Long-term survival of high-grade primary peritoneal papillary serous adenocarcinoma: a case report and literature review

BACKGROUND: Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon disease which has a high malignancy and a poor prognosis. CASE PRESENTATION: We report here a long-term survival case of PPPSC with postoperative lung metastasis. A 62-year-old female patient with PPPSC was administered two cycles of neoadjuvant chemotherapy (NAC) followed by cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) and six cycles of platinum-based (docetaxel + carboplatin) intraperitoneal chemotherapy postoperatively. The patient reached a complete remission at the completion of primary treatment. Malignant thoracic effusion and lung metastasis developed 5 months after the treatment. The patient underwent video-assisted thoracoscopic surgery plus hyperthermic intrapleural chemotherapy. CONCLUSIONS: Up to present, the patient has been survived with tumor for over 86 months with a good performance status, with only encapsulated effusion found at the latest follow-up. As a relatively new regime, the application of CRS + HIPEC in our patient has been proved example for MPE management, although more large-scale studies are needed to substantiate its efficiency and safety.