Female patients with pseudomyxoma peritonei: a single-institution clinicopathologic study of 35 cases

PURPOSE: To explore clinicopathologic/prognostic aspects of pseudomyxoma peritonei (PMP). METHODS: We reviewed records of 35 female patients with PMP treated at a single institution. RESULTS: Patients' median age was 57.0 years (range 35.0-71.0 years). Their median pre-surgery level of carbohydrate antigen 19-9 (CA-199) was 80.95 U/ml (range 0.00-1,562.10 U/ml); of carbohydrate antigen 12-5 (CA-125), 44.00 U/ml (range 0.90-231.20 U/ml); and of carcinoembryonic antigen (CEA), 17.20 ng/ml (range 2.04-211.60 ng/ml). Of the 35 patients, 23 (65.7 %) underwent cytoreductive surgery (CRS) by gynecological oncologists and 12 (34.3 %) underwent non-CRS surgeries by general gynecologists or surgeons, including one patient who refused surgical treatment beyond a diagnostic laparoscopy. After surgery, 18 patients (51.4 %) had residual lesions, 11 (31.4 %) had complete lesion removal and 6 (17.1 %)had insufficient information on residual lesion; 21 (60.0 %) had appendiceal-based tumors and 12 (34.3 %) had ovarian-based tumors. Median follow-up time was 37 months (range 1-148 months), during which 28 patients (80.0 %) had relapsed. By the end of the study, 12 patients (34.3 %) died of PMP, 16 (45.7 %) survived with disease, and 7 (20 %) survived without disease. Median progress-free survival (PFS) was 12 months (range 0.5-114.0 months). Median overall survival time was 42 months (range 5-150 months). Ovarian tumor origin, post-surgery residual lesions, preoperative CA199 > 258.9 U/ml and CA125 > 70.6 U/ml were independent predictors of PFS. CONCLUSIONS: PMP is rare in women, and has a poor long-term survival rate. Multi-center cooperation to gather more cases is needed to explore its behavior and proper treatment.