Background: Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon primary malignancy of the peritoneum. The aim of our study is to present the characteristics of such a rare entity through our case series.
Method: This is a retrospective study of nine cases of PPPSC who were treated between January 2002 and April 2007 in METAXA Memorial Cancer Hospital, Piraeus, Greece. Medical files and histopathological diagnosis of each patient were retrospectively studied.
Results: The median age of the patients was 63 years ranging from 44 up to 74 years. Clinically PPPSC presented with general abdominal discomfort in all of the patients. Three out of nine patients presented with constipation, 5/9 with distention, and 7/9 with ascites. All of the patients referred loss of appetite with weight gaining due to ascites. All the patients had pathological values of CA125 (over 35 U/ml) ranging from 125 up to 1,255 U/ml with median value 565 U/ml. Optimal debulking was possible in 3/9 of patients who were consequently treated with standard taxol-platin chemotherapy. Complete response was achieved in one woman. The median disease-free survival was 7 months and the median overall survival rate was 2.5 years.
Conclusion: PPPSC mimics ovarian papillary serous carcinoma regarding the clinical and laboratory characteristics but it has worse prognosis.