Recurrent Malignant Ovarian Steroid Cell Tumor Treated With Multiple Therapies

What happened, in summary

A premenopausal woman in her 40s had 5 months of lower abdominal pain. Imaging found a 13 cm ovarian tumor, while blood testing showed markedly elevated testosterone at 478.6 ng/dL and CA-125 at 624.6 IU/mL. She underwent total abdominal hysterectomy, removal of both ovaries and fallopian tubes, and omentectomy, leaving no visible residual tumor. Pathology identified a stage IC2 malignant ovarian steroid cell tumor, not otherwise specified.

She received 6 cycles of paclitaxel, carboplatin, and bevacizumab every 3 weeks, followed by maintenance bevacizumab. Eight months later, a 4 cm pelvic recurrence was removed surgically. The pelvis then received 50.4 Gy of radiation, and leuprorelin hormone blockade was started. A second 3 cm pelvic recurrence appeared 2 months later. Four courses of bleomycin, etoposide, and cisplatin were followed by 6 courses of etoposide and cisplatin, producing a complete PET-CT response. Olaparib was then used as maintenance treatment.

After 1 year, a 5 mm recurrence in the upper left lung was treated with partial lung resection. Tumor sequencing found somatic ARID1A, PIK3CA, TERT, and ATM mutations. She described severe fatigue and widespread pain after chemotherapy, sometimes requiring medical narcotics, but also emphasized the reassurance she gained from a responsive clinical team.