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Introduction: Ovarian granulosa cell tumor (OGCT) is a rare female pathology with few available demographic data. Besides, there are no comprehensive clinical characteristics regarding the OGCT in Iran. Thus, this study aimed to assess the clinical features and survival rate of OGCT patients in Iran to expand the scope of knowledge in this field., Materials and Methods: In this 10-year retrospective study (2013-2023), the cases were gathered from the oncologic clinic of women (Imam Khomeini Hospital, Tehran, Iran). The patients with definite OGCT diagnosis were selected based on the inclusion and exclusion criteria including medical history, interfering backgrounds, demographic data, histopathological assessment, clinical and para-clinical features, survival rates, and all previous medical reports for definite diagnosis of OGCT along with approved pathology samples., Results: The median age and BMI values of Iranian patients were 45 (19 ~ 83) years and 28.04 (19.4 ~ 48.0), respectively. The most common symptom was abdominal pain (56%) and 69.2% of cases were menopause. In 81.3% of cases, ovarian tumors were detected and metastasis was rare. Most patients (40.6%) underwent total abdominal hysterectomy and OGCT relapsing cases were seen in 13.2% of patients. The median of overall survival (OS) value using the Kaplan-Meier estimate was 52 months (95%CI:37.47-66.53), and the median of disease-free survival (DFS) was 45 months (95%CI: 28.88-61.12). There was a significant (p < 0.05) relation between chemotherapy and left oophorectomy with OS. A significant (p < 0.05) correlation was also detected among the OGCT stage and left oophorectomy with DFS., Conclusion: OS and DFS values showed that the OGCT in Iranian patients can be treated in most cases using two main procedures of chemotherapy and oophorectomy. Parallel application of both procedures and associated outcomes are suggested for future studies., (© 2024. The Author(s).)

Purpose: Granulosa cell tumors (GCTs) of the ovary are rare but clinically significant malignancies. Despite advances in treatment, recurrence has remained a substantial challenge. This study aimed to identify clinical outcomes and potential prognostic risk factors for recurrence in patients diagnosed with GCTs., Methods: In a retrospective cohort study, the ovarian cancer database of the gynecological tertiary referral cancer center, Mashhad University of Medical Sciences, Mashhad, Iran, was searched from August 2012 to August 2023 to find GCT cases. Demographic, clinical, pathological, intervention-related factors, follow-up, and survival findings were meticulously collected. Data were analyzed using SPSS v 23., Results: Ninety-two patients with GCTs, including 86 AGCT and 6 JGCT subjects, were identified. Based on further analysis of AGCT patients, most patients were ages under 50 (58.1%), clinically presented pain (32.6%), and abnormal uterine bleeding (27.9%) as the most frequent symptoms. Stages IA (64.0%) and IC (20.9%) were common. Five-year overall and progression-free survival were 98.2% and 90.8%, respectively. With a median follow-up time of 72 (0.0-180) months, disease recurrence was observed in 19 patients (23.9%), and five patients (5.4%) died of the disease. Stage IV was a hazard factor of recurrence (HR = 7.62, 95%CI (1.89-30.63); p = 0.004)., Conclusions: The present study provides valuable insights into the outcomes and potential risk factors for recurrence in ovarian AGCTs. It duplicates the importance of stage in the prognosis of AGCT patients and highlights the safety of fertility-sparing surgery in stage I and the lack of need to administer chemotherapy in stage IC., (© 2024 The Author(s). Cancer Reports published by Wiley Periodicals LLC.)

Background: Granulosa Cell Tumors (GCT) are considered the most frequent type of sex-cord stromal tumors. These tumors constitute 3-6% of neoplasms of the ovaries. GCTs are divided into 2 types: Juvenile GCT (JGCT) and Adult GCT (AGCT). Most patients are diagnosed early in the course of the disease and tend to have a favorable prognosis. In the surgical treatment of GCT, two main factors play role in the determination of feasibility of the surgery: age and tumor stage., Methods: A retrospective study was conducted on 65 consecutive female patients diagnosed with ovarian GCT at different hospitals across Lebanon who were referred to the National Institute of Pathology, Beirut-Lebanon, between January 2000 and January 2020. Then, they were divided according to types: adult versus juvenile type. Statistical analysis was carried out using Stata, version 16., Results: The incidence of GCT in a Lebanese population was 16.2 per million per year. The mean age of the studied population was 55.6 years. AGCT was the most common with a prevalence of 91% versus 19% for JGCT. Also, inhibine (the most important immunomarker) was found in 77.2% of adult cases. High mitotic index and high tumor size which are predictors for poor prognosis were respectively 20% and 36.9%. Concerning the histopathological features, Grooved nuclei and Exner bodies were less frequently observed in juvenile type (16.7% for both) compared to adult type (36.9%). Most patients with GCT were diagnosed in the early course of disease mainly due to the manifestation of the symptoms as abdominal pain, postmenopausal bleeding or intermenstrual bleeding, and the good diagnosis and screening practices in Lebanon. Regarding the recurrent cases, a significant correlation with high mitotic index (76.9%), high tumor size (92.3%) and advanced stage (46% for stage 3 and 46% for stage 4) was found with a p < 0.05., Conclusions: The incidence of GCT in the Lebanese population is 16.2 per million per year. The majority of patients with GCT in Lebanon are of Adult type representing around 90% of cases. Older age, high mitotic index and big tumor size are predictors for poor outcomes., (© 2024. The Author(s).)

Objective: ovarian granulosa cell tumor (OGCT) is a kind of infrequent ovarian malignant tumor with limited epidemiological data available. we established a predictive nomograph to verify the clinical prognosis., Methods: 1005 diagnosed with ovarian granulosa cell tumor (OGCT) were extracted from Surveillance, Epidemiology, and End Results (SEER) public database from 2000-2018. Kaplan-Meier analysis was applied to distinguish risk factors, univariate and multivariate Cox analyses were used to determine the independent prognostic factors for cancer-specific survival (CSS) of OGCT patients. The obtained prognostic variables were combined to construct a nomogram model for predicting CSS in OGCT patients., Results: Model performance was detected and evaluated with ROC curves and calibration plots. Data collected from 1005 patients were divided into two groups: training cohort(n=703,70%) and validation cohort(n=302,30%). The multivariate Cox model identified five covariates including age, marital status, AJCC stages, surgery and chemotherapy as independent interfering factors of CSS. The nomogram has shown a promising and excellent accuracy in evaluating 3 -, 5 -, 8-year CSS in OGCT patients. In terms of the CSS of the training cohort, the AUC values of the 3 -, 5 -, 8-year ROC curves were 0.819,0.8,0.819, while in terms of the CSS of the validation cohort, the AUC values of the validation cohort were 0.822,0.84,0.823, respectively. All the calibration curves showed pleasant consistency between predicted and actual survival rates. The nomogram model established in the study can improve the veracity of prognosis prediction, thereby improving the accuracy of individualized survival risk assessment, and providing targeted and constructive recommendations for specific treatment options., Conclusion: Age, advanced clinical stage, widower and without surgery therapy are independent risk factors for poor prognosis and the nomogram we constructed can help clinicians efficiently recognize high-risk OGCT patients to guide targeted therapies and improve their outcomes., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Objective: Adult-type ovarian granulosa cell tumors (AGCTs) are hormonally active neoplasms with limited epidemiological data available. We evaluated the effect of parity and postmenopausal hormone therapy (HT) use on the risk of AGCT in a population-based case-control setting., Methods: We identified all women diagnosed with AGCT during 1994-2015 (n = 505) from the Finnish Cancer Registry. For each case, five controls matched for age were selected from the National Population Registry, which also provided data on parity and ages at deliveries. Information on postmenopausal HT by different regimens (estradiol-only, sequential estrogen-progestin and continuous estrogen-progestin) was obtained from nationwide Prescription Register. The association between parity, ages at deliveries, HT use, and AGCT incidence was evaluated by odds ratios (ORs) using a conditional logistic regression model and stratified by age at index date (<55 years or ≥ 55 years)., Results: Parity and age at first or last delivery had no significant effect on AGCT risk. Systemic postmenopausal HT had been used by 20.4% of women who were later diagnosed with AGCT. The risk for subsequent AGCT was significantly decreased among users of estradiol-only therapy for at least five years (OR 0.28; 95% confidence interval 0.08-0.94) and continuous estradiol-progestin therapy for 6 months to 5 years (0.23; 0.08-0.71)., Conclusions: Unlike in epithelial ovarian cancer, AGCT development is not clearly associated with parity, and users of postmenopausal HT do not seem to carry an excess risk for AGCT formation., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To investigate the epidemiology, clinico-pathological characteristics and outcomes of patients diagnosed with malignant ovarian Sertoli-Leydig cell tumors (SLCTs) in comparison to granulosa cell tumors (GCTs)., Methods: The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database were accessed and patients diagnosed with a malignant SLCT and GCT between 1988 and 2013 were selected. Demographic and clinico-pathological characteristics were compared using the Mann-Whitney and chi-square tests. Overall (OS) and cancer-specific survival (CSS) rates were estimated with the Kaplan-Meier method and compared with the log-rank test. Cox hazard models were constructed to control for confounders., Results: A total of 175 and 1361 patients diagnosed with SLCT and GCT, respectively, were identified. Compared to patients with GCT, those with SLCT were younger (median age 32 vs. 51 years, p < 0.001) and more likely to present with larger tumors (median size 15 vs 9.5 cm, p < 0.001) confined to the ovary (77.5% vs 69.2%, p = 0.031). Patients with SLCTs had worse CSS compared to those with GCTs, p < 0.001 (5-year rate was 76.2% vs 90.7%). After controlling for the presence of extra-ovarian disease and tumor size (≤ 10 vs > 10 cm), SCLTs were associated with a worse cancer-specific mortality compared to GCTs., Conclusions: SLCTs are extremely rare, commonly arise in premenopausal patients. They are associated with a poorer prognosis compared to GCT.

Ollier disease (OD) and Maffucci syndrome (MS) are characterized by multiple enchondromas. Patients with MS also have benign vascular overgrowths that become malignant in 8.5% of cases. OD is characterized by multiple enchondromas, typically unilateral in distribution with a predilection for the appendicular skeleton. MS is characterized by multiple enchondromas bilaterally distributed in most of the cases. Both disorders feature multiple swellings on the extremity, deformity around the joints, limitations in joint mobility, scoliosis, bone shortening, leg-length discrepancy, gait disturbances, pain, loss of function, and pathological fractures. About 50% of patients with OD or MS develop a malignancy, such as chondrosarcoma, glioma, and ovarian juvenile granulosa cell tumor. To better understand the natural history of OD and MS, we reviewed 287 papers describing patients with OD and MS. We also created a survey that was distributed directly to 162 patients through Facebook. Here, we compare the review of the cases described in the literature to the survey's responses. The review of the literature showed that: the patients with OD are diagnosed at a younger age; the prevalence of chondrosarcomas among patients with OD or MS was ~30%; in four patients, vascular anomalies were identified in internal organs only; and, the prevalence of cancer among patients with OD or MS was ~50%. With these data, health care providers will better understand the natural history, severity, and prognosis of these diseases and the prevalence of malignancies in these patients. Here, we recommend new guidelines for the care of patients with OD and MS., (© 2020 Wiley Periodicals, Inc.)

Background/aim: We evaluated the incidence of uterine and breast cancer among women diagnosed with granulosa cell tumors (GCTs) of the ovary., Patients and Methods: The US Surveillance, Epidemiology, and End Results (SEER) database was accessed and patients diagnosed with a GCT and had a known follow-up between 1973-2014 were identified. Personal tumor history was extracted and patients with a previous or subsequent malignant breast or uterine tumor were identified. The expected incidence of breast and uterine cancer was calculated based on the U.S age-specific rate of breast and uterine cancer per 100,000 women. Standardized incidence ratio (SIR) with 95% confidence intervals (95% CI) were calculated for each tumor., Results: A total of 1908 cases of GCT were identified. Seventy- nine (4.14%) and 53 (2.78%) patients were diagnosed with a malignant breast and uterine malignancy. The cumulative expected number of malignant breast and uterine tumors was 27 (1.41%) and 6 (0.31%), respectively. The calculated SIR for breast and uterine malignancies was 2.96 (95%CI=2.34, 3.68) and 8.83 (95%CI=6.61, 11.56), respectively., Conclusion: An increased incidence of breast and uterine malignancies among patients diagnosed with GCTs was observed., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Objective: The aim of this study was to determine the incidence of new primary malignancies after adult-type granulosa cell tumor (AGCT) and the incidence of AGCT after breast and uterine cancer using nationwide population-based registry data., Methods: We used the Finnish Cancer Registry to identify all patients diagnosed with AGCT in 1968 to 2013 (n = 986). The number of subsequent primary malignancies among women with AGCT and the number of AGCTs in women with previous breast or uterine cancer were compared with the expected number of cases and expressed as standardized incidence ratios (SIRs)., Results: There were 122 cases of subsequent cancers diagnosed at least 6 months after the primary diagnosis of AGCT (SIR, 1.09; 95% confidence interval [CI], 0.91-1.3). In particular, the observed number of cancers of the soft tissue (SIR, 4.13; 95% CI, 1.33-12.8), thyroid (SIR, 3.42; 95% CI, 1.54-7.62), and leukemia (SIR, 2.67; 95% CI, 0.98-5.82) exceeded the number of expected cases. The SIR for breast cancers after AGCT was 1.26 (95% CI, 0.92-1.73), and the SIR for AGCT after breast cancer was 1.59 (95% CI, 1.04-2.29). The risk for subsequent AGCT was more than 2-fold in breast cancer patients younger than 50 years, and over 15 years after primary diagnosis., Conclusions: There is an increased risk for thyroid and soft tissue cancer as well as leukemia after AGCT, which may be associated with late effects of carcinogenic treatments and possibly shared risk factors. After breast cancer, the risk for AGCT was higher, which may indicate a shared hormonal etiology.

This descriptive study was carried out at Pathology Department, Shifa International Hospital from 2007 to 2016; all sex cord stromal tumours diagnosed during this time period were included. Epithelial, germ cell and metastatic tumours were excluded from the study. A total of 1254 Ovarian tumours were brought to Shifa of which47 (4%) were labeled as sex cord stromal tumours. Of these 36( 76 %)were granulosa cell tumour (adult33, juvenile3), 7 were labeled as sertoli leydig cell tumours (15%), 3 as thecoma/ fibroma group (7%)and only one case was labeled as microcystic stromal tumour of the ovary (2%). Overall age range for sex cord stromal tumours was 42 (12-71). Immunohistochemistry was done in 41 out of 47 cases. Sex cord stromal tumours of the ovary are rare tumours comprising 4% of the total. Adult Granulosa cell tumour is the commonest tumour seen in our study.

Purpose of Review: Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers., Recent Findings: Event-free and overall survival for these cancers is high, and the goals of treatment are minimization of morbidity and preservation of fertility with unilateral salpingo-oophorectomies and limited staging. Surveillance of tumor markers after surgery is helpful in monitoring for disease progression and adjuvant chemotherapy is often reserved for patients at recurrence. Recent literature supports avoiding chemotherapy even in high-grade germ cell tumors in the pediatric population.

Objective: To determine the incidence and occupational variation of granulosa cell tumours (GCTs) in Finland, Iceland, Norway and Sweden over a 60-year period, 1953-2012., Design: A longitudinal cohort study., Setting and Population: Finland, Iceland, Norway and Sweden and a total of 249 million women over a 60-year period (1953-2012). The NOCCA (Nordic Occupational Cancer Study) included 6.4 million women with 776 incident GCT cases diagnosed until the end of follow up., Methods: Incidence rates were calculated from the national cancer registries and compared using quasi-Poisson regression models. Occupation-specific standardised incidence ratios (SIRs) were calculated from the Nordic Occupational Cancer (NOCCA) database., Main Outcome Measures: Incidence rates and standardised incidence ratios., Results: The age-adjusted (World Standard) incidence rates remained quite constant: about 0.6-0.8 per 100 000 for most of the study period. The age-specific incidence was highest at 50-64 years of age. There were no occupations with significantly increased risk of GCT. Major changes in the use of oral contraceptives, postmenopausal hormonal therapy, fertility rate and lifestyle in general during the study period and among different occupational categories do not appear to have a marked effect on the incidence of GCT., Conclusion: Our findings support the concept of GCT as a primarily sporadic, not exposure-related, cancer., Tweetable Abstract: The Nordic incidence rates of GCTs show stability over time and among different occupational categories., (© 2016 Royal College of Obstetricians and Gynaecologists.)

Objective: Models to predict the probability of recurrence free survival exist for various types of malignancies, but a model for recurrence free survival in individuals with an adult granulosa cell tumor (GCT) of the ovary is lacking. We aimed to develop and internally validate such a prognostic model., Methods: We performed a multicenter retrospective cohort study of patients with a GCT. Demographic, clinical and pathological information were considered as potential predictors. Univariable and multivariable analyses were performed using a Cox proportional hazards model. Using backward stepwise selection we identified the combination of predictors that best predicted recurrence free survival. Discrimination (c-statistic) and calibration were used to assess model performance. The model was internally validated using bootstrapping techniques to correct for overfitting. To increase clinical applicability of the model we developed a nomogram to allow individual prediction of recurrence free survival., Results: We identified 127 patients with a GCT (median follow-up time was 131 months (IQR 70-215)). Recurrence of GCT occurred in 81 out of 127 patients (64%). The following four variables jointly best predicted recurrence free survival; clinical stage, Body Mass Index (BMI), tumor diameter and mitotic index. The model had a c-statistic of 0.73 (95% CI 0.66-0.80) and showed accurate calibration., Conclusions: Recurrence free survival in patients with an adult GCT of the ovary can be accurately predicted by a combination of BMI, clinical stage, tumor diameter and mitotic index. The introduced nomogram could facilitate in counseling patients and may help to guide patients and caregivers in joint decisions on post-treatment surveillance., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: To describe the outcome of adult granulosa cell tumor (AGCT) with respect to initial clinical findings, methods of surgery, and perioperative treatment., Study Design: Retrospective follow-up study., Setting: All hospitals in Jutland., Sample: 163 women diagnosed with AGCT., Methods: Follow-up by hospital data files, general practitioner, death certificate, and autopsy report. Revision of histopathology by a single pathologist., Main Outcome Measures: Survival and relapse by clinical data, stage, and type of surgery., Results: The incidence of AGCT was 1.37 per year per 100,000 women (95% CI: 1.08, 1.68). The median follow-up time was 15 years and for the 79 surviving women 22 years. Stage I was found in 94% of cases. Relapse occurred in 24% of women in stage I and 100% of the other stages. Survival in stage I was 95%, 89% and 84% after 5, 10 and 20 years respectively. Increased survival of stage I in postmenopausal women was associated with surgery including hysterectomy and bilateral oophorectomy (p<0.001). In women younger than 40 years no difference in survival was found due to type of surgery. Endometrial carcinoma was found 138 times (95% CI: 48, 275) more prevalent than the expected rate., Conclusion: The survival of women was better in AGCT than in epithelial ovarian tumor. Age and type of surgery, besides stage, influenced survival. Total abdominal hysterectomy and bilateral salpingo-oophorectomy is the recommended treatment with advancing age. At younger age less extensive surgery was associated with similar survival compared to extensive surgery, but with advancing age conservative surgery increased the risk of relapse and death., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Granulosa cell tumors are rare and comprise approximately 2% to 8% of all ovarian malignancies. Research dedicated to these tumors is rare given the low incidence. These tumors are more difficult to diagnose than epithelial ovarian tumors, and understanding how they present may aid in appropriate referral to a gynecologic oncologist. The aim of this review was to summarize the epidemiology, risk factors, and clinical presentation of granulosa cell tumors to aid in provider recognition. We will also explore current diagnostic and treatment modalities with examination of newer, novel treatments. At the end of this review, the reader should understand how to appropriately diagnose and treat these rare malignancies.

Introduction: Studies demonstrate that patient factors such as race, body composition, medical co-morbidities, and medications may be associated with cancer related outcomes independent of the patient's malignancy and related therapies for the cancer. The goal of this study is to determine demographic and prognostic factors affecting disease recurrence in women with early stage ovarian granulosa cell tumors (GCT)., Materials and Methods: This study used a dual-institution retrospective analysis of women diagnosed with GCT between 1995 and 2010. Demographics including age, race, body mass index (BMI), stage, diabetes (DM), adjuvant treatment, and progression-free survival (PFS) were extracted. Hazard ratios for recurrence were estimated by univariate and multivariate Cox regression models., Results: One hundred and four women were identified with a median age of 50 years (range 12-87 years). Fifty-five (58.5%) were Caucasian, 29 (30.9%) African American, and 10 (10.2%) others. Median BMI was 29 kg/m(2) (range 12-57 kg/m(2)). Twenty-one patients had DM. The majority of women had clinical stage I disease (95.0%), 5 (6.4%) had stage II/III disease, and 5 were unstaged. In univariate analysis among early stage disease, DM showed the strongest association with recurrence (HR=3.37, 95% CI=1.38-8.20). In multivariate analysis, DM was associated with an HR of 3.19 for recurrence (95% CI=1.08-9.44)., Conclusions: Our results emphasize that diabetes is one of the strongest predictors of recurrent disease in patients with ovarian GCTs. As this disease is characterized by long disease-free intervals prior to recurrence, this may serve as a potential chemopreventive strategy in patients felt to be at higher risk for recurrence., (© 2013.)

Objective: Concurrent presence of endometrial hyperplasia or cancer in patients with granulosa cell tumors (GCTs) is common, with reported incidences of 25.6% to 65.5%. Consequently, bilateral salpingo-oophorectomy and hysterectomy is usually recommended in patients with a GCT, but this remains debatable. Our aim was to evaluate the need for hysterectomy in patients with GCTs by studying the incidence of pathologically confirmed endometrial abnormalities at the time of diagnosis of GCT and during follow-up., Materials/methods: All cases of GCT between 1991 and 2012 were evaluated for endometrial pathology using the Dutch nationwide network and registry of histopathology and cytopathology (PALGA)., Results: A total of 1031 cases of GCT were identified at a mean ± SD age of 55 ± 17 years. The incidence of GCTs in the period 1991-2012 was 0.61 per 100,000 women per year. Concurrent endometrial cancer at the time of diagnosis of GCT was found in 58 patients (5.9%) and endometrial hyperplasia in 251 patients (25.5%), including complex hyperplasia in 89 patients (9.1%) and simple hyperplasia in 162 patients (16.5%). Long-term follow-up of 490 patients (47.5%) without a hysterectomy showed that endometrial abnormalities were found in 10 patients (2.0%) of which 2 had endometrial cancer. Interestingly, 8 (80%) of the 10 patients with endometrial abnormalities had recurrent GCT at the time of diagnosis of endometrial hyperplasia or cancer., Conclusions: Our data suggest that after surgical removal of GCT, development of an endometrial abnormality, especially cancer, is very rare. Therefore, hysterectomy is not recommended in patients with a GCT without endometrial abnormalities at the time of diagnosis.

Background: Granulosa tumors were described for the first time in 1855 by Rokitansky. These tumors are malignancies with a relatively favorable prognosis. They are characterized by a prolonged natural history and a tendency to late recurrences. The aim of this study is to investigate the epidemiological and pathological characteristics of granulosa cell tumors and to investigate the prognosis factor for recurrences., Methods: The clinical data of patients who were treated in the period from January 2003 to December 2010 at the National Institute of Oncology in Rabat, Morocco for adult granulosa cell tumors of the ovary were investigated retrospectively. Data for age, clinical manifestation, imaging, diagnosis and treatment of the patients were reviewed and analyzed. Post-operative histology was obtained for all patients., Results: Twenty-seven cases were retrieved. The median patient age was 53 years. The most common clinical manifestations at diagnosis were abdominal pain and vaginal bleeding. Mean tumor size was 14 cm. The majority of patients had stage I (63%, n = 17), while (18,5%, n = 5) had stage III, (7.4%, n = 2) had stage IV, and (11%, n = 3) of patients had an unknown stage. In the follow-up period (median = 63.44 months), five (18.51%) patients relapsed. The median time to relapse was 41.8 months, (range: 18 to 62 months)., Conclusions: Granulosa cell tumor of the ovary is an uncommon neoplasm. The adult form progresses slowly and often is diagnosed in an early stage of disease. Surgery is indicated. A prolonged post-therapeutic follow-up is necessary because of the risk of recurrences, late and exceptional for the adult form.

Introduction: Granulosa cell tumors of the ovary (GCT) are derived from the sex cords and the ovarian stroma. Their natural history however is indolent with a very favorable long-term prognosis. Their extreme rarity represents a limitation in our understanding of their natural history management, and prognosis., Material and Methods: Retrospective analysis of patient documentation treated for GCT between 1988-2008 at the Maria Skłodowska-Curie Memorial Cancer Centre, Warsaw, was performed. Clinical and pathological features of the study group, as well as methods and results of the treatment were analyzed., Results: Medical documentation of 148 patients was analyzed. The majority of patients was classified as FIGO stage 1 (87.5%). Surgery was performed as primary treatment in all cases. Forty eight patients (32,6%) were held for observation stays, whereas 57.1% were qualified to receive adjuvant treatment: chemo- or radiotherapy. Mean progression free survival was 133.5 months (11.1 years) and was significantly longer in patients treated with the chemotherapy regimen when compared to radiotherapy (148 vs. 91 months respectively; p = 0.028). Overall survival was 173,7 months and was significantly longer in patients treated with adjuvant chemotherapy vs. RTH (165 vs. 121 months; p = 0.068). Recurrence of the disease was associated with poorer prognosis., Conclusions: GCTs are potentially curable neoplasms of the ovary with low treatment failure rates. Quick diagnosis and appropriate treatment in centers experienced in ovarian cancer surgery are the necessary conditions to obtain good results. The stage of the disease remains the most important prognostic factor chemotherapy with the use of bleomycine etoposide and cisplatin should be considered in patients who require adjuvant treatment.

Aims: To analyse and describe cases of unexpected ovarian malignancy after conservative laparoscopic surgery for an apparent benign ovarian mass at our centre., Methods: This retrospective study was performed by collecting data from records of premenopausal women who underwent conservative laparoscopic surgery for benign ovarian mass at Siriraj Hospital over a 5 year period from 2006 to 2010. The rate of unexpected malignancy, histopathological type of the ovarian neoplasm and follow-up after surgery were reviewed., Results: During the study period, 1,161 patients underwent conservative laparoscopic surgery. Of these, five (0.43%) had ovarian malignancy which were diagnosed after reviewing histopathological report postoperatively. The histopathological diagnosis was borderline tumour in two patients, clear cell carcinoma, granulosa cell tumour and mucinous cell adenocarcinoma in the other three patients. All patients are alive after a mean follow-up of 9-60 months with two patients developed recurrence., Conclusion: The main concern for laparoscopic management of ovarian masses is unexpected malignancy. However, with careful patient selection, proper technique and an experienced operator, laparoscopic treatment of ovarian masses is the best approach and should be implemented, with low risk of unexpected malignancy and recurrence.

The objective of this study was to determine if follow-up is required for all ovarian tumours incidentally diagnosed in postmenopausal women, by studying the prevalence and histology of ovarian tumours in postmenopausal women undergoing hysterectomy. The histopathology of adnexa in 100 consecutive postmenopausal women who underwent an abdominal hysterectomy with bilateral salpingo-oophorectomy for various indications, was reviewed. A total of 200 adnexa were examined. Ovarian pathology was found in 62/200 (31%). Of these 34/62 (53%) were unilocular cystic tumours, 9/62 (15%) were multilocular tumours, 11/62 (18%) were solid tumours and 8/62 (11%) were uni or multilocular with solid elements. The prevalence of borderline tumours was 4% and that of malignant tumours was 5%. All tumours < 2 cm were found to be benign. All unilocular cysts < 5 cm were benign. In conclusion, a vast majority of ovarian tumours in this group of women were benign. It may be reasonable not to follow-up women with unilocular ovarian tumours < 5 cm who have a normal CA125.

Background: In Mexico, ovarian cancer represents 5.3% of cancer diagnoses in all age groups and 21% of gynecologic cancers. The states with the highest incidence of this disease Nuevo León, Mexico State and Federal District., Objective: To determine the epidemiological profile of ovarian cancer., Patients and Methods: A retrospective cross-sectional study that included all patients with complete records, diagnosed with ovarian cancer treated at the Oncology department UMAE Monterrey No. 23, January 2009 to 31 December 2009., Results: We identified 40 patients with ovarian cancer. The average age of menarche was 12.7 years, 40% were of reproductive age, 25% were nulliparous, 15% had a pregnancy and 37.5% had two pregnancies. Of the total patients, 17% had a history of breast cancer, 40% used a contraceptive method, 37% used oral contraceptives. The tumor marker CA 125 was found in 40% of patients, 63.1% had ultrasound markers for cancer. The most frequent clinical stage 1A in which they found 32% of cases. Papillary serous adenocarcinoma was diagnosed in 25% of patients, endometroid adenocarcinoma and mucinous tumor of low malignant potential was diagnosed borderline at 20%, poorly differentiated adenocarcinoma in 18% tumor granulosa cells in 7% and papillary adenocarcinoma ring cell adenocarcinoma in 5%. In total, 43% of patients received chemotherapy., Conclusion: The majority of cases tenia50 years or more. The background was the most frequent hereditary breast cancer. There were no deaths during the study.

Granulosa cell tumours are rare, potentially malignant sex cord stromal tumours of the ovary. They are unique in their presentation and histological features. Many of them are hormone-producing and this property helps them to present early unlike other epithelial ovarian cancers. As a result, most of them will be in an early stage at the time of initial diagnosis. The tumour can manifest in young girls as a juvenile form and conservative management with unilateral salpingo-opherectomy may be an option in them as 95% are unilateral. Surgery is the treatment of choice and initial staging laparatomy a determinant recurrence. Advance stage of the tumour, its size (>5 cm), mitotic figures (>10/hpf), nuclear atypia and absence of call-exner bodies are poor prognostic factors. Such tumours are characterised by late recurrences and this necessitates a prolonged follow-up. Tumour markers such as inhibin and estradiol are useful in follow-up. Chemotherapy, radiotherapy and hormone replacement therapy have very little role in the initial treatment and may be suggested in case of recurrences. With appropriate treatment, a better survival rate can be achieved as against other ovarian malignancies. Methods used for locating, selecting and synthesising data: A search of Medline and Cochrane data base for the period from 1999 to 2010 was carried out to include relevant systematic reviews, meta-analysis, randomised controlled and other clinical and rare case reports. The date of the last search was January 2010.

Purpose of Investigation: To determine risk factors for prognosis and recurrence in ovarian adult-type granulosa cell tumor (GCT)., Methods: A retrospective review of GCT cases treated at our university hospital between 1991-2006., Results: Of 39 patients with GCT, 82% had Stage I disease. The median follow-up period was 71 months. There were 12 cases of recurrence (30.8%) and seven of them had died of disease. The pelvis and liver were the most common sites of recurrence (8 and 3 patients, respectively). Interestingly lymph node recurrence was encountered in two patients. Estimated disease-free survival for five years was 82%. Stage and presence of residual tumor were calculated to be the only associated risk factors for recurrence and prognosis (p < 0.05)., Conclusion: Recurrences in GCT might be associated with stage and presence of residual tumor during primary surgery. Although rarely present during diagnosis, lymph node metastasis might be more common in recurrent disease.

Objective: This study identified risk factors for ovarian granulosa cell tumors (GCT) through a case-control study comparing women with GCT to women with epithelial ovarian cancers (OC) and general population (GP) controls., Methods: Women with GCT and OC were identified from our hospital tumor board and the Massachusetts and New Hampshire Statewide Cancer Registries between January, 1988 and November, 2008. Age, gender and county matched GP controls were identified through town books in Massachusetts and drivers' license lists in New Hampshire. Epidemiologic factors including age, race, obesity, pregnancy history, smoking, and family history were evaluated. Odds ratio (OR) was calculated and adjusted for race and age., Results: Seventy-two women with GCT, 1578 GP controls, and 1511 OC controls were identified. Patients with GCT were significantly more likely to be non-white (OR 8.49; 4.07, 17.7), obese with a BMI >30 (OR 5.80; 3.01, 11.2), and have a family history of breast (OR 2.13; 1.19, 3.80) or ovarian cancer (OR 2.89; 1.08, 7.72) than GP controls. The risk of developing GCT was significantly decreased in women who smoked (OR 0.46; 0.27, 0.78), used oral contraceptive pills (OR 0.32; 0.17, 0.63) or were parous with 1-2 (OR 0.30; 0.16-0.56) or greater than 2 births (OR 0.50; 0.27, 0.94) when compared to GP controls., Conclusion: These findings suggest an independent association between non-white race and obesity as a hyperestrogenic state in the development of GCT while parity and OCP use may be protective. An unknown familial predisposition for GCT may exist.

Background: The present study aims to characterize the women diagnosed with synchronous primary gynecological tumors with an emphasis on risk factors., Methods: A total of 21 patients were identified with synchronous primary gynecological tumors between 2000 and 2006. Demographic, clinical and pathologic data were obtained from medical records and pathology reports., Results: The majority of the study population (52.4%) was diagnosed with independent primary endometrial and ovarian tumors. The most common presenting symptoms were pelvic pain and abnormal vaginal bleeding. Tobacco use was significantly more frequent in women with synchronous cervical-ovarian tumors. There was no statistically significant difference in exogenous hormone use among patients with different synchronous tumors. Diabetes mellitus and hypertension were significantly more frequent in women with endometrial-ovarian tumors. Although the women with synchronous cervical-ovarian tumors were significantly younger and leaner, they had shorter survival periods., Conclusion: Synchronous primary gynecologic tumors are usually detected in relatively older, overweight, multiparous and postmenopausal women with personal history of diabetes mellitus or hypertension. Synchronous primary tumors of endometrium and ovary are supposed to have better prognosis as they are diagnosed at early stage and low grade.

Granulosa cell tumors of the ovary are rare neoplasms that originate from sex-cord stromal cells. The long natural history of granulosa cell tumors and their tendency to recur years after the initial diagnosis are the most prominent of their characteristics. The secretion of estradiol is the reason for signs at presentation such as vaginal bleeding and precocious puberty. Abdominal pain and hemoperitoneum, which occasionally can occur, are attributable to tumor rupture. The most common finding in pelvic examination is a tumor mass, which is subsequently confirmed with imaging techniques. Surgery is the mainstay of initial management for histological diagnosis, appropriate staging, and debulking. A more conservative unilateral salpingo-oophorectomy is indicated in patients with stage I disease and patients of reproductive age. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the appropriate surgical treatment for postmenopausal women and those with more advanced disease. The stage of disease is the most important prognostic factor associated with the risk of relapse. There are no clear conclusions regarding the role of postoperative chemotherapy or radiotherapy in stage I disease and in those with completely resected tumor. The use of adjuvant chemotherapy or radiotherapy has sometimes been associated with prolonged disease-free survival and possibly overall survival. Chemotherapy is the treatment of choice for patients with advanced, recurrent, or metastatic disease, and BEP (bleomycin, etoposide, and cisplatin) is the preferred regimen. Although the overall rate of response to treatment is high, the impact of treatment on disease-free or overall survival is unknown. Prolonged surveillance is mandatory because tumors tend to recur years after the initial diagnosis.

The objective of this study was to determine the clinicopathologic prognostic factors in adult granulosa cell tumors of the ovary. A retrospective review of the records of patients of granulosa tumors who were treated at our institute over a period of 10 years (1995-2005) was done. Clinical, pathologic, and follow-up data were collected. A total of 34 patients who were treated during this period were subjected to analysis. Cox univariate analysis and Wilcoxon's test for multivariate analysis were used as part of the SPSS software for examining the data. It was found that optimal cytoreduction (P = 0.02), presence of nuclear atypia (P < 0.001), and increased mitoses (P = 0.03) were the three factors that impacted significantly on survival. Age, stage of the tumor, parity, and size of the tumor had no significant effect on survival. Patients who received chemotherapy had a better median disease-free survival than those who did not (60 vs 48 months), but this did not reach statistical significance (P = 0.08). Optimal cytoreduction, nuclear atypia, and increased mitoses are the statistically significant prognostic factors and may be used for selecting patients for adjuvant therapy.

In 167 cases of granulosa cell tumour (GCT) in the gerbil, Meriones unguiculatus, 42.5% (71 cases) were in the ovaries, with no evidence of macroscopic lesion. In this paper, we describe the common findings in the incipient--microscopic--type of this spontaneous ovarian tumour occurring in gerbils younger than two years of age.

Of 1489 slaughtered cattle, 11 had ovarian granulosa cell tumours (GCTs). These GCTs were examined immunohistochemically for proliferating cell nuclear antigen (PCNA) and oestrogen receptor (ER) in relation to histopathological features (growth pattern, nuclear atypia and mitotic count). On the basis of nuclear atypia and mitotic count, the prognosis for GCTs with a diffuse growth pattern appeared less favourable than that for GCTs with a follicular or trabecular pattern. Increased PCNA expression was significantly associated with nuclear atypia but not with histological growth pattern or mitotic count. A novel finding was the presence of ERbeta but not ERalpha in bovine ovarian GCTs. However, ERbeta expression did not appear to be related to the histopathological features examined. The results indicate that PCNA expression may be of value in establishing the biological behaviour of bovine GCTs. However, a larger series of bovine GCTs should be examinated to assess the prognostic significance of ERbeta.

Adult granulosa cell tumors of the ovary are rare neoplasms, accounting for less than 5% of all ovarian malignancies. In addition to the tumor stage, residual disease, patient age, tumor size, extent of surgery, and also some histologic factors have been reported to be of prognostic importance. Tumor registries were screened for all patients treated between 1979 and 1998 for ovarian tumors at the University of Istanbul. There were 952 ovarian carcinomas, of which 47 were granulosa cell tumors. All charts were reviewed, and the clinical data were extracted. Prognostic factors and treatment results were evaluated retrospectively. The median follow-up was 84 (range: 6-141 months) months. According to univariate analysis, there were only two significant factors for overall survival (OS): stage and presence of residual disease. The OS of the 23 patients with early stage (mean, 122 months; median, unreached) was significantly (p = 0.0001) better than the OS of the 22 patients with advanced stage (mean, 34 months; median, 21 months). A significant difference (p = 0.0004) in OS was also observed between patients with residual (mean, 42 months; median, 21 months) and nonresidual (mean, 108 months; median, unreached) disease. In a multivariate analysis, only stage remained statistically significant (p = 0.0001). The overall 5-year survival rate was 55% and median survival after recurrence was 21 months. Despite the small number of patients, the study showed that stage and macroscopic residual disease are significant prognostic factors. The benefit of chemotherapy and radiotherapy remains controversial.

Adult granulosa cell tumor (GCT) of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to secrete sex steroids such as estrogen. Patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor-derived estrogen. In addition, GCT is a vascular tumor that may occasionally rupture and result in abdominal pain, hemoperitoneum, and hypotension, mimicking an ectopic pregnancy in younger patients. GCT is usually associated with a mass on pelvic examination that is subsequently confirmed on ultrasonography. Surgery is required for definitive tissue diagnosis, staging, and tumor debulking. In older women, a total abdominal hysterectomy and bilateral salpingooophorectomy are typically performed. In women of childbearing age, a more conservative unilateral salpingo-oophorectomy may be performed, assuming that careful staging reveals that the disease has not extended outside of the involved ovary and that a concomitant uterine cancer has been excluded. Survival of patients with GCT is generally excellent because most patients present with early-stage disease, although certain high-risk patient groups may be identified. Stage is the most important prognostic factor, with a higher risk of relapse being associated with stages II through IV disease. In addition, patients with stage I disease associated with features such as large tumor size, high mitotic index, or tumor rupture may also be at higher risk in some series. The value of postoperative adjuvant therapy for high-risk patients has not been investigated by prospective randomized trials, which are difficult to perform because of the rarity of this tumor. Nonetheless, the use of adjuvant chemotherapy or radiation has sometimes been associated with prolonged disease-free survival in patients with high-risk features. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance with serial physical examination and serum tumor markers such as estradiol and inhibin is reasonable.

Purpose of Review: Granulosa cell tumours of the ovary are an uncommon ovarian sex-cord stromal tumour. These neoplasms provide a spectrum of clinical presentations that span from the first to the tenth decade. Surgery represents the primary therapy for early stage disease; however, management of women with advanced disease is less clear. Because of their relative rarity, evidence to support decision-making in the management of granulosa cell tumours is limited. The purpose of this review is to provide the clinician with an updated knowledge of the clinical and molecular aspects of granulosa cell tumours in order to guide therapy., Recent Findings: The clinical stage, mitotic index and cellular atypia correlate most strongly with prognosis. However, these tumours may demonstrate heterogeneous genetic aberrations that can predict behaviour and response to therapy. Case series and reports suggest that postoperative combination chemotherapy is of most benefit in advanced disease. Serial measurements of serum inhibin may be helpful in the follow-up of these women, particularly in the post-menopausal group., Summary: The pathology and treatment of women with granulosa cell tumours of the ovary is complex. Such women should be managed in a multidisciplinary gynaecological oncology unit. A better understanding of the molecular pathology may assist treatment.

Introduction: Granulosa cell tumour (GCT) represents the largest group of sex-cord stromal tumours and comprises 1.5-3% of ovarian malignancy. The aim is to determine the incidence of the disease, study the profile of local patients, and assess the use of imaging studies in the diagnosis of the tumour., Material and Methods: Clinical records of 19 patients diagnosed with GCT between October 1988 and July 1997 in Kandang Kerbau Hospital (KKH) were reviewed., Results: GCT accounts for 3.5% of ovarian malignancy (54 out of 1552) in Singapore, of which 94.7% are adult GCT. In our study, patients are mainly peri/postmenopausal women (63.2%) in their 50s who experience post-menopausal bleeding. There is a high incidence of association with endometrial hyperplasia (40%). Ultrasound scans are able to predict the size and involvement of the tumour rather accurately. In our study sample, 13 patients (68.4%) presented with Stage 1 of the disease, none with Stage 2, 1 with Stage 3 (5.3%) and none with Stage 4. The other 5 patients (26.3%) were unstaged. Only one patient required adjuvant chemotherapy., Conclusion: The local data with regards to GCT is congruent with those found in foreign literature. However, in our study, there were no patients with recurrence whereas GCTs are known to be late recurring in up to 20% of patients 10-20 years after diagnosis. This is probably attributed to the relatively short period of follow-up in this study. Thus, despite the fact that there is no evidence of recurrence of disease in our current study, we still recommend a vigilant follow-up protocol on all patients as literature has proven that with early detection of recurrences, it is possible to achieve complete cure.

Ovarian granulosa cell tumour (GCT) is a rare malignancy, which has been linked to both infertility and infertility treatment with ovulation inducers. The reproductive features were analysed of 146 women with GCT diagnosed between 1956 and 1996. During the study period no changes were found in the mean age (53 years), menopausal status (59% postmenopausal), parity (32% nulliparous) or tumour size or stage at diagnosis. The clinical features in women with GCT at fertile age were compared with GCT diagnosed later in life and to population-based data. Nulliparity (50%) and history of infertility (22%) were more frequent if the tumour occurred at fertile age (n = 50). Of the 12 infertile cases, seven had anovulatory infertility (58%); 11 occurred during the era of ovulation inducers, but only five had used these drugs (clomiphene citrate in five patients, gonadotrophins in two, and tamoxifen in one patient) and no patient had undergone in-vitro fertilization. Endometrial hyperplasia was associated with GCT at all ages, while endometrial cancer was found solely after the age of 45 years. In conclusion, GCT at fertile age is associated with nulliparity and with a clinical presentation of anovulatory infertility, while GCT later in life is associated with a more normal average fertility pattern and with occurrence of endometrial cancer.

The genesis of ovarian granulosa cell tumours (GCT) has been linked to high gonadotrophin levels, and therefore also to the use of ovulation inducers and combined contraceptive pills (OC). We analysed the occurrence of GCT in the whole of Finland in 1965-1994, the period when ovulation inducers and OC became available. All women with GCT were traced from the Finnish Cancer Registry. The numbers of courses of clomiphene citrate and gonadotrophin (human menopausal gonadotrophin: HMG) and number of courses of OC used in Finland during the same period were calculated from sales statistics on these agents. In 1965-1994, 590 patients contracted GCT. The incidence of GCT declined by nearly 40% from 0.74/100000 in 1965-1969 to 0.47/100000 in 1985-1994, a fall occurring at the same time that the use of clomiphene citrate increased 13-fold, that of HMG 200-fold and that of OC 5-fold. Our nationwide data on the incidence of GCT falling concomitantly with increasing use of ovulation inducers can be seen as one piece of evidence that ovulation inducers are unlikely to cause GCT.

Juvenile granulosa cell tumor (JGCT) of the ovary has been reported to occur rarely in conjunction with Ollier's disease. We report a case of a 13-year-old girl who was noted to have a large abdominal mass at the time of excision of a symptomatic enchondroma of the right femur. Subsequent laparotomy at a tertiary care children's medical center identified a JGCT confined to the right ovary. Review of the literature reveals eight previous cases of JGCT in patients with Ollier's disease. The pathophysiology and possible common causes of both disorders is discussed.

A case of juvenile tumour of the granulosa occurred in a pregnant woman. Outcome was rapidly fatal. These tumours are classed as mesenchyma tumours of the sexual cords of the ovaries. The juvenile form occurs before the age of 30 years in 97% of the cases and has characteristic endocrine and anatomic features. The tumour remains localized in the ovary for a long period and may be treated with conservative surgery to preserve fertility in these young patients. Follow-up is aimed at detecting relapse which may occur early. Prognosis in such cases is very poor. Pregnancy does not modify the prognosis which is generally good.

Background: Juvenile granulosa cell tumors (JGCT) of the ovary are rare in children. The over-all outcome after surgery is relatively good, but the indication and type of complementary treatment for severe forms are still unclear., Population: A retrospective survey of the majority of patients with JGCT of the ovary admitted between 1965 and 1990 to 11 French oncologic pediatric centers was carried out. Medical records including surgical and histological data, were analyzed and each tumor was retrospectively classified by the same pathologist according to the Wollner classification., Results: There were 40 patients aged 7 months to 22 years (mean: 6 years); 28 were less than 10 year old at diagnosis. Three had enchondromatosis (Ollier's disease). At diagnosis, all patients presented with an abdominal tumor, 23 had developed manifestations of precocious pseudopuberty, 2 had signs of virilization after a normal puberty and 2 had secondary amenorrhea. Surgery was always the primary treatment: unilateral ovariectomy in 35 cases, bilateral in 4 and biopsy alone in 1 case. There were 21 stage I, 1 stage II, 16 stage III and 2 stage IV cases. 13 patients received combined chemotherapy and 2 abdominal radiotherapy. 34 patients were alive and disease-free 10 months to 26 years after surgery and 6 died. All 23 patients with precocious pseudopuberty had a favorable outcome., Conclusions: This study confirms earlier reports. Unilateral ovariectomy is the first-choice therapy. There is no evidence that tumors complicated by rupture and hemoperitoneum require chemotherapy. Combined chemotherapy does not appear to improve the prognosis for the rare malignant forms. The factors of good prognosis are age less than 10 years and the presence of precocious pseudopuberty.

Thirty-eight girls with malignant ovarian tumors were reviewed and studied during a 43-year period, 1945 to 1988 inclusive. The age range was 3 to 16 years. Eighteen girls were prepubertal and 16 were pubertal at diagnosis. Precocious puberty was noted in 4 children. The most common presenting symptoms and signs were pain, abdominal swelling, and pelvic mass. Emergency surgery for acute pain (? appendicitis) was needed in only 7 patients. Tumor size and cysts did not relate to histology or outcome. Tumors were classified histologically (germ cell, sex cord/stromal, epithelial tumors). Twenty-one (54%) of the patients survived 1 to 27 years (median, 8 years). Sixteen were left with ovarian tissue, 11 functioning. Diagnosis and treatment have been aided by better noninvasive radiological methods, tumor markers, newer and better chemotherapy, and pathological review and reclassification of these tumors as pediatric experience slowly increases. We can make at least four statements that differ from our past experience: (1) pure endodermal sinus tumor was previously confused histologically with embryonal carcinoma; (2) gonadoblastoma is associated with 45,X/46,Y, 45,X, or 46,Y karyotype and is known to be premalignant; (3) sex cord/stromal tumors are not necessarily malignant and metastatic behavior cannot be predicted from the histology; and (4) currently, epithelial tumors are classified as cystadenomas and are considered to be borderline malignancy. Current management should aim at both cure and preservation of fertility with conservative surgery whenever possible. The future must lie in chemotherapy.

We have found eight granulosa tumours in nine years out of 280 ovarian tumours which were collected in the maternity and neonatology unit in Rabta. The mean age of the patients was 53, the mean parity was 8. Signs of oestrogen excretion were found in all cases and metrorrhagia was the principal reason for consulting doctors in 75% of cases. There was always a palpable mass on clinical examination. Histology was usually only microfollicular. Radical surgical treatment was carried out in all cases and consisted of total abdominal hysterectomy and bilateral salpingo-oophorectomy.

The extent of tumor, as revealed by surgery in 113 primary patients with malignant granulosa-stromal cell tumors of the ovaries (granulosa cell--77, theca cell--17 and mixed theca-granulosa cell tumors--19 patients), was analysed versus duration of disease, laterality of tumor and patient age. Factors which were significantly or as a marked tendency associated with stage III and IV of disease and thus suggested poor prognosis were identified.

Data of an epidemiologic study of granulosa cell tumors in Israel are presented. During the 15-year period of the survey, 172 cases of granulosa cell tumors of the ovary were diagnosed. This represents a yearly incidence of 0.9/100,000 females. The incidence of occurrence was two times higher in women of European/American origin than in those of Asian/African origin. Most patients were 40 to 69 years old. The presenting symptoms were mainly those of abdominal distension and pain and changes in the menstrual pattern. Associated malignancy was relatively common. Carcinoma of the breast was present in 6.4% of patients and endometrial malignancy in 11%. Forty-five percent of patients were diagnosed at Stage I of the disease while 27% were detected at the advanced Stages, III and IV. Factors affecting the prognosis involved the clinical stage of the disease at diagnosis, the type of presenting symptoms, and to a limited extent, the age of the patient.

An analysis of 936 women with granulosa- and theca-cell tumors reported to the Swedish Cancer Registry between 1958 and 1972 showed crude annual incidence rates for these lesions of 0.72 and 0.74, respectively, pre 100 000 of the female population, unchanged during the period. The age-specific incidence rates, both groups taken together, increased almost linearly from 35 to 69 years. The women displayed an increased risk of developing endometrial carcinoma and malignant lymphoma, possibly also breast, colon and thyroid carcinoma.