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Hormonal based treatment of ovarian anaplastic ependymoma with anastrozole
Justin Wayne Gorski1, Jolyn Sharpe Taylor1, Jing Zhang2
1Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Unit 1362, P.O. Box 301439, Houston, TX 77230-1439, USA.
Objective:
Ovarian anaplastic ependymoma is a rare gynecologic malignancy that poses diagnostic and treatment challenges. Treatment of sub-optimally debulked disease usually portends poor prognosis. Molecular testing of tumor specimen can identify more specific targets for additional therapy such as estrogen and progesterone receptors (ER/PR).
Case:
A 29-year-old woman presented with incidental finding of large bilateral adnexal masses and elevated CA 125. Biopsy proved anaplastic ovarian ependymoma with high ER/PR expression. She underwent sub-optimal surgical debulking followed by adjuvant chemotherapy with bleomycin, etoposide and cisplatin (BEP) which resulted in a partial response. Due to extensive residual disease she has been maintained on anastrozole for over fifteen months without increased tumor burden. Targeted somatic mutation testing was negative for all high risk clinically useful variants.
Conclusion:
Aromatase inhibitors may be considered in patients with extra-axial anaplastic ependymoma and can produce prolonged stable disease.
Insights
Anaplastic ovarian ependymoma, a rare cancer, can be treated with aromatase inhibitors like anastrozole, potentially leading to prolonged stable disease in patients with estrogen and progesterone receptor-positive tumors.
Area of Science:
- Gynecologic Oncology
- Neuro-oncology
- Endocrinology
Background:
- Ovarian anaplastic ependymoma is a rare malignancy with challenging diagnosis and treatment.
- Sub-optimal debulking of ovarian anaplastic ependymoma is associated with poor prognosis.
- Estrogen and progesterone receptor (ER/PR) expression in tumors can guide targeted therapy.
Observation:
- A 29-year-old woman presented with bilateral adnexal masses and elevated CA 125.
- Biopsy confirmed anaplastic ovarian ependymoma with high ER/PR expression.
- Initial treatment with chemotherapy (bleomycin, etoposide, cisplatin) resulted in partial response.
Findings:
- Maintenance therapy with anastrozole (an aromatase inhibitor) led to over fifteen months of stable disease.
- Targeted somatic mutation testing did not reveal high-risk, clinically actionable variants.
- High ER/PR expression suggests a potential role for endocrine therapy.
Implications:
- Aromatase inhibitors may be a viable treatment option for ovarian anaplastic ependymoma, particularly in ER/PR-positive cases.
- This case highlights the potential for endocrine therapy in managing residual or recurrent disease.
- Further research is warranted to explore the efficacy of aromatase inhibitors in this rare gynecologic malignancy.