Abstract:
Sertoli–Leydig cell tumors are rare ovarian neoplasms. We report
two unusual cases with bilateral SLCTs suggesting evidence of
genetic predisposition and at high risk of recurrence. To reduce this
risk, we exploited the use of GnRH analog to lower gondadotropin
and potentially directly inhibit the tumors through expressed GnRH
receptors. We used it as maintenance antitumor therapy for 2 years after completion of chemotherapy, to cover the period of risk for
recurrence. Both patients remain in complete remission at >2 years
after completing leuprorelin therapy. Of note, both patients carry
DICER1 mutations, frequently found in pleuropulmonary blastoma
syndrome.