Vulvar Carcinoma: Diagnosis, Staging, and Treatment
Vulvar cancer accounts for only 5% of all gynecologic cancers with an incidence rate of 2.4 per 100,000 women. The most common histological type of vulvar cancer is vulvar squamous cell carcinoma (VSCC). There are two different types of VSCC with their ow
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Anne-Floor W. Pouwer, Nienke C. te Grootenhuis, Maaike H. M. Oonk, and Joanne A. de Hullu
58.1 Introduction Vulvar cancer is the fourth most common gynecologic cancer after endometrial, ovarian, and cervical cancer. It accounts for only 5% of all gynecologic cancers with an incidence rate of 2.4 per 100,000 women [1]. The most common histological type of vulvar cancer is vulvar squamous cell carcinoma (VSCC), which accounts for around 80% of all cases. Other histological types of vulvar cancer are basal cell carcinoma, melanoma, adenocarcinoma, and extremely rare types such as sarcoma and lymphoma [2]. VSCC mostly affects elderly women, with more than half of the patients aged above 70 years at the time of diagnosis [3]. Over the past few decades, the incidence of VSCC increased both in the younger and elderly population. This increase of incidence rate in younger patients is probably related to human papillomavirus (HPV), while the increase in the elderly population is due to aging.
A.-F. W. Pouwer · J. A. de Hullu (*) Department of Obstetrics and Gynaecology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands e-mail: [email protected]; [email protected] N. C. te Grootenhuis · M. H. M. Oonk Academic Medical Centre Groningen, UMCG Groningen, Groningen, The Netherlands e-mail: [email protected]; [email protected]
In this chapter, we will discuss the pathogenesis, diagnosis, staging, and treatment of VSCC.
58.2 Pathogenesis There are two different types of VSCC with their own associated premalignant lesions (Fig. 58.1). The most common type occurs in elderly women and leads to mostly differentiated keratinizing VSCC, in a background of lichen sclerosus (LS) and often differentiated vulvar intraepithelial neoplasia (dVIN) [4] (Fig. 58.2). dVIN is underreported, has a relatively brief intraepithelial phase before progression to invasive carcinoma, and is a difficult clinical and histological diagnosis. The second type of VSCC consists of mainly nonkeratinizing carcinomas, primarily affecting younger women. This type of VSCC is caused by an infection with high-risk HPV, predominantly HPV 16 and 18. These HPV-associated high- grade squamous intraepithelial lesions (HSIL) are seen adjacent to approximately 30% of the VSCC [5].
58.3 Clinical Presentation The majority of VSCC are diagnosed in the seventh decade [3]. Patients’ and doctors’ delay in diagnosis is frequent. Most patients with VSCC present with a vulvar mass, although there is often a long history of pruritus or discomfort.
© Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_58
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394 LS or ? dVIN
Keratinising VSCC
HSIL
Nonkeratinising VSCC
Normal vulva
HPV
Fig. 58.1 A drawing of the pathogenesis of VSCC. LS lichen sclerosus, ? unknown, HPV human papillomavirus, dVIN differentiated vulvar intraepithelial neoplasia, HSIL
high-grade squamous intraepithelial lesion, VS
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