Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome:A case report

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.

First Trimester Uterine Rupture: A Case Report on a Patient with Unsuspected Müllerian Duct Anomaly

Introduction: Mullerian malformations are a group of malformations that result from an alteration of embryonic development. Most cases are asymptomatic, however, in pregnant patients it has been associated with recurrent abortion or premature delivery. Case presentation: 12 + 2 weeks pregnant woman consulted for abdominal pain, vomiting and fever. During the study, a transvaginal ultrasound was performed, where two endometric cavities and a pregnancy in the right horn were observed. The evolution of the patient was torpid, reaching hypovolemic shock, resolved by an emergency laparotomy where the rupture of the right horn was found. Discussion: The bicornuate uterus is the consequence of a partial fusion of the paramesonephric ducts during the fetal development, resulting in two functional uterine horns. Patients with bicornuate uterus may be asymptomatic, a uterine rupture is a rare but potentially severe complication of the bicornuate uterus and is usually presented during first and second trimester. Conclusions: Pregnancy with Mullerian anomalies often has preterm delivery, IUGR and malpresentation, and even more severe complication as uterine ruptura, so, requires proper counselling and close monitoring during antenatal period.

Unilateral Singleton Pregnancy with Rural Vaginal Delivery in a Woman with Uterus Didelphys

Background: Uterus didelphys is a Müllerian duct anomaly which is clinically significant because only 45% of UD patients achieve term delivery and have associated increased risk of spontaneous abortion, foetal growth retardation, mal presentation, and a significant caesarean section delivery rate. Case: A 26-year-old Gravida 2 Para 1 Abortion 1 woman with uterus didelphys and associated complete, non-communicating, longitudinal vaginal septum carries a pregnancy to term in her right uterus in rural Canada. She delivers her baby at 41 weeks gestational age via vacuum assisted spontaneous vaginal delivery. Conclusion: This case report supports more recent literature that uterus didelphys should not be considered as an absolute indication for caesarean delivery. A trial of labour is conceivable with obstetrician gynaecologist involvement in more rural centers with a care plan in place.

A giant Müllerian duct cyst in the perineum:a case report

The authors present a case report of a giant Müllerian duct cyst in the perineum.A 37-year-old man presented with a mass with the size of 50 cm×40 cm×30 cm in the perineum.Computed tomography(CT)scan and magnetic resonance imaging(MRI)of the pelvis and perineum found a huge multilocular cystic mass.X-ray film of the pelvis showed a giant soft-tissue shadow of high density in the perineum.Voiding cystourethrogram did not show any cyst interlinked to posterior urethra.After related examinations,the patient underwent open surgical resection of the cyst.Pathologic examination confirmed the presence of a Müllerian duct cyst.Huge Müllerian duct cyst is uncommon in clinic.Treatments of Müllerian duct cysts depend on their sizes and symptoms.

抗苗勒管激素与抑制素B对多囊卵巢综合征的临床预测价值

目的通过分析多囊卵巢综合征(polycystic ovary syndrome,PCOS)病人与对照组的抑制素B(inhibin B,INHB)、抗苗勒管激素(anti-Müllerian hormone,AMH)及各项内分泌指标,探讨INHB、AMH对PCOS的临床预测价值及比较两组内分泌指标的特点。方法选取2016年9月至2017年5月间在首都医科大学附属北京妇产医院内分泌科就诊的PCOS病人567例为研究组,取同期就诊的年龄、原发不孕与继发不孕病人或正常体检者53例作为对照组。测量所有参与者的身高、体质量,空腹抽血查INHB、AMH、卵泡刺激素(follicle-stimulating hormone,FSH)、黄体生成素(luteinizing hormone,LH)、雌二醇(estradiol,E2)、睾酮(testosterone,T),分析PCOS组与对照组的血清INHB、AMH质量浓度及内分泌指标特点。结果 PCOS组的血清AMH质量浓度、LH、T、体质量指数(body mass index,BMI)均明显高于对照组,E2、FSH/LH低于对照组,且组间差异均有统计学意义(P<0.05)。PCOS组的INHB质量浓度高于对照组,FSH低于对照组,组间差异均无统计学意义(P>0.05)。AMH预测多囊卵巢综合征的最佳分界值(cut-off)值为4.84μg/L,敏感度为84.48%,特异度为69.81%。INHB预测多囊卵巢综合征的cut-off值为70.8 ng/L,敏感度为53.97%,特异度为67.92%,AMH与INHB联合预测(并联试验)多囊卵巢综合征的敏感度为90.29%,特异度为54.92%。结论 AMH预测PCOS的cut-off值为4.84μg/L,INHB预测PCOS的cut-off值为70.8 ng/L,且AMH与INHB的联合检测(并列试验)敏感度高于AMH单项检测,这对临床PCOS的预测有很重要的指导意义。

多囊卵巢综合征合并卵巢储备功能低下病人的内分泌特征

目的了解中国多囊卵巢综合征(polycystic ovary syndrome,PCOS)合并卵巢储备功能低下(diminished ovarian reserve,DOR)病人的发生率及内分泌特征。方法选取2015年1月至2017年1月就诊的多囊卵巢综合征病人338例及同期就诊的单纯DOR病人68例,记录所有研究对象的年龄、月经情况,测量其身高、体质量、腰围、臀围,测定基础血清卵泡刺激素(follicle stimulation hormone,FSH)、黄体生成素(luteinizing hormone,LH)、雌二醇(estradiol,E2)、睾酮(testosterone,T)、抗苗勒管激素(antiMüllerian hormone,AMH)及基础窦卵泡数(baseline antral follicle count,b AFC)。对PCOS合并DOR病人、单纯PCOS及单纯DOR病人的各指标进行方差分析。结果 1)PCOS合并DOR的病人发生率为16.9%。2)在PCOS合并DOR病人中,基础FSH>10IU/L且<40IU/L、FSH/LH>3、基础E2>80pg/m L、b AFC≤4的发生率分别为38.6%、35.1%、35.1%及36.8%。3)PCOS合并DOR病人基础FSH及睾酮浓度与单纯PCOS及单纯DOR病人之间的差异均具有统计学意义(P<0.05),PCOS合并DOR病人基础E2、FSH/LH、AMH与单纯PCOS之间的差异有统计学意义(P<0.05),而与单纯DOR之间的差异无统计学意义(P>0.05)。4)在单纯PCOS组,AMH与睾酮呈正相关(P<0.01)。在PCOS合并DOR组,AMH与FSH呈负相关,与LH呈正相关(P<0.01)。在单纯DOR组,AMH与年龄、FSH、LH、FSH/LH均呈负相关(P<0.01)。结论 PCOS合并DOR病人的内分泌特征既不同于单纯PCOS组,也不同于单纯DOR病人。

苗勒管细胞的分离及苗勒管抑制素受体定位的免疫电镜观察

本实验应用胰酶消化后的Percoll梯度分离技术,将苗勒管上皮细胞与间质细胞分离开来,然后分别进行细胞培养。一部分与同胚龄睾丸或卵巢联合培养,观察生殖腺分泌的苗勒管抑制素(MIS)对两类细胞致死率的差异;另一部分以MIS抗体为第一抗体,HRP-羊抗兔IgG或抗生物素-胶体金为第二抗体,作免疫电镜技术,显示苗勒管上皮细胞和间质细胞上MIS受体的结合位点。实验结果表明,睾丸分泌的MIS使培养的苗勒管上皮细胞致死率与对照组相比,有非常显著差异(P<0.001),对间质细胞致死率不明显,但MIS受体结合位点的免疫反应不仅出现在苗勒管上皮细胞上,也显示在间质细胞上。本文讨论了MIS的作用机理。

伴胰岛素抵抗多囊卵巢综合征患者二甲双胍治疗后血清抗苗勒管激素水平的观察

分析28例伴胰岛素抵抗（IR）的多囊卵巢综合征（PCOS）行口服二甲双胍治疗患者的临床资料.治疗前血清抗苗勒管激素（AMH）水平为（8.5±3.2） μg/L,血清睾酮及黄体生成素（LH）水平正常,二甲双胍剂量为500 mg/次、2次/d,连续3个月.治疗后AMH水平为（8.4±3.2） μg/L,治疗前后比较差异无统计学意义（P=0.148）;治疗后BMI、血糖、空腹胰岛素、稳态模型IR指数（HOMA-IR）水平[（27.6±4.1） kg/m2、（5.32±0.47） mmol/L、（93.3±17.8） pmol/L及3.17±0.71],均较治疗前降低[（29.3±4.4） kg/m2、（5.55±0.71） mmol/L、（147.7±44.1） pmol/L及5.27±1.85],差异均有统计学意义（均P〈0.05）.提示二甲双胍治疗3个月能改善IR,降低BMI,但AMH水平变化不明显.