Primary peritoneal serous papillary carcinoma: a case series

To present the clinical and laboratory characteristics, as well as the management, of patients with primary peritoneal serous papillary carcinoma (PPSPC).

This is a retrospective study of 19 patients with PPSPC who underwent debulking surgery followed by first line chemotherapy and were managed in Metaxa Memorial Cancer Hospital between January 2002 and December 2017.

The median age of the patients was found to be 66 years (range 44-76 years). Clinical presentation of PPSPC included abdominal distention and pain, constipation, as well as loss of appetite and weight gain. Two of the patients did not mention any symptomatology and the disease was suspected by an abnormal cervical smear and elevated CA125 levels respectively. Biomarkers measurement during the initial management of the patients revealed abnormal values of CA125 for all the participants (median value 565 U/ml). Human epididymis secretory protein 4 (HE4) and ratios of blood count were also measured. Perioperative Peritoneal Cancer Index ranged from 6 to 20. Optimal debulking was achieved in 5 cases. All patients were staged as IIIC and IVA PPSPC and received standard chemotherapy with paclitaxel and carboplatin, whereas bevacizumab was added in the 5 most recent cases. Median overall survival was 29 months.

PPSPC is a rare malignancy, the management of which should take place in tertiary oncology centers.