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

• ACC of Bartholin Gland
• Adenocystic Carcinoma of Bartholin Gland
• Bartholin Gland Adenoid Cystic Carcinoma

What is Adenoid Cystic Carcinoma of Bartholin Gland? (Definition/Background Information)

• Adenoid Cystic Carcinoma (ACC) of Bartholin Gland is a very rare and unusual form of cancer. It is typically observed in middle-aged and older women
• Bartholin glands are glands that are present normally on either side of the vagina. Their function is to secrete a fluid into the vagina, in order to make the vaginal opening smooth and slippery
• Factors that may influence the development of Bartholin Gland Adenoid Cystic Carcinoma are currently unknown. However, general risk factors for the development of cervical and vulvar cancers include infection by human papilloma virus (HPV), certain hormonal medications, and smoking
• 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. A diagnosis of adenoid cystic carcinoma is established based on a tumor biopsy and examination under a microscope by a pathologist
• In many cases, Adenoid Cystic Carcinoma of Bartholin Gland is confused with Bartholin gland cyst, which is a benign condition. Hence, the condition is often ignored. This may result in delayed diagnosis and metastasis to the groin (inguinal) lymph nodes
• The treatment of choice is a surgical excision with clear margins followed by radiation therapy or chemotherapy, as decided by the healthcare provider. In majority of cases, the prognosis is good with appropriate treatment
• Nevertheless, the prognosis of Adenocystic Carcinoma of Bartholin Gland depends upon many factors including the stage of the tumor and health status of the affected individual. There is also a possibility of metastasis, which may influence the course of the cancer

Who gets Adenoid Cystic Carcinoma of Bartholin Gland? (Age and Sex Distribution)

• Adenoid Cystic Carcinoma of Bartholin Gland generally affects a wide range of women in the age group 25-80 years. The average age of tumor presentation is about 49 years
• The condition is generally prevalent worldwide; all racial and ethnic groups are at risk
• Around 15% of all malignant tumors of the Bartholin glands are adenocystic carcinomas

What are the Risk Factors for Adenoid Cystic Carcinoma of Bartholin Gland? (Predisposing Factors)

There are no specific risk factors established for Adenoid Cystic Carcinoma of Bartholin Gland. However, the risk factors for cancers occurring in the cervical and vulvar region include:

• Infection with human papilloma virus (HPV)
• Lack of periodic/regular Pap smear tests
• Weakened immune system as a result of disease, such as AIDS, or due to immune-suppressing drugs
• Having the first child at a young age (before 17 years) and multiple pregnancies

Note: In the specific case of adenoid cystic carcinoma, studies have not proven conclusively that chronic smoking and excessive alcohol consumption can influence its formation.

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 Bartholin Gland? (Etiology)

The cause of Adenoid Cystic Carcinoma of Bartholin Gland is generally 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 Bartholin Gland?

The signs and symptoms of Adenocystic Carcinoma of Bartholin Gland include:

• 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
• The tumor may be single; though, it is not uncommon to find multiple tumors in an individual
• The skin lesions may appear as crusted ulcer, plaques, and nodules
• There may be pain, itching, and burning sensation
• It may ulcerate and bleed. Occasionally, after the ulcer heals, it may become ulcerated again
• In some cases, the carcinoma may appear more pigmented than surrounding skin
• Individuals with immunocompromised states have more aggressive tumors

Additionally, the tumor may cause the following signs and symptoms:

• Abnormal vaginal bleeding, vaginal discharge
• Pain during sex and bleeding after sexual intercourse
• Anemia (due to bleeding)
• Loss of weight, loss of appetite

How is Adenoid Cystic Carcinoma of Bartholin Gland Diagnosed?

A diagnosis of Adenocystic Carcinoma of Bartholin Gland is made by:

• Complete physical examination with detailed medical history evaluation
• Examination by a dermatologist using a dermoscopy, a special device to examine the skin
• Radiological studies, such as ultrasound scan, CT and MRI scans, to determine the extent of tumor spread
• Colposcopy:
  • The cervix (including the vagina and vulva) is examined with an instrument, called a colposcope. This helps the physician get a magnified view of the region
  • In order for this procedure to be performed, the individual has to lie on a table, as for a pelvic exam. An instrument, called the speculum, is placed in the vagina to keep the opening apart, in order to help the physician visualize the cervix. The colposcope is then used to get a magnified view of the inside

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.

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

In case of metastatic tumors, the following diagnostic procedures can be used to procure the tissue sample:

• 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 or open biopsy of the tumor

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 Bartholin Gland?

The possible complications due to Adenocystic Carcinoma of Bartholin Gland could include:

• Discomfort, irritability of the affected region
• Painful or uncomfortable sexual intercourse
• The tumor can metastasize to the lymph nodes of the groin and other regions of the body. Frequently, it shows perineural and lymphatic invasion
• Severe emotional and psychological stress
• There is a high frequency of recurrence of the carcinoma following treatment
• Complications that arise from cancer therapy (such as due to chemotherapy or radiation therapy)
• 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 Bartholin Gland Treated?

Early diagnosis and treatment of Adenoid Cystic Carcinoma of Bartholin Gland is important to avoid complications such as metastasis to other regions. The treatment measures may include:

• In most cases, a wide surgical excision and removal of the entire tumor is the preferred treatment option. This may be followed by radiation therapy and/or chemotherapy
• The surgical removal of the tumor depends on the size and stage of the Bartholin Gland Adenocystic Carcinoma. The surgical procedures performed could include:
  • Hemivulvectomy, which is partial removal of vulva
  • Total vulvectomy, which is complete removal of vulva: Sometimes, local lymph nodes may also be removed using total vulvectomy, with bilateral inguinal and femoral lymphadenectomy
  • If the tumor size is large, then partial pelvic exenteration may be performed. During this procedure, all the cancer tissue and surrounding pelvic tissue is removed to decrease the bulk of the tumor. This is often followed by radiation therapy and chemotherapy
• If the tumor has metastasized (in rare cases), then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
• Post-operative care is important: One must maintain minimum activity levels, 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 Bartholin Gland be Prevented?

There are no specific preventative risk factors for Adenoid Cystic Carcinoma of Bartholin Gland. Nevertheless, the general preventative factors for cancers of the cervix and vulva may include:

• Use of measures to prevent sexually-transmitted infections, such as usage of condoms, avoiding multiple sexual partners
• Avoidance of smoking
• Regular screening to detect pre-cancers:
  • The American Cancer Society recommends screening of women from age 21 years
  • A Pap smear is recommended every 3 years, from ages 21-29 years
  • From age 30-65 years, a Pap smear and HPV testing is recommended, once every 5 years
  • More frequent screenings are advised for women at a high-risk
• Vaccination against human papilloma virus (HPV):
  • Two vaccines have been studied and approved for use in the United States - Gardasil (against HPV types 6, 11, 16, and 18) and Cervarix (against HPV types 16 and 18)
  • Cervarix has been approved for use in females aged 10-25 years, while Gardasil may be used in the 9-26 years age group
  • The American Cancer Society recommends routine vaccination of girls at 11-12 years of age

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 Bartholin Gland? (Outcomes/Resolutions)

• The prognosis of Adenoid Cystic Carcinoma of Bartholin Gland is generally unpredictable and can only be assessed on a case-by-case basis. On an equal comparative note, some individuals are known to survive for decades, while others for only a few years
• Studies show that some have lived for 30-40 years after diagnosis. However, in some individuals, because of a rapid growth, the life expectancy may be substantially decreased to a few years. If metastasis (such as to the local lymph nodes) is observed, then the prognosis is guarded or unpredictable
• Despite a slow-growing growth pattern, the National Cancer Institute (USA) considers adenoid cystic carcinoma as a high-grade malignancy. Also, the tumors can recur years after surgery/radiation. Hence close follow-up for a long duration (sometimes, even for several decades) is necessary
• Many researchers believe that ACC is a high-grade malignancy with a potential for severe destruction. Regular cancer screenings and checkups (and to detect recurrences early) are extremely important
• In general, the prognosis for ACC of Bartholin Gland depends upon a set of several factors that include:
  • The size of the tumor and the extent of its invasion: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Stage of cancer: 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
  • FIGO grade of the tumor: Tumors that are graded 1 and 2 have better prognoses than grade 3 tumors
  • Cell growth rate of the cancer
  • 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
  • Individuals with bulky disease have a poorer prognosis
  • Involvement of the regional lymph nodes, which can adversely affect the 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 (like cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Individuals, who tolerate chemotherapy sessions better, generally have better outcomes

Additional and Relevant Useful Information for Adenoid Cystic Carcinoma of Bartholin Gland:

• Vulvar cancer is the fourth most common malignancy of the female reproductive tract. Vulvar cancers are very uncommon. Only 0.1% to 5% of cancers occurring in the vulva are of Bartholin gland origin
• Bartholin glands were first described by a Dutch scientist named Caspar Bartholin in 1677
• Cervical cancer, the second most common cancer in women worldwide, is a malignant (capable of invading nearby and distant tissues) tumor of the cervix, which is the lower part of the uterus/womb

The following DoveMed website links are useful resources for additional information:

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