Peritoneal inclusion cyst

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ORPHA:168816C45.1
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Overview

Peritoneal inclusion cyst, also known as benign cystic mesothelioma or benign multicystic peritoneal mesothelioma, is a rare condition characterized by the formation of thin-walled, fluid-filled cystic masses within the peritoneal cavity (the lining of the abdomen and pelvis). Despite the ICD-10 classification under malignant mesothelioma codes (C45.1), peritoneal inclusion cysts are generally considered benign or borderline neoplasms arising from the mesothelial cells that line the peritoneum. The condition predominantly affects women of reproductive age, often those with a history of prior abdominal or pelvic surgery, pelvic inflammatory disease, or endometriosis. The cysts typically develop in the pelvis and may surround or encase the ovaries and other pelvic structures. Key symptoms include chronic or recurrent pelvic or abdominal pain, abdominal distension, and the presence of a palpable abdominal or pelvic mass. Some patients may experience gastrointestinal symptoms such as bloating, nausea, or changes in bowel habits due to mass effect. The condition can be challenging to diagnose, as imaging findings may mimic ovarian cysts, lymphangiomas, or malignant peritoneal mesothelioma. Diagnosis is typically confirmed through histopathological examination showing benign mesothelial-lined cystic spaces. Treatment primarily involves surgical excision, which may range from laparoscopic drainage and cyst removal to more extensive debulking surgery depending on the size and extent of the cysts. However, recurrence rates are notably high, reported in up to 50% of cases, often necessitating repeated surgical interventions. Hormonal therapies, including gonadotropin-releasing hormone agonists and tamoxifen, have been explored as adjunctive treatments to reduce recurrence risk, though evidence remains limited. Sclerotherapy has also been attempted in some cases. Long-term follow-up is recommended due to the significant recurrence rate, though malignant transformation is exceedingly rare.

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PeritonitisHP:0002586DyspareuniaHP:0030016MetrorrhagiaHP:0100608
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Peritoneal inclusion cyst.

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No actively recruiting trials found for Peritoneal inclusion cyst at this time.

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No specialists are currently listed for Peritoneal inclusion cyst.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Peritoneal inclusion cyst.

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Common questions about Peritoneal inclusion cyst

What is Peritoneal inclusion cyst?

Peritoneal inclusion cyst, also known as benign cystic mesothelioma or benign multicystic peritoneal mesothelioma, is a rare condition characterized by the formation of thin-walled, fluid-filled cystic masses within the peritoneal cavity (the lining of the abdomen and pelvis). Despite the ICD-10 classification under malignant mesothelioma codes (C45.1), peritoneal inclusion cysts are generally considered benign or borderline neoplasms arising from the mesothelial cells that line the peritoneum. The condition predominantly affects women of reproductive age, often those with a history of prior a

How is Peritoneal inclusion cyst inherited?

Peritoneal inclusion cyst follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Peritoneal inclusion cyst typically begin?

Typical onset of Peritoneal inclusion cyst is adult. Age of onset can vary across affected individuals.