Affiliation:
1. NYU School of Medicine & Divisions of Medical Oncology & Gynecologic Oncology of the Perlmutter Cancer Center at NYU Langone Health, New York, NY 10016, USA
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.
Subject
Cancer Research,Endocrinology,Oncology,Endocrinology, Diabetes and Metabolism
Cited by
15 articles.
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