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Pleural Cancer

Disease Details

Family Health Simplified

Description
Pleural cancer, often referred to as pleural mesothelioma, is a rare and aggressive cancer that affects the lining of the lungs (pleura), typically caused by asbestos exposure.
Type
Pleural cancer, more commonly known as pleural mesothelioma, is a type of cancer that affects the pleura, which is the thin tissue lining the chest cavity and surrounding the lungs. It is primarily associated with inhalation of asbestos fibers rather than a genetic transmission. While most cases are linked to environmental exposure, a small percentage might have a genetic predisposition, but it is not typically inherited in a clear Mendelian pattern.
Signs And Symptoms
Pleural cancer, which often refers to malignant pleural mesothelioma, has the following signs and symptoms:

- Persistent chest pain
- Difficulty breathing or shortness of breath (dyspnea)
- Chronic cough
- Unexplained weight loss
- Fatigue
- Pleural effusion (accumulation of fluid in the pleural cavity)
- Difficulty swallowing (dysphagia)
- Fever or night sweats

If you experience these symptoms, it is important to consult a healthcare professional for a thorough evaluation.
Prognosis
Pleural cancer prognosis varies depending on several factors including the stage at diagnosis, patient's overall health, and response to treatment. Pleural mesothelioma, a common type of pleural cancer, typically has a poor prognosis, with many patients surviving less than a year after diagnosis. However, early detection and advances in treatment can improve outcomes for some patients.
Onset
Pleural cancer, often referred to as malignant pleural mesothelioma, typically arises after several decades following exposure to asbestos. This long latency period frequently spans 20 to 50 years after initial exposure.
Prevalence
Pleural cancer, commonly referred to as pleural mesothelioma, is a rare form of cancer. The prevalence of pleural mesothelioma is not high, with an incidence rate of approximately 1 to 2 cases per million people per year in most industrialized countries. The majority of cases are linked to asbestos exposure, which affects the lining of the lungs (pleura).
Epidemiology
Pleural cancer, also known as malignant pleural mesothelioma, is a rare and aggressive cancer that occurs in the pleura, the thin layer of tissue surrounding the lungs. Epidemiologically, most cases of pleural cancer are linked to asbestos exposure. The latency period between exposure and development of the disease can range from 20 to 50 years. It primarily affects older adults, particularly men, due to historical occupational exposure. The incidence rates vary geographically, being higher in regions with extensive use of asbestos. Despite advancements in treatment, the prognosis remains poor, with a median survival time of about 12 months post-diagnosis.
Intractability
Pleural cancer, more commonly referred to as malignant pleural mesothelioma, is considered difficult to treat and often intractable due to its aggressive nature and late diagnosis. Treatment options, including surgery, chemotherapy, and radiation therapy, can help manage the disease and improve quality of life, but they are generally not curative. The prognosis is typically poor, emphasizing the need for early detection and novel therapeutic approaches.
Disease Severity
Pleural cancer, often referred to as malignant pleural mesothelioma, is a severe and aggressive type of cancer that affects the pleura, the thin tissue layer surrounding the lungs. The severity is high, with most patients experiencing significant symptoms and a challenging prognosis. The disease can rapidly progress and is often diagnosed at an advanced stage, complicating treatment and reducing life expectancy.
Healthcare Professionals
Disease Ontology ID - DOID:5158
Pathophysiology
Pleural cancer, particularly malignant pleural mesothelioma, originates in the pleura, which is the thin tissue lining the lungs and chest cavity. It typically results from asbestos exposure. The inhaled asbestos fibers lodge in the pleura, causing inflammation and scarring over many years. This damage can lead to cellular changes and the transformation of normal cells into cancerous ones. This malignancy disrupts the normal function of the pleura, leading to a buildup of fluid (pleural effusion), pain, and impaired respiratory function.
Carrier Status
Pleural cancer is not typically associated with a genetic carrier status like some inherited conditions. It is primarily linked to environmental factors, particularly exposure to asbestos.
Mechanism
Pleural cancer, often presenting as malignant pleural mesothelioma, primarily stems from the inhalation of asbestos fibers. These fibers can lodge in the pleura, leading to chronic inflammation and genetic damage over time.

### Mechanism:
1. **Asbestos Exposure**: Inhaled asbestos fibers penetrate the lung tissue and reach the pleura.
2. **Chronic Inflammation**: Asbestos fibers cause persistent inflammation and cellular injury in the pleura.
3. **Genetic Damage**: Prolonged exposure leads to genetic mutations and chromosomal abnormalities in mesothelial cells.
4. **Uncontrolled Cell Proliferation**: Genetic damage disrupts normal cell regulatory mechanisms, resulting in uncontrolled cell growth and tumor formation.

### Molecular Mechanisms:
1. **DNA Damage and Repair Pathways**: Asbestos fibers cause oxidative stress, leading to DNA damage. The body's repair mechanisms, such as nucleotide excision repair, may be overwhelmed or become faulty.
2. **Oncogene Activation and Tumor Suppressor Inactivation**:
- **p53**: Mutations in the TP53 gene impair its role in cell cycle control and apoptosis, allowing abnormal cells to proliferate.
- **NF2**: Mutations in the NF2 gene, which encodes the merlin protein, cause loss of tumor suppressor function.
3. **Inflammatory Pathways**: Chronic inflammation induces the release of cytokines and growth factors that promote tumorigenesis. For example, TNF-α and IL-6 are key players.
4. **Cell Signaling Pathways**:
- **PI3K/AKT/mTOR Pathway**: Aberrant activation promotes cell survival and growth.
- **Wnt/β-catenin Pathway**: Dysregulation leads to increased cell proliferation and tumor development.
5. **Epigenetic Changes**: Hypermethylation of tumor suppressor genes and histone modifications can further drive malignant transformation.

Understanding these mechanisms is crucial for developing targeted therapies for pleural cancer.
Treatment
Pleural cancer, often linked with malignant pleural mesothelioma, requires a multidisciplinary approach for treatment:

1. **Surgery:** Depending on the stage, options like pleurectomy/decortication (removal of part of the lining of the lungs and chest) or extrapleural pneumonectomy (removal of a lung and surrounding tissue) may be considered.
2. **Chemotherapy:** Commonly used drugs include pemetrexed combined with cisplatin or carboplatin.
3. **Radiation Therapy:** Targeted to kill cancer cells and shrink tumors.
4. **Immunotherapy:** Drugs like pembrolizumab may be used to boost the body's immune response against cancer cells.
5. **Palliative Care:** To manage symptoms and improve quality of life.

Treatment plans are highly individualized based on the patient's overall health, the stage of cancer, and specific characteristics of the tumor.
Compassionate Use Treatment
Compassionate use treatment refers to providing patients with access to investigational drugs or therapies outside of clinical trials when no other treatment options are available. For pleural cancer, which often refers to malignant pleural mesothelioma, compassionate use treatments might involve access to experimental drugs or novel therapies still under investigation. Off-label or experimental treatments that may be considered include:

1. **Immunotherapy**: Drugs like pembrolizumab (Keytruda) or nivolumab (Opdivo), which are approved for other cancers but may be used off-label for pleural cancer.

2. **Targeted Therapy**: Drugs that target specific genetic mutations or markers, such as bevacizumab (Avastin), which may be used off-label.

3. **Gene Therapy**: Experimental approaches aiming to modify or replace defective genes involved in cancer progression.

4. **Photodynamic Therapy (PDT)**: A treatment combining a photosensitizing agent with a specific type of light to kill cancer cells, currently under investigation.

5. **CAR-T Cell Therapy**: An experimental immunotherapy approach that alters a patient's T cells to target cancer cells more effectively.

6. **Clinical Trials**: Participation in clinical trials investigating new drugs, treatment combinations, or approaches, offering access to cutting-edge therapies.

It is important to consult with healthcare providers or specialists in pleural cancer to explore these options and determine their suitability for individual cases.
Lifestyle Recommendations
Pleural cancer, also known as pleural mesothelioma, is a type of cancer affecting the pleura, the thin tissue surrounding the lungs. Here are some lifestyle recommendations for individuals dealing with pleural cancer:

1. **Quit Smoking:** If you smoke, quitting can improve overall lung health and may enhance the effectiveness of treatment.

2. **Nutritious Diet:** Consume a balanced diet rich in fruits, vegetables, lean proteins, and whole grains to support overall health and recovery.

3. **Regular Exercise:** Engage in physical activities as advised by your healthcare provider to maintain strength and improve overall well-being.

4. **Avoid Asbestos Exposure:** Minimize exposure to asbestos and other harmful substances that can aggravate the condition.

5. **Manage Stress:** Utilize stress-reduction techniques such as yoga, meditation, or counseling to manage emotional well-being.

6. **Regular Medical Follow-ups:** Maintain regular check-ups with your healthcare team to monitor the condition and manage symptoms effectively.

7. **Hydration:** Drink plenty of water to stay hydrated, which can help in managing symptoms and side effects of treatment.

8. **Rest and Sleep:** Ensure adequate rest and quality sleep to help your body heal and cope with treatment.

These recommendations should be personalized based on individual circumstances and in consultation with healthcare professionals.
Medication
Pleural cancer, often referred to as malignant pleural mesothelioma, can be treated with a variety of medications depending on the stage and specifics of the case. Common chemotherapy drugs used include pemetrexed and cisplatin, often used in combination. Other drugs that may be used are carboplatin, gemcitabine, and vinorelbine. Immunotherapy medications such as nivolumab and pembrolizumab have also shown effectiveness in treating pleural cancer. It is important to consult with a healthcare provider to determine the most appropriate treatment plan.
Repurposable Drugs
Information on repurposable drugs specifically for pleural cancer, also known as malignant pleural mesothelioma, is limited but growing. Some drugs originally approved for other cancers or conditions have shown potential in treating pleural cancer. Examples include:

1. **Pemetrexed and Cisplatin**: Originally used for other forms of cancer, this combination is a standard treatment for pleural mesothelioma.
2. **Bevacizumab (Avastin)**: Used in combination with chemotherapy, it is approved for other cancers but has shown promise in clinical trials for pleural mesothelioma.
3. **Nivolumab (Opdivo) and Pembrolizumab (Keytruda)**: Immune checkpoint inhibitors initially approved for melanoma and other cancers have shown efficacy in treating pleural mesothelioma in clinical trials.

Always consult oncologists or healthcare professionals for the most current and personalized treatment options.
Metabolites
Pleural cancer, often referred to as malignant pleural mesothelioma, does not have well-defined or specific metabolites exclusive to the disease. However, research is ongoing to identify potential biomarkers that could be used for diagnosis or monitoring. Such biomarkers may include certain proteins or metabolites that are found at different levels in patients with pleural mesothelioma compared to healthy individuals. Currently, the diagnosis primarily relies on imaging studies and biopsy rather than on specific metabolites.
Nutraceuticals
Regarding pleural cancer, there is currently no robust evidence to support the use of nutraceuticals as a primary treatment. Nutraceuticals, which are products derived from food sources with additional health benefits beyond basic nutritional value, may support overall health and wellness but should not be relied upon as a substitute for conventional cancer therapies.

As for nanotechnology (nan), it presents promising avenues in the diagnosis and treatment of pleural cancer. Nanoparticles can be engineered to deliver chemotherapy drugs directly to cancer cells, potentially improving efficacy and reducing side effects. Additionally, nanotechnology may aid in better imaging and early detection of pleural malignancies. Research is ongoing, and while these advancements hold potential, they are not yet mainstream clinical treatments. Always consult healthcare providers for personalized medical advice.
Peptides
Peptides and nanotechnology are areas of active research in the treatment of pleural cancer, which usually refers to malignant pleural mesothelioma.

1. **Peptides**: Therapeutic peptides can potentially inhibit cancer cell growth, induce apoptosis, or modulate immune responses. Some investigational approaches involve using peptides to target specific cancer cell markers or to deliver cytotoxic agents directly to the tumor.

2. **Nanotechnology**: Nanoparticles can improve the delivery of chemotherapy drugs, gene therapy agents, or imaging agents directly to tumor sites with minimal systemic toxicity. They can also be designed to enhance the efficacy of the encapsulated therapeutic agents and improve their stability.

Both peptides and nanotechnology hold promise for improving diagnosis and treatment outcomes in pleural cancer but require further clinical validation.