VULVAR CANCER (con't)
Overview of Vulvar Cancer
Cancer of the vulva, the outer portion of the vagina, is a rare kind of cancer in women, accounting for 3 to 5% of all cancers of the female genital tract. Cancer of the vulva most often affects the inner edges of the labia majora or the labia minora. Less often, cancer occurs on the clitoris or in Bartholin’s glands (small mucus-producing glands at the opening of the vagina).
Most women with cancer of the vulva are over age 50. However, it is becoming more common in women under age 40.
Types of Vulvar Cancer:
- Squamous cell carcinoma of the vulva. Squmaous cell carcinoma
is the most common cell type. Squamous cell vulvar cancers usually grow
very slowly over many years.
- Melanoma of vulva (or vulvar melanoma). Although relatively
rare, this is the second most common type of vulvar cancer. About
4% of vulvar cancers are melanoma. The three patterns of vulvar melanoma
are mucosal lentiginous melanoma (MLM), superficial spreading melanoma
(SSM), and nodular Nelanoma (NM).
- Bartholin’s gland carcinoma. Bartholin's glands
are 2 small mucous-producing glands on either side of the vaginal
opening. The most common cell type of a cancerous tumor in this area
is adenocarcinoma but, rarely, adenosquamous, transitional cell, and
small cell tumors can originate in Bartholin’s gland. Adenoid cystic carcinoma of Bartholin’s
gland is a rare variant.
- Adenocarcinoma of the vulva. Adenocarcinoma arises
from glands in the vulvar region.
- Sarcoma of the vulva. This type of vulvar cancer accounts
for only 1 to 2% of all vulvar cancers. The majority of vulvar sarcomas
raise from the smooth muscle are known as leiomyosarcoma of the vulva.
Other types are rhabdomyosarcomas which also develop from muscle cells,
angiosarcomas which develop from blood vessels, neurofibrosarcomas
which develop from nerve cells and malignant schwannoma which develops
from the serve sheath, fibrosarcoma which develops in the connective
tissue, and epithelioid sarcoma which develops from cells in the skin.
Sarcomas tend to grow more quickly than other types of cancer.
- Basal cell carcinoma of the vulva. This type of vulvar
cancer invades adjacent tissues but rarely metastasizes. Generally
the tumors are small, 1-2 centimeters.
- Paget’s disease of the vulva. In this type of
vulvar cancer adenocarcinoma cells are found in the vulvar skin. Paget’s
disease can be confined to the vulvar skin or can be associated with an
underlying invasive adenocarcinoma. Approximately 25% of patients with
vulvar Paget’s disease also have an underlying invasive adenocarcinoma
of a Bartholin’s gland or sweat gland.
- Verrucous carcinoma of the vulva. This caluliflower-like tumor is a very slow-growing type of squamous cell vulvar cancer. It has a tendency toward quite extensive local invasion but without spread to the lymph nodes or distant organs.
Other types include:
- Lymphoma of the vulva (vulvar lymphoma) Endodermal
sinus tumor of the vulva (vulvar endodermal sinus tumor)
- Merkel cell cancer of the vulva (vulvar Merkel cell
tumor) – also known as small-cell poorly differentiated neuroendocrine
carcinoma of the vulva.
- Dermatofibrosarcoma protuberans of the vulva (vulvar
dermatofibrosarcoma protuberans)
- Malignant schwannoma of the vulva (vulvar malignant
schwannoma)
- Mucinous adenocarcinoma of the vulva (vulvar mucinous
adenocarcinoma)
- Malignant fibrous histiocytoma of the vulva (vulvar
malignant fibrous histiocytoma)
- Metastatic disease to the vulva (vulvar metastases) Metastases or spread to the vulva have been reported from tumors of the endometrium, ovary, cervix, breast, kidney, urethra, and lymphoma.
Stages of Vulvar Cancer:
Stage 0
Stage 0 vulvar cancer is also known as vulvar carcinoma in situ or vulvar intraepithelial neoplasia (VIN) or vulvar squamous intraepithelial lesion(SIL). It is a very early cancer found in the vulva only and is only in the surface of the skin.
Stage I
Stage I vulvar cancer is found only in the vulva and/or the space between the opening of the rectum and the vagina (perineum). The tumor is about 3/4 inch (2 cm) or less in size.
- Stage IA vulvar cancer is a tumor confined to the vulva or perineum. It is less than 3/4 inch (2 centimeters) in diameter, and invades no more than 1 millimeter into underlying tissue.
- Stage IB vulvar cancer is confined to the vulva or perineum. It is less than 3/4 inch (2 centimeters) in diameter, and there invades more than 1 millimeter into underlying tissue. The cancer has not spread to the lymph nodes.
Stage II
Stage II vulvar cancer is found in the vulva and the space between the opening of the rectum and the vagina (perineum), and the tumor is larger than 3/4 inch (2 centimeters) but has not spread to the lymph nodes.
Stage III
Stage III vulvar cancer may be of any size with spread to the to nearby tissues such as the vagina, the lower part of the urethra (the tube through which urine passes), the anus (the opening of the rectum), or has spread to nearby lymph nodes. It has not spread to distant sites.
Stage IV
Stage IV vulvar cancer has spread beyond the urethra, vagina, and anus into the lining of the bladder and the bowel; or, it may have spread to the lymph nodes in the pelvis of to other parts of the body. Stage IV vulvar cancer is also called metastatic vulvar cancer or vulvar cancer with lung metastases, vulvar cancer with liver metastases, vulvar cancer with bone metastases, etc.
- Stage IVA vulvar cancer may involve the upper urethra, the bladder mucosa, the rectal mucosa, or the pelvic bone, and/or there are bilateral groin lymph node metastases.
- Stage IVB vulvar cancer has spread to distant sites.
Recurrent
Recurrent vulvar cancer means that the cancer has recurred after it has been treated. If it recurs in the vulva it is a local recurrence; in the nearby lymph nodes, a regional recurrence; in sites such as the lungs or bones, a distant recurrence.
Grade of Vulvar Cancer:
Grade indicates how quickly or slowly cancer cells grow. The grade is determined by the appearance of the cells under the microscope. Tumors are graded on a scale of 1 to 3.
Grade 1 - cells look most like normal tissue (called well differentiated or low-grade).
Grade 2 - cells look somewhat like normal tissue (called moderately well differentiated or moderate grade).
Grade 3 - cells appear very abnormal (called poorly differentiated or undifferentiated or high-grade). They are likely to grow more quickly and more likely to spread.
