A Study On Clinical Data Of 17 Cases With Vulvar Non-epithelial Malignant Tumors And Review Of The Literature

Objective:To summarize and analyze the incidence,clinical features,diagnosis,management,recurrence and outcome of vulvar non-epithelial malignant tumors.To provide the reference for clinical gynecologists.Methods:This study collected 159 patients who diagnosed with vulvar malignancy in Qilu Hospital of Shandong University from 2007 January to 2017 January.Among these cases,there were 18 non-epithelial vulvar malignant tumors,which 17 cases had complete clinical information.All of these 17 cases are including 10 vulvar malignant melanomas,6 vulvar sarcomas and 1 vulvar york sac tumor.Among these cases,a retrospective analysis of seventeen patients was performed.The clinical data consists of incidence,age,clinical manifestation,diagnosis,surgery,postoperative chemotherapy,recurrence and follow-up.Results:1.The incidence:In this survery,the non-epithelial vulvar malignant tumors accout for 10.7%(17/159)of all vulvar malignancy.Among these cases,there were vulvar malignant melanomas(58.8%,10/17),vulvar sarcomas(35.3%,6/17)and vulvar york sac tumor(5.9%,1/17).The incidence of the three types of all vulvar malignancy were 6.3%(10/159),3.8%(6/159),0.6%(1/159),respectively.2.The clinical characteristics:The age of 17 patients ranged from 10 to 82.The mean age and the median age were 48.4 and 52 years old respectively.The high peak age of the non-epithelial vulvar malignant tumors was from 50 to 64,accounting for 41.2%.The mean age and the median age of vulvar malignant melanomas and vulvar sarcomas were 56.6 and 57,36.7 and 36.The main clinical manifestation was vulvar mass(94.1%,16/17),sometimes associating with pain(47.1%,8/17),pruritus(23.5%,4/17).The tumor mostly occured in labium minus(41.2%)and labia majora(35.3%).70.0%patients of vulvar malignant melanomas occured in labium minus.Half of vulvar sarcomas occured in labia majora.3.The biopsy:The ratio of biopsy is 52.9%(9/17),7 of them were vulvar malignant melanoma,2 of them were vulvar sarcomas.Postoperative pathological coincidence rate is 77.8%(7/9).4.The preoperative imaging examination:41.2%(7/17)had preoperative imaging examination.29.4%(5/17)were post-operation.,including 5 cases of vulvar sarcoma and 2 cases of vulvar malignant melanoma.2 cases of vulvar sarcoma performed pelvic MRI,2 cases performed pelvic CT,1 case performed vulvar ultrasound.5.Imunohistochemistry:16 cases had immunohistochemical analysis.90%vulvar malignant melanoma had immuhistochemical analysis.The most commom immunohistochemical markers were HMB45,MelanA and S-100.All of 9 vulvar malignant melanoma showed immunoreactivity for HMB45,MelanA and S-100.The immunohistochemical markers that vulvar sarcoma used including intermediate filament proteins,epithelial tumor makers and muscle differentiation makers.The immunohistochemical markers that vulvar yolk sac tumor used were AFP,glypican-3 and SALL4.6.The surgery:Among 17 patients,there were 4 patients whose initial surgry performed in other hospitials.The remaining thirteen patients were eight vulvar malignant melanomas and five vular sarcomas.The surgery were simple vulvar neoplasm excision(52.90,9/17),radical vulvectomy(17.6%,3/17),local radical excision(17.6%,3/17).52.9%patients performed inguinal lymph node dissection.7.Postoperative adjuvant therapy:Of 17 patients,one patient lossed to follow up.The others performed post-chemotherapy accounted for 50%(8/16).5 cases of malignant melanoma had postoperative chemotherapy.The chemotherapy regimens were darcarbazine,temozolomide + cisplatin,darcarbazine + nedaplatin,cisplatin + docetaxel.The patient diagnosed synovial sarcoma and embryol rhabdomyosarcoma had IE(ifosfamide + pirarubicin)and VAC(vincristine +dactinomycin + cyclophosphamide),respectively.One vulvar york sac tumor had 6 cycles of BEP(bleomycin + etoposide + cisplatin).Interferon therapy performed in 60.0%(6/10)of vulvar malignant melanomas.8.The prognosis:All cases followed until 2017 march.Four patients had local relapse,which two of them were vulvar malignant melanomas and two was vuvlar sarcoma..Two patient who diagnosed with vulvar malignant melanoma had distant metastasis.5 cases died.Tthe survival time ranged from 8 to 96 months.The overall survival probability at 1,3,5 year was 93.3%,73.3%,73.3%,respectively.Conclusion:1.Vulvar non-epithelial malignant tumors are rare vulvar malignacy,accounting for 10.7%of vulvar malignacy.2.Immunohistochemistry play an important role on the diagnosis of vulvar non-epithelial malignant tumors.It can help to determine the grade of tumors initially,which avoid the unnecessary radical vulvectomy and chemotherapy.3.The mainstray surgery of vulvar low-grade soft tissue sarcoma is wide loval excision(2cm).The surgery of vulvar high-grade soft tissue sarcoma are recommended by radical vulvectomy.Wide local excision are recommended in vulvar malignant melanoma.