. Multiple endocrine neoplasia, type 1 (MEN1) is a hereditary condition that increases the risk of developing tumors in three different glands: pituitary, parathyroid, and pancreas. It is estimated that approximately 10% of gastrointestinal carcinoid tumors are associated with MEN1.

Race and gender: Gastrointestinal carcinoid tumors are more common among blacks than whites, and black men are at a higher risk than black women. Among whites, men and women have the same risk. Race and gender are not a significant risk factor in lung carcinoid tumors, but women tend to live longer after treatment.

Age: For gastrointestinal carcinoid tumors, the average age at diagnosis is 55 to 65. For carcinoid tumors of the appendix, the average age at diagnosis is about 40. For lung carcinoid tumors, the average age at diagnosis is between 45 and 55. Children rarely develop carcinoid tumors.

Other stomach conditions: People with diseases that damage the stomach and reduce acid production (in particular, pernicious anemia, a type of anemia characterized by very large, malformed red blood cells) have a greater risk of developing stomach carcinoid tumors.

 

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