Chi_Principles and Practice of Gynecologic Oncology 8e

16 SECTION 2 ■ Vulvar Cancer

project for HPV-associated lesions: background and consensus recommen dations from the college of American pathologists and the American society for colposcopy and cervical pathology. Int J Gynecol Pathol . 2013;32:76-115. 4. Bornstein J, Bogliatto F, Haefner HK, et al. The 2015 International So ciety for the Study of Vulvovaginal Disease (ISSVD) terminology of vulvar squamous intraepithelial lesions. J Low Genit Tract Dis . 2016;20:11-14. 5. Fergus KB, Lee AW, Baradaran N, et al. Pathophysiology, clinical man ifestations, and treatment of lichen sclerosus: a systematic review. Urology . 2020;135:11-19. 6. Krapf JM, Mitchell L, Holton MA, Goldstein AT. Vulvar lichen sclero sus: current perspectives. Int J Womens Health . 2020;12:11-20. 7. Woelber L, Bommert M, Prieske K, et al. Pelvic lymphadenec tomy in vulvar cancer—does it make sense? Geburtshilfe Frauenheilkd . 2020;80(12):1221-1228. 8. Höhn AK, Brambs CE, Hiller GGR, et al. 2020 WHO classification of female genital tumors. Geburtshilfe Frauenheilkd . 2021;81(10):1145-1153. 9. Heller DS, Day T, Allbritton JI, et al. Diagnostic criteria for differenti ated vulvar intraepithelial neoplasia and vulvar aberrant maturation. J Low Genit Tract Dis . 2021;25(1):57-70.

10. Hinten F, Molijn A, Eckhardt L, et al. Vulvar cancer: two pathways with different localization and prognosis. Gynecol Oncol . 2018;149(2):310-317. 11. Eva LJ, Sadler L, Fong KL, Sahota S, Jones RW, Bigby SM. Trends in HPV-dependent and HPV-independent vulvar cancers: the changing face of vulvar squamous cell carcinoma. Gynecol Oncol . 2020;157(2): 450-455. 12. Eva LJ, Ganesan R, Chan KK, et al. Differentiated-type vulval intraep ithelial neoplasia has a high-risk association with vulval squamous cell car cinoma. Int J Gynecol Cancer . 2009;19:741-744. 13. McAlpine JN, Kim SY, Akbari A, et al. HPV-independent differenti ated vulvar intraepithelial neoplasia (dVIN) is associated with an aggressive clinical course. Int J Gynecol Pathol . 2017;36:507-516. 14. McAlpine JN, Leung SCY, Cheng A, et al. Human papillomavirus (HPV)-independent vulvar squamous cell carcinoma has a worse prognosis than HPV-associated disease: a retrospective cohort study. Histopathology . 2017;71:238-246. 15. Horne ZD, Dohopolski MJ, Pradhan D, et al. Human papillomavirus infection mediates response and outcome of vulvar squamous cell carcino mas treated with radiation therapy. Gynecol Oncol . 2018;151:96-101.

2.5 c h a p t e r

Vulvar Cancer: Clinical Presentation, Diagnostic Evaluation, and Workup

Willemijn L. van der Kolk and M. H. M. Oonk

measurements of the primary tumor, assessment for extension to adjacent mucosal or bony structures, and palpation of the inguinal LNs. It is helpful to record the distance from vital structures such as the clitoris, urethral meatus, and anus, because these structures limit the ability to obtain adequate surgical margins. Because vul var cancers may be related to HPV, clinical examination of the va gina, cervix, and anus should also be performed as HPV may be multifocal. If no recent cervical Papanicolaou (Pap) smear has been taken, this should be performed as well (5). Patients with large or fixed tumors, and those who are difficult to examine in the clinic, may benefit from an exam under anesthesia, with cystourethros copy and proctosigmoidoscopy for tumors near the urethra or anus, respectively. Diagnosis must always be confirmed by taking a punch biopsy under local anesthesia. Selecting the most appropriate site for a biopsy can be difficult, because of coexisting premalignancies or precursors. In such cases, multiple biopsies are often required. Be cause VIN can present in different forms and can be multifocal, any new lesion should be biopsied. If a nonbiopsied or noninvasive vul var lesion does not completely resolve or is refractory to therapy, it should be biopsied to obtain a definitive diagnosis. Tissue biopsies should include the cutaneous lesion in question and representa tive contiguous underlying stroma, so that the presence and DOI can be accurately assessed. Because DOI is a central issue in the management of vulvar cancer, punch biopsies are encouraged and shave biopsies are generally discouraged. If invasion is suspected and a punch biopsy fails to confirm the clinical suspicion, then an incisional or excisional biopsy should be performed. However, complete excisional biopsies should be avoided if possible, because it may complicate a subsequent SN procedure. When the diagnosis of vulvar SCC is confirmed, further diag nostic imaging depends on the extent of disease. In patients with microinvasive vulvar SCC, no imaging is required, because chances of LN metastasis are close to zero (6). In patients with unifocal

C linical P resentation Women with VIN may present with pruritus, burning sensation, or dysuria, especially in the setting of a periurethral lesion, although up to 40% of VIN are asymptomatic. Women with invasive vulvar SCC often present with pruritus and/or discomfort in the vulvar region as well as a vulvar mass. Often, a recognizable exophytic or endophytic lesion is present, and ulceration is not uncommon. About 5% of patients present with multifocal disease (1). Most women present with an early-stage vulvar SCC (Figure 2.5-1A). Advanced-stage vulvar SCC ( Figure 2.5-1B,C ) is more often seen in older women. There is often a delay in diagnosis, which can be multifactorial. Women can delay visiting their general physician because they feel embarrassed. It is not uncommon that women are prescribed em piric topical therapies for several months, without proper examina tion of the vulva. Lanneau et al showed that among young patients with vulvar cancer (age below 45 years), almost half had symptoms for more than a year prior to diagnosis (2). Vandborg et al reported that patients with vulvar cancer had the longest delay in diagnosis of all patients with gynecologic cancer. They also showed that in the absence of blood loss, general practitioners were less likely to perform gynecologic examination, although the length of delay was shortened when examination was performed (3). A recent German study showed the potential mean delay of vulvar cancer diagnosis ranged from 186 to 328 days (4). D iagnostic E valuation The diagnostic evaluation starts with a thorough history and physi cal examination. Attention should be paid to coexisting morbidities and clinical extent of the disease. Initial evaluation should in clude bimanual pelvic examination and rectovaginal examination,

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