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Primary Intestinal Type Adenocarcinoma of Vagina

Last updated Jan. 1, 2019

Approved by: Krish Tangella MD, MBA, FCAP

Primary Intestinal Type Adenocarcinoma of Vagina is an exceedingly rare malignancy of intestinal type that arises in the vagina. The cancer may affect a wide age range of adult women.


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

  • Cloacogenic Adenocarcinoma of Vagina
  • Intestinal-Type Mucinous Adenocarcinoma of Vagina
  • Primary Vaginal Adenocarcinoma, Intestinal Type

What is Primary Intestinal Type Adenocarcinoma of Vagina? (Definition/Background Information)

  • Primary Intestinal Type Adenocarcinoma of Vagina is an exceedingly rare malignancy of intestinal type that arises in the vagina. The cancer may affect a wide age range of adult women
  • The tumor cells resemble the epithelium of the intestine when examined by a pathologist under a microscope, and hence, it is called an intestinal type tumor. However, because it resembles intestinal epithelium, the possibility of a metastasis from small bowel or large bowel (colon) should be ruled out
  • In a majority of cases, there are no risk factors or causes identified for Primary Intestinal Type Adenocarcinoma of Vagina. In a small minority of cases, the cancer is seen against a background of exposure to the chemical diethylstilbestrol (DES, a synthetic type of estrogen hormone). This is seen in a handful of cases affecting young women, whose mothers were exposed to DES during pregnancy
  • The signs and symptoms of Primary Vaginal Adenocarcinoma of Intestinal Type include urination difficulties, pain during sex, and the presence of a mass in the vagina. The complications are dependent upon the stage of the cancer and may also include complications related to treatment
  • Treatment for Primary Intestinal Type Adenocarcinoma of Vagina is mainly through surgery and other supplementary treatment measures. The prognosis depends on the cancer stage and overall health of the individual

Who gets Primary Intestinal Type Adenocarcinoma of Vagina? (Age and Sex Distribution)

  • Primary Intestinal Type Adenocarcinoma of Vagina is a very rare form a cancer that may be seen in middle-aged and older women (mostly over the age of 40 years). However, it may be manifested in a wider age group of women
  • There are no known geographical localizations; this cancer type is found worldwide among all races and ethnic groups

What are the Risk Factors for Primary Intestinal Type Adenocarcinoma of Vagina? (Predisposing Factors)

No well-established risk factors for Primary Intestinal Type Adenocarcinoma of Vagina are observed.

  • However, it can arise from villous or tubular adenoma of vagina
  • Extremely rarely, this carcinoma has been observed in association with diethylstilbestrol (DES) exposure:
    • Female children of women, who took this drug while pregnant, developed certain adenocarcinomas of the cervix and vagina more commonly than the rest of the population
    • On an average, adenocarcinoma in this (DES) context occurs at an earlier age in young women
    • The use of DES in pregnant women is mostly discontinued, and hence, the incidence of this particular type of cancer is declining

It is important to note that DES exposure does not take place in the individual affected by cancer, but occurred earlier in the individual’s mother while they were pregnant.

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 Primary Intestinal Type Adenocarcinoma of Vagina? (Etiology)

  • Currently, there are no known causes for the development of an Primary Intestinal Type Adenocarcinoma of Vagina
  • In very few cases, the cancer resulted from diethylstilbestrol-exposure. DES is a synthetically produced estrogen hormone

It is important to note that the tumor does not occur due to a sexually-transmitted infection.

What are the Signs and Symptoms of Primary Intestinal Type Adenocarcinoma of Vagina?

The signs and symptoms are associated with large tumor sizes and no particular symptoms may be noted for small tumors. The signs and symptoms of Primary Intestinal Type Adenocarcinoma of Vagina may include:

  • Presence of an enlarging vaginal tumor mass
  • The tumors may grow to large sizes; large tumors may project into the cervix or outside the vulva
  • Pain during sex
  • Abnormal vaginal bleeding and/or discharge; postmenopausal vaginal bleeding
  • Urinary tract associated signs and symptoms such as frequent urination, painful urination, blood in urine (hematuria), etc.
  • If the urinary bladder is involved, then it may obstruct the bladder and lead to retention of urine
  • Pain in the pelvic or abdominal region

How is Primary Intestinal Type Adenocarcinoma of Vagina Diagnosed?

A diagnosis of Primary Intestinal Type Adenocarcinoma of Vagina may be made by using the following resources:

  • Preliminary examination composed of:
    • Complete physical examination including pelvic exam
    • Evaluation  of medical (and family) history including history of DES exposure (in the mother), if any
  • Initial diagnosis that is made by:
    • Transvaginal ultrasound of the uterus can provide an image of the vagina and surrounding pelvic organs
    • MRI scans can be used to observe if a vaginal tumor has the characteristics of cancer, along with visualizing the cancer spread (if it has spread to other areas)
  • Plain radiographs of the chest can provide evidence if the tumor has spread to the lungs
  • CT scans are rarely used in diagnosing vaginal cancer, but can be used to determine if metastasis has occurred
  • Colposcopy:
    • The cervix (including the vagina) is examined with an instrument, called a colposcope. This helps the physician get a magnified view of the cervix
    • 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
  • A hysteroscopy may be performed to visualize and simultaneously perform the biopsy of any abnormal growth within the uterus. A hysteroscopy is performed with the aid of a tiny telescope through the uterus that allows a visualization of the area
  • A vaginal biopsy may be necessary for a definitive diagnosis. In the tissue biopsy procedure, the physician removes a sample of the tissue and sends it to the laboratory for a histopathological examination. The pathologist examines the biopsy under a microscope and arrives at a definitive diagnosis after a thorough evaluation of the clinical and microscopic findings, as well as by correlating the results of special studies on the tissues (if required)

Primary Vaginal Adenocarcinoma of Intestinal Type resembles intestinal epithelium, when examined by a pathologist under the microscope. Thus, the possibility of a metastasis from the small or large intestine must be ruled out through the following procedures:

  • Upper GI endoscopy: An endoscopic procedure is performed using an instrument called an endoscope, which consists of a thin tube and a camera. Using this technique, the radiologist can have a thorough examination of the insides of the gastrointestinal tract
  • Colonoscopy: A colonoscopy is a test that allows the physician to look at the inner lining of the colon and rectum. A typical colonoscopy involves using a thin, flexible tube (called a colonoscope), with an attached video camera, to view the colon and rectum

Note: On examination of the tumor, the morphological features of mucinous adenocarcinoma are noted.

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 possible Complications of Primary Intestinal Type Adenocarcinoma of Vagina?

The possible complications of Primary Intestinal Type Adenocarcinoma of Vagina include:

  • Ulceration of large tumor masses may get secondarily infected with bacteria or fungus
  • The rarity of the condition may cause a delayed diagnosis leading to metastasis to local and distant sites
  • The tumor may also adversely impact adjoining/surrounding structures, such as the nerves, leading to a loss of feeling
  • Side effects of chemotherapy (such as toxicity) and radiation
  • Sexual dysfunction can take place as a side effect of surgery, chemotherapy, or radiation therapy
  • Recurrence of the cancer following incomplete surgical removal

How is Primary Intestinal Type Adenocarcinoma of Vagina Treated?

Once a diagnosis of vaginal cancer has been made, the extent to which the tumor has spread is assessed, known as staging. The staging for vaginal cancer is based upon the FIGO (International Federation of Gynecology and Obstetrics) and the AJCC (American Joint Committee on Cancer) TNM staging systems.

The TNM classification for vaginal cancer is given below:

Tumor extent (T):

  • Tis: Cancer cells are only in the most superficial layer of cells of the vagina without growth into the underlying tissues. This stage is also called carcinoma in situ (CIS) or vaginal intraepithelial neoplasia 3 (VaIN 3). It is not included in the FIGO system
  • T1: The cancer is only in the vagina
  • T2: The cancer has grown through the vaginal wall, but not as far as the pelvic wall
  • T3: The cancer is growing into the pelvic wall
  • T4: The cancer is growing into the bladder or rectum or is growing out of the pelvis

Lymph node spread of cancer (N):

  • N0: The cancer has not spread to lymph nodes
  • N1: The cancer has spread to lymph nodes in the pelvis or groin (inguinal region)

Distant spread of cancer (M)

  • M0: The cancer has not spread to distant sites
  • M1: The cancer has spread to distant sites

Stage grouping: Once the T, N, and M categories have been assigned, this information is combined to assign an overall stage in a process called stage grouping. The stages identify tumors that have a similar outlook and are treated in a similar way.

Stage 0 (Tis, N0, M0):

  • In this stage, cancer cells are only in the top layer of cells lining the vagina (the epithelium) and have not grown into the deeper layers of the vagina
  • Cancers of this stage cannot spread to other parts of the body
  • Stage 0 vaginal cancer is also called carcinoma in situ (CIS) or vaginal intraepithelial neoplasia 3 (VaIN 3)
  • This stage is not included in the FIGO system

Stage I (T1, N0, M0):

  • The cancer has grown through the top layer of cells but it has not grown out of the vagina and into nearby structures (T1)
  • It has not spread to nearby lymph nodes (N0) or to distant sites (M0)

Stage II (T2, N0, M0):

  • The cancer has spread to the connective tissues next to the vagina but has not spread to the wall of the pelvis or to other organs nearby (T2)
  • The pelvis is the internal cavity that contains the internal female reproductive organs, rectum, bladder, and parts of the large intestine
  • It has not spread to nearby lymph nodes (N0) or to distant sites (M0)

Stage III - either of the following:

  • T3, any N, M0:
    • The cancer has spread to the wall of the pelvis (T3)
    • It may (or may not) have spread to nearby lymph nodes (any N), but it has not spread to distant sites (M0)

OR

  • T1 or T2, N1, M0:
    • The cancer is in the vagina (T1) and it may have grown into the connective tissue nearby (T2)
    • It has spread to lymph nodes nearby (N1), but has not spread to distant sites (M0)

Stage IVA (T4, Any N, M0):

  • The cancer has grown out of the vagina to organs nearby (such as the bladder or rectum) (T4)
  • It may or may not have spread to lymph nodes (any N)
  • It has not spread to distant sites (M0)

Stage IVB (Any T, Any N, M1):

  • Cancer has spread to distant organs such as the lungs (M1)

(Source: “The FIGO/AJCC system for staging vaginal cancer”; information provided by the American Cancer Society, February 2016)

Primary Intestinal Type Adenocarcinoma of Vagina may be treated using several methods depending on the stage of the cancer and no specific treatment protocol exists. The treatment options may include the following measures:

  • If the tumor can be surgically removed with minimal damage and mutilation of the genitalia, then a conservative surgery is attempted. Otherwise, a combination of radiation and chemotherapy may be administered to shrink and confine the tumor, before invasive procedures are sought
  • Following a surgical excision and tumor removal, a dose of chemotherapy and/or radiation therapy may be administered to kill any remaining cancer cells
  • Radiation therapy and chemotherapy may be necessary if the spread of the cancer to distant sites is noted
  • Advanced stage tumors with metastasis may be treated on a case-by-case basis; often symptomatic treatment with radiation and/or chemotherapy is attempted
  • Very infrequently, in order to treat the more aggressive tumors, a combination of stem cell transplant with chemotherapy (high-dose) may be necessitated

The treatment of Primary Intestinal Type Adenocarcinoma of Vagina involves surgery, which is the most common treatment option considered.

Surgery:

  • Vaginectomy: It is the surgical removal of a part of the vagina (called partial vaginectomy), or the entire vagina (called total vaginectomy), or the vagina and its surrounding affected structures/tissues may also be removed (called radical vaginectomy)
  • Radical trachelectomy: The surgeon removes the cervix, upper part of the vagina, and nearby lymph nodes, while preserving the ability to have children
  • Pelvic exenteration: The entire vagina, the surrounding tissues, and the pelvic lymph nodes are removed. In addition, depending on the extent of tumor spread, parts affected around the region (such as the cervix, uterus, rectum, colon, etc.) may be removed. Recovery from this surgery usually takes a long period

Chemotherapy:

  • Medications are used to kill the tumor cells, which may be given as oral pills or injected into veins
  • A combination of chemotherapy medications may be used
  • Chemotherapy may be used in addition to radiation and/or surgery, to treat cancers that have spread or recurred. When chemotherapy and radiation therapy are used together, it is called concurrent chemoradiation
  • Side effects of chemotherapy may include nausea, vomiting, hair loss, loss of appetite, diarrhea, fatigue, increased risk of infection, mouth sores, and easy bruising, depending on the drugs used

Radiation therapy:

  • This procedure uses high-energy beams to kill the cancer cells
  • These beams may be delivered from outside the body (external beam radiation therapy) or the radioactive material maybe placed inside the vagina (internal radiation therapy or brachytherapy)
  • Possible side effects may include:
    • Fatigue, nausea, vomiting, and diarrhea
    • Bladder irritation, leading to inflammation (cystitis)
    • Ovaries may be affected resulting in menstrual changes, or premature menopause
    • The vulva and vagina may be affected, causing soreness, or even scar tissue formation

Once treatment is complete, it is recommended that the individual schedule regular check-ups, based on the recommendation of the specialist treating them. 

How can Primary Intestinal Type Adenocarcinoma of Vagina be Prevented?

  • There are currently no known methods of preventing Primary Intestinal Type Adenocarcinoma of Vagina
  • Even though DES-exposure was responsible for many cases of adenocarcinoma earlier, the use of DES is decreasing steeply and the incidence of cancer caused by DES-exposure is on the decline worldwide
  • Due to its high metastasizing potential and recurrence rate, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor

What is the Prognosis of Primary Intestinal Type Adenocarcinoma of Vagina? (Outcomes/Resolutions)

  • The prognosis for Primary Intestinal Type Adenocarcinoma of Vagina 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
    • 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. However, the prognosis of  undifferentiated sarcoma is typically poor
  • According to the US National Cancer Institute (NCI) database, the 5-year survival rate for vaginal cancers range from 84% for stage I cancers, to 57% for stage IV cancers
  • 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
  • It is important to schedule and attend follow-up appointments with the healthcare provider. Many patients with metastatic or locally advanced tumors may be referred for clinical trials for experimental treatment options

Additional and Relevant Useful Information for Primary Intestinal Type Adenocarcinoma of Vagina:

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

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

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Dec. 5, 2016
Last updated: Jan. 1, 2019