What Is Adenoid Cystic Carcinoma

Enhancing Healthcare Team Outcomes

The slow-growing kinetics, along with the rarity of the condition, often hinders in early diagnosis of ACC. While the treatment is mainly surgical, it is important to consult with an interprofessional team that includes a radiologist, radiation oncologist, and a medical oncologist to tailor the best possible treatment for the patient. The radiologist plays a pivotal role in studying the images for staging purposes, which are required to decide the most appropriate surgical technique. The radiation oncologist can weigh in on the benefit of postoperative radiation, which plays a crucial part in the overall management of the patient. Lastly, although there is no defined role of systemic chemotherapy in the treatment of localized ACC, in unresectable, recurrent, and metastatic cases, chemotherapy and targeted agents are the only options. Therefore, a medical oncology consult is also advised. Although there are no strict guidelines for the overall management of the ACC, an interprofessional team approach can improve outcomes and quality of life.

About Adenoid Cystic Carcinoma

Adenoid cystic carcinoma is a rare form of adenocarcinoma, which is a broad term describing any cancer that begins in glandular tissues. In general, AdCC is found mainly in the head and neck region. It can occasionally occur in other locations in the body, including the breasts or a womans uterus. AdCC most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva scattered throughout the upper aerodigestive tract. The upper aerodigestive tract includes the organs and tissues of the upper respiratory tract, such as the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus and windpipe. Salivary glands are generally divided into 2 groups based on their size: minor salivary glands and major salivary glands. A tumor may begin in the following places in these 2 groups:

• Minor salivary glands

• Trachea the windpipe

• Major salivary glands

• Parotid glands the largest salivary gland found on either side of the face in front of each ear

• Submandibular glands found under the jawbone

• Sublingual glands located in the bottom of the mouth under the tongue

Besides being classified based on where the cancer begins, AdCC is also described based on what the tumor cells look like under a microscope. This is called the histologic variations of the tumor. The tumor can be classified as:

• cylindroma, a tumor with tube-shaped cells

• cribriform, a tumor with gaps between the cells, giving it the appearance of Swiss cheese

• solid AdCC

What Can I Expect If I Have Adenoid Cystic Carcinoma

Adenoid cystic carcinoma is a slow growing cancer. For this reason, many cases are detected early and the first round of treatment is often successful. However, its common for cancer to return many years later.

Is adenoid cystic carcinoma fatal?

ACC can be considered a life-limiting disease in the event it recurs or metastasizes. While most people respond well to their initial treatment of surgery , cancer tends to come back later on either in the same place or a different area of the body.

The overall five-year survival rate for all people with adenoid cystic carcinoma is 89%. This means that 89% of people who are diagnosed with the condition are still alive five years later. The estimated 10-year survival rate is less than 70%. Keep in mind that survival rates are only estimates. They cant tell you how long youll live or how youll respond to your personalized treatment plan. To learn more about survival rates, talk to your healthcare provider.

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Neutron And Proton Radiation Therapy

Neutron therapy is a different form of radiation therapy that uses neutrons rather than x-rays. Compared with conventional radiation therapy, neutrons can deliver 20 times to 100 times more energy along their path length, causing much greater damage to the cancer cells. Neutron radiation therapy has been tested on many different types of tumors, and its treatment for salivary gland tumors and AdCC has shown the greatest benefit.

In certain instances, neutron radiation therapy may be the first treatment for AdCC, particularly in areas of the body where it is difficult to perform surgery. However, neutron therapy carries more severe side effects than traditional radiation therapy, such as severe sore mouth and/or throat and difficulty swallowing therefore, it is generally used more often with people with an inoperable tumor or if the tumor recurs.

Medication to help increase saliva production and protect tissues may be given during this treatment. Neutrons cannot be used to treat large areas of the body for metastatic disease, but they can be used to treat an isolated metastasis that is causing problems, such as pain, blockage of part of the lung, or pressure on the spinal cord.

Neutron and proton therapy are not widely available. Talk with your doctor for information about the availability of these treatments if they are recommended for you.

Treatment Options For Adenoid Cystic Carcinoma

Adenoid cystic carcinomas are rare, slow growing cancers, so treatment at a high-volume academic medical center by highly specialized head and neck and skull base surgeons and radiation oncologists is important for the best outcomes.

Our team of skull base neurosurgeons, head and neck surgeons, radiation oncologists and other specialists work together to develop an individualized treatment plan for your needs, utilizing treatment strategies that preserve quality of life.

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Salivary Gland Genetic And Molecular Biology

Chromosomal Rearrangements

In recent years, investigations have demonstrated recurrent, nonrandom, and hallmark chromosomal rearrangements, especially translocations that characterize both benign and malignant salivary gland tumors . These translocations result in fusion oncogenes that affect the apoptotic threshold, cell cycle regulation, tumor angiogenesis, and growth independence. Similar rearrangements have been a common theme among leukemias, lymphomas, sarcomas, and more recently with various epithelial malignancies including thyroid, prostate, and breast carcinomas. Characterizing these recurring translocations and their clinical significance is an active area of investigation in hopes of identifying future prognostic and therapeutic targets. Several noteworthy observations have been made to date.

In 2003, the recurrent and hallmark t translocation involving CRTC1 at 19p13 and MAML2 at 11q21 was described by several groups demonstrating it to be a key pathogenic event that underlies the development of mucoepidermoid carcinomas .18,19 This translocation has been shown to result in the mucoepidermoid carcinoma translocated gene 1mastermind-like gene family fusion transcript that results in a transcription factor acting on the Notch and the CREB pathways .

Mutation Analysis

Dysregulation in Growth Factor Receptors

c-KIT/CD117

Juliana Müller Bark, … Chamindie Punyadeera, in, 2021

The Ngftrka Axis And Pni

TrkA serves as the membrane-bound high-affinity receptor for NGF and is expressed in multitude of lineage-unrelated cells. In normal epidermis, TrkA expression is found to be the greatest in basal keratinocytes with expression dissipating in the superficial layers of the epidermis. There are conflicting reports of TrkA expression in normal melanocytes . In epidermal keratinocytes as well as keratinocytes of the follicular epithelia, TrkA is thought to responsible for mediating NGF-induced stimulation of proliferation and inhibition of apoptosis . The proproliferative effects of NGF on cells expressing TrkA combined with the NGF-rich microenvironment surrounding nerves as a result of its secretion by Schwann cells , has led many to hypothesize a connection between TrkA and PNI.

Lanceford M. Chong MD, MPH, John G. Armstrong MD, FRCPI, in, 2010

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What Questions Should I Ask My Healthcare Provider

Its important to learn all you can about your condition so you can make informed decisions about your treatment plan and overall health. Here are some questions you might consider asking your healthcare provider:

• Where is my tumor located?
• What symptoms are possible?
• Has cancer spread anywhere else in my body?
• Will you be able to remove the tumor with surgery?
• Will I need radiation therapy?
• Can I work while undergoing cancer treatment?
• How long will treatment take?

A note from Cleveland Clinic

An ACC diagnosis can be shocking, leading to feelings of anger, sadness and frustration. If you or a loved one has been diagnosed with this disease, ask your healthcare provider about the many resources available. You may also want to join a local support group, which can be extremely beneficial for your mental and emotional health.

Who Is At Risk Of Primary Cutaneous Adenoid Cystic Carcinoma

PCACC usually affects adults with fair complexion. It typically occurs between the ages of 50 and 70 years old. It occurs in men and women equally and more frequently with increasing age.

While immunosuppression increases the risk for some other cancers such as squamous cell carcinoma , this does not increase the risk of PCACC.

Due to the rarity of this cancer, other risk factors have not yet been identified.

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Adenoid Cystic Carcinoma Symptom

Paralysis

Paralysis is the loss or impairment of voluntary muscular power. Paralysis can result from either diseases involving changes in the makeup of nervous or muscular tissue or those that are the result of metabolic disturbances that interfere with the function of nerves or muscles. Depending upon the cause, paralysis may affect a specific muscle group or region of the body, or a larger area may be involved. When only one side of the body is affected, the condition is known as hemiplegia. In other instances, both sides of the body may suffer the effects, leading to diplegia or bilateral hemiplegia. When only the lower limbs are affected by paralysis, it is called paraplegia. When all four limbs are affected, it is referred to as quadriplegia. The term palsy is sometimes used to refer to the loss of muscle power in a body part.

Recurrence And Salvage Treatments

Throughout the observation period, 35 of 50 cases who underwent radical treatments recurred. In the study of the first recurrent sites, 15 cases had locoregional recurrence , 18 had distant metastatic recurrence, and 2 had both local and distant metastatic recurrence. Throughout the observation period, overall locoregional recurrence was observed in 24 cases , and overall distant metastatic recurrence was seen in 28 cases . Major first distant metastatic sites of the 34 cases that showed distant metastasis throughout the observation period were lung in 27 cases , bone in 6 , and liver in 4 . Lung metastasis was also observed simultaneously in 1 case of bone metastasis and 3 cases of liver metastasis. The overall distant metastatic sites observed throughout the observation period showed only a slight increase in lung metastasis up to 29 cases , but a large increase in bone up to 10 cases and in liver up to 9 cases . Lung was the most common first distant metastatic site, followed by bone and liver, and both bone and liver metastases tended to increase over time.

Salvage treatments for initial locoregional recurrence in 24 cases of overall locoregional recurrence were as follows: surgery in 10 cases radiotherapy), particle-beam therapy in 4 , radiotherapy in 3, CyberKnife in 1, and BSC in 7. DSS after salvage treatment was significantly better in the salvage-surgery group than in the other-treatment groups .

Fig. 4

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Treatment For Adenoid Cystic Carcinoma

You will be cared for by a multi-disciplinary team of health professionals during your treatment for ACC.

Discussion with your doctor will help you decide on the best treatment for your cancer depending on where it is in your body whether or not the cancer has spread your age, fitness and general health and your preferences.

The main treatments include surgery, radiation therapy and chemotherapy which can be given alone or in combination.

Adenoid Cystic Carcinoma: Causes Symptoms And Treatment

Adenoid cystic carcinoma, abbreviated as ACC and also called malignant cylindroma, adenocystic carcinoma, cribriform carcinoma, is a rare type of cancer that originates in the salivary glands, or the vicinity of the head and neck, in most cases. In some instances, however, ACC can arise in other bodily organs, namely the cervix in women, skin, breast and even the prostate in men. There are three major salivary glands present in the human body parotid, sublingual and submaxillary , each of which occurs in pairs, being situated in the region of the mouth, tongue and jawbone. Their main role in the system is the synthesis and secretion of saliva, a mucous, slightly watery fluid, that is carried into the mouth via tubular passages called ducts. Saliva helps in digestion of food, breakdown of complex compounds into simple substances, as well as protects the mouth, throat, by warding off illnesses and infections from developing in the oral cavity. Yet, when ACC stems in the salivary glands and surrounding oral tissues, these functions are invariably hampered, resulting in discomforting signs of nerve pain in facial muscles, cystic lumps in the tongues surface and numbness near the lips.

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Genetic Hallmarks Of Recurrent/metastatic Adenoid Cystic Carcinoma

2Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.

3Marie-Josée and Henry R. Kravis Center for Molecular Oncology,

4Diagnostic Molecular Pathology,

5Head and Neck Service, Department of Surgery, and

6Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center , New York, New York, USA.

7Foundation Medicine, Cambridge, Massachusetts, USA.

8Department of Genetics, Yale University School of Medicine, New Haven, Connecticut, USA.

9Department of OtolaryngologyHead and Neck Surgery, UCSF, San Francisco, California, USA.

10Department of Surgery, University of Chicago, Chicago, Illinois, USA.

11Department of Genomic Medicine and

12Department of Pathology, University of Texas MD Anderson Cancer Center , Houston, Texas, USA.

13Experimental Pathology Service, MSKCC, New York, New York, USA.

14Department of Medicine,

15Department of Radiation Oncology, and

16Immunogenomics and Precision Oncology Platform, MSKCC, New York, New York, USA.

Address correspondence to: Nikolaus Schultz, Timothy A. Chan, and Luc G.T. Morris, Immunogenomics and Precision Oncology Platform, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, New York 10065, USA. Phone: 212.639.3049 Email: , , .

ASH and AO contributed equally to the manuscript.

Find articles byHo, A.in: |PubMed |

1Department of Surgery and

2Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.

Cellular Components Of Tme

Heterotypic interaction of cancer cell with other cell types including stromal cells, endothelial cells, and immune cells play a critical role in the invasion of tumor cells to surrounding matrix and further to the secondary site. Stromal cells as the main cellular component of TME significantly affect the migration and invasion behavior of cancer cells. Many studies have emphasized the importance of TME in terms of cellular components . Microfluidic models allowed for the integration of multiple cell type with biochemical and biophysical properties of TME in 2D and 3D environment which led to better mimic of in vivo condition.

Harry Quon, … David W. Eisele, in, 2016

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Survival Rates And Clinical Outcomes

Overall survival , disease-specific survival , locoregional recurrence-free survival , distant metastasis-free survival , and disease-free survival of the whole population are shown in Fig. . Five-year, 10-year, 15-year, 20-year, and 25-year OS rates were 74.0, 63.7, 41.0, 27.3, and 20.0%, respectively. Similarly, DSS rates were 76.3, 65.7, 51.2, 51.2, and 38.4%, respectively LRFS rates were 62.5, 44.2, 16.9, 16.9, and 16.9%, respectively DMFS rates were 52.0, 35.1, 19.9, 13.3, and 13.3%, respectively and DFS rates were 45.1, 25.2, 9.4, 9.4, and 9.4%, respectively. OS and DSS almost overlapped for more than 10years after starting observation the difference began to increase beyond 5% after 144.1months , and it finally became 18.4% at 300months . Of all 7 cases of intercurrent-disease deaths observed during the observation period, 5 died of other cancers , and 2 died of pneumonia . After the longest disease-free interval was observed at the time point of 169.9months , the DFS reached a plateau, and the eventual 25-year DFS was 9.4%. On the other hand, 25-year DSS was 38.4%, which differed largely from DFS. Of all the 10 long-term-surviving cases beyond 12.0years, when the difference between the DSS and OS started to be observed, 1 died of ACC , 5 were alive , and 4 died of intercurrent disease . While intercurrent-disease deaths increased as time advanced, 60% of long-term surviving cases were eventually in an ACC tumor-bearing state.

Fig. 1

Remission And The Chance Of Recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having no evidence of disease or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place, meaning it is a local recurrence, or nearby, which is a regional recurrence. If it comes back in another place, it is a distant recurrence.

When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat this type of recurrent cancer. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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