Primary peritoneal tumor

Primary peritoneal tumor refers to a group of rare neoplasms that arise directly from the peritoneum — the thin membrane lining the abdominal cavity and covering the abdominal organs — without originating from another organ such as the ovary, gastrointestinal tract, or appendix. The most well-known subtype is primary peritoneal carcinoma (PPC), also called primary peritoneal serous carcinoma or extraovarian peritoneal serous papillary carcinoma. This condition predominantly affects women and shares histological and clinical features with high-grade serous ovarian carcinoma. Patients typically present with abdominal distension, bloating, abdominal or pelvic pain, ascites (fluid accumulation in the abdomen), and sometimes bowel obstruction or weight loss. Elevated CA-125 levels are frequently observed. Because the peritoneum and the surface epithelium of the ovary share a common embryological origin (coelomic epithelium), primary peritoneal carcinoma can occur even in women who have previously undergone bilateral oophorectomy (surgical removal of both ovaries). The condition is most commonly diagnosed in postmenopausal women, typically over the age of 60. Germline mutations in BRCA1 and, to a lesser extent, BRCA2 genes have been identified as significant risk factors, and women with hereditary breast-ovarian cancer syndrome are at increased risk. Other primary peritoneal tumors include malignant peritoneal mesothelioma and desmoplastic small round cell tumor, though these are classified separately. Treatment for primary peritoneal carcinoma generally follows the same protocols as advanced epithelial ovarian cancer and includes cytoreductive (debulking) surgery combined with platinum- and taxane-based chemotherapy. Intraperitoneal chemotherapy and targeted therapies such as PARP inhibitors (particularly in BRCA-mutated cases) and bevacizumab may also be employed. Despite treatment, prognosis is often guarded due to the advanced stage at diagnosis, though outcomes are comparable to those of advanced ovarian cancer.

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