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      Recurrent granulosa cell tumor of the retroperitoneum during the COVID-19 Pandemic

      case-report

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          Highlights

          • Granulosa cell tumors (GCTs) are sex cord stromal tumors that normally arise from the ovaries.

          • Primary extraovarian GCTs are uncommon, but primary retroperitoneal GCTs are even rarer.

          • Management of extraovarian GCTs can include surgery, chemotherapy, and/or anti-hormone therapy.

          • Delays in care during the COVID-19 Pandemic may have led to advanced disease or progression of disease for cancer patients.

          Abstract

          Extraovarian granulosa cell tumors are rare with very few cases of isolated retroperitoneal granulosa cell tumors reported in the literature. Granulosa cell tumors are notorious for late recurrences and patients should have long term oncologic follow up. We describe a case of recurrent granulosa cell tumor of the retroperitoneum that originally presented as a renal mass, which has not been described before in the literature. Her management was delayed in part due to the COVID-19 Pandemic. Oncologists must be vigilant regarding the consequences of postponed care during this difficult time.

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          Most cited references13

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          Overall Survival with Fulvestrant plus Anastrozole in Metastatic Breast Cancer

          BACKGROUND We previously reported prolonged progression-free survival and marginally prolonged overall survival among postmenopausal patients with hormone receptor–positive metastatic breast cancer who had been randomly assigned to receive the aromatase inhibitor anastrozole plus the selective estrogen-receptor down-regulator fulvestrant, as compared with anastrozole alone, as first-line therapy. We now report final survival outcomes. METHODS We randomly assigned patients to receive either anastrozole or fulvestrant plus anastrozole. Randomization was stratified according to adjuvant tamoxifen use. Analysis of survival was performed by means of two-sided stratified log-rank tests and Cox regression. Efficacy and safety were compared between the two groups, both overall and in subgroups. RESULTS Of 707 patients who had undergone randomization, 694 had data available for analysis. The combination-therapy group had 247 deaths among 349 women (71%) and a median overall survival of 49.8 months, as compared with 261 deaths among 345 women (76%) and a median overall survival of 42.0 months in the anastrozole-alone group, a significant difference (hazard ratio for death, 0.82; 95% confidence interval [CI], 0.69 to 0.98; P = 0.03 by the log-rank test). In a subgroup analysis of the two strata, overall survival among women who had not received tamoxifen previously was longer with the combination therapy than with anastrozole alone (median, 52.2 months and 40.3 months, respectively; hazard ratio, 0.73; 95% CI, 0.58 to 0.92); among women who had received tamoxifen previously, overall survival was similar in the two groups (median, 48.2 months and 43.5 months, respectively; hazard ratio, 0.97; 95% CI, 0.74 to 1.27) (P = 0.09 for interaction). The incidence of long-term toxic effects of grade 3 to 5 was similar in the two groups. Approximately 45% of the patients in the anastrozole-alone group crossed over to receive fulvestrant. CONCLUSIONS The addition of fulvestrant to anastrozole was associated with increased long-term survival as compared with anastrozole alone, despite substantial crossover to fulvestrant after progression during therapy with anastrozole alone. The results suggest that the benefit was particularly notable in patients without previous exposure to adjuvant endocrine therapy. (Funded by the National Cancer Institute and AstraZeneca; ClinicalTrials.gov number, [Related object:] .)
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            Anastrozole therapy in recurrent ovarian adult granulosa cell tumors: a report of 2 cases.

            Ovarian sex cord stromal tumors are frequently hormonally active, and adult granulosa cell tumors often demonstrate estrogen receptor positivity. Thus, hormonal agents have been evaluated as potential treatments for advanced stage or recurrent adult granulosa cell tumors. Two cases of patients with recurrent adult granulosa cell tumors are presented. Each patient received multiple treatment modalities including chemotherapy and had previously progressed on leuprolide. Both patients were started on anastrozole with subsequent normalization of inhibin B levels and clinical exams. They have been maintained on treatment for 14 and 18 months, respectively, and have tolerated the drug without difficulty. Aromatase inhibitors may be a viable treatment option for women with advanced stage or recurrent ovarian adult granulosa cell tumors.
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              Leuprolide acetate for treating refractory or persistent ovarian granulosa cell tumor.

              To evaluate leuprolide acetate for treating refractory or persistent ovarian granulosa cell tumor (GCT). We treated six patients who had recurrent or persistent ovarian GCT with monthly intramuscular injections of a depot formulation of leuprolide acetate, 7.5 mg. Four patients had received prior cisplatin-based chemotherapy. Initially these patients' tumors had responded to the cisplatin, but then the disease had progressed in all cases. One of these four patients also had received prior therapy with tamoxifen that had resulted in three months of stable disease. Two patients had received no prior chemotherapy. Responses could be evaluated in five of the six patients; in the sixth patient, only survival was evaluable. Among the five evaluable patients, two achieved partial responses lasting 3 and 11 months, with disease progression-free intervals of 4 and 12 months. The other three patients (60%; 95% confidence interval [CI], 20-95%), including two whose disease had progressed on chemotherapy, had stable disease with disease progression-free intervals of 3+, 3+ and 13+ months. The objective response rate was 40% (two of five patients; 95% CI, 10-90%). Cessation of disease progression was noted in all five of the evaluable patients (100%; 95% CI, 40-100%). The sixth patient, whose disease could not be evaluated, was treated with leuprolide acetate for 24 months and at this writing had no clinical evidence of disease. The therapy was convenient to administer, and no major side effects were noted. Leuprolide acetate thus appears to have activity in patients with refractory GCT, and it may prolong the disease progression-free interval. Further trials of this relatively nontoxic and convenient therapy are warranted.
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                Author and article information

                Contributors
                Journal
                Gynecol Oncol Rep
                Gynecol Oncol Rep
                Gynecologic Oncology Reports
                Elsevier
                2352-5789
                16 March 2022
                April 2022
                16 March 2022
                : 40
                : 100961
                Affiliations
                [a ]Rutgers New Jersey Medical School Department of Obstetrics & Gynecology and Reproductive Health 185 South Orange Ave, Medical Science Building E-503, Newark, NJ 07103
                [b ]Hackensack University Medical Center Department of Hematology & Oncology, Division of Gynecologic Medical Oncology 92 2nd Street, Hackensack, NJ 07601
                [c ]Hackensack University Medical Center Department of Obstetrics and Gynecology, Division of Gynecologic Oncology 92 2nd Street, Hackensack, NJ 07601
                Author notes
                Article
                S2352-5789(22)00041-8 100961
                10.1016/j.gore.2022.100961
                8933688
                7817d680-a472-430b-8d4b-4ecca6cf6aba
                © 2022 The Author(s)

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 29 January 2022
                : 11 March 2022
                : 13 March 2022
                Categories
                Case Report

                granulosa cell tumors,extraovarian,retroperitoneal,covid-19 pandemic,delays in care

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