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Peritoneal Mesothelioma

Disease Details

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Description: Peritoneal mesothelioma is a rare cancer that develops in the lining of the abdomen, known as the peritoneum, typically associated with asbestos exposure.
Type: Peritoneal mesothelioma is a rare and aggressive cancer that develops in the lining of the abdomen, known as the peritoneum.

Type of genetic transmission: Peritoneal mesothelioma is not typically considered a hereditary disease. It is most commonly associated with environmental exposure to asbestos rather than genetic factors. Although there may be a genetic predisposition in some cases, the disease itself is not usually passed down through families in a typical genetic manner.
Signs And Symptoms: Signs and symptoms of peritoneal mesothelioma may include:

- Abdominal pain or swelling
- Nausea or vomiting
- Unexplained weight loss
- Loss of appetite
- Bowel changes, such as diarrhea or constipation
- Fatigue or general weakness

These symptoms can be vague and may resemble other medical conditions, which can make early diagnosis challenging.
Prognosis: Peritoneal mesothelioma is a rare cancer that occurs in the lining of the abdomen, known as the peritoneum. The prognosis can vary significantly based on factors like the stage of the disease at diagnosis, the patient's overall health, age, and response to treatment. Generally, the prognosis for peritoneal mesothelioma may be more favorable compared to pleural mesothelioma, especially with treatments like cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Median survival rates can range from 1 to 5 years, but there are cases where patients live significantly longer with appropriate and aggressive treatment.
Onset: Peritoneal mesothelioma is a rare cancer that forms in the lining of the abdomen (peritoneum). The onset of this disease is often associated with long-term exposure to asbestos. Symptoms typically develop several decades after initial exposure.
Prevalence: Peritoneal mesothelioma is a rare cancer, with an estimated prevalence of about 1 per million population annually. It accounts for about 10-20% of all mesothelioma cases.
Epidemiology: Peritoneal mesothelioma is a rare cancer primarily caused by asbestos exposure. The disease develops in the peritoneum, the lining of the abdominal cavity. Epidemiologically:

1. **Incidence**: It is less common than pleural mesothelioma, representing about 20-25% of all mesothelioma cases.
2. **Geographic Variation**: Higher incidence rates are observed in regions with extensive asbestos use, such as certain industrial areas in North America, Europe, and Australia.
3. **Gender**: More common in men, largely due to occupational asbestos exposure, though the gender disparity is less pronounced than in pleural mesothelioma cases.
4. **Age**: Typically diagnosed in individuals aged 50-69 years, though it can occur in younger individuals with a history of significant asbestos exposure.
5. **Latency Period**: Long latency period of 20-50 years from initial asbestos exposure to diagnosis.

Exposure to asbestos fibers is the primary risk factor, with occupational exposure being the most significant source. Other risk factors include genetic predisposition and previous radiation treatment.
Intractability: Peritoneal mesothelioma is often considered a challenging and complex disease to treat, mainly due to its rarity and aggressive nature. However, it is not entirely intractable. Various treatment options, including surgery, chemotherapy, and newer approaches like hyperthermic intraperitoneal chemotherapy (HIPEC), can improve survival rates and quality of life for some patients. The effectiveness of treatments can vary depending on factors such as the stage of the disease, the patient's overall health, and how early the disease is diagnosed.
Disease Severity: Peritoneal mesothelioma is typically a severe and aggressive form of cancer that affects the lining of the abdomen (peritoneum). The severity often depends on the stage at diagnosis and the individual's overall health. Prognosis is generally poor due to the aggressive nature of the disease, although early detection and advanced treatments may improve outcomes.
Healthcare Professionals: Disease Ontology ID - DOID:1788
Pathophysiology: Peritoneal mesothelioma is a rare cancer affecting the peritoneum, the lining of the abdominal cavity. The pathophysiology involves malignant transformation of the mesothelial cells, primarily due to asbestos exposure. When inhaled or ingested, asbestos fibers can become lodged in the mesothelium, leading to chronic inflammation, genetic mutations, and cellular damage over time. This process promotes uncontrolled cell proliferation and tumor formation within the peritoneum, causing symptoms like abdominal pain, swelling, and fluid accumulation.
Carrier Status: Peritoneal mesothelioma is not associated with a carrier status. It is primarily linked to asbestos exposure rather than inherited genetic factors.
Mechanism: Peritoneal mesothelioma is a rare and aggressive cancer that originates in the peritoneum, the lining of the abdominal cavity. The primary cause is typically exposure to asbestos fibers, which, when inhaled or ingested, can migrate to the peritoneal cavity.

**Mechanism:**
1. **Asbestos Exposure:** Asbestos fibers are inhaled or ingested and can travel to the peritoneal cavity.
2. **Inflammation and Damage:** The fibers induce chronic inflammation and cellular damage in the peritoneum.
3. **Cellular and Genetic Changes:** Persistent inflammation leads to DNA damage in mesothelial cells, promoting oncogenesis.
4. **Tumor Growth:** Mutated mesothelial cells proliferate uncontrollably, forming tumors in the peritoneal lining.

**Molecular Mechanisms:**
1. **DNA Damage and Repair Pathways:**
- Asbestos fibers cause oxidative stress and DNA damage.
- Key genes involved in DNA repair, such as BRCA1 and BRCA2, may be mutated or dysfunctional.
2. **Oncogene Activation and Tumor Suppressor Gene Inactivation:**
- Mutations in oncogenes (e.g., RAS, BRAF) promote cell proliferation.
- Inactivation of tumor suppressor genes (e.g., p53, NF2) leads to loss of cell cycle control.
3. **Epigenetic Changes:**
- Hypermethylation of tumor suppressor gene promoters.
- Histone modification altering gene expression.
4. **Chronic Inflammation:**
- Persistent inflammation activates pro-inflammatory cytokines and growth factors.
- NF-κB and other signaling pathways are perpetually activated, promoting cancer cell survival and proliferation.
5. **MicroRNA Dysregulation:**
- Altered expression of microRNAs affecting gene regulation, contributing to oncogenesis and tumor progression.

Understanding these mechanisms provides insights into the pathogenesis of peritoneal mesothelioma and potential therapeutic targets.
Treatment: Given its rarity, there are no established guidelines for the treatment of peritoneal mesothelioma. The modern approach to malignant peritoneal mesothelioma includes cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), intraperitoneal chemotherapy, and intravenous chemotherapy. These are often used in conjunction and in a complementary fashion, and this multifaceted approach has significantly improved outcomes when compared to intravenous chemotherapy alone. For instance, the reported median survival time for patients with stage IV mesothelioma as reported by the American Cancer Society is 12 months; however, with adequate cytoreduction, intraperitoneal, and intravenous chemotherapy combined, some authors report 10-year survival rates projected at nearly 75%.Multiple factors have been shown to be significant in predicting the outcome and overall survival. Age greater than 60 at surgery, more overall disease burden (defined as a PCI Archived 2016-09-02 at the Wayback Machine greater than 15), complete cytoreduction (no visible disease), and epithelioid subtype pathology have all been shown to be predictors of both mortality and disease progression. These known predictors notwithstanding, many patients with advanced peritoneal mesothelioma are still surgical candidates, and even patients with the highest possible score on the peritoneal carcinomatosis index (39) can be completely reduced to a PCI of 0 with adequate surgery.
Compassionate Use Treatment: Compassionate use treatments and off-label or experimental treatments for peritoneal mesothelioma include several approaches:

1. **Immunotherapy**:
- Drugs such as pembrolizumab (Keytruda) and nivolumab (Opdivo) have been used to target certain immune checkpoints, enabling the immune system to better fight the cancer.

2. **Gene Therapy**:
- Experimental treatments involving the alteration of genes within cancer cells are being explored. One example is the use of genetically modified viruses to attack and destroy cancer cells.

3. **Targeted Therapy**:
- Drugs that specifically target cancer cell molecules, such as bevacizumab (Avastin), may be used to inhibit blood vessel growth in tumors.

4. **Photodynamic Therapy (PDT)**:
- A treatment which involves using light-sensitive drugs that become active when exposed to light, and can destroy cancer cells.

5. **Hyperthermic Intraperitoneal Chemotherapy (HIPEC)**:
- This involves heating chemotherapy drugs and delivering them directly into the abdominal cavity, sometimes considered for compassionate use in advanced cases.

6. **CAR-T Cell Therapy**:
- Though primarily used in hematologic cancers, research is ongoing to adapt this immune cell-modifying approach for solid tumors like mesothelioma.

Patients considering these treatments should consult with their healthcare provider to understand the potential risks and benefits.
Lifestyle Recommendations: Lifestyle recommendations for individuals with peritoneal mesothelioma include:

1. **Healthy Diet**: Eat a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to maintain overall health and support the body during treatment.
2. **Regular Exercise**: Engage in regular physical activity, as tolerated, to maintain strength and improve quality of life.
3. **Avoid Alcohol and Tobacco**: Eliminate smoking and limit alcohol consumption to reduce stress on the body.
4. **Stress Management**: Practice stress-reducing techniques such as meditation, yoga, or deep-breathing exercises.
5. **Follow-up Care**: Keep regular appointments with healthcare providers for monitoring and managing the condition.
6. **Hydration**: Stay well-hydrated to support overall bodily functions.
7. **Support Networks**: Engage with support groups or counseling to help cope with the emotional aspects of the disease.
Medication: Peritoneal mesothelioma is a rare cancer that occurs in the lining of the abdomen, known as the peritoneum. Treatment often involves a combination of surgery, chemotherapy, and occasionally radiation therapy. One of the chemotherapy regimens commonly used is a combination of pemetrexed and cisplatin. Other investigational medications and treatments are being studied, but these are the mainstays of current medical treatment.

There isn't an established role for nanotechnology (nan) in the standard treatment protocols for peritoneal mesothelioma at this time, though research is ongoing in this area to explore potential benefits.
Repurposable Drugs: For peritoneal mesothelioma, some repurposable drugs under investigation include:

1. **Pemetrexed**: Originally developed for lung cancer.
2. **Bevacizumab**: Typically used in various cancers as an angiogenesis inhibitor.
3. **Nivolumab and Pembrolizumab**: Immune checkpoint inhibitors used in melanoma and lung cancer.

Repurposing these drugs aims to enhance treatment efficacy and potentially improve patient outcomes in peritoneal mesothelioma.
Metabolites: There are no specific metabolites that are unique to peritoneal mesothelioma identified as standard biomarkers for this disease. Diagnosis and monitoring typically rely on imaging studies, histopathological analysis, and markers such as mesothelin-related proteins rather than specific metabolites.
Nutraceuticals: For peritoneal mesothelioma, there is currently no strong evidence that nutraceuticals can provide significant therapeutic benefits. Nutraceuticals, which are food-derived products with potential health benefits, should not replace conventional treatments such as surgery, chemotherapy, or radiation therapy. Always consult a healthcare professional before considering any nutraceuticals as part of a treatment plan for peritoneal mesothelioma.
Peptides: Peritoneal mesothelioma is a rare and aggressive cancer that occurs in the peritoneum, the lining of the abdominal cavity.

1. **Peptides**: Research in the treatment of peritoneal mesothelioma has explored the use of peptides, including peptide-based vaccines and therapies. These peptides can stimulate the immune system to target mesothelioma cells, offering a potential therapeutic strategy.

2. **Nanotechnology (nan)**: Nanotechnology is being investigated for its potential to enhance the diagnosis and treatment of peritoneal mesothelioma. Nanoparticles can be designed to deliver drugs directly to cancer cells, improve imaging techniques for better detection, and potentially overcome drug resistance.

Further research is ongoing to optimize these approaches and improve outcomes for patients with peritoneal mesothelioma.