Vulva

-A Papanicolaou (Pap) smear of the cervix and vagina should also be examined. - Examination of the bimanual pelvic is mandatory. [1] - Radiographic studies include: Chest X-ray, intravenous pyelogram, barium enema, lymphangiogram, CT or MRI scans, and PET-CT Scan. [1] - Other studies include: cystoscopy, proctosigmoidoscopy, exfoliative cytology, colposcopy, and Schiller’s test. [1] (Table 39-1) -Laboratory Studies include: Complete blood cell count, Blood chemistry, and urinalysis. (Table 39-1) || Most cancers are squamous cell carcinomas (SCC). Verrucous carcinoma a slow-growing subtype of SCC looks like a large wart. A biopsy is needed to determine if it is benign or not growth. About 8% of vulvar cancers Most often start in the Bartholin glands. Can also form in the sweat glands of the vulvar skin. Vulvar Paget disease of the vulva is a condition in which adenocarcinoma cells are found in the top layer of the vulvar skin. Up to 25% these patients have an invasive vulvar adenocarcinoma (in a Bartholin gland or sweat gland). Occurs in females at any age, including in childhood. Basal cell carcinoma more often found on sun-exposed areas of the skin occurs very rarely on the vulva.[1] || Drain to the superficial inguinal and femoral nodes, then anterior to the cribriform plate and fascia lata. Drainage penetrates the cribriform facia to the deep femoral nodes, following the path to the external and common iliac nodes in the pelvis. *Note the most superior deep femoral nodes in known as “Cloquet’s node”[1] Typically the glans clitoris drains directly into the inguinal and deep femoral nodes. However, some drainage may bypass the superficial femoral nodes and enter directly into the pelvis and connect with the obturator and external iliac nodes.[1] || G1: The tumor cells are well differentiated (contain many healthy-looking cells). G2: The tumor cells are moderately differentiated (more cells appear abnormal than healthy). G3: The tumor cells are poorly differentiated (most of the cells appear abnormal). G4: The tumor cells are undifferentiated (the cells barely resemble healthy cells). [3] || T0: There is no tumor. Tis: The tumor is carcinoma in situ, an early cancer on the surface of the skin that has not spread to nearby tissue. T1: The tumor is only in the vulva, or the vulva and perineum (the area of skin between the anus and vagina), and is 2 centimeters (cm) or smaller. T1a: The tumor is only in the vulva, or the vulva and perineum, is 2 cm or smaller, and has spread no more than 1 millimeter (mm) into nearby structures. T1b: The tumor is only in the vulva, or the vulva and perineum, is 2 cm or smaller, and has spread more than 1 mm into nearby structures. T2: The tumor is only in the vulva, or the vulva and perineum, and is larger than 2 cm. T3: The tumor, of any size, has spread to the lower urethra and/or the vagina or anus. T4: The tumor has spread to any of the following: upper urethra, bladder mucosa, rectal mucosa, or is attached to the pubic bone.
 * **Epidemiolgy:** || Vulvar cancers most commonly occur in older women above the age of 70 years and accounts for 3% to 5% of all gynecological cancers. It rarely occurs in women under the age of 40. Vulvar cancers associated with HPV infection occur in women who are younger and are often smokers. [1] ||
 * **Etiology:** || The cause of vulvar cancer is unknown but risk factors may include a history of condyloma acuminatum, vulvar intraepithelial neoplasia, smoking and chronic vulvar dystrophies. [2] ||
 * **Signs & Symptoms:** || A mass in the vulva is the most common complaint of patients with vulvar carcinoma. Itching, bleeding, pain or tenderness may also be symptoms of vulvar carcinoma. Women with more advanced disease present with local pain, bleeding and surface drainage from the tumor. [2] ||
 * **Diagnostic Procedures:** || -Complete physical examination of the vulvar and anal area, perineum, vagina, and cervix is crucial. [1]
 * **Histology:** || Types of vulvar cancer[2]
 * Squamous cell carcinomas ** –
 * Adenocarcinoma ** –
 * Melanoma ** - 5%-8% of melanomas in women occur on the vulva usually the labia minora and clitoris.
 * Sarcoma ** – < 2% are sarcomas.
 * **Lymph node drainage:** || Lymph node metastasis is the single most important prognostic factor in vulva cancer. The different anatomical areas of the vulva drain to the same lymphatic paths except for the glans clitoris.
 * Labia, Fourchette, Perineum and Prepuce **
 * Glans Clitoris **
 * **Metastatic spread:** || Most common sites of metastatic spread are lung, liver and bone. [2] ||
 * **Grading:** || GX: The tumor grade cannot be evaluated.
 * **Staging:** || TX: The primary tumor cannot be evaluated.

NX: The regional lymph nodes cannot be evaluated. N0: Cancer has not spread to the lymph nodes. N1: Cancer has spread to lymph nodes on the same side of the body as the tumor. N2: Cancer has spread to lymph nodes on both sides of the body.

MX: Distant metastasis cannot be evaluated. M0: There is no distant metastasis. M1: There is metastasis to other parts of the body.

Stage 0: Carcinoma in situ. Stage I: The tumor is smaller than 2 cm and has not spread (T1, N0, M0). Stage IA: The tumor is smaller than 2 cm, has not spread, and is no deeper than 1 mm (T1a, N0, M0). Stage IB: The tumor is smaller than 2 cm, has not spread, and is deeper than 1mm (T1b, N0, M0). State II: The tumor is larger than 2 cm, is in the vulva or perineum or both, but has not spread to nearby tissue (T2, N0, M0). Stage III: The cancer has spread to nearby tissue (vagina, anus, urethra) and/or lymph nodes on one side of the body, but there is no distant metastasis (T1 or T2, N1, M0; T3, N0 or N1, M0). Stage IVA: The cancer has spread to lymph nodes on both sides of the body or spread into the upper part of the urethra, bladder, rectum, or pelvic bone (T1, T2, T3; N2, M0; or T4, any N, M0). Stage IVB: Any cancer that has spread to a distant part of the body (Any T, any N, M1). [2,3] ||
 * **Radiation side effects:** || Fatigue, skin reddening, wound infection or necrosis, burning with urination due to skin reaction or infection, or leg edema. [2] ||
 * **Prognosis:** || ===== Vulva cancer has an overall five-year survival rate of 75%. The prognosis depends on age, general health, stage and type of tumor. However, the five year survival rate decreases to 20% if the pelvic lymph nodes are involved. [4] ===== ||
 * **Treatments:** || ===== Surgery is the main treatment of choice for vulva cancer. The vulvar tissues and lymph nodes are removed by a procedure known as radical vulvectomy. Both radiation and chemotherapy treatments are used pre-op and post-op as well as for advanced vulvar cancer. =====

** Radiation Therapy **
Either a four field three-dimensional approach or intensity modulated radiation therapy can be used. The treatment field borders of vulva cancer includes L5/S1 superiorly and 3cm below the bottom of the ischium including the entire vulva inferiorly. The lateral field border is 2cms beyond the pelvic brim. A bolus should be used on the groin and vulva. The dose to the vulva and pelvic lymph nodes is 45-50.4Gy. For residual disease, it may boost to 76-70Gy. [2] || Small Bowel <45Gy Femoral Heads <42Gy Bladder <60Gy Rectum <60Gy Lower Vagina <75-80Gy || [2] Chao C. //Radiation Oncology Management Decisions//. 2nd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2002. [3] [] Last updated March 28, 2012. Accessed June 18, 2012.
 * **TD 5/5:** || ** Normal Tissue Tolerances (Whole Organ) ** [4]
 * **References:** || [1] Vulvar Cancer. American Cancer Society. http://www.cancer.org/Cancer/VulvarCancer/DetailedGuide/vulvar-cancer-what-causes. Accessed June 18, 2012.

[4] Hoppe TR, Phillips LT, Roach M. //Leibel and Phillips Textbook of Radiation Oncology//. 3rd Ed. Philadelphia, PA: Saunders, Elsevier; 2010. || Figure 1. Anterior simulation setup film. [4]

Figure 2. Posterior simulation setup film. [4]

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