Sertoli–Leydig cell tumor presenting hyperestrogenism in a postmenopausal woman: A case report and review of the literature

ObjectiveSertoli–Leydig cell tumor (SLCT) accounts for <0.5% of all ovarian tumors, which is unusual in postmenopausal women. Postmenopausal women with SLCT usually become virilized. We report a postmenopausal woman with SLCT presenting with hyperestrogenism.Case ReportWe report a rare case of SLCT in a postmenopausal woman aged 61 years, who presented with postmenopausal bleeding, endometrial hyperplasia and mucous polyp, elevated estradiol, and decreased follicle-stimulating hormone (FSH) and luteinizing hormone (LH) values, all suggesting hyperestrogenism. Transvaginal ultrasound revealed several small cyst locules, detected inside the right ovary, with a maximum diameter of 7 mm. The diagnosis was delayed because of the atypical clinical manifestation and negative serum tumor markers. The frozen section investigation revealed SLCT intraoperatively, which was confirmed by histopathological and immunocytochemical examination. The tumor was positive for inhibin-alpha, pancytokeratin, and p53 and in isolated tumor cells, positive for Ki-67.ConclusionThis case of SLCT suggests the existence of a new specific type of endocrine complex disease.     

Direct laparoscopic venous sampling to diagnose a small Sertoli-Leydig tumor

BACKGROUND: Sertoli-Leydig cell tumors (SLCT) constitute only 0.5% of all primary ovarian neoplasms. We report a unique diagnostic method (selective laparoscopic venous sampling) and a rare case of a contralateral second primary tumor. CASE: A 14-year-old female presented with hyperandrogenic complaints and an increased serum testosterone. Ovarian origin was confirmed by direct laparoscopic ovarian blood sampling. A right salpingo-oophorectomy was performed. The pathological diagnosis was SLCT of intermediate differentiation. Three years later, the patient presented again with an increased serum testosterone. A solid tumor in the left ovary was excised. The pathology was SLCT of intermediate differentiation. The patient remains disease-free. CONCLUSIONS: Direct laparoscopic venous sampling is used to diagnose a small SLCT in a teenage patient.     

Compensatory Ovarian Hypertrophy after Unilateral Oophorectomy: Evaluation of Ovarian Volumes in Pediatric and Adolescent Populations

Study ObjectiveLimited data exist on the morphologic and physiologic effect on the remaining ovary after unilateral oophorectomy, especially in the pediatric population. Our aim is to evaluate ovarian volumes following unilateral oophorectomy to determine whether compensatory ovarian hypertrophy occurs in the remaining contralateral ovary.DesignThis was a retrospective chart review of ovarian volume measured on ultrasounds that were completed after unilateral oophorectomy. Postoperative ovarian volumes were compared to established radiologic standards.SettingLarge tertiary care academic children's hospital in Atlanta, GA.ParticipantsFemale patients less than 21 years old who underwent unilateral oophorectomy.Main Outcome MeasuresOvarian volumes measured on postoperative ultrasounds.ResultsA total of 93 patients met inclusion criteria for this study. Serial ultrasounds were performed in slightly more than half of the patients (n = 48, 51.6%), totaling 193 postoperative ovarian volumes. The average age of oophorectomy was 10.8 years. Prior to surgery, the majority of patients presented with abdominal pain (n = 51, 54.8%) or pelvic mass (n = 51, 54.8%), and most (n = 77, 82.8%) had benign final pathology. Ovarian volumes were compared to 4 published radiologic ultrasound standards. More than 62.2% of ovarian volumes from girls who had previously had unilateral oophorectomy were larger than age-matched standard ovarian volumes.ConclusionOvarian enlargement occurs in the contralateral ovary following unilateral oophorectomy in the pediatric and adolescent population. This supports the concept of compensatory ovarian hypertrophy. This knowledge provides valuable information for interpretation of radiologic images in young female individuals who have undergone oophorectomy, and can assist with counseling on the risk of adnexal complications due to ovarian hypertrophy after unilateral oophorectomy.     

Postoperative Ovarian Morphology on Ultrasound after Ovarian Torsion—Effect of Immediate Surgery: A Retrospective Cohort Study

Study ObjectiveOur objective was to determine if ovarian surgery at the time of ovarian detorsion is associated with impaired short-term ovarian function as indicated by ovarian morphology on ultrasound when compared with detorsion alone.DesignRetrospective cohort studySettingThe Hospital for Sick Children, TorontoParticipantsPatients ≤ 18 years old with confirmed ovarian torsion from January 1, 2004, to December 31, 2018, with ovarian-sparing surgery.Main Outcome MeasuresData were collected on demographics, procedure, intraoperative findings, and postoperative ultrasound. To determine ovarian function, we compared the morphology on the postoperative ultrasound between those with surgery to the ovary and those without surgery to the ovary at the time of detorsion. We also compared the ovarian volume of affected and contralateral ovaries after detorsion and surgery to the affected ovary.ResultsOne hundred and nineteen patients met the inclusion criteria, of whom 67 (56%) had detorsion with surgery to the ovary and 52 (44%) had detorsion alone. There was no statistically significant difference in appearance on the postoperative ultrasound between these groups (P =.446). There was also no statistically significant difference on the postoperative ultrasound of affected and contralateral ovarian volumes after detorsion and surgery to the affected ovary (P = .69). Patients who underwent surgery to the ovary experienced a lower rate of recurrence; however, this did not reach statistical significance, with a P value of 0.080.ConclusionOur study demonstrates that surgery, eg cystectomy to the ovary at the time of ovarian detorsion, does not appear to impact ovarian function when compared with detorsion alone, as indicated on postoperative imaging. There was also no difference in volume of the affected and contralateral ovaries in those cases that underwent surgery at the time of initial detorsion. This evidence would support that immediate cystectomy at the time of initial ovarian detorsion is not associated with impaired ovarian function, thus avoiding the need for an interval cystectomy.     

Sertoli–Leydig cell tumors of the ovary: A Taiwanese Gynecologic Oncology Group study

ObjectiveTo report the natural history and prognosis of the uncommon Sertoli–Leydig cell tumor (SLCT) of the ovary.Materials and MethodsA 20-year retrospective review was conducted by the Taiwanese Gynecologic Oncology Group (TGOG), including nine tertiary medical centers from different regions in Taiwan. The medical records for 40 cases of ovarian SLCT were collected. Pathology reviews were carried out by a panel of expert pathologists.ResultsAfter pathological review, 17 patients were subsequently excluded because the pathology slides were unavailable in five cases, and discrepant results from the initial diagnosis were found in 12 cases (34%). For the remaining 23 patients, the median age was 41 years. The most common symptom was irregular vaginal bleeding followed by an abdominal mass or amenorrhea. Most of the tumors were unilateral and confined to the right ovary, with an average size of 8.2 cm. Preoperative serum markers were available for 12 patients and were elevated for three patients. All patients underwent primary surgery. Six patients accepted adjuvant chemotherapy, and bleomycin, etoposide, and cisplatin were used in four of them. Clinical follow-up information was available in 21 patients with a median of 19 months. Eighty-two percent of patients were alive and free of disease up to the date of the last follow-up. Two patients died of the disease.ConclusionThis study demonstrates the extreme rarity of ovarian SLCT in Taiwan. Histological discordance between the diagnosis and central review proves the need for expertise review before treatment. For an improved understanding of the biological behavior and treatment strategy for this unique tumor, international collaboration is imperative.     

Topical Hemostatic Agent May Be Mistaken for Ovarian Teratoma

BackgroundOvarian teratomas are common, as is use of topical hemostatic agents in ovarian surgery.CaseFollowing laparoscopic right ovarian cystectomy, a flowable hemostatic agent was placed in the ovarian bed. Postoperative ultrasound demonstrated an enlarged heterogeneous right ovary with solid and cystic components interpreted as residual or recurrent teratoma. Visual inspection during repeat laparoscopy revealed an irregularly shaped, enlarged right ovary with a smooth cortex. A cavity inside the ovary contained brown, shaggy material. Pathological evaluation demonstrated normal ovary and fibrin.Summary and ConclusionWe report the first case of a hemostatic agent routinely used in ovarian cystectomy postoperatively, mimicking a teratoma. Consideration of this finding is important when planning surgery in adolescent patients to optimize fertility preservation and minimize ovarian follicle damage.     

Ovarian Sertoli-Leydig cell tumor with heterologous gastrointestinal epithelium as a source of alpha-fetoprotein: a case report

Ovarian Sertoli-Leydig cell tumors (SLCT) are rare sex cord stromal neoplasms. To date there have been approximately 25 case reports of ovarian SLCT expressing alpha-fetoprotein (AFP). In such cases, AFP was immunohistochemically detected in the Sertoli cells, Leydig cells, or hepatocytes. This case report confirms heterologous gastrointestinal epithelium expression of AFP. A 20-year-old woman presented with complaints of abdominal enlargement and irregular menstrual cycles over one year. A right ovarian tumor was detected and the patient's serum AFP was elevated. A right salpingo-oophorectomy was performed. On microscopic examination, the tumor was composed of a fibrosarcoma-like area and a poorly differentiated SLCT area with heterologous gastrointestinal epithelium. Immunohistochemical analysis detected AFP in the gastrointestinal epithelium only. Postoperatively, serum AFP levels fell to normal. A recurrent tumor was discovered in the omentum after adjuvant chemotherapy, but serum AFP remained normal. A second laparotomy was performed and the recurrent tumor showed only fibrosarcoma-like features. The patient received second line chemotherapy and is currently in remission. This is the first case of AFP production by heterologous gastrointestinal epithelium in SLCT.     

Ovarian pregnancy torsion

Introduction Ovarian-cyst torsion is often seen in early pregnancy due to progesterone stimulation. It is the fifth-most-common gynecological emergency, with a reported incidence of 3%. An ectopic pregnancy implanted in the ovary is rare, occurring in only 0.5–1% of such pregnancies.Case report We presented a case of ovarian pregnancy with concomitant ipsilateral side tubal torsion managed by laparoscopy.Discussion and conclusions Varying density distribution in the ovary can predispose to ovarian torsion. Emergency surgery may offer the possibility of avoiding the ablation of functional ovarian tissue. Early detection and prompt management can preserve fertility and ovarian function. The etiology, presenting syndrome and management are discussed.     

Ovarian morphology and endocrine function in polycystic ovary syndrome

Purpose  

To assess the relationship between ovarian morphology, hormonal levels and anthropometrical characteristics in a group of patients with polycystic ovary syndrome (PCOS).     

卵巢支持莱迪细胞瘤15例临床病理分析

目的:探讨卵巢支持莱迪细胞瘤的临床特点、病理特征、治疗及预后.方法:回顾性分析我院1962年至2009年诊治的15例卵巢支持莱迪细胞肿瘤患者的临床和病理资料.结果:15例术前均未考虑本病,其中13例有男性化、女性化及去女性化表现.6例女性化表现患者同时合并雌激素相关疾病(如子宫肌瘤、子宫内膜癌、子宫内膜增生).14例术中见大体肿瘤,1例因子宫内膜癌手术,术后病理检查发现本病.肿瘤高分化6例,中分化5例(含有网状及异型成分1例),低分化4例.2例低分化肿瘤术中冰冻病理检查分别误诊为卵巢癌及卵巢颗粒细胞瘤.5例患者保留生育功能,其中3例实现生育.8例患者接受分期手术,其中1例术中发现肿瘤大网膜转移.15例中,9例中、低分化者术后接受辅助化疗.随诊2 ～ 34年,14例患者无瘤存活,1例低分化肿瘤治疗后4年复发.结论:支持莱迪细胞瘤难以及时诊断,患者的内分泌表现是提示本病的重要线索.预后良好,对于本病患者可行保留生育功能手术,但需注意低分化肿瘤的复发问题.     

Ovarian Torsion in Premenarchal Girls

Study ObjectiveTo investigate characteristics that differentiate premenarchal girls with ovarian torsion (OT) from those without OT at the time of surgery.DesignRetrospective chart review of 36 premenarchal girls who underwent 42 surgeries for either OT (n = 33) or a nontorsed ovarian mass (n = 9) from 2006 to 2017.SettingLarge, tertiary care academic institution.ParticipantsWe included patients aged 0-12 years with International Classification of Diseases, Ninth Revision codes for torsion of the ovary, adnexa, ovarian pedicle, or fallopian tube. Controls had International Classification of Diseases, Ninth Revision codes for ovarian mass or cyst, who also underwent surgery and did not have OT.InterventionsRecords were reviewed for patient characteristics including laboratory and imaging studies, surgical intervention, and pathologic diagnosis. Fischer exact test and the nonparametric Mann–Whitney U test were used for statistical analysis.Main Outcome MeasuresCharacteristics predictive of OT in premenarchal girls.ResultsTwenty-nine premenarchal patients were diagnosed with 33 episodes of OT. Nine patients underwent surgery for ovarian masses but did not have OT. All patients with OT reported abdominal pain (compared with 55.6% without OT; P < .001) and most had nausea and/or emesis (81.8% vs 33.3%; P < .009). Ultrasound findings of ovarian enlargement and decreased Doppler flow were significant in the OT group (P < .083, P < .009). There were 2 cases of malignancy in each group.ConclusionPatients with OT had significantly more nausea, emesis, and abdominal pain compared with those without OT. Additionally, 2 of 4 malignancies were found in patients with OT, indicating that malignancy should still be considered with large, complex masses.     

A Case of Many Ovarian Cystectomies

EDITORIAL COMMENT: This comment was prepared to discuss the question of whether or not a macroscopically normal ovary should be sectioned for evidence of bilaterality when the opposite ovary is found to contain a cystic teratoma. This practice is often mentioned but, in the experience of the editorial committee, seldom performed. Wedge resection of a normal ovary can result in adhesions and impairment of fertility. Readers are referred to a series of clinicopathological evaluation of286 cases of ovarian teratomas in Turkey (A). In this series bilaterality of the tumour was present in 65.9% (29 of 44) of women with bilateral adnexal masses but only in 1.1% (2 of 182) of women who had a unilateral mass and who had histological examination of a wedge resection from the macroscopically uninvolved ovary. The authors concluded that 'as the risk of bilaterality of the tumour in the normal-appearing contralateral ovary was so small, we no longer perform the routine ovarian wedge biopsy in patients with mature ovarian teratoma, in order not to decrease the fertility potential of the patient'. Readers can decide whether the case presented here is an exception to this rule!

• (A). 

  Ayhan A, Aksu T, Develioglu O, Tuncer S, Ayhan A. Complications and Bilaterality of Mature Ovarian Teratomas. Aust NZ J Obstet Gynaecol 1991; 31: 83–85.

Summary: Over a period of 6 years a young patient had 3 laparotomies resulting in the removal of 5 benign ovarian dermoid cysts and 1 ovarian mucinous cystadenoma. The occurrence of 3 dermoid cysts at I procedure and the recurrence of bilateral dermoid cysts raises questions regarding the histogenesis of these tumours. The subsequent occurrence of a mucinous cystadenoma confirms the known relationship between these benign ovarian neoplasms.     

单侧多囊卵巢与多囊卵巢综合征的比较研究

目的:探讨单侧多囊卵巢的临床病理特点及病因。方法:收集单侧多囊卵巢12例,与8例典型多囊卵巢综合征对照比较,分析其临床表现、生化参数、卵泡液中相关激素水平以及卵巢间质细胞超微结构。结果:单侧PCO患者临床表现多样,和典型PCOS比较,单侧PCO出现月经改变和不孕显著减少。单侧PCO组血清PRL和T水平显著高于正常对照组,P<0.05;LH水平显著低于PCOS组,P<0.01。单侧PCO卵泡液中T和INS的水平比正常侧卵巢组显著升高,P<0.01;LH水平显著低于PCOS组,P<0.05。单侧PCO间质细胞粗面内质网、光面内质网不同程度出现扩张,部分粗面内质网有脱粒现象;线粒体出现扩张。结论:单侧PCO是一种不同于PCOS的特殊疾病类型,其临床表现多样,对卵巢生殖和内分泌功能的损害比PCOS轻。单侧PCO与PRL和卵巢局部T异常分泌均有关。单侧PCO间质细胞超微结构改变介乎正常卵巢和典型PC0S之间。     

Ovarian Ectopic Pregnancy: Laparoscopic Excision and Ovarian Conservation

Study ObjectiveTo show a case of laparoscopic excision of an ovarian ectopic pregnancy and the technique used.SettingA university hospital (Manchester University Foundation Trust).PatientA 23-year-old primigravida presentation at 6 weeks of gestation with an ectopic pregnancy.InterventionA 23-year-old primigravida presentation at 6 weeks of gestation with a 7-day history of light bleeding and intermittent abdominal pain. The examination was unremarkable, and the serum human chorionic gonadotropin level was 7157 IU/L. An ultrasound scan showed an ectopic pregnancy in the right adnexa, and she underwent surgical management. At laparoscopy, both fallopian tubes were noted to be normal with an ectopic pregnancy within the right ovary; 20 IU argipressin diluted in 80 mL 0.9% sodium hypochlorite was injected between the normal ovarian tissue and the ectopic pregnancy to assist hemostasis and hydrodissection. An ultrasonic device was used to incise the ovarian cortex to identify a plane of dissection between the ectopic pregnancy tissue and the normal ovarian tissue. The ectopic pregnancy was excised with conservation of the ovary. The ovary was subsequently closed with absorbable sutures to ensure hemostasis. The ectopic pregnancy was removed in a bag through a 10-mm incision.Measurements and Main ResultsThe patient made an uneventful recovery. The serum human chorionic gonadotropin level in 7 days was <5, and no further medical management was indicated. Histology confirmed a primary ovarian ectopic pregnancy. Ovarian function was not assessed postoperatively; however, she conceived 6 weeks later with an intrauterine pregnancy.ConclusionThis case highlights the importance of considering nontubal ectopic pregnancies when making a diagnosis based on an ultrasound scan. Ovarian preservation with excision of ectopic pregnancy can be achieved using techniques commonly used for ovarian cystectomy. Recourse to oophorectomy should only be considered in the event of acute hemorrhage.     

Ovarian tissue cryopreservation and transplantation: scientific implications

After fresh or frozen ovary transplantation, FSH levels return to normal, and menstrual cycles resume by 150 days, coincident with anti-Müllerian hormone rising to higher than normal levels. AMH then returns to well below normal levels by 240 days, remaining as such for many years with nonetheless normal ovulation and fertility. To date, 20 babies have been born in our program from 11 fresh and 13 cryopreserved ovary transplant recipients with a live baby rate of over 70 % (11 babies from fresh and 9 from frozen). Globally, over 70 live births have been reported for both fresh and frozen ovary transplants with an approximate 30 % live birth rate. Given the rapid rise of AMH after the fall of FSH, with a subsequent AMH decrease with retention of ovarian function, it is tempting to speculate the existence of a shared mechanism controlling primordial follicle recruitment, fetal oocyte meiotic arrest, and recruitment in the adult ovary. With the massive recruitment of primordial follicles observed after human ovarian cortical tissue transplantation, which subsides to an extremely low recruitment rate, we will discuss how this phenomenon suggests a unifying theory implicating ovarian cortical tissue rigidity in the regulation of both fetal oocyte arrest and recruitment of follicles in the adult ovary. As the paper by Winkler-Crepaz et al. in this issue demonstrates, our in vivo results are consistent with the in vitro demonstration that primordial follicles in the fetal cortex are “locked” in development, resulting in meiotic arrest, which spares the oocytes from being rapidly lost all at once (Winkler-Crepaz et al., J Assist Reprod Genet, 1). Winkler-Crepaz et al. demonstrate that follicle loss after ovarian cortex transplantation is unlikely due to ischemic apoptosis, but rather from a “burst” of primordial follicle recruitment. In vivo, primordial follicles are normally resistant to further development or activation to prevent oocyte depletion. The dense fibrous ovarian cortex, through as yet unresolved mechanisms, arrests the further continuation of meiosis and also prevents a sudden depletion of all resting follicles in the adult ovary. Intrinsic tissue pressure is released after cortical tissue transplantation, temporarily resulting in a rapid follicle depletion. These results are consistent with the observation that once the ovarian reserve is reduced in the graft, the rate of recruitment diminishes and the ovarian tissue exhibits a relatively long duration of function.     

Another case of Sertoli-Leydig cell ovarian tumor (arrhenoblastoma). Review of the literature

In reporting another case of Sertoli-Leydig cell tumor (SLCT) treated by conservative management, the principal characteristics of these tumors are reviewed and a general review is made: SLCT (arrhenoblastomas) are rare endocrine tumors (1 p. cent of tumors of the ovary). They most frequently occur in young women, are unilateral in 98 p cent of cases, and are manifest after a long period by endocrine signs (75 p. cent of cases) whose most frequent combination is: amenorrhea-hirsutism-hoarse voice. They are classified in four histological types which have different prognoses. Their potential development varies and is variously assessed: these are tumors with a low incidence of malignancy (mean 18 p. cent). Under certain conditions, this permits the institution of conservative therapy conserving future fertility. Malignant types of tumors are treated in the same way as other malignant ovarian tumors: surgery is the main tool; radiotherapy is often insufficient and chemotherapy often provides remission more frequently than cure (80 p. cent 5 year survival).     

Ovarian size in postmenopausal women

Ovarian volumes have been determined by pelvic ultrasonography in 2246 apparently healthy postmenopausal women of whom 2221 were included in the statistical analysis. Factors associated with gonadal size have been identified, and reference ranges for derived indices have been determined for use (in association with criteria for abnormal morphology) in a screening programme for ovarian carcinoma. The right ovary was present in 98.9% of subjects and the left in 99.1%. The mean (SD; range) of right and left ovarian volumes were 3.58 (1.40; 1.00-14.01) and 3.57 (1.37; 0.88-10.9) ml respectively. Significant predictors of ovarian volume were years since the menopause, weight, parity, age at menopause, a history of hormone replacement therapy, and previously diagnosed breast cancer. Abnormal ovarian volumes were assessed from a score equal to the (observed mean log volume (MLV) minus the predicted MLV)/0.327. A simplified nomogram has been prepared for routine clinical use. The relative abnormality of one ovary was assessed from a ratio score equal to loge (larger ovarian volume/smaller ovarian volume)/0.211 compared with the 99th centile for the Gaussian distribution.     

Ovarian size in postmenopausal women

Summary. Ovarian volumes have been determined by pelvic ultrasonography in 2246 apparently healthy postmenopausal women of whom 2221 were included in the statistical analysis. Factors associated with gonadal size have been identified, and reference ranges for derived indices have been determined for use (in association with criteria for abnormal morphology) in a screening programme for ovarian carcinoma. The right ovary was present in 98·9% of subjects and the left in 99 · 1 %. The mean (SD; range) of right and left ovarian volumes were 3·58 (1·40; 1·00–14.01) and 3·57 (1·37; 0·88–10·9) ml respectively. Significant predictors of ovarian volume were years since the menopause, weight, parity, age at menopause, a history of hormone replacement therapy, and previously diagnosed breast cancer. Abnormal ovarian volumes were assessed from a score equal to the (observed mean log volume (MLV) minus the predicted MLV)/0·327. A simplified nomogram has been prepared for routine clinical use. The relative abnormality of one ovary was assessed from a ratio score equal to log_e (larger ovarian volume/smaller ovarian volume)/0·211 compared with the 99th centile for the Gaussian distribution.     

Ovarian transplantation by selective microvascular anastomoses in the rabbit

Summary. Autotransplantation of the ovary by microvascular anastomoses was performed in 42 rabbits by three different procedures: orthotopic replantation with selective end-to-end anastomoses of the ovarian vessels; heterotopic transplantation in the groin area, with anastomosis of the ovarian vessels on the inferior epigastric vessels; heterotopic transplantation in the groin with the ovary included in a peritoneal sac to prevent adhesions. Three weeks later, at day 3 of a pseudo-pregnancy, the grafts were examined and morphology, presence of adhesions and ovulation were compared with those of a control group. The long-term vascular patency rate was 78%. The ovulation rate was 71.5% in the orthotopic group, 50% in the heterotopic group and 83.5% in the heterotopic group with the ovary in a peritoneal sac. Subcutaneous transplantation of the ovary within a peritoneal sac provides a new and useful experimental model for the study of tubo-ovarian function and for recovery of postovulatory oocytes.