Carcinoid Syndrome - Symptoms, Treatment, Forms, Stages, Diagnosis

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Carcinoid syndrome

The content of the article:

1. Causes and risk factors
2. Forms of the disease
3. Symptoms
4. Diagnostics
5. Treatment
6. Possible complications and consequences
7. Forecast
8. Prevention

Carcinoid syndrome is a complex of symptoms that occurs due to the release of hormones by carcinoid tumors (the most common neoplasms from neuroendocrine cells) and their entry into the bloodstream. Carcinoid tumors are relatively rare and slowly progressing neoplasms. Carcinoid syndrome occurs in 15-20% of patients with carcinoids. All age categories of the population are susceptible to the development of the pathological process.

A characteristic feature of carcinoid syndrome is sudden flushing of the skin of the face, neck and chest.

Causes and risk factors

Carcinoid syndrome usually occurs against the background of endocrinologically active neoplasms in the ileum and rectum, appendix or other parts of the gastrointestinal tract, as well as in the pancreas, bronchi, etc.

Forms of the disease

Depending on the clinical manifestations, the following forms of pathology are distinguished:

• latent carcinoid syndrome (in the absence of clinical manifestations, but altered biochemical parameters of blood and urine);
• carcinoid syndrome without identifying the primary focus of the neoplasm and its metastases;
• carcinoid syndrome with the identification of the primary focus of the neoplasm and its metastases or only metastases to the liver.

Depending on the location, carcinoid syndrome is distinguished with a tumor of the anterior, middle or posterior part of the digestive tube.

Symptoms

Symptoms depend on the hormones secreted by the tumor. The clinical picture of carcinoid syndrome in intestinal neoplasms, as a rule, manifests itself only when they metastasize to the liver.

A characteristic sign of pathology is a sudden hyperemia of the skin of the face, neck and chest, caused by a rush of blood. Often, skin hyperemia occurs against the background of food intake, alcohol consumption, and distress. The nature of hyperemia differs depending on the location of the carcinoid tumor. In patients with intestinal neoplasms, hyperemia may be accompanied by a sensation of heat, and with the progression of the disease, the skin may acquire a cyanotic hue during attacks.

When the tumor is localized in the stomach, hyperemic spots may appear on the skin, accompanied by severe itching. In the case of localization of a carcinoid tumor in the bronchi, attacks have a more severe and prolonged (up to several days) course, may be accompanied by tremor and disorientation in space.

Symptoms of carcinoid syndrome

In addition to hyperemia, the appearance of spider veins or vascular networks on the skin (mainly in the nasolabial triangle, on the cheekbones) is characteristic.

Diarrhea is another common manifestation of carcinoid syndrome. The frequency of bowel movements can vary from several to several dozen times a day. Diarrhea is often accompanied by abdominal pain and gastrointestinal cramps.

In patients with developed valvular heart disease, heart murmurs occur.

In addition, with carcinoid syndrome, there may be:

• wheezing;
• dyspnea;
• lacrimation;
• swelling around the eyes;
• increased salivation;
• decreased libido;
• hypotension;
• tachycardia;
• oliguria, etc.

Diagnostics

To make a diagnosis of carcinoid syndrome, carry out:

• collection of anamnesis;
• determination of the excretion of 5-hydroxyindoleacetic acid (a product of the metabolism of a carcinoid neoplasm) in daily urine;
• biochemical analysis of blood and urine;
• Ultrasound of internal organs;
• computed or magnetic resonance imaging;
• positron emission tomography;
• phlebography, selective arteriography;
• endoscopic examination;
• biopsy with morphological, histological and immunohistochemical examination of the biopsy.

One of the types of diagnostics of carcinoid syndrome is ultrasound of internal organs

To avoid false positive results in laboratory analysis of urine, three days before the study, foods that contain serotonin, as well as drugs that can distort the results of the analysis, are excluded from the patient's diet.

Electrocardiography and echocardiography are done to detect heart damage.

If the diagnosis is in doubt, tests with pentagastrin, catecholamines, alcohol, etc. are carried out in order to provoke specific seizures ("hot flashes").

It is necessary to carry out a differential diagnosis with idiopathic anaphylaxis, side effects from taking medications, manifestations of menopause, irritable bowel syndrome, etc.

Treatment

Radical surgical treatment of carcinoid tumors and its metastases eliminates the clinical manifestations of carcinoid syndrome. With an unresectable neoplasm, the impossibility of surgical intervention due to the general condition of the patient or for other reasons, as well as to eliminate signs of carcinoid syndrome with an undetected tumor, conservative treatment is indicated.

The best treatment for carcinoid syndrome is to remove the tumor that caused it.

Symptomatic therapy of the manifestations of carcinoid syndrome is carried out using drugs that inhibit the production of hormones by a carcinoid tumor, as well as glucocorticoids, antidepressants, etc.

Possible complications and consequences

In some cases, carcinoid syndrome is complicated by a carcinoid crisis, which can occur against the background of:

• serious surgical intervention;
• stress;
• instrumental diagnostic examination;
• chemotherapy;
• anesthesia during surgery.

Signs of a carcinoid crisis:

• a sharp change in blood pressure (hypertension or hypotension);
• deterioration of the general condition;
• frequent noisy breathing;
• tachycardia, threadlike pulse;
• disorders of the central nervous system, etc.

A carcinoid crisis occurs acutely and, in the absence of an urgent complex of intensive care measures, it can turn into shock and coma.

Forecast

The prognosis for carcinoid syndrome depends on the prevalence of the oncological process. In the presence of only a primary neoplasm and its successful treatment, the average five-year survival rate is 95%; in patients with multiple distant metastases, this indicator does not exceed 20%. In the case of involvement of regional lymph nodes in the pathological process, the five-year survival rate decreases to 60-70%.

With a successful surgical intervention, the prognosis is favorable.

The prognostic factor in carcinoid syndrome is the proliferative activity of the cells of the primary neoplasm. In carcinoid tumors with a low proliferation index, the prognosis is more favorable.

Prevention

Specific measures to prevent carcinoid syndrome have not been developed. Non-specific preventive measures include:

• healthy lifestyle;
• sufficient physical activity;
• rejection of bad habits;
• balanced balanced nutrition;
• avoiding the influence of oncogenic substances on the body (including occupational hazards);
• timely diagnosis and treatment of internal diseases, etc.

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Anna Aksenova Medical journalist About the author

Education: 2004-2007 "First Kiev Medical College" specialty "Laboratory Diagnostics".

The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!