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Carcinoid Syndrome And Hormone-secreting Tumors

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What Is A Carcinoid Tumor

Carcinoid Syndrome – causes, symptoms, diagnosis, treatment, pathology

Cancer starts when cells change and grow out of control. The changed or abnormal cells often grow to form a lump or mass called a tumor. Cancer cells can also grow into nearby areas. And they can spread to other parts of the body. This is called metastasis.

Carcinoid tumors are also called neuroendocrine tumors . Its a rare type of cancer. Carcinoid tumors tend to grow slowly. Few people with these tumors have symptoms. So you may have a carcinoid tumor for many years and never know it.

Carcinoid tumors can grow anywhere in your body where there are hormone-producing cells. Hormones are chemical messengers that travel through your blood. Most carcinoid tumors form in the digestive tract. This is likely because there are more neuroendocrine cells there than anywhere else in the body.

Heres where carcinoid tumors are most often found:

  • Small intestine

How Is Carcinoid Tumor Treated

Treatment for each person will be unique. You should go to an expert in neuroendocrine tumor treatment to decide the best approach for your tumor. You can contact MyPART for help finding experts near you.

Surgery: If you have a carcinoid tumor, you may have surgery to remove the tumor and some surrounding tissue. Surgery is the best option for treating carcinoid tumor and preventing it from spreading.

When the carcinoid tumor is large or the cancer cells have spread to other parts of the body other treatments may include:

Somatostatin analogs: Somatostatin analogs are a type of treatment that may stop your body from making too many hormones. This may slow down the growth of the tumor when cancer cells have spread to other part of the body

Targeted therapy: Targeted therapy is a type of treatment that uses drugs that target certain genes or proteins to kill cancer cells. Neuroendocrine tumor cells have receptors on the surface of the cells called somatostatin. A type of targeted therapy called peptide receptor radionuclide therapy can target these cells.

Chemotherapy: is a type of treatment that uses stronger drugs to kill fasting growing cells.

Symptoms Caused By The Hormones

Typical symptoms of carcinoid syndrome include:

  • diarrhoea, tummy pain and loss of appetite
  • flushing of the skin, particularly the face
  • fast heart rate
  • breathlessness and wheezing

These symptoms may come on unexpectedly, as the hormones can be produced by the tumour at any time.

Some people may also develop carcinoid heart disease, where the heart valves thicken and stop working properly.

There’s also a risk of developing a rare but serious condition called a carcinoid crisis, which involves severe flushing, breathlessness and a changes in your blood pressure.

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Types Of Carcinoid Tumors

Here are the areas of the body where carcinoid tumors are usually found:

  • Small intestine

Carcinoid tumors behave differently depending on how quickly they grow, if they spread to other areas of your body, and if they make too much hormone. Types of carcinoid tumors include:

  • Slow-growing tumors. These tumors are the most common type. They usually remain small, under about an inch wide. They dont grow quickly or spread to other areas of the body.

  • Faster-growing tumors. These tumors may grow more rapidly, grow larger, and spread.

  • Hormone-secreting tumors. These functioning carcinoid tumors produce hormones including serotonin. The effect of serotonin and other hormones causes the symptoms known as carcinoid syndrome.

Figure 45treatment Algorithm For 90ytrium Microsphere Brachytherapy


Kennedy A et al. Int J. Radiation Oncology Biol. Phys. 2007

90Yttrium based therapy is effective at controlling carcinoid symptoms, but has not been shown to improve survival . In general, these patients undergo the procedure in three stages:

  • Mapping stage when the arterial anatomy of the liver is evaluated and potential shunting to the lung and foregut is identified and minimized by selective coiling of involved vessels
  • 2.Treatment of individual lesions or disease dominant liver lobe.

    3.Treatment of remaining liver as needed.

    In general, these procedures are performed as an outpatient and are well tolerated with pain or peptic ulceration as the major complications. Patients with poor hepatic synthetic function are not candidates.

    Peptide Receptor Radiotherapy

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    How Is Carcinoid Tumor Diagnosed

    Some people with carcinoid tumors have symptoms, but others dont. The symptoms of carcinoid tumor depend on where the tumor is inside the body.

    Patients with carcinoid tumor of the appendix usually have symptoms of appendicitis, such as pain in the abdomen. They may be diagnosed later with carcinoid tumor if the doctor removes the appendix and finds a tumor. Patients with carcinoid tumor in other parts of the digestive tract may have symptoms such as:

    • Pain in the abdomen

    Patients with carcinoid tumor in the lungs may have symptoms such as:

    • Trouble breathing
    • Coughing up blood

    Sometimes these symptoms are diagnosed as pneumonia by mistake.

    In rare cases, patients with carcinoid tumor may develop carcinoid syndrome. Carcinoid syndrome is a problem that develops from the tumors making hormones. Symptoms include:

    • Feeling flushed
    • Nausea and vomiting

    Lab Tests: If you have symptoms of carcinoid tumor, your doctor will order lab tests of your urine or blood to check your hormone levels.

    Imaging: Your doctor will use scans such as CT and MRI to see where the tumor is and how big it is. Different types of PET scans can also help find more fast-growing neuroendocrine cancer cells.

    Biopsy: To check if the tumor is carcinoid tumor your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope and run other tests to see what kind of tumor it is.

    Carcinoid Syndrome: Symptom Management

    Background The carcinoid syndrome is a symptom complex resulting from hormone secretion in patients with carcinoid tumors. Carcinoid tumors typically grow slowly, and patients may live for many years following diagnosis. CS becomes manifest only when sufficient concentration of hormones reach the systemic circulation, most commonly in the presence of liver metastases. This Fast Fact will focus on managing the symptoms of CS.

    Symptoms and Causes The CS includes the complex of: flushing, diarrhea, abdominal cramping, cyanosis, bronchoconstriction, and symptoms of right heart failure. Compared with the general population and other cancer patients, CS sufferers may also be at increased risk for cognitive impairment. Other specific symptoms depend on the hormones the tumor secretes. Serotonin-secreting tumors cause diarrhea and cramping bradykinin and histamine secretion lead to flushing and cyanosis. Carcinoid tumors may also produce somatostatin, norepinephrine, dopamine, gastrin, vasoactive intestinal peptide, and other hormones. Drugs that block the hormonal secretion can help to control the symptoms of carcinoid syndrome.

    Interferon Interferon alpha is effective in controlling both diarrhea and flushing, although it is inferior to the somatostatin analogs. The dose is 3-9 mU subcuteneously three to seven times per week. Interferon alpha therapy is often limited by its side effects: fever, anemia, thrombocytopenia, neutropenia, fatigue, depression, and flu-like symptoms.

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    How Is A Carcinoid Tumour Treated

    It is recommended that multidisciplinary teams at referral centres give guidance on the management of the patient. The main aim should be to keep the patient disease and symptom-free for as long as possible and to maintain a good quality of life.

    The definitive treatment to cure a carcinoid tumour is by surgical removal of the tumour. However, as most of these tumours are detected incidentally or show features of spread to other organs, surgical cure of the tumour may not be possible in many patients.

    Injections of octreotide , which is an equivalent of a naturally produced hormone called somatostatin, may help fight the symptoms of excess hormone production and even possibly slow down the growth and spread of the tumour.

    In selected cases, chemotherapy or other radiotherapy treatments or blocking the main artery to the liver may be used to reduce the total number of tumour cells in the blood circulation. This then controls the excess hormone production and improves symptoms.

    Table 14 Value Of Measurement Of Neurokinin A

    Carcinoid Syndrome | Serotonin-Secreting Tumors | 5-Minute Review ð?
    Neurokinin A
    § Developed highly sensitive and specific radioimmunoassay § Found very high levels of NKA-like activity in tumor tissue from four patients with mid-gut carcinoids ⢠Regul Pept. 1986 Jan 13:183-96 § Patients in whom NKA levels continue to rise despite treatment with somatostatin analogs have poorer prognosis ⢠⢠Turner et al. Gut. 2006 Nov 55:1586-91 NKA should be considered another of the substances secreted by mid-gut carcinoid and is an important marker for prognosis

    Substance P has been found in tumor extracts and plasma from patients with carcinoid tumors and, in one reported case, was useful for tumor localization . Neurokinin A, its amino- terminally extended form, neuropeptide K, and SP are a group of peptides with common biologic properties . Norheim and colleagues measured peptide responses to PG or ingestion of food or alcohol in 16 patients with metastatic carcinoid tumors and demonstrated two-fold or greater increases in neurokinin A and neuropeptide K in 75% of patients, as well as variable increases in SP in approximately 20% of patients . Additional information on neurokinin A is shown in Table 14.

    A summary of biochemical markers which may be useful for each carcinoid tumor type is shown in Table 12.

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    Figure 52rational For Mtor Inactivation

    mTOR mediates downstreaming signaling from IGFi/SSA, VEGFi and VEGF TKIs which is responsible for angiogenesis, bioenergy, growth and proliferation of cell. Activation of mTOR pathway occurs through PI3K/AKT/RAS. While inhibitors of mTOR pathway includes PTEN/NF1. Inactivation of mTOR pathway alone or combined with RAD001 therapy is implicated in inhibition of neuroendocrine.

    Inhibitors of mTOR have shown early activity in number of cancers including neuroendocrine tumors . The mechanism whereby everolimus inhibit growth NETS is shown in .

    Thirty seven patients with progressive neuroendocrine tumors were treated with mTOR inhibitor temsirolimus in one phase II study. The response rate was 5.6% with 63.9% of patients showing partial response or stable disease . RAD001 is a novel agent that is being studied in the treatment of neuro-endocrine tumours, and is known to interact with mTOR. Everolimus, is an oral inhibitor of the mammalian target of rapamycin . Everolimus has been studied as an adjuvant to SAs in patients with advanced NET, although it has not been FDA approved for this indication. The combined effect of mTOR inhibitors RAD001 and depot octreotide showed a partial response rate of 12% in carcinoid patiets while 37% of patients had > 50% reduction in chromogranin A . A Phase I study by Awada showed that daily therapy with RAD001 plus letrozole exhibited anti-tumor activity in breast cancers .

    Management Summary

    Causes Of Carcinoid Tumors

    The exact cause of carcinoid tumors is not known. Generally, cancer forms when a cell develops mutations in its DNA, resulting in the abnormal growth and division of the cell. The divided cells accumulate to form the tumor and invade the healthy tissues resulting in the spread of cancer.

    In people with or with a family history of MEN 1 , there is a risk of developing intestinal and bronchial carcinoid tumors.

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    Treatment For Carcinoid Tumors

    The focus of carcinoid tumor management is symptom control, biochemical control, tumor control, and improving the quality of life.

    Management of carcinoid tumors uses various approaches.

    1. Cytoreductive Surgery

    Cytoreductive surgery is the cornerstone in the management of carcinoid tumors. The surgery aims to improve survival and control symptoms by destroying or removing the tumor. It includes tumor resection, fulguration, and cryotherapy. Local excision may be successful for smaller tumors, but more radical surgery might be required in patients with larger tumors.

    Surgery can reduce tumor bulk and prevents other local or systemic effects. Thus, surgery improves the quality of life and hormone-related symptoms and can prolong survival in some patients.

    2. Systemic treatment

    Systemic treatment includes therapy with somatostatin analogs, cytotoxic agents, and interferon alfa. Other agents are administered as required, for example, loperamide or diphenoxylate for diarrhea and H1 or H2 blockers for histamine-secreting tumors.

    3. Radiotherapy

    • Tumor-targeted treatment with radioactive octreotide derivatives is associated with tumor shrinkage in some tumors. Recent evidence suggests that combining radioisotope with concurrent systemic therapy might be more effective.

    What Can I Do To Help Myself

    Clinical presentation of the carcinoid syndrome

    There are things you can do yourself to manage some of the symptoms of carcinoid syndrome.

    Generally, you should avoid triggers of flushing, such as:

    • foods containing the substance tyramine, such as aged cheese and salted or pickled meats

    Some medications, such as selective serotonin reuptake inhibitor antidepressants, may make symptoms worse by further increasing your levels of serotonin but never stop taking medication without seeking medical advice.

    If you have diarrhoea, it’s important to keep drinking little and often to avoid dehydration.

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    What Are The Signs And Symptoms Of A Carcinoid Tumour

    Carcinoid tumours may be discovered by chance, for example, from a routine chest X-ray or during surgery being performed on the gut for another reason, and they can produce very few symptoms. However, they do sometimes cause signs and symptoms and these can be divided into several groups:

  • A growing tumour these effects depend on the location of the tumour. For example, a carcinoid tumour in the lung might cause a cough with some blood possibly being coughed up. A carcinoid tumour in the gut may result in abdominal pain.
  • Secretion of hormones sometimes, particularly when there are tumour cells in the liver, the hormones released by the tumour can cause symptoms like flushing of the skin , diarrhoea and wheezing. These symptoms occur only if the tumour cells have spread to the liver.
  • Long-term raised hormone levels some of the substances released by carcinoid tumours can cause the development of fibrous tissue in nearby organs and this may lead to heart valve problems, breathlessness and swollen ankles. The fibrous tissue surrounding intestinal carcinoid tumours can lead to formation of adhesions and obstruction of the intestine. Production of large amounts of these hormones can also use up a large part of the bodys vitamin B stores resulting in nerve damage, particularly in the feet and lower legs.
  • Neuroendocrine Syndrome In Bronchial Carcinoid Tumors

  • Affiliations: Department of Thoracic Surgery, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania, Department of Thoracic Surgery, Marius Nasta National Institute of Pneumophtisiology, 050159 Bucharest, Romania, Department of Urology, Fundeni Clinical Institute, 022328 Bucharest, Romania, Department of Anesthesiology, Fundeni Clinical Institute, 022328 Bucharest, Romania, Department of Oncology, Prof. Dr. Al. Trestioreanu Institute of Oncology, 022328 Bucharest, Romania, Department of Internal Medicine, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania, Department of Visceral Surgery, Ponderas Academic Hospital, 021188 Bucharest, Romania, Department of Cardiac Surgery, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania, Department of Urology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania, Department of Anaesthesia and Intensive Care, Ilfov County Emergency Hospital, 022104 Bucharest, Romania, Department of Obstetrics and Gynecology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
  • Article Number: 200
  • This article is mentioned in:



    Patients and methods

    The final diagnostic of carcinoid tumor wasestablished on the specimens retrieved during bronchoscopy or onthose which were obtained after surgical resection.


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    What Hormones Do Carcinoid Tumors Secrete To Produce The Symptoms Of Carcinoid Syndrome

    In general it can be stated that practically all carcinoid syndrome cases exhibit some increase in at least one of the many endocrine chemical products elaborated by the tumor and producing the syndrome. These include not only blood serotonin and urine 5HIAA but also chromogranin A, Neuron Specific Enolase, Pancreatic Polypeptide, Calcitonin, Substance P, Neurokinin A, Prostaglandin A,E and D, Histamine and Pancreastatin.

    Furthermore there are a number of look alike syndromes such as Zollinger Ellison, VIPoma and Mast Cell Disease which can mimic carcinoid and be suspected by respectively measuring Gastrin, VIP, Histamine and Tryptase. Medullary Thyroid Carcinoma can cause a carcinoid-like syndrome and usually produce Calcitonin, CEA and at least one of the prostaglandins. Certain pituitary tumors make prolactin and can be associated with carcinoid like features. Prolactin is useful in this diagnosis.


    Figure 22positron Emission Tomography Scan

    Carcinoid syndrome – Symptoms and treatment. Carcinoid Heart Disease

    Appropriate Use Criteria for Somatostatin Receptor PET Imaging in Neuroendocrine Tumors

    Summary of Recommendations

    MIBG Scanning

    The first report of 131 I-MIBG for the imaging of a carcinoid tumor was that of Fischer and colleagues in 1984, in which hepatic metastases that were seen as photopenic areas on a 99mTc-phytate liver scan concentrated 131 I-MIBG . Since this initial description, there have been a number of reports of successful imaging of carcinoid tumors using 131 I-MIBG. The number studied are far less than those reported for pheochromocytomas or neuroblastoma but it probably is fair to say that the sensitivity is significantly lower . The overall sensitivity is calculated to be 55%. Because MIBG is taken up by a wide variety of neuroendocrine tumors, specificity depends on the certainty of the clinical and biochemical diagnosis. In the correct clinical context, this is well over 95% for pheochromocytomas and neuroblastoma, but it is clearly less for carcinoid tumors. 131 I-MIBG scanning offers information that is additive to the information gained by SRS imaging. In some patients SRS scanning is negative and other lesions light up on MIBG scanning. In other patients SRS imaging and MIBG scans both are positive or negative. In the case where both scans are positive patients may be candidates for future therapy with 131 I-MIBG or PRRT with radiolabeled somatostatin analogs.

    Other Diagnostic Methods

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    How Are Carcinoid Tumors Grouped

    Carcinoid tumors differ a lot depending on how fast they grow, where they start, if they spread to other parts of the body, and if they make too much hormone.

    Carcinoid tumors might be described based on how fast they grow:

    • Slow-growing tumors. These are the most common type. They often stay small. They dont grow quickly or spread to other parts of the body.

    • Faster-growing tumors. These tumors grow faster, grow larger, and spread.

    • Hormone-secreting tumors. These functioning carcinoid tumors produce hormones including serotonin. The effect of serotonin and other hormones causes the symptoms known as carcinoid syndrome.

    Another way to describe them is by where they start. For instance, it might be a GI neuroendocrine tumor or a liver carcinoid tumor. The stage of the tumor defines if it has spread.

    These tumors can also be grouped based on whether they make hormones:

    • Functional tumors. These make hormones or other substances that cause symptoms.

    • Nonfunctional tumors. These tumors are more common. They dont make hormones or dont make enough to cause symptoms.

    All these things are found out while diagnosing carcinoid tumors.

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