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Acta Obstetricia Et Gynecologica... Oct 2021Ovarian granulosa cell tumor (GCT) is a rare, low-grade malignant tumor that accounts for 70% of the sex cord-stromal tumors. It has two histopathologic types with...
Ovarian granulosa cell tumor (GCT) is a rare, low-grade malignant tumor that accounts for 70% of the sex cord-stromal tumors. It has two histopathologic types with different clinical and biologic features: adult GCT and juvenile GCT. Most women diagnosed with the adult GCT have a favorable prognosis, with a 5-year survival rate of 97%-98%, but adult GCT has a feature of late relapse; the recurrence time could be more than 20 years after diagnosis. Juvenile GCT has a survival rate of 97% in stage I and a 5-year survival rate of 0%-22% in advanced stage with earlier recurrence than adult GCT. Consequently, the scenario emphasizes the need for early diagnosis, standardized treatment protocols, and long-term follow up. However, there is a lack of consensus regarding accurate diagnosis of GCT and adjuvant treatment. Furthermore, GCT tends to occur in young women, which emphasizes the viability of fertility-sparing surgery. The current review performed a systematic literature review of 60 articles to summarize the latest advances in GCT, with an emphasis on the molecular pathogenesis and survival after fertility-sparing surgery. We found that young women with fertility-sparing surgery had a desirable reproductive and survival outcome compared with those undergoing radical surgery.
Topics: Female; Fertility Preservation; Granulosa Cell Tumor; Humans; Neoplasm Recurrence, Local; Ovarian Neoplasms; Survival Analysis
PubMed: 34027996
DOI: 10.1111/aogs.14189 -
Molecular Cancer Research : MCR May 2023Adult-type granulosa cell tumors (aGCT) are rare ovarian sex cord tumors with few effective treatments for recurrent disease. The objective of this study was to...
UNLABELLED
Adult-type granulosa cell tumors (aGCT) are rare ovarian sex cord tumors with few effective treatments for recurrent disease. The objective of this study was to characterize the tumor microenvironment (TME) of primary and recurrent aGCTs and to identify correlates of disease recurrence. Total RNA sequencing (RNA-seq) was performed on 24 pathologically confirmed, cryopreserved aGCT samples, including 8 primary and 16 recurrent tumors. After read alignment and quality-control filtering, DESeq2 was used to identify differentially expressed genes (DEG) between primary and recurrent tumors. Functional enrichment pathway analysis and gene set enrichment analysis was performed using "clusterProfiler" and "GSVA" R packages. TME composition was investigated through the analysis and integration of multiple published RNA-seq deconvolution algorithms. TME analysis results were externally validated using data from independent previously published RNA-seq datasets. A total of 31 DEGs were identified between primary and recurrent aGCTs. These included genes with known function in hormone signaling such as LHCGR and INSL3 (more abundant in primary tumors) and CYP19A1 (more abundant in recurrent tumors). Gene set enrichment analysis revealed that primarily immune-related and hormone-regulated gene sets expression was increased in recurrent tumors. Integrative TME analysis demonstrated statistically significant depletion of cancer-associated fibroblasts in recurrent tumors. This finding was confirmed in multiple independent datasets.
IMPLICATIONS
Recurrent aGCTs exhibit alterations in hormone pathway gene expression as well as decreased infiltration of cancer-associated fibroblasts, suggesting dual roles for hormonal signaling and TME remodeling underpinning disease relapse.
Topics: Adult; Female; Humans; Granulosa Cell Tumor; Tumor Microenvironment; Neoplasm Recurrence, Local; Hormones
PubMed: 37068116
DOI: 10.1158/1541-7786.MCR-22-0623 -
Healthcare (Basel, Switzerland) Oct 2021Granulosa cell tumors are rare ovarian tumors that can arise during pregnancy. We present a new case of recurrent adult granulosa cell tumor (AGCT) in pregnancy and a... (Review)
Review
Granulosa cell tumors are rare ovarian tumors that can arise during pregnancy. We present a new case of recurrent adult granulosa cell tumor (AGCT) in pregnancy and a systematic review of the literature. The new case described is a 41-year-old woman G5P1122 with a prior history of AGCT that was referred to our center at 29 weeks because of a symptomatic abdominal mass, compatible with a possible recurrence of AGCT. At 36 + 3 weeks, she underwent a cesarean delivery for preterm labor and a total hysterectomy with a radical surgical staging. A healthy female infant was delivered. The patient received a platinum-based chemotherapy, with a 26-month follow-up negative for recurrence. Analyzing our case with the four identified by the literature review, three were recurrent and two were primary AGCT. Only one required surgery for AGCT at 15 weeks, while another underwent chemotherapy in pregnancy. In the other three cases, surgery for AGCT was done at the time of cesarean delivery. There were three cases of preterm delivery. All five pregnancies resulted in the birth of live babies with weight adequate for gestational age. In conclusion, AGCT diagnosed in pregnancy is rare, reported in only five cases. All gave birth to live babies in the third trimester, and maternal outcome at up to 18 months showed no recurrence.
PubMed: 34828500
DOI: 10.3390/healthcare9111455 -
Journal of Surgical Case Reports Apr 2022Granulosa cell tumors of the ovary are sex cord stromal tumors. Sex cord stromal tumors make up 8% of ovarian tumors. Ninety percent of sex cord stromal tumors of the...
Granulosa cell tumors of the ovary are sex cord stromal tumors. Sex cord stromal tumors make up 8% of ovarian tumors. Ninety percent of sex cord stromal tumors of the ovary are granulosa cell tumors. There are two types of granulosa cell tumors: juvenile and an adult type (Jamieson and Fuller. Molecular pathogenesis of granulosa cell tumors of the ovary. 2012;:109-44). This article provides a literature review outlining histopathology, immunocytochemistry and cytogenic characteristics of adult granulosa cell tumor of the ovary and a case report of synchronous adult granulosa cell tumor of the ovary and endometrial adenocarcinoma.
PubMed: 35474951
DOI: 10.1093/jscr/rjac109 -
Taiwanese Journal of Obstetrics &... Nov 2021Adult-type granulosa cell tumors (GCT) are sex cord-stromal tumors and often accompanied with abdominal distention and hyperestrogenism-related symptoms. Adult-type...
OBJECTIVE
Adult-type granulosa cell tumors (GCT) are sex cord-stromal tumors and often accompanied with abdominal distention and hyperestrogenism-related symptoms. Adult-type GCT-presenting ascites and pleural effusion is extremely rare.
CASE REPORT
A 56-year-old perimenopausal woman presented with abdominal distention and abnormal vaginal spotting. Ultrasound and abdominal computed tomography showed a complex cystic mass in the left ovary accompanied with bilateral pleural effusion and ascites. The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, left pelvic lymph node dissection, omentectomy and appendectomy. Final histopathological diagnosis was adult-type GCT. The patient had postoperative hormone and anti-angiogenesis agent therapy with free of disease.
CONCLUSION
Ovarian cystic complex mass accompanied with ascites and pleural effusion often results from malignant ovarian tumors or benign ovarian fibroma. Based on the aforementioned report, the rare types of ovarian tumors, such as adult-type granulosa cell tumor of the ovary should be taken into consideration.
Topics: Antineoplastic Agents, Hormonal; Ascites; Bevacizumab; Cytoreduction Surgical Procedures; Female; Gonadotropin-Releasing Hormone; Goserelin; Granulosa Cell Tumor; Humans; Laparotomy; Meigs Syndrome; Middle Aged; Ovarian Neoplasms; Ovary; Pleural Effusion; Salpingo-oophorectomy; Treatment Outcome
PubMed: 34794749
DOI: 10.1016/j.tjog.2021.09.028 -
Granulosa Cell Tumor of the Ovary: A Retrospective Study of 31 Cases and a Review of the Literature.International Journal of Surgical... 2018Adult granulosa cell tumors (AGCTs) are the most common sex cord-stromal tumors. Unlike epithelial ovarian tumors, they occur in young women and are usually detected at... (Review)
Review
BACKGROUND
Adult granulosa cell tumors (AGCTs) are the most common sex cord-stromal tumors. Unlike epithelial ovarian tumors, they occur in young women and are usually detected at an early stage. The aim of this study was to report the clinical and pathological characteristics of AGCT patients and to identify the prognostic factors.
METHODS
All cases of AGCTs, treated at Salah Azaïz Institute between 1995 and 2010, were retrospectively included. Kaplan-Meier's statistical method was used to assess the relapse-free survival and the overall survival.
RESULTS
The final cohort included 31 patients with AGCT. The mean age was 53 years (35-73 years). Patients mainly presented with abdominal mass and/or pain (61%, = 19). Mean tumor size was 20 cm. The majority of patients had a stage I disease (61%, = 19). Two among 3 patients with stage IV disease had liver metastasis. Mitotic index was low in 45% of cases ( = 14). Surgical treatment was optimal in almost all cases (90%, = 28). The median follow-up time was 14 years (1-184 months). Ten patients relapsed (32%) with a median RFS of 8.4 years (6.8-9.9 years). Mean overall survival was 13 years (11-15 years). Stage I disease and low-to-intermediate mitotic index were associated with a better prognosis in univariate analysis (resp., = 0.05 and = 0.02) but were not independent prognostic factors.
CONCLUSION
GCTs have a long natural history with common late relapses. Hence, long active follow-up is recommended. In Tunisian patients, hepatic metastases were more frequent than occidental series. The prognosis remains good and initial staging at diagnosis is an important prognostic factor.
Topics: Adult; Aged; Antineoplastic Agents; Chemotherapy, Adjuvant; Female; Granulosa Cell Tumor; Humans; Hysterectomy; Middle Aged; Neoplasm Staging; Ovarian Neoplasms; Ovariectomy; Retrospective Studies; Salpingectomy
PubMed: 29796312
DOI: 10.1155/2018/4547892 -
American Journal of Cancer Research 2022Adult-type Granulosa Cell Tumor of the Ovary (AGCT) is a relatively rare subtype of ovarian cancer, accounting for 2-4% of all ovarian cancer. AGCT originates from... (Review)
Review
Adult-type Granulosa Cell Tumor of the Ovary (AGCT) is a relatively rare subtype of ovarian cancer, accounting for 2-4% of all ovarian cancer. AGCT originates from proliferating normal preovulatory granulosa cells (GCs) and retains several features of those GCs. The hormonal features of AGCT explain the clinical manifestations and provide reliable markers for early diagnosis and recurrence prediction of the disease. Most AGCT patients are diagnosed at an early stage and usually demonstrate a better prognosis than patients with other types of ovarian cancer. Surgery is crucial for both initial and post-relapse treatments, whereas adjuvant therapy is still in the exploratory stage. In 2009, a population-based screening makes an exciting step, about 97% of AGCT has somatic missense mutations in the transcription factor FOXL2 gene and the FOXL2 mutation is considered to be a molecular characteristic of AGCT. Unfortunately, the FOXL2 mutation does not fully explain the development of AGCT. Ongoing research is focusing on signalling pathways in the molecular pathogenesis of AGCT to identify the possible pathogenetic factors and signal transduction pathways and provide a theoretical basis for targeted treatment. Postoperative recurrence of ovarian AGCT is common and is associated with a high mortality rate, which necessitates regular follow-up. The life management of postoperative patients is also crucial, which requires multidisciplinary experts to design recurrence treatment from the perspective of patients and implement meaningful treatment measures.
PubMed: 36119817
DOI: No ID Found -
Abdominal Radiology (New York) May 2023Adnexal masses during pregnancy are a relatively uncommon entity. Their clinical management is challenging given the overlapping features of certain entities on imaging... (Review)
Review
Adnexal masses during pregnancy are a relatively uncommon entity. Their clinical management is challenging given the overlapping features of certain entities on imaging and histopathology, which can mimic malignancy, and the potential side effects to the mother and fetus, whether expectant management versus surgery is pursued. Ultrasonography with Doppler evaluation is the modality of choice for evaluating adnexal masses during pregnancy. Magnetic resonance imaging is the second-line modality useful when US findings are inconclusive/indeterminate. Most adnexal masses in pregnant patients are benign in origin (e.g., functional cysts, mature cystic teratoma, decidualization of endometrioma), but a few are malignant in origin (e.g., dysgerminoma, granulosa cell tumor). Most cases of adnexal masses are asymptomatic, but complications such as ovarian torsion can occur. This review aims to familiarize the radiologist with the imaging of adnexal lesions during pregnancy so that the radiologist can identify ovarian cancer. Specifically, the review will detail the most common benign and malignant adnexal masses in pregnancy, mimickers, and their corresponding imaging findings on US and MRI.
Topics: Pregnancy; Humans; Female; Ovarian Neoplasms; Adnexal Diseases; Magnetic Resonance Imaging; Granulosa Cell Tumor; Dermoid Cyst
PubMed: 36538079
DOI: 10.1007/s00261-022-03768-y -
Acta Obstetricia Et Gynecologica... Jun 2023This study aimed to describe the clinicopathological characteristics of recurrent adult granulosa cell tumor and identify the risk factors for recurrence.
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.
Topics: Female; Adult; Humans; Retrospective Studies; Granulosa Cell Tumor; Neoplasm Recurrence, Local; Ovarian Neoplasms; Prognosis
PubMed: 37012211
DOI: 10.1111/aogs.14558 -
Journal of Education & Teaching in... Jan 2022A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated...
UNLABELLED
A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated extensive abdominal free fluid with a large, heterogeneous mass in the suprapubic region. This was confirmed on computed tomography (CT) of the abdomen and pelvis. The patient was urgently taken to the operating room for exploratory laparotomy where a ruptured tumor was discovered. The patient was eventually diagnosed with ovarian juvenile granulosa cell tumor. POCUS can be vital in guiding the recognition of rare pediatric conditions in the emergency department and should be considered in patients where there is concern for an acute abdomen.
TOPICS
Abdominal pain, ascites, ovarian juvenile granulosa cell tumor, point-of-care ultrasound.
PubMed: 37483405
DOI: 10.21980/J8035H