Ovarian adult-type granulosa cell tumor: focusing on endocrine-based therapies. (8th October 2018)

• Record Type: Journal Article
• Authors: Yang, Annie D; Curtin, John; Muggia, Franco
• Is Part Of: International journal of endocrine oncology. Volume 5:Number 2(2018)
• Publication Date: 2018-10-08
• Subjects: anastrazole -- antimullerian hormone -- aromatase -- endocrine therapies -- FOXL2 -- granulosa cell tumors -- inhibin B -- letrozole; Endocrine glands -- Cancer -- Periodicals; 616.9944005
• DOI: 10.2217/ije-2017-0021
• Languages: English
• ISSNs: 2045-0869

Abstract

Adult-type granulosa cell tumors (GCTs), although rare, are the most commonly diagnosed neoplasms arising in the endocrine-active ovarian stroma. They are characterized by excessive production of estrogens, antimullerian hormone and inhibins. In 2009, a specific mutation in FOXL2 was identified to be pathognomonic of GCTs. How dysregulation of this transcription factor, resulting in upregulation of aromatase, leads to unchecked proliferation, and progression to a malignancy, remains unclear. The key pathological and clinical feature of GCTs that affects their usually favorable outcomes is a diagnosis of greater than Stage 1 disease at presentation. Chemotherapy is given as adjuvant upon an advanced stage diagnosis; however, its effect on survival upon recurrence is modest. On the other hand, aromatase inhibitors also lead to tumor regression and are suitable for long-term maintenance.