Primary peritoneal serous borderline tumors (PPSBT) are rare neoplasms that can present as an incidental finding at laparoscopy and raise concern regarding a primary ovarian tumor with peritoneal implants. The aim is to present an overview of all reported cases of PPSBT, including a case from our own department, with a focus on clinical presentation, diagnosis, therapeutic options, and prognosis. A search for articles containing various terminologies describing PPSBT was performed via PubMed. We included English and French language publications from 1966 to May 2019. All identified manuscripts (N = 15) were reviewed completely. To date, 229 cases of PPSBT have been reported in literature. Most patients present with infertility or abdominal pain. Diagnosis is based on histopathology since appropriate imaging or specific tumor markers are lacking. The main therapy applied in the majority of cases was simple (74 cases, 32.3%) or extended (136 cases, 59.4%) resection. Prognosis seems good, independent of the extent of surgery, with recurrence rates below 25%, and follow-up periods from 5 months to > 30 years. PPSBT is a rare condition, often found in women of reproductive age with a history of infertility or abdominal pain. In most cases, the diagnosis is established incidentally during laparoscopy or laparotomy followed by histopathology. Considering the good prognosis even with incomplete resection, limited surgery to retain fertility might be good initial options. Since the course of the disease can comprise decades, a long-term follow-up is crucial.
