Last Updated January 4th, 2021
What is Carcinoid syndrome?
The carcinoid syndrome is a specific type of syndrome that occurs with carcinoid tumours. Such tumours can occur in various parts of the body including the intestine, appendix and bronchial tubes in the lung. A small percentage of people affected by the tumours show signs of this syndrome which results from the secretion of a few chemicals from the tumours. These hormonal chemicals can vary depending on the nature of the tumour and its location. Around 10 per cent of patients with carcinoid tumours suffer from malignant carcinoid syndrome.
Origin of Carcinoid tumours
Carcinoid tumours are of neuroendocrine origin and the rise from primitive stem cells in the body. In general, the hormones secreted by these tumours are neutralised by the liver before they can cause any change in the body. In some rare cases, the tumour or cancer will have advanced and spread to the liver. The liver may also fail to check the spread of the disease. The chemicals secreted include serotonin and a range of other peptides. Some of the major symptoms of this condition are hot and red facial flushing, diarrhoea and wheezing. Carcinoid tumours have similar symptoms to other conditions like menopause or asthma.
In some cases, cancer need not have spread into the liver. Tumour cells may also migrate from the lungs to the liver. The exact cause of these tumours remains unknown. If the tumour is detected at an early stage, it might be possible to remove it. Otherwise, there is no cure for the condition. Some treatments can control the symptoms and help a person live longer.
The symptoms of carcinoid syndrome
There are no specific symptoms when a tumour gets formed, and in many cases, there are no symptoms at all. In the case of carcinoid syndrome, some symptoms are evident. These are listed below:
Flushing of skin
The flushing of skin is a major symptom. The skin in the face and the upper parts of the body turns red and feels hot. The skin turns purple or pink in shades. A flushing episode can last for a few minutes to a few hours. Some of the factors that might trigger flushing episodes include stress, exercise or alcohol consumption.
The formation of skin lesions can also be a part of the symptom. There can be a thin network of veins appearing on the nose or around the lips.
Another symptom is diarrhoea that occurs frequently. Abdominal cramps can also accompany this symptom.
Difficulty in breathing
There is difficulty in breathing, as happens in an asthmatic attack. This is accompanied by wheezing and shortness of breath, and it can occur in conjunction with flushing of the skin.
Specific periods when the heartbeat turns very fast can also be linked with carcinoid syndrome. There can also be sudden drops in blood pressure for no apparent reason.
There is also weight loss that is accompanied by malnutrition, weakness and muscle or joint pain. The patient can also gain weight in some cases.
In advanced stages, the syndrome can damage the heart valves, which can lead to congestive heart failure. Severe diarrhoea can also lead to the excretion of vital salts from the body, leading to a life-threatening electrolyte imbalance. Other symptoms that can occur include severe stomach pain, blockage of the arteries in the liver, digestive problems, heart palpitations and excess peptide excretion in the urine. A combination of multiple symptoms can result in a carcinoid crisis that can be life-threatening. However, not all the features are listed above will be present together in most cases of carcinoid syndrome.
The tumours can also secret a hormone called gastrin which affects the production of acid in the stomach. It results in Zollinger-Ellison syndrome, which causes irritation of the lining of the stomach and leads to the formation of stomach ulcers. Bleeding from these ulcers can result in other complications.
Some carcinoid tumours produce ACTH (adrenocorticotropic hormone), which results in the over secretion of cortisol from the adrenal glands. Consequently, this might trigger the development of Cushing syndrome, which comes with a wide range of symptoms. Some of the symptoms include:
- Weight gain
- Muscle fatigue
- High blood sugar that can result in diabetes
- High blood pressure
- Higher generation of body and facial hair
- A bulge of fat on the back of the neck
- Stretch marks in the skin.
Diagnosis of carcinoid syndrome
Almost two-thirds of the carcinoid tumours occur in gastrointestinal tracks, and they are most common in the rectum, small intestine and stomach. The doctor can try to find the causes behind the symptoms, and in case of no specific cause, carcinoid syndrome is suspected. The following tests act as a confirmatory diagnosis:
A urine test helps to detect substances like 5-hydroxyendolacetic acid that forms when the body breaks down serotonin. It is an indication that the body is breaking down the excess serotonin produced by the tumours.
Blood tests detect components like protein chromogranin A that are released by the tumours.
Imaging tests locate the tumour, and to checks the extent to which the disease has progressed. These may include abdominal CT scans or an MRI or even a nuclear medicine scan.
In some cases, endoscopy is performed to diagnose the problem in the intestine. Apart from that, colonoscopy also detects the presence of any abnormal growth in the large intestine.
A PET scanner is also used along with an injection of radioactive glucose in a vein. Malignant cells absorb large amounts of glucose and hence, are easier to visualise.
Tissue samples can be collected during endoscopy or colonoscopy and a biopsy conducted over the same.
Cancer generally spreads either through the tissue or the blood or the lymph system. After the tumour is detected, staging follows to determine the stage of the disease. It sets the stage for the development of a treatment plan. However, in the case of gastrointestinal carcinoid tumours, the stage is not considered important. It is classified by whether the tumour requires removing by surgery or it has spread widely.
Causes behind carcinoid syndrome
The syndrome arises from the tumours, and the underlying cause behind the formation of tumours is still unclear to medical science. In general cancerous cells are formed from an abnormal mutation in the cell DNA that promotes uncontrolled growth. The continuous growth of the cells results in the formation of a tumour. Even though smoking and food habits are risk factors for this condition, further studies are needed to confirm the same. These tumours are generally slow-growing and release hormones like serotonin, bradykinins, tachykinins and prostaglandins.
These tumours are rare, and in the USA, only around 27 people in a million are affected by them. Among these 27, only 10 per cent will develop carcinoid syndrome. While people of all age groups are equally affected by tumours in the gastrointestinal tracks, the middle-aged patients are most affected. Some risk factors for carcinoid tumours are listed below.
Older people have a higher chance of getting affected by this condition than the younger generation.
Those with a family history of multiple endocrine neoplasia, type I (MEN I) have a higher risk of formation of carcinoid tumours. In some cases, glands of the endocrine system get affected by multiple tumours.
Treatment of carcinoid syndrome
The most fundamental treatment for Carcinoid syndrome is the surgical removal of the tumour, along with any metastates. In case the tumour has spread to the lungs, processes like lobectomy, sleeve resection or pneumonectomy are performed. In case the tumour is dislodged in the intestine, it may be necessary to remove a particular area, followed by resection. When the liver is infected, this necessitates surgical excision. Certain modern techniques like cryoablation and radiofrequency ablation are also useful. In many cases, chemotherapy prevents metastases. Once the tumour gets removed, periodic surveillance is needed to avoid any renewed growth.
Management of carcinoid syndrome entails the use of medication. Many times, a synthetic form of the pancreatic hormone, somatostatin called Octreotide, is injected into the patient. Multiple injections may be given for a period of several weeks. Antidiarrheal and anticholinergic medications also get administered. The patient must restrict their consumption of alcohol and certain food items that can trigger symptoms.
Some medicines that make the defence system of the body perform better are also used to halt the growth rate of the tumours. Before progressing along the path of any treatment, it is essential to learn about the side effects and balance the pros and cons. Some of the factors that determine the best possible treatment approach include: The organ which has been affected
- The tumour size and location
- Whether it has spread to lymph nodes, liver, bones, or other organs
- Any other medical condition that the patient is suffering from
- The severity of the symptoms that are being caused by the tumour
Many patients also choose complementary or alternative methods of treatment apart from the main course of treatment. These include the use of vitamins, herbs, and special diets. In many cases, methods like as acupuncture, reiki and Ayurveda are also used. These methods can help reduce the symptoms and provide relief. Since their use is not based on scientific evidence, there can also be harmful effects. It is necessary to consult the doctor before opting for any alternative medical processes.
Managing carcinoid syndrome
While handling a life-threatening disease is challenging at all levels, some effective steps can be taken for overall betterment. Once the syndrome is detected, medication provides relief from the bothersome symptoms. However, the detection process of the condition can also be stressful for the patient. It is important to discuss the details with your doctor to get clarity about any doubts.
It is necessary to find as much information about the condition from reliable sources so that you can take the right steps towards getting better. This will also help you to make better decisions about the treatment processes.
One can also get in touch with support groups that have people suffering from carcinoid syndrome. The views and experience of such people can help a patient in facing the challenges by picking up the right suggestions.
It is also important to follow a healthy lifestyle which will have an overall positive impact on health. Consuming a healthy diet is an important step that needs to be taken. Avoiding a stressful life, light exercising, getting sufficient sleep are some steps that can be taken to cope with the conditions. Sticking to the treatment plan can be challenging, but with the right level of help and support from the family, it can be achieved. It is also necessary to adhere to the treatment plan as prescribed by the doctor. One can also join support groups near their area for moral support.
Carcinoid syndrome is a rare but serious condition that is often difficult to diagnose as the symptoms are not consistent. Apart from the standard treatments, there are new forms of treatments that are coming up for carcinoid tumours. It is important to discuss the options with your doctor and take a second opinion if time and situations allow. There is also supportive care available to deal with pain and other symptoms arising from the treatments. A good understanding of the entire process helps in the effective treatment of this complex condition.
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