原发卵巢Müllerian腺肉瘤1例及文献回顾

报道复旦大学附属妇产科医院于2022年收治的原发性卵巢Müllerian腺肉瘤(Müllerian adenosarcoma,MA)1例,并进行文献复习,分享卵巢腺肉瘤的诊治经验。患者,女性,29岁,未婚育,外院行腹腔镜下卵巢病损切除术,术中探查见“右侧卵巢体积增大,下极可见大小约11 cm×8 cm的菜花样肿块,无包膜,质地似鱼肉样,完全切除肿块送检”。术后经北京某(三甲)医院及我院病理科会诊提示卵巢腺肉瘤,遂行全面分期手术:腹腔镜下右侧输卵管卵巢切除术、左侧卵巢囊肿剥除术、大网膜切除术、腹膜多点活检术;宫腔镜下宫颈管占位切除术。术后予紫杉醇+异环磷酰胺化疗4周期。患者化疗结束后定期随访,目前术后近2年随访期间无复发征象。

子宫Müllerian腺肉瘤5例临床病理分析

目的探讨子宫Müllerian腺肉瘤的临床病理学特征。方法收集5例子宫Müllerian腺肉瘤的临床资料,行HE、免疫组化EnVision法染色,分析其临床病理学及免疫表型特征,并复习相关文献。结果5例患者年龄41~63岁,中位年龄44岁。镜下肿瘤呈分叶状结构,富于细胞的间质呈乳头状和息肉状突入囊性扩张的腺腔内或压迫良性腺体呈成角的裂隙。轻~中度异型的间质细胞围绕腺体呈套袖样增生。免疫表型:肿瘤间质细胞ER、PR、SMA均弥漫阳性,腺体周围CD10阳性,Ki-67增殖指数3%~30%。结论子宫Müllerian腺肉瘤常表现为阴道异常出血,多为低度恶性肿瘤,手术治疗为主,不良预后与肿瘤过度生长、脉管内癌栓、FIGO分期等有关。

Müllerian Adenosarcoma of the Vaginal Stump: A Case Report and Literature Review

Müllerian adenosarcomas are rare mixed tumors with low malignant potential, and occur mainly in the uterus. Primary adenosarcomas arising in vaginal endometriosis are even more rarely reported. We report a very rare case with Müllerian adenosarcoma arising from vaginal endometriosis. A 43-year-old nulliparous woman has a history of endometriosis. Due to two parallel fistulas in cervix, severe endometriosis and adenosarcoma, she has undergone radical and multiple surgeries. After the surgery, persistent vaginal vault masses were noticed, and eight resections of the polyps at vaginal stump were performed. For the first six resections, all the pathological examinations showed endometriosis. And the last two resections demonstrated vaginal adenosarcoma. Transition between Müllerian adenosarcoma and endometriosis was gradual. Finally, adenosarcoma associated with endometriosis was diagnosed from recurrent vaginal masses. To our knowledge, this report is the sixth case that primary adenosarcomas arising in vaginal endometriosis. But chemo-therapy and radiation therapy were not taken and the patient remained in a good condition throughout the 9-year follow-up period. In addition, we present a review of the literature. The diagnosis and treatment of the tumor are also discussed.

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.

Müllerian腺肉瘤的临床及超声特点分析

目的：探讨Müllerian腺肉瘤（MA）的临床与超声特点。方法：回顾性分析复旦大学附属妇产科医院2007年11月至2014年7月25例经手术病理检查证实为MA患者的临床病史及超声表现。结果：125例MA患者平均年龄48.2±13.8岁,10例为绝经后妇女。主要临床表现为异常阴道流血（22例）,下腹痛（1例）及体检发现异常占位（2例）。病变部位在子宫内膜20例（80%）、宫颈3例（12%）、宫腔和宫颈1例（4%）以及盆腔内1例（4%）。16例单纯MA,5例伴性索样分化,4例伴肉瘤成分过度生长。其FIGO分期为ⅠA 8例、ⅠB 14例、ⅠC 1例,ⅢA 1例。2MA位于子宫的患者超声图像显示宫腔或（及）颈管内单个大小不等（最大径12~95mm,平均40.1±24.5mm）的息肉样占位,部分脱垂到阴道内（24例）;形态不规则,边界模糊（8例）或尚清晰（16例）;实性为主者22例（88%）,囊实性者2例（8%）,而1例（4%）位于盆腔内者是囊性为主、边界不清的低回声肿块。彩色多普勒血流显像：条索状血流信号7例,星点状血流信号者12例,彩色血流信号不明显者6例。结论：MA是一种少见的低度恶性肿瘤,其临床及超声表现无特异性,需要病理检查确诊。熟悉其临床及超声表现有助于加强对MA的认识,从而正确诊断。

子宫Müllerian腺肉瘤伴肉瘤过度生长临床病理分析

目的 探讨子宫苗勒管腺肉瘤伴肉瘤过度生长（Müllerian adenosarcoma of the uterus with sarcomatous overgrowth，MASO）的临床病理特征、生物学行为及预后。方法 对3例MASO采用光镜、免疫组化染色观察，并复习相关文献。结果 3例患者分别表现为不规则阴道出血、绝经后阴道出血及子宫增大。大体上，宫腔内见息肉状肿物，直径2～13cm，2例浸润浅肌层，1例浸润深肌层达浆膜。组织学上，3例MASO均见典型腺肉瘤结构，即由良性腺体和低度恶性肉瘤样成分混合而成，其中纯粹肉瘤成分比例均超过25％，并且肉瘤级别比相邻的腺肉瘤高；例2含有异源成分。3例肉瘤成分均显示vimentin阳性，而EMA、CD10、desmin、SMA、S-100蛋白等均阴性。3例均行全子宫＋双侧附件切除术，1例进行了盆腔淋巴结清扫。2例术后分别随访3年及5年均无瘤生存，1例术后1个月死亡。结论 MASO是一种少见的Müllerian混合上皮和间质肿瘤，具有独特的临床病理形态特征；与经典的子宫Müllerian腺肉瘤相比，它更具有侵袭性，预后差。

宫颈Müllerian腺肉瘤临床病理观察

目的探讨子宫颈Müllerian腺肉瘤(MAs)的临床病理特征、免疫表型、鉴别诊断及治疗。方法对1例宫颈MAs行光镜、免疫组化检查,复习临床资料及相关文献。结果本例瘤组织上皮成分为宫颈管腺体,腺体成裂隙状,间质细胞分布不均,在腺体周围呈袖套样分布,伴轻~中度细胞异型性,核分裂象>7个/10HPF。免疫组化间质细胞ER和PR(+),SMA、desmin和CD10部分(+),h-Caldesmon(-)。结论宫颈MAs较罕见,诊断主要依靠组织形态特征,免疫组化有助于鉴别诊断和确定异源性成分,组织中有无间质过度生长和肌层浸润影响患者预后,也是临床术式选择和是否辅助治疗的主要依据。

宫颈原发性Müllerian腺肉瘤4例临床病理分析

目的探讨宫颈原发性Müllerian腺肉瘤的临床病理特征,提高对该瘤的认识。方法对4例发生于宫颈的Müllerian腺肉瘤进行光镜、免疫组化标记检查,并复习临床资料及相关文献。结果临床上患者以阴道不规则出血为主要症状。镜下肿瘤由良性腺上皮成分和肉瘤性间质成分组成;腺体常扩张,异型性较明显的间质成分突入腺腔内形成息肉状结构,腺体周围间质成分比较密集,围绕腺体形成"袖套样"结构;核分裂象分布不均(1～15个/10 HPF)。免疫表型没有特异性。结论宫颈原发性Müllerian腺肉瘤是一种罕见的肿瘤,诊断主要依靠组织形态学特征,免疫组化标记主要用于确定异源性成分及与其他肿瘤的鉴别。

年轻未孕女性子宫Müllerian腺肉瘤5例临床病理分析

目的:探讨年轻未孕子宫Müllerian腺肉瘤患者的临床病理特征,提高对该病的认识。方法:分析5例年轻未孕子宫Müllerian腺肉瘤患者的临床表现及病理组织学特点。结果:5例患者均以经量增多伴阴道不规则流血为初诊主诉,子宫均增大,4例有息肉样物脱出宫颈。肿瘤由良性腺上皮成分和肉瘤性间质成分组成。免疫表型,肉瘤成分普遍Vimentin阳性,大部分CD10阳性,部分Desmin、SMA阳性。5例中术前诊断3例;4例经术后病理检查证实。治疗主要采用次广泛全子宫+单侧附件+盆腔淋巴结切除,术后预防性化疗。结论:年轻未孕子宫Müllerian腺肉瘤的基本形态由良性腺上皮和肉瘤性间质混合组成。对年轻未孕患者出现阴道不规则流血并伴有息肉样物脱出宫颈,且伴子宫增大时要警惕子宫Müllerian腺肉瘤。治疗以手术为主,可辅以化疗。

子宫Müllerian腺肉瘤临床病理分析

目的通过对Müllerian腺肉瘤的临床表现、镜检、免疫组化及鉴别诊断的总结分析,提高对Müllerian腺肉瘤的认识水平.方法回顾性分析总结本院2009年至2018年期间收治的8例子宫Müllerian腺肉瘤的临床表现、镜下形态及免疫组化结果.结果Müllerian腺肉瘤多见于中老年女性,临床表现常为异常阴道流血、阴道排液或肿块突入阴道.大体多为息肉状肿块.形态上,显著增生的富细胞性间质构成息肉状突起,形似叶片,腺体周围更富于细胞,显得更为致密("袖套样").免疫组化,间质成分表达CD10、ER、PR.结论Müllerian腺肉瘤易漏诊或误诊,形态上富细胞性间质围绕良性腺体聚集是镜下诊断的重要依据,鉴别诊断包括:腺纤维瘤、非典型性息肉样腺肌瘤、低级别子宫内膜间质肉瘤、癌肉瘤、宫颈的腺泡状横纹肌肉瘤等病变鉴别.

女性生殖系统恶性Mllerian混合瘤临床病理分析

目的 探讨女性生殖系统恶性M櫣ｌｌｅｒｉａｎ混合瘤的发生、免疫表型和鉴别诊断?椒ā《?16例子宫、输卵管和卵巢恶性M櫣ｌｌｅｒｉａｎ混合瘤进行临床病理和免疫组织化学观察?峁?16例患者中 ,恶性M櫣ｌｌｅｒｉａｎ混合瘤发生于子宫颈 2例 ,子宫体 11例 ,输卵管 1例 ,卵巢 2例。同源性 9例 (5 6 % ) ,异源性 7例 (44 % )。癌转移 7例 (44 % ) ,其中异源性转移 5例 (71% ) ,以肉瘤成分转移为主。免疫表型 :在 14例中所有癌成分CK和所有肉瘤成分Vim呈阳性表达 ,4 3%病例的肉瘤成分呈CK和Vim双相表达 ,Des 9例阳性 (6 4 % ) ,MG 4例阳性 (2 9% ) ,CD6 85例阳性 (36 % )。结论 女性生殖系统恶性M櫣ｌｌｒｉａｎ混合瘤可能来源于中胚层 ,多发生于子宫 ,其异源性肿瘤恶性度高 ,转移快。肿瘤的全面检查和免疫表型可作为鉴别诊断的重要参考依据。

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.

Diagnosis and laparoscopic excision of accessory cavitated uterine mass in a young woman:A case report

BACKGROUND Accessory and cavitated uterine mass(ACUM)is an uncommon form of connate Müllerian anomaly seen in young and nulliparous women,which presents as chronic periodic pelvic pain and severe dysmenorrhea.The entity is often underdiagnosed due to a broad differential diagnosis,including rudimentary uterine horn,true cavitated adenomyosis and degenerating fibroids.CASE SUMMARY A 22-year-old woman who presented with severe dysmenorrhea and was initially misdiagnosed with cystic adenomyosis.Gynecological examination and ultrasonography were performed.The patient underwent laparoscopic excision of the mass and histopathological examination confirmed the diagnosis.Postoperatively,the patient did well,with no further dysmenorrhea.CONCLUSION ACUM is difficult to diagnose.A correct diagnosis can be made only after excision and histopathological evaluation.Surgical excision is necessary and can be carried out by laparoscopy.

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.

Rapid Recurrence of Unilateral Endometrioma in a Teenager with a Noncommunicating Rudimentary Horn and Unicornuate Uterus

A 17-year-old nulligravida woman presented to the emergency department complaining of a six months’ history of severe dysmenorrhea. The patient underwent a laparoscopic excision of a right ovarian endometrioma in another hospital one year ago, 24 months after menarche at 14 years of age. Transvaginal ultrasonography, magnetic resonance imaging, hysteroscopy as well as laparo- scopy revealed a unicornuate uterus with a noncommunicating right rudimentary horn, a dilated right fallopian tube and a novel 40 mm right ovarian endometrioma. An excision of the right fallopian tube, the rudimentary horn and the endometrioma was performed. Since endometriosis may be originated by retrograde menstruation, an obstructive müllerian malformation should be ruled out at the diagnosis of endometriosis, particularly in adolescents. It may lead clinicians to provide an initial definitive treatment for avoidingrapid recurrent endometriosis.

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。

子宫苗勒氏腺肉瘤5例临床病理分析

目的分析子宫苗勒氏腺肉瘤（uterine mllerian adenosarcoma,UMA）的临床病理特点,以期提高对其诊断、鉴别诊断和治疗的水平。方法回顾性分析5例子宫苗勒氏腺肉瘤的临床表现、病理特征、治疗方法和预后。结果患者年龄19~77岁,平均46岁,绝经前后分别为3例和2例。临床主要特点为阴道异常流血。组织学特征主要为良性或非典型腺上皮伴有肉瘤间质,其中2例伴有肉瘤成分的过度生长,另一例间质肉瘤伴有性索样分化。免疫表型：肿瘤上皮CK、EMA呈强阳性表达,ER、PR、CA125呈弱阳性表达;肉瘤间质Vi m呈弥漫强阳性,CD10、CD99呈局灶强阳性;ER、PR、SMA、Inhibin-α、CR、S-100等呈局灶弱阳性;Des阴性。5例均采用全子宫＋双侧附件切除手术治疗,3例术后加用化疗。术后随访1例死亡,1例复发,3例无瘤生存。结论子宫苗勒氏腺肉瘤常以阴道异常流血为临床表现,治疗以手术为主,预后的不良因素：子宫外播散,超过子宫肌壁1/2以上的深层肌层侵犯以及肉瘤成分的过度生长。

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。

骶尾部皮肤纤毛囊肿1例并文献复习

目的探讨骶尾部皮肤纤毛囊肿(cutaneous ciliated cyst,CCC)的临床病理学特征、诊断、鉴别诊断及预后。方法回顾性分析1例骶尾部CCC的临床病理学、组织学发生及免疫表型特征并复习相关文献。结果患者女童,11岁,发现骶尾部臀裂处赘生物、盆腔MRI尾骨后软组织内边界清楚的囊肿。镜下见真皮层及皮下组织内囊肿形成,与表皮不相连,囊腔内上皮细胞呈乳头状生长,可见鳞状上皮化生,衬覆上皮类似输卵管上皮,由假复层纤毛柱状上皮及分泌细胞组成,未见胞质内分泌物及顶质分泌,未见肌上皮细胞。免疫表型:上皮细胞表达ER、PAX-8,不表达GCDFP-15、TTF-1。结论骶尾部CCC属罕见的良性囊肿,该例起源于异位的Müllerian上皮,确诊主要依靠病理学形态及免疫表型,手术切除后不复发。

Primary omental malignant mixed Müllerian tumor in a 67-year-old woman

背景 大网膜原发苗勒氏管混合瘤很少见 ,迄今为止仅报道了 2例 ,具有其特殊临床特征。病例 女性 ,6 7岁 ,G6P5 ,主因腹痛、腹胀 15天就诊于北京大学人民医院妇科。体格检查发现一直径 12厘米腹部包块 ,活动度差。行肿瘤及大网膜大部分切除 ,全子宫加双附件切除术。术后病理示大网膜原发恶性苗勒氏管混合瘤。术后顺铂、吡喃阿霉素、足叶已甙化疗一疗程 ,因严重骨髓抑制 ,未继续化疗 ,术后 8个月发现肿瘤肝转移。结论 原发性恶性苗勒氏管混合瘤预后差 ,现今手术仍是最有效的治疗手段 ,化疗及放疗效果不确切。