Enormous Cystic Tumor of Peritoneal Psammocarcinoma Exhibiting Complete Response to Cisplatin and Cyclophosphamide after Suboptimal Cytoreduction: Case Report and Review of the Literature

Abstract

Psammocarcinoma is a serous peritoneal tumor arising from the ovary or the peritoneum and characterized by low-grade nuclear features, extensive psammoma bodies, and invasiveness. Only 62 cases have ever been documented, 30 primary peritoneal and 32 primary ovarian, most of which presented as small tumors. Adjuvant therapies, including chemotherapy and radiation, were performed in 12 of the primary peritoneal cases, without any clear evidence of benefit. We present a case of an unusually large primary peritoneal psammocarcinoma with unexpected outcome. The patient was a 38-year-old woman with a tumor of the peritoneum which adhered densely to the uterus and rectum and developed into the intra-abdominal cavity and retroperitoneal space. After adhesiolysis of the tumor and rectum, suboptimal surgical reduction left a 4 cm × 2 cm tumor segment. Postoperative chemotherapy, consisting of paclitaxel and carboplatin (TC) for 1 course, and cyclophosphamide and cisplatin (CP) for 5 courses, was conducted. The residual tumor responded completely to the chemotherapy and the patient is alive today, with no evidence of disease 15 months after the surgery. Our case implies that CP therapy is a potential regimen of postoperative remission-induction therapy for suboptimally resected primary peritoneal psammocarcinoma.

Case Report: Fortuitous discovery of primary peritoneal psammocarcinoma

“…8 Although most of PPP seem to display an indolent clinical course, some may be very aggressive, with distant metastases. 7 One of the strengths of our study is that we were able to demonstrate with certainty that the primary origin of the psammocarcinoma was peritoneal and not ovarian. However, its major limitation consists in the lack of follow-up.…”

“…3 Chemotherapy is thought to have a poor efficiency in PPP. 16 However, follow-up adjuvant chemotherapy is required, at least for residual diseases and for PPP with aggressive behavior like in the case reported by Akbulut et al 7,21 Given their rarity, we do not dispose enough elements to judge the scalability of PPPs. However, PPP is likely to have a high potential towards peritoneal recurrence, even after suitable treatment.…”

“…[3][4][5][6] In the majority of cases, PPP has a favorable prognosis, although some may show recurrences and metastases. 7 We report a rare case of primary peritoneal psammocarcinoma (PPP) in a 74-years-old female, discovered fortuitously within a laparotomy for gallbladder lithiasis.…”

Long-Term Follow-Up of Tamoxifen Treatment and the Use of Imaging in Psammocarcinoma: A Case Report, Review of the Literature and Discussion of Diagnostic and Therapeutic Challenges

“…In the case of residual disease, only four of the reports (presented with individual details for each patient) have a follow-up period of more than a year. In this subgroup, there are two reports where the patient was treated with chemotherapy [16,35]. Takahashi et al present a case with a complete response to combination therapy after suboptimal cytoreductive surgery [35].…”

“…In this subgroup, there are two reports where the patient was treated with chemotherapy [16,35]. Takahashi et al present a case with a complete response to combination therapy after suboptimal cytoreductive surgery [35]. Debbagh et al presented a case where surgical exploration revealed peritoneal carcinosis and an ovarian mass, although without long-term follow-up [36].…”