Malignant Ovarian Sertoli-Leydig Cell Tumor Localized With Selective Ovarian Vein Sampling

Sertoli-Leydig cell tumors (SLCT) are rare, comprising less than 0.5% of ovarian neoplasms. They are most often diagnosed in premenopausal women and may produce androgens, resulting in hirsuitism, voice deepening, frontal balding, terminal hair growth, and clitoromegaly. SLCT are malignant in 15%–20% of cases. We discuss a 25-year-old patient with persistent hyperandrogenemia. Noninvasive imaging cannot conclusively differentiate between SCLT and other diagnoses such as polycystic ovary syndrome, ovarian hyperthecosis, idiopathic hyperandrogenism, idiopathic hirsuitism, and 21-hydroxylase-deficient nonclassic adrenal hyperplasia. Selective ovarian vein sampling revealed a 15-fold greater testosterone production from the right ovary compared with the left, which guided appropriate surgical management.     

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.     

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.     

卵巢上皮性肿瘤的临床病理特征及其细胞周期素D1和p53蛋白表达的研究

目的研究卵巢上皮性癌（卵巢癌）和交界性上皮性肿瘤的临床病理特征及其细胞周期素D1（cyclin D1）和p53蛋白表达的情况,探讨卵巢癌和交界性上皮性肿瘤在发病机制上的联系。方法分析45例卵巢癌（卵巢癌组）和54例卵巢交界性上皮性肿瘤（交界性肿瘤组）的临床病理资料,采用免疫组化法检测两组组织中cyclin D1、p53蛋白的表达情况,并分析其与临床病理特征的相关性。结果（1）临床病理特征：①年龄：交界性肿瘤组平均年龄为42．5岁（14～82岁）,中位数年龄41岁;卵巢癌组平均年龄为53．5岁（26～80岁）,中位数年龄51岁。②分期：按国际妇产科联盟（FIGO）分期标准,交界性肿瘤组Ⅰ期48例、Ⅱ期3例、Ⅲ期3例;卵巢癌组Ⅰ期6例、Ⅱ期8例、Ⅲ期26例、Ⅳ期5例。③病理类型：交界性肿瘤组以黏液型为主[占56％（30／54）],其次为浆液型[其中普通型11例,微乳头型5例;占30％（16／54）];卵巢癌组以浆液型（其中低度恶性19例,高度恶性3例）为主[占49％（22／45）]。④病理分化程度：卵巢癌组高分化5例,中分化17例,低分化或未分化23例。⑤预后：交界性肿瘤组5年生存率为98％,卵巢癌组为51％,两组比较,差异有统计学意义（P=0．000）。（2）cyclin D1和p53蛋白的表达及其与卵巢癌和交界性肿瘤临床病理特征的相关性：卵巢癌组cyclin D1和p53蛋白的阳性表达率分别为31％（14／45）和56％（25／45）,p53蛋白表达强度与病理分化程度呈正相关（r=0．320,P=0．032）;交界性肿瘤组cyclin D1和p53蛋白的阳性表达率分别为69％（37／54）和6％（3／54）。其中,普通型浆液性交界性肿瘤与高度恶性浆液性癌比较（两者cyclin D1蛋白阳性表达率分别为91％和26％,p53蛋白分别为0和58％）,差异有统计学意义（P〈O．01）;而微乳头型浆液性交界性肿瘤与低度恶性浆液癌比较（两者cyclin D1蛋白阳性表达率分别为3／5和2／3,p53蛋白分别为1／5和1／3）,差异则无统计学意义（P〉0．05）。结论cyclin D1蛋白的过度表达常见于卵巢浆液性交界性肿瘤及低度恶性浆液性癌组织中,而p53蛋白的过度表达更多见于高度恶性浆液性癌组织中。卵巢浆液性交界性肿瘤与高度恶性浆液性癌具有不同的发病机制,而微乳头型浆液性交界性肿瘤与低度恶性浆液性癌的关系可能更为密切。     

Recurrent extra-uterine pregnancies. 63 cases treated at the Hospital Center of Libreville from 1985 to 1989]

Ectopic implantation of the fertilised ovum, whether for the first time or as a recurrence, is not uncommon in this department. In a study in the Libreville Hospital group from 1985 to 1989, the authors found 828 cases of ectopic pregnancy (EP) including 63 recurrences, i.e. 7.61 per cent. These recurrences most often involved young women (26 per cent of cases) and nullipara (30%). The chief common etiology for the first occurrence remained tubal infection (57.28 per cent of cases). Treatment consisted most often of radical macrosurgery because of the advanced state of tubal disease, thus leading to the permanent sterilisation of 48 patients, i.e. 71.2 per cent of recurrences. Nineteen patients had a history of previous conservative surgery, including 8 homolateral recurrences (43 per cent). Reducing the incidence of such pathology essentially involves the prevention of gynecological infections, and in particular sexually transmitted diseases, as well as the possibility of early diagnosis of EP. Despite the fact that it seriously compromises the subsequent fertility of these young patients, total salpingectomy remains the treatment of choice for avoiding recurrences.     

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.     

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.     

Comparison of two groups of patients with serous and mucinous types of borderline ovarian tumors

OBJECTIVE: The retrospective comparison of two groups of patients with the most common types of borderline ovarian tumors (BTO): serous and mucinous. METHODS: All patients were treated between 1978 and 2000 at the 2nd Department of Obstetrics and Gynecology Medical University of Gdańsk. The first group consisted of 58 patients with serous BTO. The second group consisted of 48 patients with mucinous BTO. These groups were compared according to such parameters: age at diagnosis, parity, clinical symptoms, localization of tumor, tumor size, type of operation, stage of disease, utilization of adjuvant therapy, presence of recurrence and 5-year survival. Statistical comparisons were made by the chi 2 test. The clinical life table analysis was performed to determine 5-year survival and comparisons were made by means of the Long Rank tests. RESULTS: The mean age of serous BTO was 53.1 +/- 12.8 and of mucinous BTO 43.3 +/- 17.9 (p = 0.0000). In the first group 15.5% and in the second 43.8% women were nullipara (p = 0.0602). The main symptom of serous BTO was pain--53.5% patients and that of mucinous BTO increasing girth--47.9% patients (p = 0.0456). The mean size of serous BTO was 13.7 +/- 6.9 cm and that of mucinous BTO was 20.3 +/- 9.5 cm (p = 0.004). 43 patients (89.6%) with mucinous and 28 patients (48.3%) with serous BTO were at I A stage and 9 patients (15.5%) with serous and only one (2.1%) with mucinous were at III stage (p = 0.0008). The mucinous BTO--affected were more frequently treated conservatively than serous BTO--ones which were rather treated radically with using adjuvant therapy (p = 0.003). Residual disease was observed mainly in patients with serous BTO. 5-years survival was better in patients with mucinous BTO, 87.7% and 85.2% respectively (p = 0.0001). CONCLUSIONS: 1. The mucinous BTO occurred more frequently than serous BTO in women of the childbearing age. 2. The extraovarian disease was observed more often with serous BTO than the mucinous BTO. 3. The mucinous BTO was usually bigger than serous BTO and mainly affected only one ovary. 4. The mucinous BTO was more frequently treated conservatively than serous BTO which were rather treated radically with use of adjuvant therapy. 5. The recurrence rate was higher and the 5-year survival was worse in patients with serous BTO.     

Molecular genetic evidence supporting the neoplastic nature of the Leydig cell component of ovarian Sertoli-Leydig cell tumors.

Sertoli-Leydig cell tumors (SLCT) comprise less than 1% of ovarian tumors. The nature of the Leydig cells has been a subject of controversy and it is unclear whether they are clonally related to the neoplasm or instead proliferate as a non-neoplastic response to the Sertoli-cell component. Twelve ovarian SLCT were identified and hematoxylin and eosin and unstained sections were prepared from formalin-fixed, paraffin-embedded tissue blocks. Tissue samples were microdissected from normal tissue, the Sertoli cell component, and the Leydig cell tumor component using the laser capture microdissection method. If present, tissue was also obtained from any heterologous component. Genomic DNA was extracted from the samples and polymerase chain reaction was used to amplify polymorphic sites at 5 loci: D16S402, TP53, IFNA, D17S855, and D11S1318. X-chromosome inactivation (HUMARA) analysis was also performed. LOH and/or nonrandom X-chromosome inactivation was observed in at least 1 of the 6 amplified loci in the Leydig cell component of 10 of the 12 tumors. LOH and nonrandom X-chromosome inactivation patterns of the Sertoli cell component and Leydig cell component were compared. Concordant allelic loss and/or matching X-chromosome inactivation patterns were observed in 8 (67%) of the 12 tumors. In 7 of these tumors similar LOH or X-chromosome inactivation was observed at 1 site. In 1 tumor similar LOH and/or X-chromosome inactivation was observed at 2 sites. Three tumors had heterologous components. The heterologous components similarly shared LOH/X-chromosome inactivation with the Sertoli cell components at 1 site in 1 case and 2 sites in 2 cases. These data suggest that, at least in some cases, the Leydig cell component of SLCT is neoplastic rather than reactive in nature, and shares a common clonal origin with the coexisting Sertoli cell component. Similarly, the heterologous components, when present, appear to share clonal origin with the Sertoli cell components.     

Current views on treatment of the ovarian granulosa-cell tumor

The granulosa-cell tumor (folliculoma) is a rare type of ovarian neoplasm, accounting for 5% of all cases. It is the most common type of sex cord-stromal tumors, diagnosed in 70% of cases. The granulosa-cell tumor is a hormone active one, originating from granulosa cells which produce estradiol. Overproduction of estradiol is helpful in the diagnosis of the tumor because of its numerous symptoms. There are two types of folliculoma: juvenile (5%) and adult (95%). The juvenile type is mostly recognized (90%) in FIGO I stage and has a better prognosis. Operation is often a sufficient way of treatment in this group. Tumors in higher stages are more aggressive and must be treated further. The adult folliculoma is more aggressive in its nature. Patients with the disease diagnosed in higher stages must be treated by adjuvant radiotherapy or chemotherapy. Recurrence appears often many years after the treatment and has high mortality. Many old (platinum) and new (taxans) agents are active when used in treatment of this type of tumor. Randomized study must be made to establish standard therapy of granulosa-cell tumor. Currently, the most frequent way of treatment is chemotherapy with BEP (Blemycyna, Etopozyd, Cisplatyna).     

Endocrine tumors of the uterine cervix: incidence,demographics, and survival with comparison to squamous cell carcinoma

OBJECTIVE: The aim was to describe the epidemiology of endocrine tumors of the cervix in comparison with invasive squamous cell carcinomas using population-based data reported to the Surveillance, Epidemiology and End-Results (SEER) program. METHODS: Retrospective analysis of actively followed cases reported to SEER from 1973 to 1998. Incidence, demographic characteristics, and survival were compared for endocrine and squamous tumors. RESULTS: There were 239 cases of endocrine tumors and 18,458 cases of invasive squamous cell carcinoma of the cervix included in the study. Mean age at diagnosis was 49 years for endocrine tumors versus 52 years for squamous cell carcinoma (P < 0.01). Endocrine tumors were more likely to present at a later FIGO stage (P < 0.01), and to have lymph node involvement at diagnosis (57 vs 18%, P < 0.01) compared to squamous cell carcinoma. Observed median survival for women with endocrine tumors was 22 months versus 10 years for women with squamous cell carcinoma. Age and FIGO stage-adjusted hazards of death were 1.84 times greater for endocrine tumors than for squamous cell carcinoma (95% CI 1.52-2.23). At all stages of disease, survival was worse for women with endocrine tumors compared to women with squamous cell carcinomas. CONCLUSIONS: Endocrine tumors of the cervix are extremely aggressive and survival is poor regardless of stage at diagnosis.     

Klinische Pathologie des Mammakarzinoms

The pathological characterization of breast cancers today extends beyond traditional typing, staging, and grading. Via immunohistochemistry, intrinsic subtypes, luminal A and B, HER2, and triple-negative types can be determined, which in most cases has a greater impact for the prognosis and prediction of therapy than does traditional histologic typing and stage. With regard to the luminal subtypes, the proliferation marker Ki67 provides a frequently used tool for discriminating between the prognostically favorable A type, which can be sufficiently treated by endocrine therapy alone, from the unfavorable B type, which will need additional chemotherapy. However, with Ki67 there is no commonly accepted threshold for low and high risk. Gene expression profiles are increasingly used for a more precise evaluation of prognosis in breast cancer. Different gene expression profiles are available that reveal only a moderate congruence in risk prediction. Neoadjuvant concepts of therapy with preoperative cytotoxic or endocrine medication offer the opportunity to evaluate the in vivo response of tumors. Complete and partial pathological remission after chemotherapy or dynamic Ki67 reduction following endocrine therapy represents new prognostic and predictive markers provided by pathological tissue examination.     

The changing patterns of occurrence and management in primary mediastinal tumors and cysts in the People's Republic of China

Primary mediastinal tumors and cysts (MTC) constitute a common problem in thoracic operations done in the People's Republic of China. Data from 4,357 instances of MTC diagnosed in China between 1963 and 1985, including 307 from our own facility, are presented. Teratoid tumors are the most common of the MTC in the northern part of China contrasting with thymomas and neurogenic tumors which are the most common in the southern part of China; a finding that notes a significant difference (p less than 0.01). Beyond this, the distribution of MTC is different than in North America. In patients we studied, we were able to predict the correct histopathologic diagnosis 80 per cent of the time by considering the history of the patient and the results of a conventional roentgenogram of the chest. This was increased to 91.4 per cent when needle biopsy was added. Optimal therapy for MTC is complete surgical resection. We present a scheme for localization of the various types of MTC and an operative approach.     

Ⅲ～Ⅳ期子宫内膜异位症患者保守性手术后的结局分析

目的探讨Ⅲ～Ⅳ期子宫内膜异位症患者保守性手术后的复发和妊娠结局。方法回顾性分析90例Ⅲ～Ⅳ期子宫内膜异位症患者的临床资料,均行保留子宫和部分卵巢的保守性手术。16例患者单纯行保守性手术（术后未用药,A组）,52例术后予孕三烯酮治疗（B组）,22例术后予促性腺激素释放激素激动剂（GnRHa）治疗（C组）。3组中不孕症患者分别是10、15和10例。随访24～84个月（中位时间是48．5个月）,比较3组患者的术后复发、妊娠情况及妊娠结局。结果A、B、C3组的复发率分别是18．8％（3／16）、30．8％（16／52）和13．6％（3／22）,3组比较,差异无统计学意义（P=0．247）;3组的平均复发时间分别是术后63．8、63．3和47．6个月,3组比较,差异无统计学意义（P=0．376）。3组中不孕症患者,术后累积妊娠率分别是70．0％（7／10）、66．7％（10／15）和60．O％（6／10）,3组间比较,差异无统计学意义（P=0．890）;术后首次妊娠距手术的时间间隔比较,差异无统计学意义（P=0．092）;而且65．2％（15／23）是在术后1年内自然妊娠。结论保守性手术后采用孕三烯酮或GnRHa巩固治疗未能延缓复发时间,也不能降低复发率。手术能改善Ⅲ～Ⅳ期患者的生育力,但术后药物治疗不能提高妊娠率。     

Tubal histology in extra-uterine pregnancies

141 patients affected by tubal pregnancy have been treated by total salpingectomy at the Port-Royal university clinic between the 1st January 1977 and the 31st January 1984. Tubal pregnancy occurred in the majority of cases in a pathological Fallopian tube, of which the histological state was unrelated to the clinical observations made at laparotomy. Ampullary pregnancy (88% of cases) is secondary to a pathological ampulla in 94 p. cent of cases. The latter was chronic salpingitis in 92 p. cent of cases. Isthmic pregnancy (12% of cases) appears to us to be secondary to isthmic pathology in all cases, and is generally associated with ampullary lesions. There also chronic salpingitis preponderates, whilst endometriosis is very rare. The great frequency of pre-existing lesions which we have noted explains perfectly the high level of recurrences in situ observed after conservative surgery of the gravid tube.     

Safety of conservative management and fertility outcome in women with borderline tumors of the ovary

OBJECTIVE: To assess the safety of fertility-sparing treatment and the remaining chance of childbearing after surgery. DESIGN: Retrospective clinical study. DESIGN: Gynecology department of a university teaching hospital. PATIENT(S): Seventy-five women underwent surgical management in our institution between 1986 and 2001 for borderline tumors of the ovary. INTERVENTION(S): Fifty-nine patients were treated by radical, fertility-compromising surgery. The remaining 16 patients underwent conservative surgery, preserving the uterus and at least some functional ovarian tissue. Seven unilateral adnexectomies, one simple cystectomy, and two adnexectomies associated with contralateral cystectomy were performed. MAIN OUTCOME MEASURE(S): Recurrence, survival, and pregnancy rates. RESULT(S): The observed recurrence rates after radical and conservative surgery were 0.0% and 18.7%, respectively. No disease-related deaths occurred in any group; there is no significant difference in survival rates. We can report 12 pregnancies in 7 of 11 women who underwent fertility-sparing management and who wished to become pregnant. CONCLUSION(S): In certain circumstances, conservative management offers a safe solution for borderline tumors of the ovary. Recurrence is noted significantly more often after this type of treatment, but all cases of recurrent disease can be detected with close follow-up and can be treated accordingly. No significant change in survival rates was found. Moreover, the pregnancy rate in women desiring pregnancy, those treated conservatively, was as high as 63.6%.     

Clinical review of 55 cases of malignant ovarian germ cell tumors

PURPOSE OF INVESTIGATION: A retrospective analysis of 55 cases of malignant germ cell tumors in a 20-year period was done to evaluate the impact of conservative surgery and adjuvant treatment on survival and fertility. METHODS: Fifty-five cases of malignant ovarian germ cell tumors (MOGCTs) were studied. Mean age was 22 years. Dysgerminoma was the most common histotype (45%). RESULTS: Thirty-nine patients (71%) presented with FIGO surgical Stage I disease. Fertility-sparing surgery was performed in 39 (71%) women. Postoperative systemic chemotherapy was administered to 40 women (73%), 27 (68%) had received conservative treatment. One woman developed renal failure after the first cycle of chemotherapy and died a few days thereafter and there was one case of bleomycin-induced death due to pulmonary fibrosis. There were eight (14.5%) clinical recurrences. Overall survival rate for relapsing women was 75% (6/8). The recurrence rate for women treated conservatively was 15%, and it was 13% for those treated radically. With a median follow-up of 129 months the overall survival rate for the entire study-population was 90.9%. Eleven pregnancies occurred in 36 women treated with fertility-sparing surgery who were of child-bearing age. CONCLUSION: The management of MOGCTs with fertility-sparing surgery is a safe, practicable treatment option. The majority of these patients can retain normal ovarian function and reproductive potential after chemotherapy treatment.     

Descriptive epidemiology of gynecologic and breast cancers]

The epidemiology of gynecological and breast cancers are better known in France as a result of the mortality data provided by INSERM and the mortality data obtained from the French Tumor Register. Breast cancers are the most common form of cancer in women, accounting for about 30 p. cent of tumors (excluding skin cancers) followed by cancers of the uterine cervix, uterine body and the ovary. The change in incidence shows a definite reduction in the number of uterine cancers over the past 10 years, whereas the incidence of breast cancers is rising by 1 to 2 p. cent per year. Mortality due to breast cancer has risen steadily in France since 1950, particularly in higher age groups. At birth, the risk of developing a breast cancers is 7 p. cent, i.e. one woman in 14 will develop a breast cancer. The figures for cancers of the uterus and ovary are much lower. Survival curves for various types of cancer confirm the steady decline in survival for breast cancers, whereas for cancers of the cervix, uterine body and ovary, mortality rates stabilize after 5 years. The risk of a secondary cancer remains very high for breast tumors, and half the cases of a secondary tumor involve a contralateral breast tumor. In general, there is an increased risk of a secondary cancer after a primary gynecological tumor.     

Effect of cellular DNA content on the prognosis of epithelial ovarian cancers

OBJECTIVE: To assess the cellular DNA status of epithelial ovarian cancers with regard to clinicopathological findings and its effect on prognosis. MATERIALS AND METHODS: Twenty-six consecutive patients with a diagnosis of epithelial ovarian cancer who had been treated by primary surgery and six courses of platinum-based chemotherapy were enrolled in this study. Second-look laparotomy (SLL) was performed in all cases following confirmation of the clinical remission state. Surgical stage, tumor grade, initial tumor volume, residual tumor volume, histopathologic differentiation, and SLL findings were analyzed in correlation with DNA ploidy and DNA index. DNA analysis was performed via DNA flow cytometry through paraffin-embedded tissue specimens. RESULTS: Of 26 patients, flow cytometric studies revealed 16 aneuploidy cases (61.5%). DNA index values ranged from 1.1 to 1.82 (average 1.29 +/- 0.28). The flow cytometry coefficient of variation mean value was set to 6.7. Taking the cutoff value of 1.2 for DNA indices, a fairly good correlation was detected between DNA ploidy and DNA indices (p < 0.001). The aneuploidy incidence was found to be high in advanced and poorly differentiated tumors (p < 0.05). There was statistically more residual tumor volume in aneuploid tumors during primary cytoreductive surgery and also higher recurrence rates following six courses of chemotherapy compared with diploid tumors (p < 0.05). No significant correlation was detected between the histopathologic subtypes and tumor volume (p > 0.05). Residual tumor volumes were larger in cases with DNA indices of 1.2 yielding higher residual tumor volume following surgery and being in good correlation with SLL results (p < 0.05). The mean survival rates of cases with aneuploid tumor and a DNA index of >1.2 were low compared to those with diploid tumors and DNA indices of <1.2 tumors (p < 0.05). CONCLUSION: DNA ploidy and DNA indices are important prognosticators for malignant epithelial ovarian tumors. They should be evaluated together with the patient's clinical status and other prognostic factors.     

Ovarian sex cord-stromal tumors in children and adolescents

Ovarian sex cord-stromal tumors (OSCSTs) are a heterogeneous group of tumors that develop from the gonadal non-germ-cell component. Despite recent advances in the clinical and histopathologic diagnosis of OSCSTs, a high degree of uncertainty remains with regard to adequate therapy, particularly in patients presenting with microscopic or macroscopic tumor spread. We review the currently available data on the biology and histology of OSCST in children and adolescents. In addition, we summarize the data from our clinical, histopathologic and genetic analyses of patients that were prospectively reported to the German MAKEI protocols for treatment of nontesticular malignant germ cell tumors. Among these patients, juvenile granulosa cell tumors (JGCTs) constitute the most frequent histologic subtype, followed by Sertoli-Leydig cell tumors (SLCTs) and sclerosing stromal tumors. Patients with JGCT and SLCT show greater mitotic activity than do all those with other histologic types. Furthermore, high mitotic activity is associated with adverse outcome. In addition, prognosis correlates with tumor stage according to the International Federation of Obstetrics and Gynecology. Nevertheless, we observed a favorable response to cisplatin-based chemotherapy in the majority of stage II and III tumors. For the whole cohort of 62 patients, event-free survival was 0.87 +/- 0.05 months and overall survival 0.88 +/- 0.05. Genetic analysis of 27 tumors available for comparative genomic hybridization analysis revealed normal profiles in the majority of tumors and whole chromosomal gain, such as a gain of 12 in single tumors, with no consistent pattern with regard to histology or clinical outcome. This analysis confirmed that most OSCSTs present at a low tumor stage and that prognosis in these patients is excellent. Most important, patients at high risk can be identified through clinical and histopathologic analysis, and the majority can be treated successfully with adjuvant cisplatinum-based chemotherapy. Based on this analysis, a prospective study on OSCST in children and adolescents began recruiting cases in 2005.