Adenoid cystic carcinoma
What is adenoid cystic carcinoma?The physician, when assuming an adenoid cystic carcinoma, speaks of a malignant tumor.
Adenoid cystic carcinoma is a malignant tumor. However, the tumor rarely occurs. Adenoid cystic carcinoma is derived from glandular tissues; predominantly the adenoid cystic carcinoma is diagnosed in the head or neck area.
The most common manifestation sites include the salivary glands; As a result, adenoid cystic carcinoma predominantly occurs in the parotid, mandibular or even small salivary glands. From time to time physicians have been able to detect further localizations in recent years.
These include adenoid cystic carcinoma in the lacrimal glands, nose and sinuses, trachea, larynx, lungs, skin, bronchi, mammary gland, cervix, external auditory canal, prostate or even bartholin Gland could form. Characteristic is the slow growth of adenoid cystic carcinoma at the beginning.
However, the tumor has a pronounced tendency to infiltrate, affecting predominantly the surrounding tissue. For this reason, adenoid cystic carcinoma can only rarely be completely removed, so that again and again new tumor formations can occur.
The tumor was described by Robin and Laboulbene; It was not until 1856 that the tumor was documented in more detail by Theodor Billroth, a surgeon. For this reason, the adenoid-cystic carcinoma is still called cylindroma today, since the tumor cells have a cylindrical shape. The term adenoid cystic carcinoma was first used by James Ewing, a pathologist.
So far, there are no known causes why it even comes to the development of adenoid cystic carcinoma. Also, there is no evidence of environmental or genetic risk factors, which sometimes favor the formation of adenoid cystic carcinoma. However, at the molecular genetic level, especially in aggressive and advanced adenoid cystic carcinomas, physicians were able to detect inactivation of the so-called p53 tumor suppressor gene.
New studies also claim deletion of certain chromosomal regions (such as region 1p32-36). In this region, the most common genetic deviations that have been observed in association with adenoid cystic carcinoma have been identified.
Symptoms, complaints & signs
Those affected complain mainly about paralysis that can occur in the entire face. In addition, swelling of the cervical lymph nodes occurs. Sometimes also diffuse pain or discomfort may occur.
Due to the fact that it is a very rare tumor, adenoid cystic carcinoma is often diagnosed late. The advantage is that the tumor grows slowly, but at the same time comes to a disadvantage: Even if the tumor has a slow growth, it can still scatter, so that sometimes even other organs of cancer cells are affected.
Diagnosis & History
The physician can only make a definitive diagnosis after the tumor tissue has been taken (fine needle aspiration, biopsy) and subsequently a microscopic examination carried out by pathologists. In the field of propagation diagnostics, predominantly imaging techniques such as magnetic resonance tomography, computed tomography or positron emission tomography are used.
In histology, adenoid cystic carcinoma shows a highly differentiated picture; the tumor is mainly known for its very infiltrative growth behavior. The growth pattern is glandular or sieve-like. Adenoid cystic carcinoma is growing slowly but permanently, so the 5-year survival rate of 89 percent is favorable, but the 10-year survival rate is just 65 percent.
The 15-year survival rate is just 40 percent. For the course of the disease, the histological patterns of growth, tumor size, localization and also the clinical stage, the question of whether bone involvement is present and also the status of surgically placed resection margins are of major importance.
If a cribriform or tubular growth pattern is present, there may be a favorable disease course; Lymph node involvement is possible, but rarely occurs (5 to 25 percent). Fine metastases, which subsequently become lodged in the lungs, brain, bones or liver, are possible. That it comes to other tumors is therefore in 25 to 55 percent of all cases reality. If there is a scattering of the tumor cells, the survival rate - within the first five years - is just 20 percent.
Adenoid cystic carcinoma is a rare malignant salivary gland tumor that manifests in the head and neck area. It grows slowly from the palate along the blood vessels and nerves. The symptom is noticeable by a strong painful swelling. In acute cases also by means of facial paralysis.
To balance the cause, the person concerned needs immediate medical help. As further complications, the tumor can form tumors in the lymph nodes, which spread to the brain, esophagus, and lungs. If the person does not treat the symptom, the infestation can also spread to the skin, prostate and cervix.
Women are more affected by the symptom than men. The age of the patients is between 30 to 70 years of age and hardly occurs in childhood. Due to the persistently recurring nature of adenocarcinoma, complete healing is not possible. A detailed pathogenesis of whether the symptom arises genetically or environmentally, is not yet known.
Adenoid cystic carcinoma is extensively removed during surgery. The therapy depends on the diagnostic findings. Since, due to the scattering of the tumor, usually no complete removal is feasible, a combined radiotherapy is recommended for confinement. The form of therapy can burden the psyche and physical compatibility of the patient and requires intensive care.
When should you go to the doctor?
The typical warning signs of adenoid cystic carcinoma are facial paralysis and swelling of the cervical lymph nodes. Sometimes there are also diffuse pains and discomfort, which increase as the disease progresses. Should one or more of these symptoms be noticed, it is advisable to go to the doctor immediately. A quick medical evaluation can significantly improve the survival rate. It is therefore advisable to speak with the family doctor at the first complaint, for which there is no other explanation.
In case of a suspected carcinoma also appropriate specialists should be consulted. This is especially true if the person has already had cancer once in the past. Genetic predispositions should also be considered and, if necessary, discussed with the doctor. Adenoid cystic carcinoma must always be diagnosed with a doctor and then treated with radiotherapy. The medical clarification of the cancer should be done as early as possible to improve the healing prospects and to exclude complications.
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Treatment & Therapy
If the physician diagnoses that it is an adenoid cystic carcinoma, he will initially prefer the complete removal of the tumor. The patient must undergo a surgical procedure. The tumor is removed with sufficient safety distance to the surrounding tissue.
To reduce the relapse rate, postoperative radiotherapy is ordered. Chemotherapy is not recommended; So far there is no effective chemotherapy for adenoid cystic carcinoma. So-called combination therapies, ie those consisting of chemotherapy and radiotherapy, are only used in a few cases - mainly for testing.
Outlook & Forecast
The prognosis for adenoid cystic carcinoma in the head or neck is estimated differently when radiotherapy is used. First, it states that the tumor does not start on it. The value of so-called neutron irradiation is becoming increasingly positive.
Unfortunately, a cure is often not possible. Complete tumor removal is usually not possible because the tumor grows along nerves and blood vessels. New tumorous developments are therefore frequently observed. Rapid tumor growth with secondary tumors is rarely documented.
In most cases, adenoid cystic carcinoma grows very slowly. However, this tumor is prone to metastasis in the lungs and bones. The recurrence tendency is relatively high. These factors make the prognosis appear in a bad light at first glance.
A survival rate of five years is around 75%. The ten-year survival rate is still 30% and the twenty-year survival rate is still 10%. If daughter tumors occur in the lungs, the average survival time is three and a half years. It should be noted that in the late, rapidly infiltrating stage mostly only a palliative therapy seems possible. Especially when the metastasis has started.
Since there is no information on the development of adenoid cystic carcinoma, no preventive measures are known. It is important that - if the physician provides the diagnosis of adenoid cystic carcinoma - an immediate treatment is started.
In most cases, the possibilities or measures of follow-up for this disease are very limited. First and foremost, a quick and, above all, an early diagnosis is very important so that there is no further deterioration or other complaints and complications. This tumor significantly reduces the life expectancy of the affected person in most cases.
As a rule, even after a successful treatment and removal of the tumor further investigations are advisable so that further tumors can be detected at an early stage. In most cases, the disease is treated by surgical removal of the tumor. After such an operation, the person concerned is dependent on bed rest.
Efforts or other stressful activities should definitely be avoided so as not to unnecessarily burden the body. In most cases, patients also depend on the help and support of friends and acquaintances. This also applies to chemotherapy, in which most patients require comprehensive support from their relatives. This includes the mental support of the person concerned.
You can do that yourself
Adenoid cystic carcinoma is a malignant cancer that usually has a severe course. The patients can support the treatment by changing the lifestyle. In addition to dietary and sporting measures, sometimes alternative healing methods are recommended.
For example, massages or acupuncture help with pain relief and are therefore an important part of the therapy. Patients should discuss alternative treatments with the appropriate physician so that they can be optimally tailored to conservative therapy. In addition, general measures such as the renunciation of alcohol and caffeine apply.
In order not to irritate the esophagus, should be dispensed with hot, hot, cold and sour food as far as possible. It is recommended to have a diet that can be supplemented with dietary supplements and appetizers, as far as the disease leads to malnutrition.
The patients should also talk to other people affected. Talking with other cancer patients not only makes it easier to deal with the disease, but also often shows other ways to improve well-being and thereby increase the quality of life in the long term. Finally, in adenoid cystic carcinoma, the voice must be spared, as this is usually already severely damaged by the tumor.