Cancer that forms on the outer surface of the vulva is called vulvar cancer. It is a rare condition — only about 6,400 new cases are diagnosed in the United States each year.

What is vulvar cancer?

Vulvar cancer occurs when abnormal cells form and grow in the vulvar tissues. These tissues are part of the vulva, which includes several parts of female genitalia:

• 阴道内外唇(大阴唇和小阴唇)
• Clitoris
• 阴道开口周围的组织
• 尿道(这是尿出来的地方)

Types of Vulvar Cancer

外阴癌最常见于大阴唇和小阴唇. Most vulva cancers are squamous cell carcinomas, a type of cancer that starts in the skin cells. 外阴鳞状细胞癌形成时呈薄状, 阴道表面的扁平细胞发生变化(突变). 突变的细胞可以迅速繁殖.

以下是其他不太常见的外阴癌类型.

• Melanoma: The second-most common type of vulvar cancer typically presents as a pigmented lesion on the vulva. 无色素病变也可发生.
• Basal cell carcinoma: 这占外阴癌的2%到7%.
• Bartholin腺癌: 高达5%的外阴癌起源于Bartholin腺, 哪个是阴道开口里面的一个小腺体.
• Sarcoma: 这占外阴癌的1%到2%. 这些软组织肉瘤包括平滑肌肉瘤, rhabdomyosarcomas, liposarcomas, angiosarcomas, neurofibrosarcomas, 上皮样肉瘤和未分化/未分类肉瘤.
• 外阴佩吉特病: 这占外阴癌的不到1%.

外阴癌的成因及危险因素

Most cases of vulvar squamous cell carcinoma result from two common risk factors — 人乳头瘤病毒(HPV) 慢性炎症或自身免疫过程.

• Human papillomavirus: About half of vulvar squamous cell carcinoma cases are caused by exposure to HPV, which is the most common sexually transmitted infection (STI) in the United States — 43 million people are infected each year. 大多数感染这种病毒的人在20岁出头时就接触到了这种病毒, 但癌症可能在暴露多年后发展. There is a safe and effective vaccine to reduce the risk of cancer from HPV. HPV疫苗适用于9至45岁的人群, 但最好在接近9岁时接种疫苗. HPV感染可导致癌前病变和癌性外阴病变.
• 慢性炎症或自身免疫过程: Skin conditions such as lichen sclerosus can lead to development of precancerous and cancerous vulvar lesions that are unrelated to HPV infection.

Other risk factors can also increase a person’s chance of developing vulvar cancer, including:

• Age: 大约80%的外阴癌是在50岁以上的女性中诊断出来的. 
• HIV infection: 人类免疫缺陷病毒(HIV)会导致艾滋病，破坏免疫系统. This increases the risk of getting HPV and may also affect the body’s ability to find and destroy cancer cells before they spread.
• Lichen sclerosus: This condition causes skin around the vulva to become irritated, thin and itchy. Only about 4% of women with this condition eventually develop vulvar cancer.
• Melanoma: A family history of melanoma 或几个不典型痣增加外阴癌的风险.
• Other genital cancers: 在生殖器有其他癌症, 最常见的子宫颈癌, 会增加患外阴癌的机会吗.
• Smoking: 吸烟会增加患外阴癌的风险. 感染HPV的人吸烟的风险更大.

Having any risk factor — or even more than one — does not mean you will develop vulvar cancer, 但这会增加患外阴癌的可能性. 这就是为什么定期检查和盆腔检查很重要. 你的医生可以在这些检查中寻找外阴癌的早期迹象.

Vulvar Cancer Symptoms

The signs of vulvar cancer vary depending on the type of cancer and where it starts. 最常见的外阴癌, squamous cell carcinoma, 通常有明显的症状, including:

• 数周或数月不愈合的开放性伤口
• 月经来潮不是正常月经的一部分的出血或分泌物
• 长得像花椰菜或生殖器疣
• 痒或灼烧感
• 表面粗糙的明显肿块或凸起
• 阴道外侧疼痛
• 变色(皮肤比周围的皮肤更浅或更深), 或者看起来比平时更红或更粉)

Symptoms of Bartholin gland carcinoma may be different than symptoms of other types of vulvar cancer. 你可能会注意到阴道开口附近有一个肿块. 然而，这个肿块可能是囊肿，而不是癌变.

Vulvar Cancer Diagnosis

The best way to diagnose vulvar cancer is by having regular wellness exams with a primary care doctor or a gynecologist. During these exams, the doctor will look for common signs and symptoms of vulvar cancer and may order tests if something looks suspicious.

• Colposcopy: During a colposcopy, a magnifying device is used to get a better look at the skin cells around the vulva to spot signs of cancer. 如果出现可疑区域，将进行活组织检查.
• Biopsy: The vulva area is numbed and a small sample of tissue is removed and sent to a lab, 病理学家在哪里确定癌症的存在. 活检是确诊外阴癌的必要条件.
• Imaging: Imaging tests may be ordered if your doctor thinks your vulvar cancer may have spread to other areas of the body. Tests include CT scans, MRI or 正电子发射断层扫描(PET).

Vulvar Cancer Stages

Doctors use cancer staging to describe whether and how far cancer has spread. 这可以帮助他们制定外阴癌的最佳治疗方案.

• Stage 1: 癌症局限于外阴.
• Stage 2: 癌症已经扩散到阴道下部, 尿道(连接膀胱的小管道)或肛门.
• Stage 3: Cancer has spread to the upper urethra, vagina, bladder or rectal mucosa, or to nearby lymph nodes.
• Stage 4: Advanced stage vulvar cancer is fixed to bone and has ulcerated lymph nodes or distant metastases. It may have also spread to distant lymph nodes or other parts of the body.

Vulvar Cancer Treatment

以下是外阴癌最常见的治疗方法.

• Surgery: 这样做是为了从外阴切除病变. 它可以包括一个简单的, 外阴切除术大面积局部切除或切除外阴的大部分, 取决于病变的位置和大小. The surgeon also takes out some healthy cells around the cancer to help be sure that all of the cancerous cells are removed.
• Lymph node biopsy: 确定癌症是否已经扩散到淋巴结是很重要的. 对于非常早期的外阴癌, 不需要切除淋巴结, but for any tumor that is bigger than 2 centimeters or that has a depth of invasion greater than 1 millimeter, surgical assessment of lymph nodes either through sentinel lymph node biopsy or complete lymphadenectomy is required.
• Radiation therapy: High-powered energy beams are directed at the tumor to kill cancer cells or shrink a tumor before surgery.
• Chemotherapy: Medication kills cancer cells that have spread to other areas of the body. Your oncologist may recommend chemotherapy alone or in combination with radiation and other treatments.
• Immunotherapy: Drugs activate your body’s natural immune system to find and destroy cancer cells.

Vulvar Cancer Prognosis

The prognosis for vulvar cancer varies, depending on how far cancer has spread. 外阴癌的五年存活率为:

• 86% when cancer is only in the vulva and it has not spread to lymph nodes or local (nearby) tissues
• 53% when cancer cells have spread to nearby lymph nodes, but not to other parts of the body
• 19% when the cancer has spread (metastasized) to distant organs or lymph nodes