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Granular cell tumors, uncommon soft tissue growths, predominantly manifest in the subcutaneous and tongue areas, while those in the gastrointestinal tract are scarce and develop slowly. Patients typically show no distinct clinical symptoms and are hard to differentiate from gastrointestinal mesenchymal tumors, smooth muscle tumors, neural sheath tumors, and rhabdomyosarcomas using endoscopy. This paper details a case of a granular cell tumor in the stomach addressed through endoscopic submucosal dissection, focusing on its endoscopic attributes and clinicopathological traits., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

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.)

Aim: To determine the role of preoperative MRI in the diagnosis and treatment of patients with granulosa cell tumors (GCTs) of the ovary., Materials and Methods: Twenty-four patients who were operated on between 2018 and 2022 and who were pathologically diagnosed with GHT and met the inclusion criteria were retrospectively examined. The findings were compared with the patients' demographic data, symptoms, surgical findings (laterality, stage, lymph node involvement, endometrial pathology, tumor size), and CA-125 levels., Results: The final cohort included 24 patients with a mean age of 54.71 ± 16.52. All the patients had the pathological diagnosis of adult type GCT. In the morphological evaluation, the most common finding was a solid-cystic mixed type (14 patients, 58.3%), while intratumoral hemorrhage signal was observed in 10 patients (41.7%). In the majority of cases (91.7%), the mass showed regular contours. The honeycomb/Swiss cheese sign was detected in 54.2% of the cases. When the T1 and T2 signal of the solid component of the mass were examined relative to the myometrium, the majority of GCTs appeared isointense on both sequences (83.3% and 62.5%, respectively). The mean ADC value of the solid component obtained from diffusion-weighted imaging was 0.78 ± 0.15 × 10 -3 . Pelvic fluid was observed in 41.7% of the cases. The average endometrial thickness was 9.74 ± 6.43 mm. Thickened endometrium more than 9 mm was observed in 9 out of the remaining 21 patients (42.9%)., Conclusion: Understanding the key imaging features for GCTs plays an essential role in the diagnosis and guiding the treatment effectively., (© 2024 Japan Society of Obstetrics and Gynecology.)

Adult-type Granulosa cell tumor of the testis is a rare subtype of sex cord-stromal tumors, with fewer than 100 cases reported. The typical clinical presentation is an asymptomatic, painless testicular mass. We report a case of a 16-year-old male with adult-type testicular Granulosa cell tumor who presented with a palpable, painless right testicular mass, and subsequently underwent right inguinal radical orchiectomy. This report contributes to the growing body of literature regarding this rare diagnosis, furthering our understanding of clinical, imaging, and histological findings of its presentation., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of competing interest The authors declare that they have no competing interests.

Background: This retrospective study aims to evaluate the clinical course and long-term outcomes of patients diagnosed with adult granulosa cell tumors (AGCT)., Methods: The study analyzed a cohort of 112 AGCT patients with a median follow-up of 87 months. Data regarding disease-free survival (DFS), overall survival (OS), recurrence rates, and prognostic factors were collected and analyzed. Surgical interventions, including lymphadenectomy and cytoreductive surgery, were assessed for their impact on outcomes., Results: The study revealed favorable long-term outcomes, with a 5-year DFS of 85% and a 10-year DFS of 83%. Additionally, a 5-year OS of 100% and a 10-year OS of 96% were observed. Recurrence occurred in 13.4% of cases, with advanced stage and positive peritoneal cytology identified as independent poor prognostic factors for DFS. Lymph node involvement was rare, and routine lymphadenectomy did not improve outcomes. Conservative surgery showed comparable DFS rates to definitive surgery in early-stage disease. However, cytoreductive surgery was crucial for advanced and recurrent tumors, with complete tumor resection enhancing survival outcomes., Conclusion: The study underscores the importance of vigilant follow-up and individualized treatment strategies for AGCT patients. Despite the retrospective nature of the analysis, the substantial patient cohort and meticulous surgical interventions contribute valuable insights into AGCT management. Prospective multicenter studies are warranted to further elucidate prognostic factors and optimize treatment approaches for this rare malignancy., (© 2024. The Author(s).)

Background: Few studies have evaluated the role of cytoreductive surgery in patients with recurrent adult granulosa cell tumors of the ovary. Despite a multitude of treatment modalities in the recurrent setting, the optimal management strategy is not known. Cytoreductive surgery offers an attractive option for disease confined to the abdomen/pelvis. However, few studies have evaluated the role of surgery compared with systemic therapy alone following the first recurrence and subsequent disease progressions., Objective: This study aimed to determine the impact of secondary, tertiary, and quaternary cytoreductive surgery on survival outcomes in recurrent adult granulosa cell tumors of the ovary., Study Design: This is a multicenter, retrospective cohort study evaluating patients with recurrent adult granulosa cell tumors of the ovary enrolled in the MD Anderson Rare Gynecologic Malignancy Registry from 1970 to 2022. Study inclusion criteria consisted of histology-proven recurrent disease, at least 1 documented recurrence, and treatment/treatment planning at the MD Anderson Cancer Center or Lyndon B. Johnson General Hospital. The primary exposure was cytoreductive surgery, and the outcomes of interest were progression-free survival and overall survival. Survival analyses were restricted to eligible patients with resectable disease without medical barriers to surgery at each progression episode. Demographic and clinicopathologic characteristics were summarized using descriptive statistics. Progression-free survival (after first, second, and third progression) and overall survival were estimated with methods of Kaplan and Meier, and were modeled via Cox proportional hazards regression. Multivariable analyses were performed for progression-free survival after first progression and overall survival., Results: Among the 369 patients with adult granulosa cell tumors of the ovary in the registry, 149 patients met the study inclusion criteria. Secondary cytoreductive surgery was associated with a significant improvement in progression-free survival on univariable (hazard ratio, 0.37; 95% confidence interval, 0.17-0.81, P=.01) and multivariable analyses (hazard ratio, 0.42; 95% confidence interval, 0.19-0.92; P=.03). Those who underwent secondary cytoreductive surgery had a significantly improved median overall survival compared with those who did not undergo cytoreductive surgery (181.92 vs 61.56 months, respectively; P=.002). Overall survival benefit remained statistically significant on multivariable analysis (hazard ratio, 0.28; 95% confidence interval, 0.11-0.67; P=.004). Tertiary cytoreductive surgery was similarly associated with a significant improvement in progression-free survival (hazard ratio, 0.43; 95% confidence interval, 0.26-0.70; P=.001). Despite a similar trend, quaternary cytoreductive surgery was not associated with a significant improvement in progression-free survival (hazard ratio, 0.74; 95% confidence interval, 0.42-1.26; P=.27)., Conclusion: Among those with resectable disease and no medical contraindications to surgery, cytoreductive surgery may have a beneficial impact on progression-free survival and overall survival in patients with recurrent adult granulosa cell tumors of the ovary., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: The main feature of adult granulosa cell tumors (AGCT) is their capacity to secrete hormones, with nearly all of them capable of synthesizing oestradiol. The primary goal of this study is to identify synchronized endometrial pathologies, particularly endometrial cancer, in AGCT patients who had undergone a hysterectomy., Materials and Methods: The study cohort comprised retrospectively of 316 AGCT patients from 10 tertiary gynecological oncology centers. AGCT surgery consisted of bilateral salpingo-oophorectomy, hysterectomy, peritoneal cytology, omentectomy, and the excision of any suspicious lesion. The median tumor size value was used to define the relationship between tumor size and endometrial cancer. The relationship between each value and endometrial cancer was evaluated., Results: Endometrial intraepithelial neoplasia, or hyperplasia with complex atypia, was detected in 7.3% of patients, and endometrial cancer in 3.1% of patients. Age, menopausal status, tumor size, International Federation of Gynecology and Obstetrics stage, ascites, and CA-125 level were not statistically significant factors to predict endometrial cancer. There was no endometrial cancer under the age of 40, and 97.8% of women diagnosed with endometrial hyperplasia were over the age of 40. During the menopausal period, the endometrial cancer risk was 4.5%. Developing endometrial cancer increased to 12.1% from 3.2% when the size of the tumor was >150 mm in menopausal patients (p = 0.036)., Conclusion: Endometrial hyperplasia, or cancer, occurs in approximately 30% of AGCT patients. Patients diagnosed with AGCT, especially those older than 40 years, should be evaluated for endometrial pathologies. There may be a relationship between tumor size and endometrial cancer, especially in menopausal patients., (© 2024 Japan Society of Obstetrics and Gynecology.)

Competing Interests: Competing interests: None declared.

Introduction: Adult-type granulosa cell tumors (AGCTs), which account for 2% to 5% of all malignant ovarian tumors, are rare sex cord-stromal tumors that usually secrete excess estrogens, but they can also secrete androgens., Patient Concerns: We report 2 patients of childbearing age with AGCT who presented with the complaint of abnormal menstruation and elevated luteinizing hormone (LH), and mildly elevated testosterone., Diagnosis: The ovarian tumors had hormonal activity., Interventions: The 2 patients underwent laparoscopic left adnexectomy. The second patient underwent 4 cycles of chemotherapy with paclitaxel and carboplatin as adjuvant treatments., Outcomes: Their postoperative pathology confirmed AGCTs. Also, their menstrual cycle returned to normal, with normal serum LH and testosterone levels. There was no sign of recurrence., Conclusion: The cases suggest that elevated serum LH levels may be a sign of unknown tumors in cases of oligomenorrhea or secondary amenorrhea. It is useful to evaluate the serum levels of inhibin B and anti-Müllerian hormone to improve the early recognition of ovarian granulosa cell tumors., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Granulosa cell tumor (GCT) is a rare ovarian malignancy that represents only 2-3% of all cases. There are two subtypes of GCT: juvenile/JGCT (5% of cases) and adult/AGCT (95% of cases). This study aimed to describe a series of 6 GCT cases. The 6 study patients were managed from June 2011 to November 2022 in a private oncology clinic located in Teresina (PI), Brazil. At diagnosis, the mean patient age was 47 years, and symptoms in 5 patients (83%) were pelvic pain and/or increased abdominal volume. The majority of the patients (N=4/67%) had no comorbidities or findings related to GCT on physical examination. The mean tumor size was 11 cm. Five (83%) tumors were stage Ia and one tumor (17%) was stage III. Regarding tumor subtype, 5 (83%) were AGCT and 1 (17%) was JGCT. Surgical treatment consisted of unilateral salpingo-ophorectomy in 2 patients (33%), total hysterectomy and bilateral salpingo-ophorectomy in 3 patients (50%), and cytoreduction (suboptimal) in 1 patient (17%). After a mean follow-up period of 62.7 months, 5 patients (83%) are still alive and free of disease. One (17%) died from disease progression after 126 months. In the current study, disease-free overall survival was 83%, in a mean follow-up period of 62.7 months., Competing Interests: The authors declare no competing interests., (Copyright: Rafael Everton Assunção Ribeiro da Costa et al.)

Objective: To evaluate the effectiveness of hormonal therapy (HT) in patients with recurrent adult ovary granulosa cell tumors. Methods: The clinical and treatments features of the patients who received HT were studied retrospectively. The efficacy and safety of HT were evaluated. The Kaplan-Meier technique was used to conduct survival analysis. Results: The research involved a total of thirteen patients. The median age of the participants was 49 years (range: 34-61). Since diagnosis, the median number of surgeries has been three (range: 2-8). At least one chemotherapy regimen has been administered to 12 (92.3%) patients. Ten of the patients (76.9%) had at least two metastatic areas. Lung metastases were found in two (15.4%) of the patients. Inhibin B levels were elevated in 81.2% of patients before hormone treatment. The patients received different HTs (Leuprolide acetate + anastrozole-three patients, leuprolide acetate + tamoxifen-six patients, only anastrozole-three patients, only tamoxifen-one patients). The median progression-free survival was found 17.7 months (95 % CI: 14.7-20.6). In four (33.4%) patients, an overall response (complete or partial) was identified. A stable response was observed in eight (66.7%) patients. Conclusions: HT is effective in pretreated individuals with recurrent ovarian granulosa cell tumors, according to this research. Despite the limited number of patients and treatment variability, disease control was achieved in all patients. Also, we found that Inhibin B levels were associated with treatment response., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Aim of the present analysis is to compare the impact of antihormonal therapy versus cytotoxic chemotherapy versus a watch a wait approach on disease-free survival (DFS) in the adjuvant setting of patients who underwent complete cytoreductive surgery(CRS) for recurrent adult type granulosa cell tumours of the ovary (GCT). Moreover, we wished to identify prognostic risk factors for recurrence. We included recurrent GCT-patients who underwent CRS resulting in total macroscopic tumour clearance, treated in two gynaecological cancer centres over a 20-year period (2000-2020). CRS was performed for 51 recurrences in 26 GCT-patients. Adjuvant systemic treatments were as follows: chemotherapy in 21 cases, hormonotherapy in 10 cases, no systemic treatment in 20 cases. There were no statistically significant differences in DFS between chemotherapy, hormonotherapy and no systemic treatment: median DFS was 57, 36 and 57 months, respectively (p = 0.616). Extra-pelvic and/or multifocal tumour dissemination were found to be independent predictive factors for subsequent recurrences. In the cases with both lower and upper abdominal involvement (n = 18), patients who received chemotherapy (n = 9) had longer DFS than those who had hormonotherapy (n = 2) or no adjuvant therapy (n = 7) at all: median DFS was 36, 13 and 15 months, respectively (p = 0.9). Our findings do not encourage the administration of adjuvant therapy following complete CRS for GCT-relapse. Selected high-risk patients with disseminated disease may derive clinical benefit from additional chemotherapy, larger-scale multicentre studies are warranted to define treatment algorithms for this rare disease., (© 2024. The Author(s).)

Juvenile granulosa cell tumors (JGCTs) are rare, though carry significant burden of morbidity and mortality. A 15-year-old menstruating female with abdominal pain was diagnosed with a large 22.3 cm pelvic mass. CA-125 and LDH were elevated. Exploratory laparotomy was undertaken due to lesion size, and left salpingo-oophorectomy with omentectomy was completed. Pathology confirmed JGCTs with focal disruption, consistent with Stage IC disease. Six weeks postoperatively, the patient experienced recurrent abdominal pain and ultrasound revealed a 7.9 cm right ovarian cystic structure. Given size and nodularity, management was discussed with a multidisciplinary team. Serial ultrasounds demonstrated resolution of the cyst. Workup for ovarian masses in pediatric patients has added complexity of fertility preservation. Once ovarian torsion is ruled out, imaging and laboratory studies are completed to characterize the mass. In pediatric patients with cancer of the Mullerian structures and risk of infertility, decision-making can be challenging and is best managed with a multidisciplinary approach., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Juvenile granulosa cell tumor (JGCT) is an uncommon ovarian tumor. There are only a few cases in the literature that depict the cytomorphology of JGCT at the primary/metastatic site. We described the fine-needle aspiration cytology of a recurrent metastatic JGCT of the anterior abdominal wall, 5 years post-surgery (total abdominal hysterectomy with bilateral salpingo-oophorectomy)., (© 2023 Wiley Periodicals LLC.)

Objective: Adult granulosa cell tumors (AGCTs) are rare malignancies that accounts for approximately 1% of ovarian neoplasms. As there are currently no well-recognized models for predicting relapse-free survival (RFS), we performed a clinicopathological analysis to identify risk factors for AGCT recurrence., Methods: We investigated 130 patients with pathologically diagnosed AGCT as confirmed by the presence of the characteristic FOXL2 C402G mutation., Results: Most patients had International Federation of Gynecology and Obstetrics stage I disease (n = 122, 95.3%). The 10-year RFS rate was 31.4% (22/70) and mean 10-year RFS was 74.4 (95% CI, 65.2-83.7) months. Ten patients experienced recurrence beyond the 10-year follow-up period. Undergoing fertility sparing surgery, an estrogen receptor-α (ERα) score (>0.25), and a Ki-67 index >15% were independent risk factors for recurrence in patients with stage I disease (bias-corrected C-index: 0.776). We constructed a nomogram with well-fitting calibration plots; the areas under the curve (AUCs) for 5-, and 10-year RFS prediction were 0.883 and 0.906 respectively. A simplified model with 3 predictive factors (ERα score, Ki-67 index, and primary surgical procedure) and 2 risk stratification subgroups (low- and high-risk) was constructed; its AUCs for 5-, and 10-year RFS prediction were 0.825 and 0.850 respectively. Kaplan-Meier survival curves showed significant differences in 10-year RFS between the low- and high-risk groups (p < 0.001)., Conclusions: The type of primary surgical procedure, ERα score, and Ki-67 index are independent predictors of recurrence for patients with stage I AGCT. Our predictive model based on these factors showed good performance., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The adult granulosa cell tumor of the testis is a rare sex-cord/stromal tumor, with a potentiality for late recurrence and metastasis. Because of its rarity, this tumor is poorly understood, particularly in terms of its molecular features. As a result, it is necessary to register each occurrence in order to study the evolution of this rare malignancy and develop therapeutic strategies., Methods: A 50-year-old man discovered a painless right testicular mass unexpectedly, and the mass steadily expanded for 2 months. Ultrasonography showed a 5.2 cm × 4.0 cm × 3.6 cm mass in the right testicle. A right radical orchiectomy was performed on September 7, 2016. The pathologic diagnosis was a testicular adult granulosa cell tumor. The post-computed tomography scans and bone scintigraphy ruled out distant metastases. A high-throughput sequencing of 520 cancer-related genes revealed FOXL2 C134W, CDKN2A E87Gfs*24, TP53 S183*, TERT c.-124C > T, and H3F3A K28R mutations in this case. Because the patient stated he would be unable to return to the hospital for a follow-up appointment on time, he elected to have 4 cycles of adjuvant chemotherapy BEP (bleomycin, etoposide, and cisplatin) after the right radical orchiectomy., Results: The patient has not had a clinical recurrence or metastasis in 6 years., Conclusion: Surgery together with adjuvant chemotherapy may be useful treatment options for these individuals with malignant tendencies who are unable to visit the hospital for a follow-up appointment on time. Adult testicular granulosa cell tumors have a relatively complex genetic profile; their etiology is linked to a number of common driver genes, including TERT, CDKN2A, TP53, and H3F3A., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Leiomyomas are the most common benign tumors of smooth muscle origin in women. They are most frequently found in the submucosal tissue of the uterine corpus; however, they also occur in other areas of the uterus, including the cervix. Their size usually varies between 0.5 to 1.0 cm; however, they can reach great dimensions. A strong correlation between the onset and growth of leiomyomas and estrogen levels was observed. Granulosa cell tumor (GCT) is an infrequent sex cord-stromal ovarian neoplasm. Despite their malignancy, GCTs have a good long-term prognosis. In this study, we present a unique case of coincidence of 2 tumors: leiomyoma of rare location (cervix uteri) and extraordinary size (9, 04 cm diameter) with an adult granulosa cell tumor., Patient Concerns: A 67-year-old Caucasian woman was transported from an emergency ward to a gynecological surgery department due to a massive vaginal hemorrhage., Diagnoses: Preliminary examination showed a presence of an enormous uteri cervix tumor., Interventions: Initially, the patient underwent physical and ultrasound examinations. To prevent further bleeding, an urgent surgery (hysterectomy) with bilateral salpingo-oophorectomy was performed., Outcome: Postoperative histopathological examination revealed a cervical leiomyoma and the incidental occurrence of an adult GCT in the right ovary., Lessons: This case shares an interesting coincidence between a rare variant of leiomyoma and GCT. The study suggests that the potential reason for this can be estrogen secreted by the GCT, which causes the enormous size of the patient's cervical leiomyoma and the severe vaginal bleeding. Therefore, we advise it is important in abnormal cases to search for other hidden explanations, as in cases of GCT., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: Identifying prognostic factors and evaluating the impact of adjuvant chemotherapy in patients with sex cord stromal tumors (SCST) is crucial. In this study, we aimed to address these challenges., Methods: We conducted a retrospective analysis of data from 13 centers of the French Rare malignant gynecological tumors (TMRG) network. We enrolled 469 adult patients with malignant SCST who received upfront surgery since 2011 to July 2015., Results: 75% were diagnosed with adult Granulosa cell tumors, and 23% had another subtype. With a median follow-up of 6.4 years, 154 patients (33%) developed a first recurrence, 82 (17%) two recurrences, and 49 (10%) three recurrences. Adjuvant chemotherapy was administered in 14.7% of patients at initial diagnosis. In relapse, perioperative chemotherapy was administered in 58.5%, 28.2%, and 23.8% of patients, respectively, in the first, second, and third relapse. In the first-line therapy, age under 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival (PFS). Chemotherapy had no impact on PFS in early-stage disease (FIGO I-II). The PFS was similar using BEP or other chemotherapy regimens (HR 0.88 [0.43; 1.81]) in the first-line therapy. In case of recurrence, PFS was statistically prolonged by complete surgery, but perioperative chemotherapy use did not impact PFS., Conclusion: Chemotherapy use did not impact survival in the first-line or relapse setting in SCST. Only surgery and its quality demonstrated benefit for PFS in ovarian SCST in any lines of treatment., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Mixed sex cord-stromal tumors, which consist of poorly differentiated Sertoli cells and Leydig cells and juvenile granulosa cell tumor tissue, are extremely rare. Most of these tumors are unilateral and stage I at the time of diagnosis; nonetheless, according to the available relevant English-language literature, these tumors maintain a malignant potential. We herein report a case involving a 15-year-old girl diagnosed with a mixed sex cord-stromal tumor (gynandroblastoma with juvenile granulosa cell tumor component). Left salpingo-oophorectomy was initially performed, and the diagnosis of a juvenile granulosa cell tumor was established. Right salpingo-oophorectomy was performed 1 year later, at which time the specimen showed a different growth pattern involving epithelioid cells and tubules, resembling a Sertoli-Leydig cell tumor. Immunohistochemical staining was performed and the specimen was compared with that obtained 1 year earlier. We concluded that the tumors were linked and most likely constituted a gynandroblastoma (mixed form of sex cord-stromal tumor). Although this is an extremely uncommon ovarian tumor, it should be considered when diverse tumor morphology is identified. Bilateral metachronous involvement of the ovaries is possible. The grade of the Sertoli-Leydig cell component may influence the prognosis of such a tumor.

Introduction: This study aimed to describe the clinicopathological characteristics of recurrent adult granulosa cell tumor and identify the risk factors for recurrence., Material and Methods: Seventy recurrent adult granulosa cell tumor patients treated in Peking Union Medical College Hospital between 2000 and 2020 were retrospectively reviewed. The primary outcomes were progression-free survival after first recurrence (PFS-R), overall survival after first recurrence (OS-R) and recurrence frequency. The Kaplan-Meier analysis, univariate and multivariate Cox proportional hazard analysis, and the Prentice, Williams and Peterson counting process (PWP-CP) model were adopted., Results: There were 70 patients included in the study, and recurrence occurred twice in more than 71% of patients, and 49.9% of patients relapsed ≥ three times. The recurrence pattern in over half of the patients at first recurrence was multifocal and distant disease, and abdominal or pelvic mass and liver metastasis were the most common. The 5-year PFS-R was 29.3%, and the 10-year PFS-R was 11.3%; the 5-year OS-R was 94.9%, and the 10-year OS-R was 87.9%. Kaplan-Meier analysis demonstrated that patients with distant recurrence and PFS1 (PFS when first recurrence occurred) ≤60 months had worse PFS-R (p = 0.017, 0.018), and patients with PFS-R ≤ 34 months had worse OS-R (p = 0.023). It demonstrated that PFS1 ≤ 60 months (hazard ratio, HR 1.9, 95% confidence interval [CI]: 1.1-3.4, p = 0.028) was an independent risk factor for PFS-R, and local lesion at recurrence (HR 0.488, 95% CI: 0.3-0.9, p = 0.027) was an independent protective factor for PFS-R. In addition, it demonstrated that PFS-R ≤ 33 months (HR 5.5, 95% CI: 1.2-25.3, p = 0.028) was an independent risk factor for OS-R. The PWP-CP analysis showed that laparoscopic operation (at each operation) could significantly increase recurrence times (p = 0.002, HR = 3.4), and no existence of gross residual lesion (R0) at each recurrence operation could significantly decrease recurrence frequency (p < 0.001, HR <0.001)., Conclusions: The recurrence pattern in patients with recurrent adult granulosa cell tumor was characterized as late and repeated, multifocal, and distant relapse. It has been demonstrated that PFS1 ≤ 60 months and distant lesion at recurrence are independent risk factors for PFS-R, and PFS-R ≤ 33 months is an independent risk factor for OS-R. The PWP-CP model showed that the transabdominal approach and surgery reaching R0 could significantly decrease the recurrence frequency., (© 2023 The Authors. Acta Obstetricia et Gynecologica Scandinavica published by John Wiley & Sons Ltd on behalf of Nordic Federation of Societies of Obstetrics and Gynecology (NFOG).)

Rationale: Adult granulosa cell tumors (AGCT) mainly secret estrogen, but few androgens. It is rarer to have amenorrhea and hyperandrogenemia as clinical features. Here, we report a rare case of right side AGCTs with amenorrhea and hyperandrogenemia in a 19-year-old female., Patient Concerns: The 19-year-old patient was admitted to our hospital due to amenorrhea for more than 1 year, and discovery of pelvic mass for 4 months. The gynecological ultrasound and computed tomography (CT) cannot define the nature of the mass. Surprisingly, an elevation in testosterone levels was also measured., Diagnosis and Interventions: The present patient underwent laparoscopic right salpingo-oophorectomy and partial omentectomy and biopsy of the peritoneum., Outcomes: After the surgery, the testosterone value was down to normal. The patient menstrual cramps on August 13, 2021. Her clitoris is smaller than the front. Up to August 1, 2022, there was no obvious sign of recurrence., Lessons: Androgen-secreting AGCT is rare. We hope that this case can strengthen gynecologists' early diagnosis and treatment of this disease and improve the prognosis., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Androgen-producing granulosa cell tumor in adolescent girl is rare condition and clinical characteristics are not fully elucidated., Case Presentation: Seventeen years old girl complained of secondary amenorrhea was referred to our out-patient consultation. Markedly elevated serum testosterone, LH, and AMH levels were noted. Mild hirsutism and clitoromegaly were presented. Transabdominal ultrasonography and MRI revealed cystic mass occupied pelvic cavity probably originated from left ovary. Right ovary showed polycystic appearance. Laparoscopic left ovarian cystectomy was performed. After the surgery, her menstruation resumed along with normalized hormonal parameters, and clinical hyperandrogenism were improved. Since the scarcity of cellular lining of inner cyst wall, definitive pathological diagnosis was difficult. After the consultation with gynecological pathologist, the tumor was diagnosed as sex cord stromal tumor, highly suspicious for adult granulosa cell tumor. Residual left salpingo-oophorectomy was performed by additional laparoscopic surgery. Her serum testosterone and AMH levels were remained low with regular menstrual cycles and no evidence of recurrence., Conclusions: Androgen-producing cystic granulosa cell tumor is rare gynecological disorders, which need both gynecologic oncological and endocrinological approach. Its clinical manifestations may bring some clues to the pathogenesis of ovulatory dysfunctions, such as polycystic ovary syndrome., (© 2023. The Author(s).)

BACKGROUND Juvenile-type granulosa cell tumors (JGCTs) are a rare subtype of sex cord stromal tumor with a characteristic histology that is commonly found in the first 3 decades of life. It most commonly presents with symptoms of hyperestrogenism, which may present as precocious pseudopuberty or as menstruation-related symptoms, allowing for early detection of the tumor. CASE REPORT We present the case of a 12-year-old girl who presented to her primary care provider (PCP) with secondary amenorrhea with intermittent abdominal pain, who underwent an ultrasound for further evaluation, which revealed a large incidental pelvic mass. She was admitted to the Emergency Department (ED) and had findings of galactorrhea and hyperprolactinemia on examination. Imaging studies demonstrated a large ovarian mass measuring 15.0×9.0×18.8 cm that was resected, and subsequent pathology results showed JGCT stage 1A. CONCLUSIONS Prognosis of granulosa cell tumors (GCT) largely depends on its initial size, stage at diagnosis, residual tumors after surgery, and the subtype of GCT. If the patient is of reproductive age, fertility-sparing surgical options must be considered and patients must be regularly monitored for recurrence. JGCTs can present with minimal to no symptoms of precocious puberty in young girls but may present with amenorrhea, which may be considered normal for their developmental age. Although JGCTs are rare, they are important to include in differential diagnoses of younger female patients with abdominal pain, especially if accompanied by hormonal irregularities.

A male patient in his late 20s was admitted to the hospital after presenting with left abdominal, back and scrotal pain that had begun approximately 2 weeks earlier. He had a history of a stable left testicular mass for 3 years, and a physical exam revealed a non-tender, firm left testicular mass and a mild left varicocele. Testicular tumour markers were normal, but a scrotal ultrasound revealed a 2 cm hypoechoic left testicular lesion. Staging imaging showed no retroperitoneal adenopathy or pulmonary metastases.The patient underwent left radical inguinal orchiectomy with no evidence of extratesticular or spermatic cord involvement. His surgical pathology revealed a left pT1a 2.3 cm adult granulosa cell tumour of the testis with no lymphovascular invasion. The tumour was positive for inhibin and negative for OCT3/4, supporting the diagnosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aim: To present the clinicopathologic findings of the second case of androgen-secreting adult granulosa cell tumor (AGCT) in a woman with polycystic ovary syndrome (PCOS) and discuss in the light of the literature. Methods: Description of a case and discussion of the literature. Results: A patient with oligomenorrhea, amenorrhea and hirsutism who was diagnosed as PCOS and treated by oral contraceptive for three years, then left ovarian solid and liquid mass was found and pathologically confirmed to be androgen-secreting AGCT after left oophorectomy. She got regular menstrual cycle and gave birth naturally, but clinical features of PCOS reappeared after breastfeeding. Conclusion: Androgen-secreting AGCT and PCOS have similar clinical features of hyperandrogenism, it is difficult to diagnose androgen-secreting AGCT when both diseases occur in the same patient. If the size of cystic mass in androgen-secreting AGCT is too small to differentiate from PCOM on imaging, pathological examination after surgery may be the only way to find the disease.

Background: Ovarian adult granulosa cell tumours are low-grade malignant sex cord-stromal neoplasm with a low recurrence rate. Prognostic factors for recurrence include tumor stage, tumor rupture in Stage I neoplasms and the presence of residual tumors after surgery. However, in recurrent tumors, prognostic factors for overall survival (OS) are lacking. In the present paper, we conducted a systematic meta-analysis with the aim to assess prognostic factors for OS in patients with recurrent GCT., Methods: Electronic databases were searched for all studies assessing prognostic factors in recurrent adult granulosa cell tumor of the ovary. Student T test, Fisher's exact test and Kaplan-Meier survival analysis with long-rank test were used to assess differences among groups; a p value < 0.05 was considered significant., Results: Eleven studies analyzing 102 recurrent tumors were included in the systematic review. Tumor stage and localization of recurrent tumors were significantly associated with OS on Kaplan-Meier analysis; Cox regression analysis showed a HR of 0.879 for the stage II, of 3.052 for the stage III, and of 2.734 for stage IV tumor was significantly associated with OS (p = 0.037); observed HRs for abdominal and thoracic locations were of 2.405 and of 4.024, respectively., Conclusions: In conclusion, the present article emphasizes the prognostic significance of tumor stage > II and extrapelvic anatomic sites of recurrences in patients with recurrent granuolase cell tumors of the ovary., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Study Objective: The objective of the study was to analyze the oncological outcomes and prognostic factors in patients with early-stage juvenile granulosa cell tumor (JGCT) who underwent fertility-sparing surgery., Design, Setting, and Participants: All patients with early-stage JGCT who underwent fertility-sparing surgery between January 1995 and December 2017 were reviewed retrospectively., Interventions: None., Main Outcome Measures: The risk factors for recurrence and death in patients with early-stage JGCT were identified., Results: Thirty-five patients were eligible for the current study. The median age was 17 years (range: 4-30 years), and 10 patients were premenarchal. Nine patients were International Federation of Obstetrics and Gynecology stage IA, and 26 were stage IC. Eight subjects underwent complete staging, whereas 28 had no staging at their initial surgery. In those without initial staging, 3 patients who received initial unilateral salpingo-oophorectomy (USO) and 6 with initial cystectomy underwent secondary surgery for staging or USO with staging, respectively. At the time of the secondary staging operation, 1 of the subjects (treated with USO at initial surgery) was found to be stage III and was excluded from this study. Therefore, 8 patients underwent secondary staging surgery performed by laparoscopic surgery, and none of them suffered recurrence during follow-up. Fourteen of the 16 patients with complete staging surgery underwent lymphadenectomy, and none of them had lymph node metastasis. Thirty-one patients received adjuvant chemotherapy after surgery. Eight patients had disease recurrence after a median follow-up time of 51 months (range: 6-229 months), with a median time to recurrence of 4.5 months (range: 2-52 months). Six patients died of their disease. The 5-year disease-free and overall survival rates were 74.8% and 84.3%, respectively. Univariate analysis showed that incomplete staging surgery was associated with increased risk of recurrence (P = 0.029). Adjuvant chemotherapy was not associated with disease-free survival. Four patients had a total of 6 pregnancies, resulting in 6 live births., Conclusion: Complete surgical staging is recommended for early-stage JGCT, but lymph node dissection can be omitted. Laparoscopic restaging surgery is feasible for patients with incomplete staging at initial surgery. However, the prognosis of patients with relapsed JGCT remains poor., (Copyright © 2021. Published by Elsevier Inc.)

Estrogen modulates platelet activation and aggregation, and it increases the levels of the von Willebrand factor, factors II, VII, VIII, and X, and of fibrinogen, all of which increase the risk for thromboembolism. We report the case of a 59-year-old woman, postmenopausal for 4 years, not using hormone replacement therapy, who was admitted into the emergency room with shortness of breath and increased abdominal volume. After physical examination and imaging and biochemical tests, she was diagnosed with pulmonary thromboembolism and a large left adnexal tumor. The patient was promptly received full anticoagulation therapy for the pulmonary thromboembolism. High levels of estradiol (810.4 pg/mL), anti-Mullerian hormone (16.39 ng/mL), inhibin (11250 pg/mL), and suppressed FSH (<0.16 IU/L) led to a suspicion of granulosa-cell tumor. After clinical stabilization, she underwent to an exploratory laparotomy with total hysterectomy and bilateral adnexectomy to treat the pelvic tumor. Pathologic report confirmed a granulosa-cell tumor.

Background: Pregnancy complicated with juvenile granulosa cell tumor (JGCT) is very rare; thus, the experience on clinical diagnosis and management is limited., Cases: Two patients presented with abdominal pain, two were incidentally discovered, one by ultrasonography, and one during a caesarian section. One case received an emergency caesarian section because of tumor rupture at 38th week's gestation, the rest were treated at full term and no abnormalities were detected in the newborns. Three cases received further staging surgery, two of which received postoperative adjuvant chemotherapy. No patient had recurrent disease after a follow-up period spanning from 13 to 57 months., Conclusion: In the absence of emergency, surgery can be delayed without affecting the fetus. More research is needed to determine the value of chemotherapy in FIGO stage I patients., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Only a few cases of early-stage ovarian granulosa cell tumor (GCT) with lung metastasis have been previously documented in the literature. We present a case of stage IA adult-type ovarian GCT with late pelvic recurrence and lung metastasis and review the relevant literature., Case Report: A 71-year-old female who underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy for an ovarian GCT in her early 50s presented with pelvic recurrence 16 years later. Lung metastasis was accidentally discovered during a preoperative computed tomography scan of the pelvic tumor. The patient received surgical resection and adjuvant chemotherapy. She remains alive and healthy without evidence of disease after 12 months of follow up., Conclusion: This case illustrates the importance of long-term follow-up for early-stage GCTs. It also highlights the requirement of extended examination for possible extra-abdominal/pelvic tumors., Competing Interests: Declaration of competing interest The authors declare no conflicts of interest., (Copyright © 2021. Published by Elsevier B.V.)

This is a report on a case of CA19-9-producing cancer of esophagogastric junction with rectal cancer and a suspicion of Krukenberg tumor, a metastasized ovarian tumor that would mean an inoperable condition of cancer progression if that were true. This was a case of a woman in her 60s who was diagnosed with double cancers at the esophagogastric junction and rectum with a swollen left ovary. She had a laparoscopic bilateral salpingo-oophorectomy to get a histologic diagnosis, which should affect the subsequent therapeutic strategy because metastasis to the ovary meant an inoperable cancer progression. The resected ovary was diagnosed as juvenile granulosa cell tumor, but not Krukenberg tumor. Thus, subsequent curative surgeries, such as thoracolaparotomy for esophagogastric junction cancer and robot-assisted surgery for rectal cancer, were performed. Immunohistochemical examination revealed that the expression of CA19-9 was strongly observed in the tumor of esophagogastric junction, but not in the tumors of rectum or ovary. Furthermore, serum CA19-9 was drastically decreased after the resection of esophagogastric junction cancer. In aggregate, this esophagogastric junction cancer met the criteria of CA19-9-producing gastric cancer defined by Okinaga et al. So far, 46 cases of CA19-9-producing gastric cancer including this case have been reported in Japanese literature. Interestingly, this case had another characteristic of juvenile granulosa cell tumor, one of borderline malignant sex cord-stromal tumors rarely found in adults.

Objectives: The aim of this study is to share of the 20-year experience of a tertiary center about juvenile granulosa cell tumor (JGCT) and describe clinic manifestations, treatment, and outcome of patients who diagnosed JGCT., Material and Methods: Five patients who diagnosed juvenile granulosa cell tumor between 2000 and 2020 were included in the study. The demographics, clinical findings and outcomes were retrospectively evaluated. Of the 5 patients in our study, one was in the premenarcheal girl. The common complaint in all of our patients was abdominal swelling. In preoperative imaging methods, all patients had unilateral adnexal mass and no signs in favor of metastasis. All patients were staged according to FIGO classification for ovarian tumors; 3 of patients had stage IA disease, one of patients had stage IC1 and one of patients had stage IC2. All patients underwent different surgecal procedures which is appropriate for their clinical manifestations. In addition to surgery 2 patients received adjuvant chemotherapy., Results: The median follow-up period of the patients was 60 mounts and recurrence was observed in two patients who were reoperated. We have no patients who died due to this disease., Conclusions: Possible diagnosis of juvenile granulosa cell tumor should be kept in mind in a patient of young age with unilateral adnexal mass with benign features.

This is a case of a 73-year-old woman who first presented in 2020 with a fullness in her abdomen. After several thorough investigations and unforeseen complications, the fullness was diagnosed as a granulosa cell tumour. In 2003, she had been diagnosed with a granulosa cell tumour of the ovary. Complete excision was performed, however she was not given a follow-up appointment after the procedure. This case highlights the importance of frequent follow-up of these tumours, the high recurrence rate, the severe complications which may result and the awareness of possible variations in this tumour's histologic appearance., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Granulosa cell tumors (GCTs) account for less than 2% of all ovarian malignancies and are the second most common ovarian sex cord stromal tumors after fibroma/thecomas.GCTs occur most frequently in postmenopausal women with a peak age of 50 to 55, are usually diagnosed in their early stages, and have a good prognosis. GCTs usually present with features of hyperestrogenism, with an average size is 10 to 15 cm., Patient Concerns: A 31-year-old nulligravida diagnosed with polycystic ovarian syndrome (PCOS) 10 years prior, had a 20-mm mass in her right ovary found on ultrasonography 2 years ago. She had been taking dienogest 2 mg for 2 years for a misdiagnosed endometrioma, but over a 2-year course, the mass increased to 50 mm., Diagnoses: An ultrasound scan revealed a 47 × 37-mm round solid mass in the right ovary with a spongiform appearance and little vascularity. The pathologic findings showed an adult-type granulosa cell tumor with necrosis and hemorrhage. The tissue stained positive for inhibin-α, Wilms' tumor-1, CD56, and negative for cytokeratin 7., Interventions: We finally performed right salpingo-oophorectomy, endometrial biopsy, peritoneal biopsy, and partial omentectomy. The pathological findings were adult-type granulosa cell tumor. The International Federation of Gynecology and Obstetrics staging was IA. The patient did not require additional treatment., Outcomes: Surprisingly, her normal menstruation returned 2 weeks after the operation, and she had a normal pregnancy and parturition. The patient had been followed-up regularly for 3 years following the surgery. The patient has not experienced any complications and has remained disease-free., Lessons: GCTs should be considered in the differential diagnosis if a female patient with PCOS and amenorrhea shows a unilateral small solid mass. They are extremely rare malignant ovarian tumors that must be differentiated from other benign ovarian tumors, especially endometriomas and dermoid cysts. It was difficult for us to suspect a granulosa cell tumor because the patient already had PCOS symptoms such as mild hirsutism and amenorrhea. This case highlights the importance of physicians being aware of and suspicious for GCTs in similar cases, along with knowing their characteristics in considering possible differential diagnoses., Competing Interests: The authors alone are responsible for the content and writing of this article. The authors declare no conflicts of interest. The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: The aim of this study was to elucidate the clinicopathological features of ovarian granulosa cell tumors (GCTs) and to identify the prognostic factors., Methods: The Japanese Society of Gynecologic Oncology (JSGO) conducted an observational retrospective cohort study of women with GCTs enrolled in the Gynecological Tumor Registry of the Japan Society of Obstetrics and Gynecology (JSOG) between 2002 and 2015. Clinicopathological features, including lymph node metastasis, were evaluated. In addition, we performed a prognostic analysis of patients between 2002 and 2011 for whom survival data were available. Kaplan-Meier and multivariate Cox proportional hazards analyses were performed., Results: We identified 1426 patients with GCTs. Of the 222 patients who underwent lymph node dissection, 10 (4.5%) had lymph node metastasis. The incidence of lymph node metastasis in patients with pT1, pT2, and pT3 was 2.1%, 13.3%, and 26.7%, respectively (p < 0.001). Prognostic analysis was performed on 674 patients. In the multivariate Cox regression analysis, residual disease after initial surgery (hazard ratio (HR) = 10.39, 95% confidence interval (CI) = 3.15-34.29) and lymph node metastasis (HR = 5.58, 95% CI = 1.62-19.19) were independent risk factors for cancer-specific survival., Conclusions: In the initial surgery for GCTs, lymph node dissection can be omitted if the operative finding is pT1. In cases of pT2 or higher, lymph node dissection should be considered. Debulking is critical for achieving no gross residual tumor at the end of the surgery., Competing Interests: Declaration of Competing Interest No potential conflict of interest relevant to this article was reported., (Copyright © 2021 Elsevier Inc. All rights reserved.)