Vulva

​ In 2009, an estimated 3,580 women in the United States will be diagnosed with vulvar cancer. It is estimated that 900 deaths from this disease will occur this year. Vulvar cancer accounts for about 4% of cancers in female reproductive organs and 0.6% of all cancers in women. Its incidence is increasing in young women because of its association with the human papillomavirus (HPV). 2 Over 70% of vulvar malignancies arise in the labia majora and minora, 10% to 15% in the clitoris and 4% to 5% in the perineum and fourchette. The vestibule, bartholins gland and the clitoral prepuce are unusual primary sites, each accounting for less than 1% of vulvar cancers. Up to 20% of patients are asymptomatic. ⁵ || Age. The majority of women diagnosed with vulvar cancer are older than 50. However, a significant percentage, (15%) of women younger than 40 develop vulvar cancer. Generally, vulvar cancer in younger women is associated with HPV infection and smoking. Vulvar cancer in older women is most often associated with lichen sclerosus (a rare skin condition; see below) or changes in certain genes.  HPV infection.  Research indicates that infection with this virus is a risk factor for vulvar cancer. HPV is most commonly passed from person to person during sexual activity. There are different types, or strains, of HPV, and some strains are more strongly associated with certain types of cancers. HPV may be responsible for about one-third to two-thirds of vulvar cancers. Many types of cancer caused by HPV are associated with precancerous conditions (changes in cells that may, but do not always, become cancer) that develop before the cancer. Smoking. Smoking may increase a woman’s risk of developing vulvar cancer if she has HPV. Immune system deficiency. Women with lowered immune systems have a higher risk of developing vulvar cancer. A lowered immune system can be caused by immune suppression from corticosteroid medications, organ transplantation, treatment for other types of cancer, or human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS). When a woman has a lowered immune system, her body is more likely to develop infections, including HPV. Lichen sclerosus**.** This condition affects the vulvar skin, making it thin and itchy. About 4% of women with lichen sclerosus develop vulvar cancer. Precancerous conditions. Precancerous conditions of the vulva, cervix, or vagina, or melanoma elsewhere on the body, can increase a woman’s risk of developing vulvar cancer 2 || Special studies: exfoliative cytology of cervix and vagina colposcopy and directed biopsies cytoscopy proctosigmoidoscopy Radiographic studies: chest radiographs intravenous pyelogram barium enema lymphangiogram CT or MRI of pelvis and abdomen Lab Studies: CBC blood chemistry urinalysis 3
 * Epidemiology: || Survival is most dependent on the pathologic status of the inguinal nodes. In patients with operable disease without nodal involvement, the overall survival (OS) rate is 90%; however, in patients with nodal involvement, the 5-year OS rate is approximately 50% to 60%. Risk factors for node metastasis are clinical node status, age, degree of differentiation, tumor stage, tumor thickness, depth of invasion, and presence of capillary-lymphatic space invasion. Overall, about 30% of patients with operable disease have nodal spread. A multi-factorial analysis of risk factors in squamous vulvar cancer demonstrated that nodal status and primary lesion diameter, when considered together, were the only variables associated with prognosis. Patients with negative inguinal nodes and lesions no more than 2 cm had a 98% 5-year survival rate, while those with any size lesion with three or more unilateral nodes or two or more bilateral nodes had a 29% 5-year survival rate. 1
 * Etiology: || The following factors may raise a woman’s risk of developing vulvar cancer:
 * Signs & Symptoms: || Women with vulvar cancer may experience the following symptoms. Sometimes, women with vulvar cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your doctor.
 * A lump or growth in or on the vulvar area
 * A patch of skin that is differently textured or colored than the rest of the vulvar area
 * Persistent itching, pain, soreness, or burning in the vulvar area
 * Painful urination
 * Bleeding or discharge that is not menstrual blood
 * An ulcer that persists for more than one month
 * A change in the appearance of an existing mole (specific to vulvar melanoma)
 * Wart-like growths (similar to genital warts) 2 ||
 * Diagnostic Procedures: || General history and physical exam

Clinical history and a complete physical evaluation. Assessment of the vulvar, anal, and perineal areas, as well as a thorough inspection of the vagina and cervix. A pap smear of the cervix and vagina. Biannual pelvic examination. Assessment of the regional lymph nodes. Note that there is a substantial margin of error in vulvar lymph node assessment. Chest radiographs. CT or MRI may aid in determining tumor extent and evaluating pelvic and periaortic lymph nodes. Other studies include cystoscopy, proctosigmoidoscopy, barium enema, and intravenous pyelogram when indicated. 4 || Paget's disease is associated with invasive aprocine carcinoma in approximately 20-30% of cases. Squamous cell carcinoma comprises over 90% of invasive lesions of the vulva. Most are well differentiated with keratin formation, and 5-10% are anapestic. Two variants of squamous cell carcinoma that are not found frequently are adenosquamous and basiloid carcinoma. These tumors are usually expohytic and well differentiated but and rarely metastasize. Verrucous carcinoma is extremely rare and has a very low incidence of lymph node metastasis. Basal cell carcinoma is occasionally reported. Adenoid cystic carcinoma of the Bartholin's gland is about 0.1% of all vulvar malignancies. Most Adenocarcinomas originate from the Barholin's gland or from bulboadnexal structures associated with Paget's disease. Some may originate from the periurethral Skene's gland. Bartholin's gland carcinoma occasionally may be squamous cell when it originates near the orifice of the duct, papillary if it arises from the transitional epithelium of the duct, or adenocarcinoma when it arises from the gland itself. Melanoma accounts for 2-9& of vulvar malignancies. Nodular and superficial spreading are two varieties that are described. Vulvar sarcomas are extremely rare with leiomyosarcoma being the most common. Other varieties include neurofibrosarcoma, rhabdomyosarcoma, fibrosarcoma, and angiosarcoma. Metastatic carcinomas to the vulva can come from the uterine cervix, the endometrium, the ovary, the urethra, to the vagina. 4 || Lymph from the glans clitoris drains to the inguinal nodes and the deep femoral nodes. Some lymphatics from the clitoris may enter the pelvis directly, connecting with the obturator and external iliac nodes and bypassing the femoral area. 4 ||
 * Histology: || Carcinoma in situ is a pre-invasive form of vulvar malignancy.
 * Lymph Node Drainage: || The lymph node drainage from the labia, fourchette, perineum, and prepuce go into the superficial inguinal and femoral lymph nodes. They penetrate the cribiform fascia and reach the deep femoral nodes. From these, lymph drains into the external and common iliac lymph nodes.
 * Metastatic Spread: || The most common metastatic sites are lung, liver and bone. 5 ||
 * Grading: || **GX:** The tumor grade cannot be evaluated.
 * G1:** The tumor cells are well differentiated (contains 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). 6 ||
 * Staging: || Staging systems are International Federation of Gynecology and Obstetrics or American Joint Committee on Cancer. 5

|| Moist Desquamation: Dose is usually 30-40Gy, time frame is around 3 to 4 weeks from beginning of treatment. 8 || Survival of patients after being treated for vulva cancer. 9 || Superior:L4-L5 (includes upper portion of common iliac chain) Inferior: includes entire vagina and pelvic lymph nodes Lateral: 1-2cm past femoral heads(includes inguinal and adjacent femoral lymph nodes) Boost: 5-10 Gy given to positive inguinal lymph nodes for a total of 70-75Gy. 8
 * Radiation Side Effects: || Radiation side effects of the vulva include moist desquamation. Late radiation side effects of the vulva include skin changes of atrophy and telangiectasis. 7
 * Prognosis: || The most important prognostic factor is lymph node metastasis. 8
 * Treatments: || Vulva Treatment Borders: __AP/PA__ (Usual dose is 1.6-1.8 daily to 60-65Gy using 4-6MV in frog-leg position with anterior with bolus)

Treatment decisions for vulvar carcinoma. 9 || 2 || **__ References __** 1. [|www.cancer.gov/cancertopics/pdq/treatment/vulvar.com] Accessed Febuar 2, 2010 2. [|www.cancer.net/patient/cancer+types/vulvar.com] Accessed Febuary 2, 2010 3. Perez CA, Brady LW, eds. //Principals and Practice of Radiation Oncology//, 3rd ed. Philadelphia: Lippincott-Raven. 1998. 4. Chao KS, Perez CA., Brady LW. //Radiation Oncology - Management Decisions//. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002. 5. Chao KS, Perez CA., Brady LW. //Radiation Oncology - Management Decisions//. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002 6 . AJCC Cancer Staging Manual, Sixth Edition (2002) // published by Springer-Verlag New York, [|//www.cancerstaging.net//]////.// Accessed February 1, 2010, Table 1&2 7. Gunderson and Tepper. Clinical Radiation Oncology. 2nd Edition. Philadelphia, PA: Churchill Livingstone.2007. 8. Chao KS, Perez CA., Brady LW. //Radiation Oncology - Management Decisions//. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002. 9. Philip Rubin, //Clinical Oncology - A Multidisciplinary Approach for Physicians and Students//. 7th edition. Philadelphia, PA: W.B. Saunders Company. 1993. 10. Hand CM, Kim SJW, Waldow SM. Overview of radiobiology. In: Washington CM, Leaver D, eds. //Principles and Practice of Radiation Therapy//. 2nd ed. St. Louis, MO: Mosby; 2004:80-81. 11. Treatment plans and treatment planning photos courtesy of the University of Colorado Hospital, 2010.
 * TD5/5: || According to Washington and Leaver, et. al. the main organs of interest in the treatment fields for vaginal would be rectum, bladder and urethra and possibly intestine. The TD5/5 for rectal tissue is 6000 cGy which could result in ulceration or stricture. The tolerance dose for small bowel is much lower at 4500 cGy. Intestine exposed to 45 Gy or higher could result in sequelae of ulcer, perforation of hemorrhage. Bladder and urethra TD5/5 is 6000 cGy which could result in contracture or strictures. 10 ||
 * Treatment Plans and Fields || Treatment plan information and initial treatment fields used on a patient in 2009 at the University of Colorado Hospital. Note how the inferior borders of the treatment fields are well below the ischial tuberosities in order to flash over any external disease and the perineum. 11