Nephron

Adenoid Cystic Carcinoma of Nasal Cavity

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Ear, Nose, & Throat (ENT)
Diseases & Conditions
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Contributed byKrish Tangella MD, MBAMar 30, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • ACC of Nasal Cavity
  • Adenoid Cystic Carcinoma of Nasal Cavity and Paranasal Sinuses
  • Adenocystic Carcinoma of Nasal Cavity

What is Adenoid Cystic Carcinoma of Nasal Cavity? (Definition/Background Information)

  • Adenoid Cystic Carcinoma (ACC) of Nasal Cavity is a very rare and unusual form of cancer. It is typically observed in middle-aged and older adults
  • A diagnosis of adenoid cystic carcinoma is established based on a tumor biopsy and examination under a microscope by a pathologist. Because of its rarity, it is often considered to be an ‘orphan tumor’. The rarity can pose diagnostic challenges and treatment difficulties
  • The risk factors and cause of Adenoid Cystic Carcinoma of Nasal Cavity are generally unknown. Genetic abnormalities have been observed in some tumors involving the MYB and NFIB genes
  • Most tumors are painless and grow very slowly on the body, but an erratic/random growth pattern may be observed; involvement of the surrounding nerves may also be noted. In many cases, during the early stages (sometimes for many years), no significant signs and symptoms are noticed apart from the presence of a swollen lump on the face
  • Some tumors may present headaches, nosebleeds, cause congestion of the sinuses, and partial hearing impairment. Adenoid Cystic Carcinoma of Nasal Cavity is known to metastasize to the local lymph nodes and lungs
  • The treatment of choice is a surgical excision with clear margins followed by radiation therapy. In case of spread of cancer to other regions, chemotherapy may be considered by the healthcare provider. Close follow-up is necessary for several years/decades after treatment
  • The prognosis depends upon a set of several factors including the stage of the tumor, age of the individual, the size of the tumor, and many other factors. In general, due to the unpredictable nature of Adenoid Cystic Carcinoma of Nasal Cavity, its prognosis is evaluated on a case-by-case basis

Who gets Adenoid Cystic Carcinoma of Nasal Cavity? (Age and Sex Distribution)

  • Adenoid Cystic Carcinoma of Nasal Cavity is normally observed in middle-aged and older adults; average age 55 years. Although, individuals of a wide age range may be affected
  • Both males and females are affected, though the condition is slightly more common in females than males (female-male ratio is 3:2)
  • It is a very rare form of cancer. But, there is no predilection for any particular racial or ethnic group

What are the Risk Factors for Adenoid Cystic Carcinoma of Nasal Cavity? (Predisposing Factors)

  • The risk factors for Adenoid Cystic Carcinoma of Nasal Cavity are currently not well-established
  • Studies have not proven conclusively that smoking and excessive alcohol consumption are risk factors for ACC

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Adenoid Cystic Carcinoma of Nasal Cavity? (Etiology)

The cause of development of Adenoid Cystic Carcinoma of Nasal Cavity is unknown. Some tumors have displayed a fused gene product of chromosome 6 and chromosome 9, called MYB-NFIB gene. This fused gene is caused by the translocation of chromosome 6 and 9.

  • In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor
  • Many cancer types are caused by genetic mutations. These can occur, due to inherited mutations, or mutations that occur due to environmental factors
  • The transformation of normally healthy cells into cancerous cells may be the result of genetic mutations. Mutations allow the cancer cells to grow and multiply uncontrollably to form new cancer cells
  • These tumors can invade nearby tissues and adjoining body organs, and even metastasize and spread to other regions of the body
  • A poor diet and lack of physical activity may also contribute to the cause of cancer. Unhealthy food choices and avoiding physical activities can increase one’s likelihood of developing health risks, which could ultimately lead to cancerous tumor growth

What are the Signs and Symptoms of Adenoid Cystic Carcinoma of Nasal Cavity?

Adenoid Cystic Carcinoma of Nasal Cavity may present the following signs and symptoms:

  • ACC is a slow-growing and painless tumor with a potential to invade into the surrounding tissues. In many individuals, often no significant signs and symptoms are seen for many months and years
  • The tumor develops slowly and the growth rate varies from one individual to another. Often individuals have tumors growing for 5 years or more, after which they may present symptoms
  • An unpredictable growth pattern is recognized; the tumor grows slowly during the initial stages, but a sudden rapid growth may be visible after a certain time duration
  • The tumor infiltrates into the surrounding nerves and can cause pain, numbness, and tingling sensations
  • Usually a single mass is observed; a swollen lump may be seen on the face
  • Large tumors may cause a pressure effect by compressing adjoining structures and organs
  • During the later stages, the tumor may cause the following:
    • Headaches
    • Nasal cavity inflammation and obstruction
    • Bleeding from the nose
    • Paranasal and nasal sinus congestion
    • Dry or stuffy nose
    • Hearing difficulties
  • Large tumors may ulcerate and bleed and become painful

How is Adenoid Cystic Carcinoma of Nasal Cavity Diagnosed?

A diagnosis of Adenoid Cystic Carcinoma of Nasal Cavity may involve the following:

  • A thorough medical history and physical examination
  • ENT examination
  • Plain X-ray of the head
  • CT or CAT scan with contrast of the head and neck region may show the presence of a. This radiological procedure creates detailed 3-dimensional images of structures inside the body
  • MRI scans of head and neck: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor
  • Whole body PET scans to determine how far the cancer has spread to other organ systems

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include: 

  • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor and the tumor may be misdiagnosed. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of the tumor
  • Open biopsy of the tumor

Tissue biopsy:

  • A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Note: A differential diagnosis, to eliminate other tumor types is considered, before arriving at a conclusion.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Adenoid Cystic Carcinoma of Nasal Cavity?

The complications of Adenoid Cystic Carcinoma of Nasal Cavity may include the following:

  • Ulceration of the tumor can lead to secondary infections of bacteria and fungus
  • Compression of the underlying nerve, which can affect nerve function
  • The tumor can metastasize to the nearby/local lymph nodes
  • In some occasions, tumor metastasis to distant sites including liver, lung, and bones, are observed. Metastasis often occurs due to a delay in diagnosis or due to a misdiagnosis
  • Recurrence of the tumor after a long period of time (years) on incomplete removal
  • Side effects of chemotherapy (such as toxicity) and radiation
  • Damage to the muscles, vital nerves, and blood vessels, during surgery
  • Post-surgical infection at the wound site is a potential complication

How is Adenoid Cystic Carcinoma of Nasal Cavity Treated?

The treatment of Adenoid Cystic Carcinoma of Nasal Cavity usually involves surgery, which is the first treatment option considered. This is followed by radiation therapy. In case of metastasis, chemotherapy may be employed by the healthcare provider.

  • In most cases, a wide surgical excision and removal of the entire tumor is the preferred treatment option. This is usually followed by radiation therapy
  • If the tumor has metastasized, then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
  • Post-operative care is important: A minimum activity level is ensured, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important and encouraged

Clinical trials: In advanced stages of cancer progression, there may be some newer treatment options, currently on clinical trials, which can be considered for some patients depending on their respective risk factors.

How can Adenoid Cystic Carcinoma of Nasal Cavity be Prevented?

  • Currently, there are no known methods to prevent Adenoid Cystic Carcinoma of Nasal Cavity occurrence
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Adenoid Cystic Carcinoma of Nasal Cavity? (Outcomes/Resolutions)

  • The prognosis of Adenoid Cystic Carcinoma of Nasal Cavity is generally unpredictable and can only be assessed on a case-by-case basis. In some individuals, the life expectancy is long, even after metastatic stage (advanced disease)
  • Studies show that some have lived for 30-40 years after diagnosis. However, in some, because of a rapid growth, the life expectancy may be substantially decreased to a few years. The tumors can recur years after surgery/radiation
  • Also, despite a slow-growing growth pattern, the National Cancer Institute (USA) considers adenoid cystic carcinoma as a high-grade malignancy. Hence close follow-up for a long duration (sometimes, even for several decades) is necessary
  • The prognosis of ACC of Nasal Cavity depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Individuals with bulky disease may have a poorer prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
    • Progression of the condition makes the outcome worse
  • An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
  • The combination chemotherapy drugs used, may have some severe side effects (such as cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Tolerance to the chemotherapy sessions is a positive influencing factor

Additional and Relevant Useful Information for Adenoid Cystic Carcinoma of Nasal Cavity:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/cancer/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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