BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with H...BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.展开更多
BACKGROUND Primary vaginal cancer is rare and most vaginal tumors are metastatic,often arising from adjacent gynecologic structures.Primary vaginal cancers are also more common among postmenopausal women and most of t...BACKGROUND Primary vaginal cancer is rare and most vaginal tumors are metastatic,often arising from adjacent gynecologic structures.Primary vaginal cancers are also more common among postmenopausal women and most of these are squamous cell carcinomas,with adenocarcinomas being relatively rare.Vaginal bleeding is the most common clinical manifestation of vaginal adenocarcinoma.About 70%of vaginal adenocarcinomas are stage I lesions at the time of diagnosis,for which radical surgery is recommended.However,more advanced vaginal cancers are not amenable to radical surgical treatment and have poor clinical outcomes.Optimal treatments modes are still being explored.Here,we report a rare case of stage IIb primary vaginal adenocarcinoma for which an individually designed vaginal applicator for after-loading radiotherapy was used to achieve good tumor control.CASE SUMMARY A 62-year-old woman presented to our clinic after 3 months of abnormal postmenopausal vaginal bleeding.Gynecological examination,computed tomography(CT),and positron emission tomography-CT showed a large mass(about 5 cm)on the anterior vaginal wall.Colposcopy biopsy confirmed adenocarcinoma of vaginal origin.After three cycles of carboplatin plus paclitaxel chemotherapy,the lesion partially shrunk.The patient then received external irradiation of 45 gray(gy)in 25 fractions,which further reduced the vaginal lesion,followed by after-loading radiotherapy of 30 gy in 5 fractions with an individually designed vaginal applicator.Three months later,magnetic resonance imaging showed a slight thickening CONCLUSION Primary vaginal adenocarcinoma is rare,and prognosis is poor in most vaginal cancers of locally advanced stages,which cannot be treated with radical surgery.Better tumor control can be achieved with an individualized vaginal applicator that allows administration of a higher radical dose to the tumor area while protecting normal tissues.展开更多
文摘BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.
文摘BACKGROUND Primary vaginal cancer is rare and most vaginal tumors are metastatic,often arising from adjacent gynecologic structures.Primary vaginal cancers are also more common among postmenopausal women and most of these are squamous cell carcinomas,with adenocarcinomas being relatively rare.Vaginal bleeding is the most common clinical manifestation of vaginal adenocarcinoma.About 70%of vaginal adenocarcinomas are stage I lesions at the time of diagnosis,for which radical surgery is recommended.However,more advanced vaginal cancers are not amenable to radical surgical treatment and have poor clinical outcomes.Optimal treatments modes are still being explored.Here,we report a rare case of stage IIb primary vaginal adenocarcinoma for which an individually designed vaginal applicator for after-loading radiotherapy was used to achieve good tumor control.CASE SUMMARY A 62-year-old woman presented to our clinic after 3 months of abnormal postmenopausal vaginal bleeding.Gynecological examination,computed tomography(CT),and positron emission tomography-CT showed a large mass(about 5 cm)on the anterior vaginal wall.Colposcopy biopsy confirmed adenocarcinoma of vaginal origin.After three cycles of carboplatin plus paclitaxel chemotherapy,the lesion partially shrunk.The patient then received external irradiation of 45 gray(gy)in 25 fractions,which further reduced the vaginal lesion,followed by after-loading radiotherapy of 30 gy in 5 fractions with an individually designed vaginal applicator.Three months later,magnetic resonance imaging showed a slight thickening CONCLUSION Primary vaginal adenocarcinoma is rare,and prognosis is poor in most vaginal cancers of locally advanced stages,which cannot be treated with radical surgery.Better tumor control can be achieved with an individualized vaginal applicator that allows administration of a higher radical dose to the tumor area while protecting normal tissues.
