Malignant mesothelioma is a rare form of cancer that affects the thin cell wall lining of the body's internal organs and structures. This lining is known as the mesothelium.
Pleural Mesothelioma (covering of the lungs)
Peritoneal Mesothelioma (covering of abdomen)
Pericardial Mesothelioma ( covering of heart)
It is of three varieties:
Asbestos exposure is a known risk factor for development of malignant mesothelioma. Asbestos is a natural, yet toxic, mineral that was frequently used across a wide variety of industries. Microscopic asbestos fibers enter the body via the lungs or by means of ingestion. Once inside the human body, the durable asbestos fibers are unable to be broken down or expelled, resulting in a harmful inflammation and scarring of the mesothelium. This scarring lays the groundwork for malignant mesothelioma.
No other genetic, dietary or geographic factors or variation have been reported.
Trouble breathing, pain under the rib cage, distension of abdomen due to ascitis, lumps in the abdomen, unexplained weight loss. The symptoms depend on the extent of involvement. Nowadays, a common mode of diagnosis is incidental finding on laparoscopy for infertility or other procedures.
Signs and Symptoms:
Imaging Scan: CT Scan, MRI, PET CT usually picks up the disease.
Biopsy: Cytology cannot diagnose a mesothelioma. A biopsy is essential and the goal standard for the diagnosis of mesothelioma. A staging laparoscopy is ideal since it helps get adequate biopsy sample and assess the extent of peritoneal disease.
Blood Tests: Tumor marker levels like Ca 125, Ca 19-9 and CEA are advisable but not diagnostic.
How is it diagnosed?
Factors that affects the prognosis:
The stage of the disease like the extent of peritoneal involvement, involvement of lymph nodes, extra-abdominal spread, etc.
Whether the tumor can be removed completely by surgery.
The patient's age and general health, including lung and heart health.
Aggressiveness of the disease as assessed by histopathology – epitheloid variant has better outcomes compared to the mixed or sarcomatoid varieties.
The prognosis and treatment options depend on the following:
Procedure to confirm the diagnosis
A laparoscopy is often necessary for a biopsy and evaluation of the extent of disease. Occasionally, a thoracotomy or laparotomy (open surgery) may be necessary for the purpose.
Staging of Malignant Peritoneal Mesothelioma
The standard TNM method does not apply for Staging of Malignant Peritoneal Mesothelioma . Advanced research in this field has set up a new staging criteria depending upon the PCI (Peritoneal Carcinomatosis Index). PCI staging helps in evaluating the prognosis of the disease. Another important factor is the involvement of the lymph nodes.
Treatment of Malignant Peritoneal Mesothelioma:
Malignant peritoneal mesothelioma (MPM) is an aggressive neoplasm that arises from the lining mesothelial cells of the peritoneum and spreads extensively within the confines of the abdominal cavity. Morbidity and mortality are almost entirely due to disease progression within the peritoneum and not distant metastatic spread
Surgery is possible for patients diagnosed with earlier-stage disease. In many cases, surgical treatment for mesothelioma will be coupled with chemotherapy regimens.Choosing the right mesothelioma doctor is an important first step in planning for treatment.
Cytoreductive surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC).
This is the treatment of choice in patients with a peritoneal mesothelioma. It is the only treatment modality that offers a possibility of long-term survival. Luckily, this surgery has high success rates and is also used to treat other abdominal malignancies. The key factor that determines the outcome is the complete surgical removal of all visible tumour.
The CRS is a “Maximal Effort Surgery” that involves systematic, laborious and thorough physical removal of all visible tumour tissue. Often, this surgery can go on for several hours. This, theoretically, reduces a stage IV disease to an RO (no residual tumor status) status with no macroscopic or visible disease. The microscopic disease that may be left behind after such a long surgery is then further eradicated by the Hyperthermic Intra-peritoneal Chemotherapy ( HIPEC ). Studies have shown that this combination treatment more than quadruples the survival compared to traditional treatment methods and is therefore considered as the treatment of choice for malignant mesothelioma of the peritoneum. It has also been shown that high volume centres have better outcomes in terms of survival and complication rates compared to other centres. Dr. Sanket Mehta currently has treated perhaps the largest number of peritoneal mesothelioma patients in the country.
Recent studies have shown that when CRS and HIPEC is performed upfront, the survival rates are the highest. This means that giving chemotherapy before surgery has in fact a detrimental effect on survival. Chemotherapy before surgery is generally reserved for patients in poor general condition or the extent of the disease is such that complete surgical removal may not be possible. In such patients, chemotherapy is given before surgery in the hope that the general condition of the patient will improve and the disease extent will become operable. All the larger series from experienced centers have consistently shown a survival approaching 5 years when treated with CRS and HIPEC.
Chemotherapy, also known as systemic therapy, uses oral or infusion-based medications to kill cancer cells throughout your body. Chemotherapy is used both before as mentioned before, as well as after surgery, and in patients who cannot undergo surgery. Though not curative, it has a proven survival benefit. It is also used in the palliative setting to reduce pain and improve quality of life.
The most commonly used chemotherapy drugs for mesothelioma are Cisplatin combined with Premetrexed ( Alimta ). Other combinations include Gemcitabine, carboplatin or oxaliplatin.
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