Overview
Primary peritoneal carcinoma (PPC), also known as primary peritoneal serous carcinoma or extra-ovarian peritoneal serous papillary carcinoma, is a rare malignancy that arises from the peritoneum — the thin membrane lining the inner wall of the abdomen and covering most abdominal organs. This cancer is histologically and clinically very similar to advanced-stage epithelial ovarian cancer (particularly high-grade serous carcinoma), but it occurs in the peritoneal lining itself rather than in the ovaries. PPC can develop even in women who have previously had their ovaries removed (oophorectomy). It predominantly affects women, typically in the postmenopausal age group. The disease primarily affects the abdominal cavity and peritoneal surfaces, and may spread to involve the omentum, bowel surfaces, diaphragm, and other abdominal structures. Key symptoms include abdominal distension and bloating, abdominal or pelvic pain, ascites (fluid accumulation in the abdomen), early satiety, changes in bowel habits, unexplained weight loss or gain, and fatigue. Because symptoms are often nonspecific, PPC is frequently diagnosed at an advanced stage. Elevated CA-125 levels are commonly observed and used as a tumor marker. Treatment for primary peritoneal carcinoma follows protocols similar to those used for advanced epithelial ovarian cancer. The standard approach includes cytoreductive (debulking) surgery aimed at removing as much visible tumor as possible, followed by platinum-based and taxane-based combination chemotherapy. Intraperitoneal chemotherapy may also be considered. Targeted therapies, including bevacizumab (an anti-angiogenic agent) and PARP inhibitors (particularly in patients with BRCA1 or BRCA2 germline mutations), have shown benefit and are increasingly incorporated into treatment regimens. Patients with BRCA mutations may have improved responses to platinum-based chemotherapy and PARP inhibitor maintenance therapy. Despite treatment, the prognosis remains guarded due to the typically advanced stage at diagnosis, though outcomes are generally comparable to those of advanced ovarian serous carcinoma.
Also known as:
Clinical phenotype terms— hover any for plain English:
Multifactorial
Caused by a mix of several genes and environmental factors
Late onset
Begins later in life, typically after age 50
FDA & Trial Timeline
10 eventsCentre Oscar Lambret — NA
Massachusetts General Hospital — PHASE2
Institute of Hematology & Blood Diseases Hospital, China
Second Affiliated Hospital, School of Medicine, Zhejiang University — PHASE2
AbbVie
Beijing Biotech — PHASE1, PHASE2
Essen Biotech — PHASE1, PHASE2
Tongji Hospital
AbbVie — PHASE2
Sciensano — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
4 availableElahere
indicated for the treatment of adult patients with folate receptor-alpha (FRα) positive, platinum-resistant primary peritoneal cancer, who have received one to three prior systemic treatment regimens
Rubraca
indicated for the maintenance treatment of adult patients with a deleterious BRCA mutation (germline and/or somatic)-associated recurrent epithelial ovarian, fallopian tube, or primary peritoneal canc…
indicated for the maintenance treatment of adult patients with a deleterious BRCA mutation (germline and/or somatic)-associated recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy
Zejula
for the maintenance treatment of adult patients with deleterious or suspected deleterious germline BRCA-mutated recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a …
for the maintenance treatment of adult patients with deleterious or suspected deleterious germline BRCA-mutated recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy
Avastin
Epithelial ovarian, fallopian tube, or primary peritoneal cancer: in combination with carboplatin and paclitaxel, followed by Avastin as a single agent, for stage III or IV disease following initial s…
Epithelial ovarian, fallopian tube, or primary peritoneal cancer: in combination with carboplatin and paclitaxel, followed by Avastin as a single agent, for stage III or IV disease following initial surgical resection
Treatment Centers
8 centersFred Hutch/University of Washington Cancer Consortium
📍 Seattle, Washington
👤 Rosa Nadal Rios, MD, PhD
Stanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🏥 NORDBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
Financial Resources
2 resourcesTravel Grants
No travel grants are currently matched to Primary peritoneal carcinoma.
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5 articlesCaregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Common questions about Primary peritoneal carcinoma
What is Primary peritoneal carcinoma?
Primary peritoneal carcinoma (PPC), also known as primary peritoneal serous carcinoma or extra-ovarian peritoneal serous papillary carcinoma, is a rare malignancy that arises from the peritoneum — the thin membrane lining the inner wall of the abdomen and covering most abdominal organs. This cancer is histologically and clinically very similar to advanced-stage epithelial ovarian cancer (particularly high-grade serous carcinoma), but it occurs in the peritoneal lining itself rather than in the ovaries. PPC can develop even in women who have previously had their ovaries removed (oophorectomy).
How is Primary peritoneal carcinoma inherited?
Primary peritoneal carcinoma follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Primary peritoneal carcinoma typically begin?
Typical onset of Primary peritoneal carcinoma is late onset. Age of onset can vary across affected individuals.
Are there clinical trials for Primary peritoneal carcinoma?
Yes — 20 recruiting clinical trials are currently listed for Primary peritoneal carcinoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Primary peritoneal carcinoma?
25 specialists and care centers treating Primary peritoneal carcinoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Primary peritoneal carcinoma?
1 patient support program are currently tracked on UniteRare for Primary peritoneal carcinoma. See the treatments and support programs sections for copay assistance, eligibility, and contact details.