From the Guidelines
Primary peritoneal serous carcinoma is a rare and aggressive type of cancer that closely resembles ovarian cancer in its appearance, behavior, and treatment approach, and is characterized by peritoneal carcinomatosis in females with serous or undifferentiated adenocarcinoma histology, often with elevated CA125 and BRCA1/2 mutations. This cancer primarily affects women, particularly those in post-menopausal age, and can occur even in women who have had their ovaries removed 1. The disease typically presents with symptoms similar to ovarian cancer, including abdominal distension, pain, bloating, changes in bowel habits, and sometimes ascites (fluid buildup in the abdomen). Diagnosis usually involves imaging studies, blood tests for tumor markers like CA-125, and ultimately surgical biopsy.
Key Characteristics
- Peritoneal carcinomatosis in females with serous or undifferentiated adenocarcinoma histology
- Often with elevated CA125 and BRCA1/2 mutations
- Closely resembles ovarian cancer in appearance, behavior, and treatment approach
- Primarily affects women, particularly those in post-menopausal age
- Can occur even in women who have had their ovaries removed
Treatment Approach
Treatment generally follows protocols similar to ovarian cancer, consisting of:
- Cytoreductive surgery to remove as much visible tumor as possible
- Chemotherapy with platinum-based agents (such as carboplatin) and taxanes (like paclitaxel)
- Optional addition of bevacizumab and poly (ADP-ribose) polymerase (PARP) inhibitor maintenance therapy in responding patients 1 The disease is often diagnosed at an advanced stage because early symptoms can be vague or absent, which contributes to its generally poor prognosis. The exact cause remains unclear, though genetic factors, particularly BRCA gene mutations, may increase risk. Recent guidelines, such as those from the ESMO 1, provide a framework for diagnosis, treatment, and follow-up, emphasizing the importance of a multidisciplinary approach to manage this complex disease.
From the Research
Definition and Characteristics
- Primary peritoneal serous carcinoma (PPSC) is a rare primary malignancy that diffusely involves the peritoneum, indistinguishable clinically and histopathologically from primary serous ovarian carcinoma 2.
- PPSC is an extremely rare malignancy that arises from the peritoneal epithelium and is similar to serous ovarian carcinoma 3.
- It is characterized by a unique range of clinical features and imaging findings, although it shares many clinical, histologic, and imaging features with serous ovarian carcinoma 4.
Diagnosis and Clinical Presentation
- A diagnosis of PPSC is typically made based on the Gynecology Oncology Group criteria; however, a correct differential diagnosis of PPSC is difficult preoperatively 3.
- The most common presenting symptoms are abdominal distension and ascites 5.
- Pretreatment CA125 levels are significantly elevated in most patients 5.
- Imaging modalities used in the evaluation of PPSC include ultrasound, CT, MRI, and PET/CT 4.
Treatment and Prognosis
- PPSC is often inoperable, and chemotherapy and palliative care are the only offered treatment 2.
- Cytoreductive surgery combined with pre/postoperative platinum-based chemotherapy may be effective for PPSC patients 5.
- The prognosis of PPSC is often equally poor as its primary ovarian counterpart, with presentations common in late stages of disease 4.
- The median overall survival of PPSC patients is around 21 months 5.