When referring to adenoid cystic carcinoma, physicians refer to it as a malignant tumor. Although the tumor is relatively rare, it is fatal in many cases. Mainly because adenoid cystic carcinoma spreads, so other organs can be affected by the cancer cells. The 5-year survival rate is 89 percent; the 15-year survival rate is only 40 percent.
What is adenoid cystic carcinoma?
When referring to adenoid cystic carcinoma, physicians refer to it as a malignant tumor. Adenoid cystic carcinoma is a malignant tumor. However, the tumor rarely occurs. Adenoid cystic carcinoma is derived from the glandular tissues; predominantly, adenoid cystic carcinoma is diagnosed in the head or neck area. Among the most common sites of manifestation are the salivary glands; thus, adenoid cystic carcinoma occurs predominantly in the parotid, mandibular, or even minor oral salivary glands. In recent years, physicians have also been able to identify other localizations. These include adenoid cystic carcinoma in the lacrimal glands, nose and paranasal sinuses, trachea, larynx, lungs, skin, bronchi, mammary gland, cervix, external auditory canal, prostate or even Bartholin’s gland. Characteristic is the slow growth of adenoid cystic carcinoma at the beginning. However, the tumor shows a marked tendency to infiltrate, predominantly affecting the surrounding tissue. For this reason, adenoid-cystic carcinoma can rarely be completely removed, so that new tumor formations can always 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, adenoid-cystic carcinoma is still called cylindroma today because the tumor cells have a cylindrical shape. The term adenoid-cystic carcinoma was first used by James Ewing, a pathologist.
Causes
To date, there are no known causes of why adenoid cystic carcinoma develops in the first place. Likewise, there is no knowledge of environmental or even genetic risk factors that sometimes promote the formation of adenoid cystic carcinoma. However, physicians have been able to identify an inactivation of the so-called p53 tumor suppressor gene at the molecular genetic level, especially in aggressive and already advanced adenoid-cystic carcinomas. New studies also refer to a deletion of certain chromosomal regions (such as the region 1p32-36). This region has so far been the site of the most frequent genetic abnormalities observed in association with adenoid cystic carcinoma.
Symptoms, complaints, and signs
Affected individuals predominantly complain of paralysis, which can occur throughout the face. Furthermore, swelling of the cervical lymph nodes occurs. Occasionally, diffuse pain or even paresthesias may also occur. Due to the fact that it is a very rarely occurring tumor, adenoid cystic carcinoma is often diagnosed quite late. The advantage is that the tumor grows slowly, although there is a disadvantage here at the same time: even if the tumor has a slow growth, it can still spread, so that sometimes other organs are already affected by cancer cells.
Diagnosis and course
Physicians can only make a definitive diagnosis after the tumor tissue has been removed (fine-needle aspiration, biopsy) and subsequently examined under a microscope, which is performed by pathologists. In the field of spreading diagnostics, imaging methods such as magnetic resonance tomography, computer tomography or positron emission tomography are predominantly used. In histology, adenoid cystic carcinoma shows a highly differentiated picture; the tumor is mainly known for its very infiltrative growth pattern. The growth pattern is glandular or sieve-like. Adenoid cystic carcinoma grows slowly but permanently, so while the 5-year survival rate is favorable at 89 percent, the 10-year survival rate is just 65 percent. The 15-year survival rate is just 40 percent.The histological growth pattern, tumor size, localization and also the clinical stage, the question of whether there is bone involvement and also the status of the surgically placed resection margins, are of essential importance for the course of the disease. If a cribriform or tubular growth pattern is present, there is sometimes a favorable course of disease; lymph node involvement is possible but occurs rarely (5 to 25 percent). Fine metastases that subsequently invade the lungs, brain, bones or liver are possible. The fact that other tumors develop is therefore a reality in 25 to 55 percent of all cases. If the tumor cells spread, the survival rate – within the first five years – is just 20 percent.
Complications
Adenoid cystic carcinoma is a rather rare malignant salivary gland tumor that manifests in the head and neck region. It grows slowly from the palate along blood vessels and nerves. The symptom is noticeable by a severely painful swelling. In acute cases also by means of facial paralysis. In order to determine the cause, the affected person requires immediate medical attention. As further complication consequences the tumor can form tumors in the lymph nodes, which spread to the brain, esophagus and lungs. If the affected person does not have the symptom treated, the infestation can also spread to the skin, prostate and cervix. Women are more often affected by the symptom than men. The age range of the patients is from 30 to 70 years of age and it hardly occurs in childhood. Due to the persistent recurrent nature of adenocarcinoma, a complete cure is not possible. A detailed pathogenesis, whether the symptom is genetic or environmental, is not yet known. The adenoid cystic carcinoma is removed over a large area during a surgical procedure. The therapy depends on the diagnostic findings. Since complete removal is usually not feasible due to the spread of the tumor, combined radiation therapy is recommended for containment. This form of therapy can stress the patient’s psyche and physical tolerance and requires intensive care.
When should you see a doctor?
The typical warning signs of adenoid cystic carcinoma are facial paralysis and swelling of the cervical lymph nodes. Sometimes diffuse pain and discomfort are also present, and these symptoms increase as the disease progresses. Should one or more of these symptoms be noticed, an immediate visit to the doctor is recommended. Rapid medical clarification can significantly improve the survival rate. It is therefore advisable to talk to your family doctor at the first symptoms for which there is no other explanation. If carcinoma is suspected, appropriate specialists should also be consulted. This applies in particular if the person concerned has already developed cancer in the past. Genetic predispositions should also be considered and discussed with the physician if necessary. In any case, adenoid cystic carcinoma must be diagnosed by a physician and subsequently treated with the aid of radiation therapy. Medical evaluation of the cancer should be done as early as possible to improve the chances of cure and to rule out complications.
Treatment and therapy
If the physician determines that the cancer is adenoid-cystic carcinoma, he or she will initially favor complete removal of the tumor. This will require the patient to undergo a surgical procedure. The tumor is removed – with sufficient safety distance to the surrounding tissue. Postoperative radiation therapy is ordered to reduce the recurrence rate. Chemotherapy, on the other hand, is not recommended; to date, there is no effective chemotherapy against adenoid cystic carcinoma. So-called combination therapies, those consisting of chemotherapy and radiotherapy, are used only in a few cases, mainly for testing.
Outlook and prognosis
The prognosis for adenoid cystic carcinoma of the head or neck when radiotherapy is used varies. On the one hand, it is said that the tumor does not respond to it. The value of a so-called neutron irradiation is increasingly judged positively. Unfortunately, a cure is often not possible.Complete tumor removal is usually not successful because the tumor grows along nerves and blood vessels. New tumorous developments are therefore frequently observed. Rapid tumor growth with daughter tumors is rarely documented. Usually, adenoid cystic carcinoma grows very slowly. However, this tumor tends to metastasize to the lungs as well as the bones. The tendency to recur is also relatively high. At first glance, these factors cast the prognosis in a poor light. A five-year survival rate is about 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, usually only palliative therapy seems possible. Especially when metastasis has begun.
Prevention
Because there is as yet no knowledge of how adenoid cystic carcinoma develops, no preventive measures are known. What is important is that – if the medical professional makes the diagnosis of adenoid cystic carcinoma – immediate treatment is started.
Follow-up
In most cases, the options or measures of aftercare for this disease are very limited. First and foremost, a quick and especially an early diagnosis is very important in this case, so that there is no further deterioration or other complaints and complications. Due to this tumor, the life expectancy of the affected person is significantly reduced in most cases. As a rule, even after successful treatment and removal of the tumor, further examinations 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 a surgical intervention, the affected person is dependent on bed rest. Efforts or other stressful activities should be avoided in any case in order not to put unnecessary strain on the body. In most cases, patients are also dependent on the help and support of friends and acquaintances. This also applies to chemotherapy, during which most patients require comprehensive support from their relatives. This also includes psychological support for the affected person.
What you can do yourself
Adenoid cystic carcinoma is a malignant cancer that usually takes a severe course. Sufferers can support the treatment by changing their lifestyle habits. In addition to dietary and exercise measures, alternative healing methods are sometimes recommended. For example, massages or acupuncture help to relieve pain and are thus an important part of the therapy. Patients should discuss alternative treatment methods with the doctor in charge so that they can be optimally coordinated with conservative therapy. In addition, general measures such as abstaining from alcohol and caffeine apply. In order not to further irritate the esophagus, spicy, hot, cold and sour foods should be avoided as far as possible. A light diet is recommended, which can be supplemented with food supplements and appetite stimulants if the disease leads to malnutrition. Sufferers should also talk to other people affected by the disease. Talking to other cancer patients not only makes it easier to cope with the disease, but also often reveals further possibilities for improving well-being and thus also enhancing quality of life in the long term. Lastly, in the case of adenoid cystic carcinoma, the voice must be spared, as it is usually already severely affected by the tumor.
