Rare Ovarian Neuroendocrine Cancer Treated With Surgery and Chemotherapy

What happened, in summary

A 48-year-old woman sought care after 3 months of increasing abdominal discomfort and a palpable pelvic mass. Imaging showed a 16 cm complex mass arising from the left ovary. There was no ascites or visible peritoneal disease, chest CT showed no metastatic disease, and gastrointestinal evaluation did not identify another primary cancer. She underwent exploratory surgery with hysterectomy, removal of both ovaries and fallopian tubes, pelvic and para-aortic lymph-node dissection, omentectomy, and adhesiolysis. The ovarian mass had ruptured and involved the surface, but all 13 removed lymph nodes were negative for metastasis. Pathology confirmed Grade 3 large cell neuroendocrine carcinoma, a rare ovarian cancer. Additional testing supported the ovary as the primary site rather than a metastasis from the gastrointestinal tract. After surgery, she received 6 cycles of cisplatin and etoposide chemotherapy. Molecular testing of the tumor identified a somatic BRCA2 mutation. The tumor was also microsatellite instability-high and had a high tumor mutational burden. Blood testing did not find a germline BRCA2 mutation. These findings pointed to possible future treatment options beyond the surgery and chemotherapy she had already received. Follow-up with CT imaging every 3 to 6 months and serial CA-125 testing showed no evidence of recurrence over 2 years.