Well-Differentiated Papillary Mesothelioma

What is Papillary Mesothelioma?



Well-differentiated papillary mesothelioma (WDPM) of the peritoneum is a rare subtype of peritoneal epithelioid mesothelioma which typically has low malignant potential. This form of mesothelioma, in contrast with the conventional diffuse malignant mesothelioma (DMM), is considered to be of low malignant potential, or not likely to spread or invade other parts of the body.

It most commonly occurs in young women lacking a history of asbestos exposure. Only 38 female patients with peritoneal WPDM have been reported in the literature, and no uniform treatment recommendation has been established.

Well-Differentiated Papillary Mesothelioma.

Department of Pathology, Duke University Medical Center, Durham, North Carolina 27710, USA. butno001@mc.duke.edu

Well-differentiated papillary mesothelioma is an unusual variant of epithelial mesothelioma considered to be of low malignant potential.

The majority of previously reported cases developed in the peritoneum of young women without a history of asbestos exposure. The authors report 14 cases of well-differentiated papillary mesothelioma, seven of which originated in the pleura, six in the peritoneum, and one in the tunica vaginalis. Eleven of the patients were male and three were female, with an average age at presentation of 58 years (range 32-82 years). Six of the patients had a quantifiable history of asbestos exposure. Of the nine cases with complete follow-up, six had clinically indolent disease, one showed resolution after adjuvant chemotherapy, one pursued an aggressive course, and one died of other causes. These findings indicate that well-differentiated papillary mesothelioma is a rare variant of mesothelioma with a variable clinical prognosis that is etiologically related to asbestos exposure in some cases.

PMID: 11688466 [PubMed - indexed for MEDLINE

Macroscopic features: Multiple grey white nodules usually less than 20mm in diameter.

Microscopic features:

Well defined stout papillary structures with myxoid cores, lined by bland, flattened, single layer of cuboidal or columnar mesothelial cells. Subnuclear vacuolation may be noted. Mitotic figures are rarely present. In some cases there may be limited invasion of the submesothelial layer. Deep invasion is usually not seen.

Differential diagnosis:

1. Reactive mesothelial hyperplasia (assocciated with inflammation) ;
2. Serous neoplasia of peritoneum ( cellular stratification, atypia and mitotic figures) ;
3. Primary and seconday adenocarcinoma (mucin histochemistry and immunohistochemistry are useful ) ;
4. Diffuse mesothelioma with a prominent tubulopapillary epithelial component (diffuse growth pattern & cytological atypia).
   

Papillary Mesothelioma Treatments

Due to the rare occurrence of this disease, a recommendation of uniform treatment has yet to be established. The typical forms of treatment that have been documented in various studies are similar to traditional mesothelioma treatments and have included surgery and multiple forms of chemotherapy. Patients and their families often have numerous questions about the best way to combat mesothelioma and we offer a comprehensive packet with information about treatment options and top doctors, catered to each patient’s specific diagnosis. Please click here to receive this complimentary packet overnight.

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Peritoneal Mesothelioma Treatment

Your choice of treatment for peritoneal mesothelioma will depend on a number of factors. These include:

• The stage of your cancer
• Any other medical conditions you may have
• Your general fitness

Treatment of Peritoneal Cancer:

1. Surgery. Surgery is not possible for everyone with peritoneal mesothelioma. If surgery is possible, the operation is called a peritonectomy. This means removing the peritoneum (the lining of the abdomen) where the mesothelioma has started growing. In recent years some doctors have been developing a technique known as cytoreductive surgery for peritoneal mesothelioma. Cytoreductive surgery involves the surgeon carrying out up to 6 different peritonectomy procedures, to remove as much of the cancer as possible. At the same time, or shortly after, chemotherapy may be given straight into the peritoneal cavity.
2. Medical Management. In addition to the symptoms of abdominal pain, distension and ascites peritoneal mesothelioma can be associated with hypoalbuminemia, night sweats, inguinal and umbilical hernia, and hypercoagulability. A new drug combination such as cisplatin plus pemetrexed that have shown promise in pleural mesothelioma may also be effective in peritoneal mesothelioma. Immunotherapeutic agents such as interferon and various cytokines may have a role in treating this disease especially when the amount of disease is minimal.
   
3. Chemotherapy. Chemotherapy uses anti-cancer drugs, which are usually injected into a vein. For mesothelioma, chemotherapy may be given directly into the abdomen. Depending on the type of chemotherapy drugs used, this treatment can be given weekly or every two to three weeks. It can be given at the same time as cytoreductive surgery, or soon after. The doctor has to make a small cut in the wall of your abdomen. Then the doctor puts a tube called a catheter through the opening and into your tummy (abdomen). The chemotherapy is given into your abdominal cavity through the catheter. Some studies have suggested that this can work better if it is heated to a few degrees above body temperature first. This is called hyperthermic intraoperative intraperitoneal chemotherapy (HIIC).
4. Radiation Therapy. Radiation may be targeted directly at cancer cells or can be used for palliative reasons such as to reduce pain or shortness of breath or to control the spread of the tumor. This internal radiation therapy has a number of advantages, including the ability for doctors to treat a small area with a higher dose of radiation in a shorter amount of time. Brachytherapy may be either temporary or permanent.
5. Supportive care (Palliative care)
   Unfortunately peritoneal mesothelioma is often diagnosed when it is quite advanced. Some people may be too ill to cope with intensive chemotherapy. But you can still have treatment to try to relieve symptoms such as pain, weight loss and other symptoms such as fluid in the abdomen.
   With more advanced peritoneal mesothelioma, fluid may collect inside your abdomen. If too much fluid collects, it makes your abdomen swell. This can be uncomfortable and heavy.
   
   
   
   You can have the fluid drained off. This is called abdominal paracentesis. It is sometimes called an ascitic tap.
   
   Your care will be managed by a palliative care team. This is a team of doctors and nurses who are expert in controlling the symptoms of advanced cancer. The team may also include a physiotherapist and a dietician. By: Cancer Help
6. Clinical Trial.

• Promoting new drug.
• Perfused Heated Cisplatin. This study will test the effectiveness of an experimental treatment for peritoneal cancer involving surgical removal of the tumor, perfusion of the abdomen during surgery with a heated solution of the drug cisplatin, and post-surgery combination chemotherapy in the abdomen with fluorouracil (5-FU) and paclitaxel.

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Type of Mesothelioma

Types of Malignant Mesothelioma:

1. Pleural Mesothelioma. Pleural mesothelioma is a cancer of the lining of the lungs, called the "pleura". Lung cancer, however, is different from pleural mesothelioma because lung cancer is a malignant tumor that originates in the lungs, whereas pleural mesothelioma occurs in the lining of the lungs.
2. Pericardial Mesothelioma. Pericardial mesothelioma is a cancer in the lining that surrounds the heart, called the pericardium.
3. Peritoneal Mesothelioma. Peritoneal mesothelioma is a cancer in the lining that surrounds the stomach or abdomin, called the peritoneum.

There are three cell types of mesothelioma:

1. Epithelioid accounts for 50% to 70% of all cases. It also has the best prognosis.
   
2. Sarcomatoid accounts for 10% to 15% of all cases. It is a more aggressive form of mesothelioma.
3. Biphasic or Mixed accounts for 10% to 15% of cases. This form has characteristics of both epithelioid and sarcomatoid mesotheliomas but is associated with worse survival than epithelioid mesothelioma.

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