Serous peritoneal psammocarcinoma with an aggressive course: a case and review of the literature.

Background: Primary serous peritoneal psammocarcinoma (PSPP) is a rare variant of serous carcinoma characterized by massive psammoma body formation and low-grade cytological features. Patients with serous psammocarcinoma have a protracted clinical course and relatively favourable prognosis, although a more aggressive course of PSPP may occur.

Case Presentation: A 52-year-old woman suffering from abdominal pain with ascites and serum CA-125 level substantially elevated underwent an exploratory laparoscopy which revealed bulk disease. The pathology report detected PPSP at the FIGO stage IIIC. The patient received neoadjuvant chemotherapy (3 courses of paclitaxel/pegylated liposomal doxorubicin/carboplatin). Optimal interval debulking surgery was performed as the next step, followed by three courses of adjuvant chemotherapy (paclitaxel/carboplatin). Due to the fact that the patient had residual disease, at the second-look surgery she received consolidation therapy with intraperitoneal and intravenous chemotherapy carboplatin. Eight months after the completion of treatment the patient developed disease recurrence in the peritoneum. Palliative surgery (enterostomy) was performed. Furthermore, the patient received two lines of chemotherapy consisting of cyclophosphamide/cisplatin and then gemcitabine. After twenty five months she developed brain metastases, treated with palliative radiotherapy. The patient died twenty-eight months since her primary presentation of PSPP.

Conclusion: PSPP is an infrequent variant of epithelial cancer with favourable prognosis. The disease may however, take a more aggressive course. Thus, an aggressive therapy is required to postpone the progression.