Feto-maternal outcome of pregnancy complicated by vulvar cancer: a systematic review of literature.

Vulvar cancer is an extremely rare complication during pregnancy, and its effect on pregnancy and survival is not well understood. A systematic literature review was conducted in order to examine the fetal and maternal outcomes and optimal management of pregnancy complicated by vulvar cancer. PubMed/MEDLINE were used to identify case reports with searching keywords "pregnancy" and "vulvar cancer" between January 1955 and February 2014 that identified 36 cases for analysis. Mean age was 30.7. The most common presenting symptom and gestational age were vulvar mass/swelling (75.0%) and the second trimester of pregnancy (54.8%), respectively. Vulvar biopsy at the time of initial presentation to care during pregnancy was performed in only 46.7% of cases. Among delayed cases for biopsy, mean duration of delay was 12.8 weeks and the majority had a delay for more than 8 weeks (62.5%). The majority of vulvar cancer was squamous histology (47.2%) and stage I disease (60.0%). Vulvectomy and inguinal-femoral lymphadenectomy were performed in 97.1% and 63.9%, respectively. Abdominal delivery was recorded in 46.2% of cases. Live birth and full term delivery rates were 96.3% and 74.0%, respectively. For survival analysis, delay in diagnosis and advanced stage disease were commonly associated with decreased disease-free survival (5-year rate, delay in diagnosis >8 versus ≤8 weeks, 0% versus 69.1%, hazard ratio (HR) 7.86, 95% confidence interval (CI) 2.03-30.6, p=0.001; and stage III-IV versus stage I-II, 0% versus 59.8%, HR 3.35, 95% CI 1.16-9.68, p=0.011) and overall survival (5-year rate, delay in diagnosis >8 versus ≤8 weeks, 0% versus 67.1%, hazard ratio (HR) 14.8, 95% CI 1.77-124, p=0.001; and stage III-IV versus stage I-II, 0% versus 86.4%, HR 8.22, 95% CI 2.06-33.2, p<0.001). In conclusion, while the majority of cases resulted in good pregnancy outcomes, diagnosis of vulvar cancer during pregnancy is frequently delayed. Since delayed diagnosis is a significant prognosticator of decreased survival outcomes, early recognition is integral in the management of pregnancy complicated by vulvar cancer.