Vulvar cancer is a rare malignancy that occurs in the female genital tract. Vulvar cancer studies have shown links between the HPV virus and lichen sclerosus in women diagnosed with vulvar cancer. The majority of vulvar cancers are squamous cell carcinoma. Differentiated VIN is more likely to progress to squamous cell carcinoma versus HGSIL. In most cases, women present with a palpable mass in the vulva area, dysuria, and spotting. For treatment, IMRT tends to be the most successful treatment for local cancer of the vulva. The aim of this research is to present a case study and compare to typical presentation of vulvar cancer. In conclusion, vulvar cancers present in women with a history of HPV or lichen sclerosus.
Keywords: vulvar cancer, HPV, squamous cell carcinoma
Vulvar cancer is a rare malignancy that occurs in the female genital tract. This disease commonly occurs in women over the age of 70 years. Over the years, vulvar cancer has been linked to two specific factors in women. In this case study narrative, vulvar cancer presents with common signs and symptoms, histopathology, and similar treatment principles.
A recent clinic case presented a 70-year-old Caucasian woman with squamous cell carcinoma of the left labia minora of the vulva. The patient presented to the doctor with signs and symptoms of dysuria, minimal spotting, and “knot” inside the vagina. The mass was friable, but hard and excoriated. A PAP smear was performed, which showed significant HGSIL. HGSIL is a high grade squamous intraepithelial lesion associated with human papillomavirus (Khieu and Butler, 2018). The patient has a history of smoking for nearly 30 years. She is also married with two daughters. At the age of 25, the patient had a bilateral salphingoophorectomy and hysterectomy at age 63. It is not explained as to why a bilateral salphingoophorectomy was performed. The patient’s daughter was diagnosed with breast cancer five years ago but is now in remission. No other family members have been diagnosed with ovarian or breast cancer.
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The patient will receive 60 Gy in 30 fractions to gross vulvar disease. Immobilization will be uniquely fitted with vac-lock device formed to shape of patient’s body. Patient will be placed in supine position with legs in frog-leg position. MLC blocking devices will be designed to protect critical structures such as skin, bladder, bowel, and rectum. The radiation plan consists of vulvar boost 4-field box after treatment, consisting of 1,400 cGy daily in 7 fractions at 200 cGy. Before boost, the vulva will be treated in AP/PA field at 4,600 cGy in 23 fractions at 200 cGy.
Epidemiology and Etiology of Vulvar Cancer
Among the gynecologic diseases, vulvar cancer is the fourth most common malignancy of the female reproductive tract (Alkatout, et al., 2015). Majority of vulvar tumors are composed of squamous cell carcinomas that tend to grow slowly (Washington and Leaver, p. 748, 2016). Before progressing to squamous cell carcinoma, these cancers will be in a precancerous state called vulvar intraepithelial neoplasm (Washington and Leaver, p. 748, 2016). The most common sites for vulvar cancers occur in the labia minora and majora, but the tumors can also be found on the clitoris and perineum. In recent studies performed over the years, there are two possible links to vulvar cancer. The first possible link could be HPV, or human papillomavirus. This could be an infection that causes VIN (Alkatrout, et. al., 2015). The second possible link could be lichen sclerosis or vulvar inflammation. Due to the inflammation, this causes an itch-scratch cycle that can lead to squamous cell hyperplasia (Alkatrout, et al., 2015). Some other risk factors associated with vulvar cancer include multiple sexual partners, sexual intercourse starting at a young age, a low socioeconomic status, and previous infection to HPV (Washington and Leaver, p. 748, 2016). With the case study, the patient presents with a mass on the labia minora, history of HPV virus and smoking, which are linked to vulvar cancer.
Pertinent Anatomy and Patterns of Spread
The vulva is located on the outer part of the female genitals, which includes the opening of the vagina, the labia majora (outer lips), the labia minora (inner lips) and the clitoris. The vulvar lies in close contact with the vagina, rectum and bladder. Each of the organs can tolerate different amounts of radiation. The bladder and rectum’s maximum tolerance dose is 47 Gy. The vagina’s maximum tolerance dose is 60-70 Gy.
Vulvar cancers can spread in several different methods. The first method is by direct extension into surrounding organs. Another route of spread can be to the regional lymph nodes of hematogenous route. As the tumor continues to grow, the depth of lymph node involvement increases. Lymphatic node status is an important prognostic factor in vulvar cancer (Washington and Leaver, p. 748, 2016). With vulvar cancers, it tends to spread in order from inguinal lymph nodes to pelvic lymph nodes (Washington and Leaver, p. 748, 2016).
Detection and Diagnosis
Women with vulvar cancers normally present with a palpable mass on the vulva, painful urination, and vaginal bleeding (Washington and Leaver, p. 248, 2016). The most common site of cancer in the vulva is the labia majora. The first step in vulvar cancer would be a physical examination, such as a pelvic exam, which feels the uterus, ovaries, cervix and vagina for irregularities. It is possible the doctor will perform a PAP or HPV test. If a mass is detected, the doctor will perform a biopsy to see if the mass contains cancerous cells. To determine if the cancer has metastasized, a CT scan will be performed. It can be helpful in deciding whether a sentinel lymph node procedure should be done to check groin lymph nodes for cancer spread (American Cancer Society). SLN can be recommended for women in early stages of vulvar cancer, instead of an inguinofemoral lymphadenectomy (Alkatrout, et al., 2015). MRI can be beneficial in addition to CT scan to view enlarged lymph nodes or tumor growth in the area.
Surgery is the main treatment for vulvar cancer. Most early stage cancer can be treated with a wide local incision, where the only the cancer and an edge of healthy skin are cut out. According to the American Cancer Society, more extensive cancers might require more surgery, such as a vulvectomy, which could be part or all of vulvar to be removed (Cancer of the Vulva, n.d.). How much removed depends on how far the cancer has spread into nearby organs.
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Squamous cell carcinoma makes approximately 95% of malignant tumors involving the vulva (Alkatrout, et. al., 2015). This can be grouped into different types of vulvar squamous cell carcinoma: warty, basaloid, and keratinizing (Alkatrout, et. al.,2015). This most common subtype to occur is keratinizing, which occurs in postmenopausal women. The basaloid and wart tend to occur in premenopausal women associated with VIN. The second most common neoplasm of the vulvar is melanoma (Alkatrout, et al.,2015). These lesions tend to occur on the labia minora or the clitoris. There are many other histological subtypes, such as verrucous carcinoma, basal cell carcinoma, acantholytic squamous cell carcinoma, Bartholin’s gland cancer and Paget’s disease (Alkatrout, et al., 2015).
Vulvar cancer can be staged using the American Joint Committee on Cancer TNM staging system and the International Federation of Gynecology and Obstetrics FIGO staging system. Both of these systems stage vulvar cancer on the size of the tumor, lymph node involvement, and distant spread. (Alkatrout, et al., 2015). Lymphatic node status is the most important factor for diagnosing vulvar cancers (Washington and Leaver, p. 248, 2016).
Role of Radiation Therapy Treatment
In the early 2000s, the most common methods to treat vulvar cancers were with two dimensional or three-dimensional conformal radiotherapy with AP/PA photon beams. A central black would be used to spare the surrounding healthy organs (Sciacero, et al., 2015). Technology has now advanced to IMRT, which allow delivery of radiation in a more precise manner than conventional 2D-3D techniques. IMRT creates a dose falloff between the target and healthy tissues, which allows the normal tissues to be spared from damage (Sciacero, et al., 2015). Along with radiation therapy treatment, some studies used the chemotherapy drug, Cisplatin or even high dose brachytherapy with Iridium-192 (Rao, et al., 2017). HDR brachytherapy was performed three to four weeks after completion of IMRT treatment. The combined dose to the vulva was 7120 cGy (Rao, et al., 2017). Chemotherapy treatment was used to treat patients with locally advanced disease.
Simulation and Treatment Principles
Radiation therapy is expanding in the role of treatment for vulvar cancer. Preoperative radiation treatment is becoming a more common option with women diagnosed with advanced cancer (Alkatrout, et al., 2015). Tumor shrinkage and complete responses have been reported due to preoperative radiation (Alkatrout, et al., 2015). Patient with vulvar cancers are usually simulated in the supine position with their legs in the frog-leg position using a vac-lock immobilization device. This immobilization device ensures the patient is setup accurately for each treatment. In addition, using the frog-leg position prevents dose to the soft tissues of the thighs with an AP-PA field (Washington & Leaver, p. 248, 2016). A bolus can be used on the vulva to eliminate a cold spot. If the patient has had surgery, it is important to wire the scar and lesion, and positive nodes. When creating a radiation treatment plan for the vulvar, it is crucial to develop a wide anterior field to include all the pelvic nodes in the area, such as the inguinal lymph nodes (Washington and Leaver, p.248, 2016). The posterior field will be opposite using a narrow field to prevent dose to femoral heads (Washington and Leaver, p. 248, 2016). The total dose of radiation varies between 60-70 Gy. IMRT is the most recent radiation therapy being used due to decrease toxicity to surrounding healthy organs.
Assessment and management of treatment side effects
When treating patients with radiation therapy, side effects can occur. The most common side effects are fatigue, moist desquamation, sensitive and sore in the pelvic area. Chronic side effects could include lymphedema to the lower extremities and narrowing of the vagina (Cancer of the Vulva, n.d.)
In conclusion, vulvar cancer has a positive prognosis due to the continued research in different methods of treatment. Research should be continued since young women become sexually active at a young age. As a future radiation therapist, it provides insight to provide better treatment and quality of life for patients suffering from vulvar cancer.
- Alkatout, I., Günther, V., Schubert, M., Weigel, M., Garbrecht, N., Jonat, W., & Mundhenke, C. (2015). Vulvar cancer: Epidemiology, clinical presentation, and management options. International Journal of Womens Health,305. doi:10.2147/ijwh.s68979
- Cancer of the vulva. (n.d.). Retrieved from https://www.cancervic.org.au/cancer-information/types-of-cancer/vulvar-vaginal-cancers/vulvar_cancer.html
- Hinten, F., Molijn, A., Eckhardt, L., Massuger, L., Quint, W., Bult, P., . . . Hullu, J. D. (2018). Vulvar cancer: Two pathways with different localization and prognosis. Gynecologic Oncology,149(2), 310-317. doi:10.1016/j.ygyno.2018.03.003
- Khieu, M., & Butler, S. (2018). Cancer, Squamous Cell, High Grade Squamous Intraepithelial Lesion (HGSIL). National Center for Biotechnology Information. Retrieved from https://www.ncbi.nlm.nih.gov/books/NBK430728/.
- Rao, Y. J., Chundury, A., Schwarz, J. K., Hassanzadeh, C., Dewees, T., Mullen, D., . . . Grigsby, P. W. (2017). Intensity modulated radiation therapy for squamous cell carcinoma of the vulva: Treatment technique and outcomes. Advances in Radiation Oncology,2(2), 148-158. doi:10.1016/j.adro.2017.02.006
- Sciacero, P., Cante, D., Piva, C., Borca, V. C., Petrucci, E., Gastaldi, L., . . . Franco, P. (2016). The Role of Radiation Therapy in Vulvar Cancer: Review of the Current Literature. Tumori Journal,103(5), 422-429. doi:10.5301/tj.5000529
- Washington, C. M., & Leaver, D. T. (2016). Principles and practice of radiation therapy.
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