Clinicopathological And Prognostic Analysis Of Vulvar Malignant Tumors

Objective:To evaluate the role of clinicopathological parameters on prognosis in vulvar squamous cell carcinoma (VSCC) and to explore the rationality of the new FIGO staging system (2009) in Chinese population.Method:Patients with VSCC were recruited in this study who underwent the wide local excision/radical vulvectomy and bilateral inguinofemoral lymphadenectomy between November1990and March2012in Fudan University Shanghai Cancer Center. Clinical data was recorded and histopathologic slides were reviewed. The clinicopathological parameters include tumor size, location, depth of tumor invasion, lymph node metastasis, number and size of positive lymph nodes, and extracapasular spread. Patients were followed up for survival analysis. All the cases were restaged according to new FIGO staging system (2009). Prognostic predicting value was compared between the new (2009) and old (1994) FIGO staging system. The5-year overall survival (OS), relapse-free survival (RFS) and cause-specific survival (CSS) were calculated using Kaplan-Meier curves. Log-rank test was employed to evaluate the differences among groups.Results:Data from171patients with VSCC were eligible for analysis. The median age was60years (rang31-91years). According to the old FIGO staging system (1994),40patients were stage Ⅰ,46patients were stage Ⅱ,61patients were stage Ⅲ and24patients were stage Ⅳ. According to the new FIGO staging system (2009),86patients were stage1,13patients were stage Ⅱ,65patients were stage Ⅲ and7patients were stageⅣ. Of the171patients, lymph node metastasis was identified in71(41.5%) patients, involvement of surgical margins was confirmed in9patients (5.3%) and lymphvascular invasion was found in8patients. The median follow-up duration was56months (rang4-264months).49patients (28.7%) died of VSCC and35patients (20.5%) died of other diseases.80patients (46.8%) had recurrence. For all the patients, the5-year and10-year OS was64.2%and49.9%respectively. The5-year and10-year CSS was70.9%and68.8%respectively. The5-year and10-year RFS was54.8%and49.7%respectively. Univariate analysis showed that age, tumor size, lymph node metastasis and staging were associated with prognosis. According to the old FIGO staging system, the5-year OS was77.9%for stage Ⅰ,74.7%for stage Ⅱ,63.4%for stage Ⅲ and23.3%for stage Ⅳ, respectively(P<0.001). After restage according to the new FIGO staging system (2009),76patients (44.4%) was down staged. The5-year OS was76.1%for stage I,84.6%for stage Ⅱ,51.0%for stage Ⅲ and0for stage Ⅳ, respectively (P<0.001). Thus, the survival was also not evenly distributed among the4new stages. However, it was suggested that patients with lymph node metastasis had poorer survival than patients without. The5-year OS. CSS and RFS of patients with lymph node metastasis was46.7%,50.7%and33.9%, respectively, while the5-year OS. CSS and RFS of patients without lymph node metastasis was76.8%.85.6%and70.4%, respectively (P<0.001). In addition, the survival was decreasing with the increasing number of positive lymph nodes. Among the23patients with1positive lymph node,23patients with2positive lymph nodes,13patients with3positive lymph nodes and12patients with four or more positive lymph nodes, the5-year OS was72.9%.45.7%.38.5%and0, respectively (P<0.001), the5-year CSS was82.1%.48.2%.38.5%and0, respectively(P<0.001)., and the5-year RFS was55.3%.31.6%.23.1%and0, respectively (P=0.004). For the49patients with unilateral inguinal lymph node metastasis and22patients with bilateral inguinal lymph node metastasis, the5-year OS was56.3%and25.5%(P=0.007), the5-year CSS was62.1%and25.5(P=0.001) and the5-year RFS was41.5%and17.0%(P=0.013). However, after adjust with the number of lymph nodes in stage ⅢB, the prognosis of patients with unilateral and bilateral inguinal lymph node metastasis were the same. The5-year OS was39.4%and25.0%, respectively (P=0.555), the5-year CSS was42.4%and25.0%, respectively (P=0.261), and the5-year RFS was22.5%and17.8%, respectively (P=0.802). Among the patients with lymph node metastasis, the5-year OS. CSS and RFS for patients without extracapsular spread was66.0%.73.0%and56.9%, respectively. In comparison, none of the patients with extracapsular spread survived more than5years (P<0.001).According to the old FIGO stage (1994), the survival was similar among the patients with stage IB and Ⅱ, the5-year OS was77.0%and74.7%(P=0.848), the5-year CSS was86.8%and83.0%(P=0.883) and the5-year RFS was81.7%and71.5%,(P=0.817). It was reasonable for the new FIGO staging system (2009) to combine the two stages. In stage Ⅲ of the old FIGO staging system (1994), patients with lymph node metastasis had poorer survival than patients without. The5-year OS was84.6%and57.4%(P=0.019). In stage Ⅲ of the new FIGO staging system (2009), the5-year OSs CSS and RFS for patients with stage ⅢA was77.1%、84.8%and62.1%, respectively, the5-year OS、CSS and RFS for patients with stage ⅢB was33.0%,34.6%and20.2%, respectively, and none of the patients with stage ⅢC survived more than5years (P<0.001for OS, P<0.001for CSS and P=0.001for RFS).Conclusion:The patient’s age, lymph node metastasis and staging are prognostic predictors for VSCC. According to the new FIGO staging (2009), a part of the patients were down staged. Although the survival is not evenly distributed among the4new stages, it is reasonable to combine the old stage Ⅰ and stage Ⅱ and to differentiate the old stage Ⅲ based on the status of lymph node metastasis in the new stage system (2009). Subdivision of stage Ⅲ in the new FIGO staging system (2009) provides indeed a better reflection of prognosis based on the number and the size of metastatic lymph nodes and the presence of extracapasular spread. Objective:To characterize the clinicopathological features and evaluate the treatment outcomes for cases of primary extramammary Paget’s disease of the vulva (EMPDV).Methods:The medical records and pathology slides were reviewed and analyzed for43patients with primary EMPDV.Results:The mean age of the patients was68.6years (range,52-85). Intraepithelial EMPDV, invasive EMPDV and EMPDV with adnexal adenocarcinoma were observed in33(76.7%),7(16.3%) and3(7.0%) cases, respectively. Varied surgical procedures were initially performed in35(81.4%) cases. A positive incision margin was observed in16cases (47.0%). Definitive radiotherapy at a median dose of60Gy was performed in8(18.6%) patients. Six patients received postoperative radiotherapy due to a positive margin or lymph node metastasis after surgical excision. During a follow-up period of6-169months (median,54), recurrence was observed in12(34.3%) patients. Nine (75.0%) patients underwent repeated surgery and3(25.0%) patients received radiotherapy. Long-term overall survival was observed in patients with intraepithelial EMPDV. The median overall survival was124.5months in intraepithelial cases,70.8months in invasive cases and21.3months in cases with adnexal adenocarcinoma (log rank, P=0.032).Conclusions:Intraepithelial EMPDV accounted for the majority of primary cases and had a better prognosis. Surgical excision was the standard curative treatment for EMPDV. Radiotherapy was an alternative choice for patients with medical contradiction or surgical difficulties. Postoperative radiotherapy could be considered in cases with positive surgical margin or lymph node metastasis. Recurrence was common and repeated excision was often necessary.