Premalignant and malignant disease of the vulva and vagina

Author(s):  
Linda Rogers ◽  
Maaike Oonk ◽  
Ate van der Zee

Vaginal intraepithelial neoplasia is a rare, premalignant condition of the vagina, which is caused by persistent infection with oncogenic strains of the human papillomavirus (HPV). It occurs either concurrently with intraepithelial neoplasia of other parts of the anogenital tract, or can develop after treatment of cervical lesions or pelvic irradiation. It can be difficult to diagnose and treat, due to the proximity of surrounding structures such as the bladder and rectum, and the need to preserve sexual function. Squamous carcinoma of the vulva is the most common vulval malignancy. It may arise from two distinct types of vulval intraepithelial neoplasia (VIN). Vulval extramammary Paget’s disease is a rare intraepithelial adenocarcinoma which accounts for less than 2% of primary vulval tumours. VIN and Paget’s disease are treated in order to relieve symptoms, such as severe pruritus, to exclude invasive disease, and to decrease the risk of developing cancer. Specialist follow-up in multidisciplinary clinics, with access to conservative surgery and reconstruction, as well as psychosexual support, are important in the management of women with vulval premalignant disease. Vulvar and vaginal cancer are rare gynaecological malignancies that occur predominantly in elderly women. Where the cornerstone of vulvar cancer treatment is surgery, radiotherapy is the most common treatment given in vaginal cancer. Vulvar cancer treatment has undergone significant modifications during the last decades, all with the aim to reduce treatment-related morbidity without compromising survival rates. The introduction of the sentinel node procedure has been a major advantage in the treatment of this disease.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e17029-e17029
Author(s):  
Shin Nishio ◽  
Takeo Shibata ◽  
Satoshi Yamaguchi ◽  
Hiroyuki Kanao ◽  
Atsumi Kojima ◽  
...  

e17029 Background: Vulvar cancer is a rare malignancy in women. During the past 30 years, large surveys of vulva cancer have not been performed in Japan. We therefore conducted a multicenter study to clarify the clinicopathological features of vulva cancer in Japan. Methods: In this multicenter retrospective cohort study, the clinical data of patients with vulva cancer were surveyed. The medical records of patients with vulvar cancer patients treated between 2001 and 2010 were retrospectively reviewed after obtaining approval from the Institutional Review Board of each institution. Survival analysis was performed using Kaplan-Meier curves. The effects of the clinical factors on overall survival were investigated using a Cox regression model. Results: A total of 1082 patients treated in 108 centers were studied. The median age was 72 years (range, 20 to 96). The disease stage was stage I in 415 patients (38.3%), stage II in 249 (23%), stage III in 255 (23.6%), and stage IV in 163 (15.1%) (FIGO 2009). The diagnosis was squamous cell carcinoma in 779 patients (72%), Paget’s disease in 158 (14.6%), adenocarcinoma in 63 (5.8%), and others in 82 (7.6%). Positive lymph nodes were found in 237 patients (21.9%). The median tumor diameter was 35 mm (range, 1 to 180). The 5-year overall survival was 86% in stage I, 74.7% in stage II, 48.2% in stage III, and 39.3% in stage IV (P < 0.001), and that according to histology was 63.9% in squamous cell carcinoma, 57.1% in adenocarcinoma, 79.7% in Paget’s disease, and 85.4% in others. The hazard ratio was 0.51 in patients with a histology of Paget’s disease or others (vs. squamous cell carcinoma or adenocarcinoma; P = 0.001; 95% CI, 0.35-0.75), 2.14 in patients with a the number of positive lymph nodes 2 or more (vs. 0 or 1; P < 0.001; 95% CI, 1.50-3.05), 2.10 in patients with a tumor diameter of ≥35mm (vs. < 35mm; P = 0.001; 95% CI, 1.36-3.25). Conclusions: Treatment outcomes in Japanese patients with vulvar cancer were similar to those reported previously. However, squamous-cell carcinoma, adenocarcinoma, positive lymph nodes, and bulky tumors were associated with poor outcomes. Multidisciplinary treatment might be required in patients with these characteristics. Clinical trial information: UMIN000017080.


1976 ◽  
Vol 62 (5) ◽  
pp. 529-535 ◽  
Author(s):  
Bruno Salvadori ◽  
Giuseppe Fariselli ◽  
Roberto Saccozzi

Of 100 cases of Paget's disease of the breast admitted to the National Cancer Institute of Milan from 1940 to 1974, 91 were statistically evaluated. They were divided in two groups according to presence or absence of a palpable nodule. The results of surgical treatment in terms of 5 and 10 year survival rates were 59 and 44 %, respectively, with a median survival of 9 years. For the two separate subgroups, those with a palpable nodule were 38 and 22 % for 5 and 10 years, respectively, while those without a palpable nodule were 92 and 82 % for 5 and 10 years, respectively. For the two groups the median survival was 3.6 and 16.4 years, respectively. The extent of surgery should be dependent on the presence or absence of palpable nodules under the nipple. For the two groups (with and without) extended radical mastectomy and the Patey-Dawson mastectomy are recommended.


1996 ◽  
Vol 89 (12) ◽  
pp. 699-701 ◽  
Author(s):  
J A Tidy ◽  
W P Soutter ◽  
D M Luesley ◽  
A B MacLean ◽  
C H Buckley ◽  
...  

Women with vulval intraepithelial neoplasia (VIN), lichen sclerosus (LS) and Paget's disease are referred either to gynaecologists or to dermatologists. We have ascertained the caseloads, referral patterns and treatment modalities used in the two specialties. A postal questionnaire was sent to 540 consultant gynaecologists and 225 consultant and senior registrar members of the British Association of Dermatologists. 350 gynaecologists and 161 dermatologists returned completed questionnaires. The workload of LS and Paget's disease was evenly distributed, with 54% of dermatologists and 58% of gynaecologists seeing more than six cases of LS per annum and less than 1% seeing more than five cases of Paget's disease. 92% of responding gynaecologists saw at least one case of VIN per year whereas 43% of dermatologists saw no cases. Patients with VIN and Paget's were referred to gynaecologists for treatment by 66% of dermatologists. Both groups are equally prepared to treat LS. Indications for treatment of VIN and LS were suspicion of invasion and symptoms. Local excision of VIN is the treatment of choice by both gynaecologists and dermatologists. LS is predominantly treated with topical steroids but gynaecologists also use topical oestrogen and testosterone. The great majority of responders favoured establishing a national register to study the outcome of vulval lesions.


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