Introduction
Primary peritoneal serous carcinoma (PPSC) is an infrequent type of cancer initially documented in 1959. This cancer is similar to serous ovarian carcinoma. This resemblance can be due to the common Mullerian origin from the same embryonic mesoderm. This has suggested a potential genetic link between primary peritoneal serous carcinoma and hereditary breast-ovarian cancer, particularly involving BRCA (breast cancer gene 1) mutations. There will be a prominent mass located in the pelvic peritoneum. Abdominal distension, abdominal discomfort, and pain are the commonly noticed symptoms of primary peritoneal serous carcinoma. These symptoms can sometimes overlap with the symptoms of peritoneal carcinomatosis. Most of the cases have been notably identified at advanced stages.
What Is the Etiology Behind Primary Peritoneal Serous Carcinoma?
The origin of this disease is still under research and unclear. It appears that primary peritoneal serous carcinoma may be linked with hereditary breast-ovarian cancer syndrome, as the frequency of BRCA mutations in cases of ovarian and peritoneal cancer is comparable. Two theories have been suggested regarding the cause of primary peritoneal cancer. One theory suggests that the tumor can develop from the malignant change of the nests formed from the ovarian tissue remnants that are left within the peritoneum during embryonic gonadal migration. Another theory suggests that primary peritoneal cancer can arise from a secondary mesodermal Müllerian system that exists in the peritoneal mesothelium. These two studies infer different explanations for the origin of primary peritoneal cancer.
What Is the Incidence of Primary Peritoneal Serous Carcinoma?
Primary peritoneal serous carcinoma, being a rare form of cancer, has been accounted as ovarian cancer initially for about 7% of cases. It is characterized by the spread of cancer throughout the pelvic and abdominal areas with no involvement or little involvement of the ovaries. This rare form of cancer has been frequent in Caucasian women who were nearing or at their menopause (end of menstrual cycle) years, usually in the 7th decade. Women who have a hereditary history of ovarian carcinoma have a 10-fold heightened risk of developing primary peritoneal cancer (PPC) and need monitoring despite preventive oophorectomy.
What Are the Variants of Primary Peritoneal Cancer?
Primary peritoneal cancer (PPC) consists of many variants, including
-
Malignant Mesothelioma (MPM): A cancer affecting the mesothelium, a thin tissue covering the chest wall, lung, and abdomen.
-
Leiomyosarcomas: A rare type of cancer affecting the smooth muscles and commonly seen in the uterus or abdomen.
-
Extraovarian Primary Peritoneal Carcinoma: A type of adenocarcinoma that originates from the lining of the pelvis and abdomen, also known as peritoneum.
-
Leiomyomatosis Peritonealis Disseminata: A benign lesion of smooth muscles that can rarely turn malignant, often affecting premenopausal women.
-
Multicystic Mesothelioma: A benign tumor arising from the peritoneum and often affecting women in their reproductive age.
-
Desmoplastic Small Round Cell Tumor: An infrequent and aggressive tumor affecting young adults and adolescents. Desmoplastic round small-cell tumor is usually seen in young adults and adolescents with a median age of 19 years.
It predominantly occurs in Caucasians, accounting for about 85 % of cases. This cancer can occur due to a specific translocation, t(11;22)(p13;q12), and it is classified within the Ewing's sarcoma tumor family. Uncommon sites of spread, including the main bronchus and brain parenchyma, as well as lymph node involvement, have been reported in cases of primary peritoneal carcinoma. This shows that the cancer has the potential to metastasize to atypical locations beyond the primary peritoneal and ovarian regions. The symptoms during the onset are nonspecific and varied and observed as
-
Abdominal pain.
-
Loss of appetite.
-
Early fullness after eating.
-
Bloating.
-
Weight loss.
Imaging tests reveal an abdominal mass, and also lymph node enlargement is a common finding. Liver metastasis might be detected at the initial diagnostic phase. Unfortunately, this tumor is known to be aggressive and has a poor prognosis. This tumor is managed through neoadjuvant chemotherapy, especially using the P6 protocol, which includes drugs like Ifosfamide, Cyclophosphamide, Etoposide, Vincristine, Doxorubicin, and Mesna. After this, the next step involves a vigorous surgical procedure to remove the tumor. Additional therapy may include hyperthermic intraperitoneal chemotherapy (HIPEC). For both adult and pediatric cases, comprehensive whole abdominal radiotherapy is necessary to improve the overall treatment outcome. When there is a recurrence, palliative chemotherapy can be employed.
How Is Primary Peritoneal Serous Carcinoma Diagnosed and Treated?
The diagnosis of this condition follows Gynecology Oncology Group (GOG) criteria that include the normal or abnormal size of the ovaries, the widespread involvement of extra ovarian sites compared to the ovarian surfaces, and histopathological characteristics similar to epithelial ovarian cancer. Diagnosing primary peritoneal serous carcinoma before surgery can be a significant challenge, with cases often mistaken for omental metastasis, peritoneal carcinomatosis from the gastrointestinal tract, or ovarian malignancy.
PET/CT (positron emission tomography/computed tomography) plays an important role in the accurate diagnosis by delineating the extent of metabolically active disease and identifying distant metastases. This imaging technique is especially important in primary peritoneal serous carcinoma diagnosis as it helps in differentiating primary peritoneal serous carcinoma from ovarian cancer.
A combination of chemotherapy and surgical resection is the recommended treatment for primary peritoneal serous carcinoma, with platinum-based chemotherapy being the most often used treatment. Recent studies have suggested that intraperitoneal chemotherapy has provided a survival advantage compared to other treatments that only involve surgery or a combination of chemotherapy and surgery. This finding highlights the benefits of intraperitoneal chemotherapy in enhancing results for patients with conditions like primary peritoneal serous carcinoma.
What Is the Prognosis?
The survival time of patients with primary peritoneal serous carcinoma is found to be 11 to 17 months. A study has shown that there is a mean 5-year survival rate of 40 % in high-grade serous carcinoma. The stage of the disease, the debulking status post-surgery, and the response to chemotherapy and other treatment methods are important prognostic factors to be considered. Overall, there has been a maximum cervical of 89 months in a female patient who underwent two visits of neoadjuvant chemotherapy, which was followed by cytoreductive surgery, which was combined along with six cycles of platinum-based intraperitoneal chemotherapy and HIPEC (hyperthermic intraperitoneal chemotherapy) postoperatively.
Conclusion
Primary peritoneal serous carcinoma is one of the rare forms of cancer and is challenging to diagnose. Though it has been difficult to diagnose, people at risk of developing this disease, like menopausal women, should be aware of the symptoms and seek professional advice in case they encounter any abnormal symptoms. An early diagnosis of this condition can help in early intervention, thereby helping improve the quality of life of patients and improve survival rates.
