Adenoid Cystic Carcinoma Research Foundation or ACCRF is situated in the US state of Massachusetts, at a place called Needham.  It is a charitable organization that works with the goal of accelerating development of advanced therapies for ACC treatment.

ACC being one of the most rare cancer types might make the doctors face challenges like unavailability of basic research, limited funding and minimum researcher focus. The experts involved with Adenoid Cystic Carcinoma Research Foundation work together for overcoming all these challenges taking the advantage of fresh, lower cost technologies resulted from successful medical studies.

For accomplishing the mission, ACCRF has implemented strategies based on 3 goals; they are:

• Building community involving interested researchers
• Generating innovative and effective action plans
• Mobilizing the  adenoid cystic carcinoma patients for offering support to the research

All the plans made by ACCRF are made expansively with the intent of making them active globally. The projects running currently include basic research, drug screening etc. Ultimate goal of all these projects is supporting the clinical trials.  The funded researches belong to countries like United States, United Kingdom, Sweden and China.

ACC is one of the most common varieties of tumor that is known to affect the salivary gland and breed tumors in this region. ACC is, in most cases, a slow growing condition which makes it possible for individuals that develop to have a much lower rate of mortality in the early stages.

There have been reports of many Adenoid Cystic Carcinoma symptoms. Some of these symptoms include facial swelling, hoarseness and difficulty swallowing, formation of lumps in areas of the face, ears, jaw tongue and mouth, drooping eyelids and changes in vision or speech. Symptoms may also include problems of the respiratory system.

Although these symptoms may be directly linked to the present of ACC many of them may also be associated with other conditions or illness as well so if you experience any of these symptoms it is important that you seek the advice of a medical professional to determine if ACC may be the actual cause of your symptoms.

Although the Adenoid Cystic Carcinoma symptoms are numerous this does not necessarily mean that individuals afflicted will experience any or all of these symptoms. If you are experiencing any unusual or unexplained symptoms it is always a good idea to check with your doctor to determine if a problem exists. In some cases people have gone for an extended length of time without any symptoms which makes early detection of this condition difficult.

The word prognosis often refers to the treatability of a disease and the chances of success with those treatments. In the case of Adenoid Cystic Carcinoma the prognosis depends on several factors such as the size and variety of the tumor involved, what treatment option is used and how early the tumor was detected. Another factor may include how fast the tumor is spreading. In order to determine this it may be necessary to make several trips to a physician for regular monitoring of the growth of the tumor.

In any case early detection is often the key to successful and effective treatment of any cancer including ACC. The earlier a problem is located then the sooner a treatment plan can be discussed and executed.

If you experience any of the Adenoid Cystic Carcinoma symptoms or any other symptoms that can’t be explained by some other illness or condition, then it is vitally important that you promptly schedule an appointment with your doctor to determine the possibility of the presence of ACC.

There are many symptoms that may be experienced by individuals in relation to ACC. These symptoms include paralysis of the face or nerves, lumps around the face, ears, jaw and mouth, drooping eyelids, changes in vision or hearing, hoarseness or difficulty swallowing and problems related to the respiratory system. If this condition affects the lung area some additional symptoms that may be encountered include coughing, shortness of breath and wheezing.

There are two common treatments for ACC. Adenoid Cystic Carcinoma surgery is the most common course of treatment for this disease. Another treatment that may be used is radiation also known as radiotherapy; this treatment is often given after surgery and may also be referred to as adjuvant or palliative radiotherapy. This treatment may be used alone in cases where surgery is not a viable option due to location, size or general condition of the individual.

What type of adenoid cystic carcinoma surgery is used is dependent on the location of the tumor in question. When the tumor is located in the breast area the tumor and some surrounding tissue is generally removed through a surgical procedure. Generally this will be followed by radiotherapy to ensure that no cancerous cells remain and in most cases the individual has a good chance of making a full recovery.

Although surgery is most often the first choice of treatment in adenoid cystic carcinoma when the area affected is located within the head or neck this makes surgery difficult.  The reason for this is because tumors in this area are often likely to spread along the nerve tracts.

Although in many cases surgery is the best course of action this does not always mean that it will correct the problem. Even with surgery tumors may reappear in previously affected areas and in some cases even in areas that were not previously affected. Radiotherapy often helps to reduce the chances of this. This is why radiotherapy is often used in conjunction with adenoid cystic carcinoma surgery. In cases where surgery is not possible radiotherapy alone may be an effective treatment.

Even if it is surgery and radiotherapy is performed and it has been determined that these treatments were effective in removing the tumor in the affected area, it is still important to maintain regular checkups with your health care provider in order to be certain that this condition does not recur. If there is a recurrence of the tumor or if tumors develop in other areas it may become necessary to undergo these treatments again for the new or resurfacing tumor.

Even after an extended period of time has passed, if new symptoms are experienced it is important to be rechecked for development of new tumors.

ACC is one cancer that is known to infect and spread through the nerves. This is also known as perineural invasion. Infection of the lymph nodes is not a common occurrence with this type of cancer. ACC more commonly affects women than men.

The most common methods of treatment generally include surgery and then radiation. At this time there have been no drug therapies that have been reported to be effective.

At this time it is unknown what causes Adenoid Cystic Carcinoma. It is not believed to be associated with common risk factors such as alcohol consumption and smoking. It is also not known to be associated with heredity as other cancer may be.

In most cases, cancers are determined by their appearance under a microscope. Adenoid Cystic Carcinoma has been reported to have three different types of cells. These include tubular, solid and cribiform. In some cases it has even been noted that all three variations have appeared in within one tumor. It is suggested that a tumor can go from cribiform to solid over the progression and time. Solid type may suggest a more aggressive cancer and may in turn result in a higher mortality rate. In order for a tumor to be classified as solid it should be approximately 30% solid.

Each case of ACC is different based on the particular person that is afflicted with it. In some people it may move very slowly while others it may spread at a much faster rate. Some of the things that may have an effect on the progression of ACC include the type of tumor and how large it is, as well as how quickly it was detected.

There are numerous symptoms that have been reported to be associated with Adenoid Cystic Carcinoma including nerve paralysis, changes in vision and speech, respiratory problems, drooping eyelids and hoarseness or difficulty swallowing. Another symptom that you may experience is painful lumps in various locations such as about the face, in the mouth, under the jaw as well as under the tongue, in the roof of the mouth and in front of the ears.

Although there are quite a few symptoms relating to Adenoid Cystic Carcinoma not all individuals will experience all of these symptoms and some people who develop ACC may not have any of the symptoms for long periods of time.

The doctor would take into consideration the following four aspects while deciding on the test to be performed for diagnosis, namely:

• How old the patient is and any presence of medical problem.
• The kind of cancer that is doubted.
• How severe the signs are appearing.
• Any past test outcomes in case carried out.

Along with conducting a comprehensive physical examination of the person, the below mentioned tests would be carried out for diagnosing the presence of AdCC.

• Biopsy Procedure
  A biopsy involves removing a miniscule quantity of tissue sample for microscopically evaluating the presence of cancer. There are other testing methods which could detect cancer presence, however solely the biopsy procedure could help in making an explicit identification. The tissue sample extracted during the biopsy procedure is evaluated by a doctor proficient in the interpretation of lab tests and analysing cell, tissue and organ samples for diagnosing ailments (pathologist).The biopsy method could be carried out by employing a fine needle biopsy or by surgically excising some or most of the tumor present at the site. AdCC tumors are typified by a distinguishing outline in which bunches of epithelial cells enfold and optionally permeate ducts or glandular constitution in the organ. Often AdCC is diagnosed subsequent to the operative excision of a tumor that was firstly believed to be non-malignant.
• Imaging Techniques
  Imaging tests chiefly comprising of MRI or magnetic resonance imaging and computed tomography (CT or CAT scans) are beneficial in helping doctors to check the size of the tumor before deciding on any surgery. A PET or positron emission tomography could additionally be employed for ascertaining whether the tumor has metastasized or reached other body parts.
• MRI or Magnetic Resonance Imaging
  This imaging method employs magnetic fields instead of X-rays for rendering in-depth pictures of the interiors of the body. A particular kind of contrasting dye could be intravenously administered to the patient for facilitating lucid images. An MRI is immensely beneficial in detecting perineural multiplication or tumor growing along the nerve branching in case of AdCC.
• CT or CAT scan
  A CAT scan produces 3-D imagery of the interior parts of the body employing an X-ray device. The assistance of a computer is then taken for merging the pictures captured converting them into a complete, cross-sectional outlook that lucidly reveals any anomalies or tumor growths. At times, a contrasting medium is administered intravenously for providing clear images.
• PET scan
  A PET scanning method is a means of creating images of internally placed structures. A miniscule quantity of radioactive matter is given via injection form which on absorption by the body emits energy that could be picked up by the scanning device assisting in image production.

Below explicated are the widely employed treatments for adenoid cystic carcinoma, though patients are egged on to opt for participating in clinical trials when deciding on the line of treatment. Clinical trials are research studies performed for testing any new-fangled treatment for proving its safety quotient, efficacy and probably enhanced standard treatment.

1. Surgery

Surgical excision of the tumor is the line of treatment in case of AdCC, when carried out adhering to utmost safety standards and has likelihood of having a favourable result. A surgical oncologist is one proficient in cancer treatment performing the surgery. During surgical intervention, the tumor along with marginal tissues is taken out. In case the marginal tissues excised at the time of surgery contain no indication of malignancy in about two mm of the tissue adjoining the tumor, then it is an indicator of complete expurgation of the tumor and offers the optimal prospects of success in treating the location where the cancer originated.

As AdCC often has a tendency of spreading along the nerve branches, hence examination and establishing if nerves are affected is done by the doctor. A tumor that is considerably involving the nerves requires urgent removal.

The side effects associated with surgery differ dependent on the location of the surgery. Trepidations associated with all surgeries involving the parotid glands is that the nerves of the face could  be damaged or severed during surgery leading to facial drooping. Occasionally, grafting procedure of a facial nerve could be employed as a restorative means subsequent to surgery for the removal of the tumor. The degree of surgery being performed would differ by the tumor location and the inner structures that have been affected.

2. External-Beam Radiation Therapy

Radiation therapy employs high-power x-ray beams or other matter for obliterating malignant cells. The prevalent form of radiation therapy offered is known as external-beam radiation therapy administered from a device located outside the body. A specialist doctor (radiation oncologist) is the one who offers the treatment that is done generally as an adjuvant therapy (post-surgery therapy) for eradicating any infinitesimal remnant cancerous cells in the body following surgery.

Radiation therapy is generally not a pre-operative procedure in case of AdCC as several specialists believe that there is enhanced healing process of the operative wounds when radiation therapy is not offered before surgery.

3. IMRT or Intensity modulated radiation therapy

A particular process of eternal-beam radiation therapy called as IMRT or intensity modulated radiation therapy facilitates delivery of radiation therapy dosages of greater efficacy while sparing as much as normal cells as possible thus resulting in lesser side effects.

As radiation therapy is a target-specific treatment hence the side effects experienced would be dependent on what site the radiation therapy was offered. Usually when the treatment is performed on the head and neck regions, then side effects experienced could include soreness in the mouth or throat area, discomforting sensation and trouble during ingestion. The skin could show some form of reaction or burns that would be excruciating. Due to the outcome of radiation therapy on healthy tissues, particularly in the oral or throat area, such persons are persuaded to undergo prophylactic treatment prior to the radiation treatment commencing, conducted by a dental specialist who has received training in oncology.

Long-standing side effects faced due to radiation therapy differ in accordance to the locations that are part of the treatment. However, these could comprise of inadequate salivation leading to parched sensation in the mouth and deteriorating condition of the teeth. In few situations, a medicine for stimulating the parotid saliva gland and for increasing and restoring salivation is offered. Presently, amifostine or Ethyol – a medicine used for safeguarding the tissues at the time of radiation therapy is being researched. Scar formation or fibrosis (hardness or thickness) of the skin and muscles could arise for months or years following radiation therapy that could hinder lesions from being healed, rigidity in the neck and difficulty in ingestion. Other long-standing side effects which could arise comprise of hastened constriction of the carotid arteries that carry blood supply to the upper extremities of the body and could lead to hypothyroidism.

Entrants for radiation therapy are egged on to having a meeting with an experienced speech pathologist before undergoing the treatment, so that apt exercises could be carried out for maintaining potency and suppleness of the muscles linked with speech and ingestion.

4. Neutron and proton radiation therapy

Neutron therapy is diverse from radiation therapy and has a delivering capacity of twenty to hundred times greater energy in their course span leading to more annihilation of cancer cells in comparison to the traditionally employed radiation therapy. Neutron radiation therapy has been tried on several varying forms of tumors, and with those tumors inflicting the salivary glands especially AdCC appears to show the maximum benefits. In particular scenarios, neutron therapy could be the opted treatment in case of AdCC, especially in hard to reach places of the body where surgery could not be performed.  Nevertheless, neutron therapy holds more acute side effects in comparison to tradition radiation therapy, like acute soreness in the oral area and at times the throat and problems with ingestion, and hence is usually employed more frequently among individuals having ailments that could not be operated upon or disease that has relapsed. Medicines for facilitating salivation and protecting tissues could be offered in the course of the treatment. Neutrons could not be employed for the treatment of wide-spanning regions of the body in case of the disease having metastasized, however they could be utilised for treating disease spread which is one-off and leading to niggling symptoms like painful sensation, acting like blockages to part of the lung or exerting pressure on the spinal cord.

Proton radiation or proton beam therapy is a form of external-beam radiation therapy that employs protons instead of X-rays. During states of intense energy, protons have a capacity of destroying cancer cells. The benefits of proton therapy is the capability of better steering the manner of delivery of the radiation, thus immensely lowering the harm caused to the adjoining healthy tissues. But there is still dearth of extensive investigation in the usage of proton radiation therapy in case of AdCC.

Neutron or proton therapy is still not commonly obtainable and one has to speak to the doctor for enquiring about where such treatments are available.

5. Chemotherapy

Chemotherapy employs cancer-killing drugs offered by the medical oncologist. A typical chemotherapy schedule comprises of a particular set of cycles offered over a particular time span. Chemotherapy is usually not utilised in case of AdCC as this form of cancer seems to show lesser responsiveness to chemotherapy in comparison to other cancer forms. However, merging chemotherapy and radiation therapy is being explored for treating AdCC.

As AdCC is an atypical disease, and generally the presently obtainable chemotherapy has partial benefits, hence those having AdCC are recommended to participate in clinical trials for treating this condition.

AdCC prevalently is found in the salivary glands that comprise of a collection of secretory cells dispersed all through the inner areas of the URT or upper respiratory tract hence could be found in the regions of the oral palate, nasopharyngeal area, the base of the tongue, the internal mucosa lining of the oral cavity, laryngeal and tracheal area and main salivary glands located on the face.

Irrespective of where it originates, it has a tendency of spreading all along the nerves or via the blood. Only in five to ten percent of the cases it is observed to metastasize to the lymph nodes, the prevalent region of spreading being the lungs.

AdCC has been observed to stay dormant for protracted spans of time following which display proliferation surges. Nonetheless, AdCC could turn belligerent in few individuals which make the path of AdCC capricious.

Figures

AdCC is mostly detected among individuals aged forty to sixty years of age, though even childhood AdCC has been found. Female gender is more prone to be detected with AdCC as compared to males.

The rate of progression of the AdCC tumor is mostly gradual and individuals could have lengthy existence with metastatic ailment, but a belated relapse is prevalent and could arise several years subsequent to the foremost treatment.

Risk Factors

Though the reason for AdCC arising is unclear, however there has been some proof which indicates that the p53 tumor suppressor gene – a gene which curbs cell development by supervising the rate of cell division, that is in some way in an inactive state during the advanced and belligerent AdCC types.

Adenoid Cystic Carcinoma Symptoms

Several individuals could feel some symptoms whereas others could be totally asymptomatic. The foremost signs arise depending on the site of the tumor.
Foremost arising salivary glands lesions could seem non-tender, generally gradually-progressing, mass formation in the oral or facial area. As there are several salivary glands beneath the mucosa that line the oral area, throat and sinus, hence lumpy appearance might be from this tumor form.

The signs could comprise of:

• Ingesting problems.
• Gruffness.
• Dulling pain.
• A lump or nodule formation close to the ears or beneath the jaws.
• Facial nerve getting paralysed.
• Loss of sensation of the facial area or the tongue.
• A lumpy appearance beneath the tongue or on the roof of the mouth.