Abstract

Peritoneal papillary serous adenocarcinoma is a rare müllerian tumor type that is histologically similar to ovarian serous carcinoma. The low incidence of peritoneal papillary serous adenocarcinoma has hindered the establishment of a standard of care for this disease. A 56-year-old woman, who had undergone an abdominal hysterectomy 25 years earlier, presented with a vaginal nodule and palpable pelvic mass. She underwent debulking surgery and was diagnosed with stage IIIC peritoneal papillary serous adenocarcinoma. After 6 cycles of paclitaxel/carboplatin and 10 cycles of liposomal doxorubicin/cyclophosphamide, laparotomy revealed microscopic disease. This heavily pretreated patient then started topotecan therapy (1.0 mg/m²/day, ×5 days). After several dosing adjustments because of fatigue and malaise, weekly topotecan 4.0 mg/m² was determined to be the best-tolerated dosage. The patient ultimately received 14 weekly cycles of topotecan. After 12 weekly cycles, she achieved a complete response, which was maintained for 25 weeks with weekly topotecan therapy with a subsequent recurrence. Topotecan was reinstated. She maintains a disease-free interval of 58 weeks. Weekly topotecan demonstrated antitumor activity and was well tolerated in a patient with recurrent peritoneal papillary serous adenocarcinoma.