Young Woman With Rare Small Cell Ovarian Cancer and Abdominal Recurrence

What happened, in summary

A 30-year-old woman came to the emergency department with vomiting, groin and lower abdominal pain, fatigue, weight loss, poor appetite, and intermittent nausea. Imaging showed a large left ovarian mass. She underwent exploratory surgery with removal of the tumor, hysterectomy, removal of both ovaries and fallopian tubes, omentectomy, and pelvic and para-aortic lymph-node dissection. Pathology diagnosed small cell carcinoma of the ovary, hypercalcemic type, a rare ovarian cancer. The tumor showed loss of SMARCA4 expression with retained SMARCB1 expression. A microscopic tumor deposit was present in the omentum, while 33 lymph nodes were negative and chest imaging showed no distant metastasis. The disease was classified as Stage III. She received 3 cycles of cisplatin and etoposide over 3 months. Eighteen months later, surveillance imaging showed a large pelvic-abdominal recurrence involving surrounding structures. She received 5 more cycles of chemotherapy, but the tumor continued to grow. She later declined further treatment because of poor tolerance and disease burden. The cancer caused an enterocutaneous fistula and extensive abdominal involvement. Palliative care was discussed. She left the hospital and died at home within a week, 26 months after her original diagnosis.