 Introduction/Background Vulvar cancer is a rare gynecologic malignancy. In the United States, it is estimated that approximately 6,120 women will present with vulvar cancer, and 1,350 will succumb to their disease in 2020 [1]. Most patients are diagnosed with early-stage disease, and the majority of tumors originate in the labia majora [2,3]. The 5-year survival rate is 86% for patients with vulvar-confined disease but is reduced to 57% for patients with regional lymph node metastases, and 17% for patients with distant metastases [4]. Squamous cell carcinoma (SCC) is the predominant histotype of vulvar cancer, accounting for 90% of cases; thus, it is the focus of this discussion. Up to 69% of vulvar cancer is attributed to chronic human papillomavirus (HPV) infection, in particular high-risk strains such as HPV-16 and HPV-18 [5]. Other risk factors include older age, tobacco use, chronic inflammation of the vulva, and immune-compromised state [6]. The International Federation of Obstetrics and Gynecology (FIGO) and The American Joint Committee on Cancer (AJCC) TNM systems are both used to stage vulvar cancer and are closely aligned [7-9]. Initial evaluation of patients with vulvar lesions consists of careful clinical examination and punch biopsies of all suspicious vulvar lesions. Care must be taken to include the underlying stroma and to avoid necrotic areas. Lesion size, location relative to the midline, relationship to the adjacent organs (urethra, vagina, anus), and presence of multifocal disease are noted on physical examination. Clinical palpation of groin lymph nodes is performed, although this approach is limited by the high false-negative rate [10]. The status of inguinofemoral lymph nodes (IFLNs) is the most important prognostic factor in vulvar cancer. The likelihood of lymph node metastases is estimated by primary tumor size, depth of stromal invasion, and presence of lymphovascular space invasion [11-15].
Staging and Follow up of Vulvar Cancer.  Introduction/Background Vulvar cancer is a rare gynecologic malignancy. In the United States, it is estimated that approximately 6,120 women will present with vulvar cancer, and 1,350 will succumb to their disease in 2020 [1]. Most patients are diagnosed with early-stage disease, and the majority of tumors originate in the labia majora [2,3]. The 5-year survival rate is 86% for patients with vulvar-confined disease but is reduced to 57% for patients with regional lymph node metastases, and 17% for patients with distant metastases [4]. Squamous cell carcinoma (SCC) is the predominant histotype of vulvar cancer, accounting for 90% of cases; thus, it is the focus of this discussion. Up to 69% of vulvar cancer is attributed to chronic human papillomavirus (HPV) infection, in particular high-risk strains such as HPV-16 and HPV-18 [5]. Other risk factors include older age, tobacco use, chronic inflammation of the vulva, and immune-compromised state [6]. The International Federation of Obstetrics and Gynecology (FIGO) and The American Joint Committee on Cancer (AJCC) TNM systems are both used to stage vulvar cancer and are closely aligned [7-9]. Initial evaluation of patients with vulvar lesions consists of careful clinical examination and punch biopsies of all suspicious vulvar lesions. Care must be taken to include the underlying stroma and to avoid necrotic areas. Lesion size, location relative to the midline, relationship to the adjacent organs (urethra, vagina, anus), and presence of multifocal disease are noted on physical examination. Clinical palpation of groin lymph nodes is performed, although this approach is limited by the high false-negative rate [10]. The status of inguinofemoral lymph nodes (IFLNs) is the most important prognostic factor in vulvar cancer. The likelihood of lymph node metastases is estimated by primary tumor size, depth of stromal invasion, and presence of lymphovascular space invasion [11-15].