Papillary mesothelioma is an uncommon form subtype of epithelial mesothelioma. Also known as well-differentiated papillary mesothelioma (WDPM), it is marked by a slow growth pattern. It most often forms in the peritoneum in the abdomen and is usually benign with a good prognosis.
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What Is Papillary Mesothelioma?
Papillary mesothelioma, or WDPM, is a rare cell subtype of epithelial mesothelioma. All cases of mesothelioma are diagnosed with a cell type: epithelial or sarcomatoid (or biphasic, a mix of the two).
There are also many subtypes within these primary cell types. WDPM is an epithelial mesothelioma. Epithelial cells are cells that line tissues and organs. Compared to sarcomatoid or biphasic mesotheliomas, epithelial mesothelioma is less aggressive and has a better prognosis.
WDPM Characteristics and Facts
Because it is so rare, information about WDPM is limited. This is some of what the experts know about papillary mesothelioma from patients studied:
- Cells in papillary mesothelioma are round and form finger-like projections called papillae.
- Most cases of WDPM have been diagnosed in women.
- Most cases of WDPM are peritoneal mesothelioma, the type of mesothelioma that develops in the abdomen.
- Most people who develop WDPM are women between 30 to 40 years of age who’ve never been exposed to asbestos.
- The age of WDPM patients ranges from mid-20s to 70s.
- WDPM is almost always benign, or noncancerous.
- A few cases of pleural and testicular WDPM have been recorded.
- In very rare cases, WDPM tumors form psammoma bodies, areas of calcification.
- Recent studies have found that WDPM is genetically different from malignant mesothelioma.
Is Papillary Mesothelioma Always Benign?
WDPM is almost always benign, meaning the tumors are not cancerous. They may grow large and cause issues but do not spread to other tissues or organs.
Some cases of WDPM that were diagnosed as malignant might be misdiagnosed. It is difficult to diagnose rare mesotheliomas accurately.
WDPM can also become malignant over time. This is a rare occurrence but always possible, so diagnosis and treatment are important.
What Causes Well-Differentiated Papillary Mesothelioma?
Studies of groups of patients with WDPM find that most have no known history of asbestos exposure. In one study, six of 27 patients were exposed to asbestos. This included male patients, who have a higher chance of past asbestos exposure in the workplace than women.
Because there is no strong connection between WDPM and asbestos exposure, no one knows for sure what causes it.
WDPM Symptoms
Symptoms of this type of mesothelioma vary according to each person and where the tumors are. The majority of WDPM patients report there aren’t any symptoms. The few symptoms reported include:
- Pain in the abdomen or pleura due to fluid build-up
- Pelvic pain
Since symptoms are so rare, WDPM is typically found during surgery for other medical conditions unrelated to papillary mesothelioma.
Diagnosing WDPM
Since symptoms are so rare, WDPM is usually found during surgery or an exam for other conditions unrelated to papillary mesothelioma, such as a pelvic exam or an unrelated CT scan.
Although the tumors can be detected via a pelvic exam or CT scan, a biopsy is the only accurate way to diagnose papillary mesothelioma.
A biopsy entails a surgeon removing a piece of tissue from the area of the body where the tumor was detected. Once the tissue is removed, it’s sent to a lab, where a pathologist views the cells in the tissue under a specialized microscope.
Pathologists look for papillary (finger-like) structures when diagnosing papillar mesothelioma. They can also perform immunohistochemical tests to look for biomarkers. Because WDPM is so rare, no definitive markers are used for diagnosis.
WDPM is rare and difficult to diagnose. It is easy to misdiagnose it as other conditions, with similar characteristics and more common than WDPM:
- Adenoid tumors
- Tuberculosis peritonitis
- Other papillar carcinomas
- Peritoneal carcinomatosis
- Reactive mesothelioma hyperplasia
How Is WDPM Treated?
Doctors usually approach any type of mesothelioma with multimodal treatment. This means they use a combination of therapies that may include standard and emerging treatments.
If WDPM is benign, treatment is often straightforward, with surgery followed by chemotherapy to reduce the risk of a recurrence.
Cytoreductive Surgery
Cytoreductive surgery is a standard treatment for many patients with peritoneal mesothelioma. Surgeons remove as much of the tumor tissue as possible during this procedure.
Exploratory laparotomy is sometimes performed before a patient undergoes cytoreductive surgery.
Exploratory laparotomy allows physicians to determine the extent and location of the tumors and, if necessary, schedule a cytoreductive surgery to remove the tumors.
Some physicians feel that an exploratory laparotomy is too risky to perform on a disease that’s still considered benign.
Cytoreductive surgery typically takes 10 to 12 hours, but most patients are admitted a day before testing, X-rays, and preparation.
Surgery might be the only treatment for some patients, but most benefit from chemotherapy afterward.
Chemotherapy
Surgery alone usually isn’t enough to effectively battle cancer cells, even with a disease considered less invasive than other forms of mesothelioma. In some cases of WDPM, chemotherapy alone has been successful.
Carboplatin is the most common medication used for chemotherapy for those with WDMP and has proven to be effective in many instances. Some studies show that the disease never resurfaced after patients underwent chemotherapy with carboplatin.
Some patients receive a combination of surgery and chemotherapy, typically heated chemotherapy. This involves heating the medication to a little higher than the patient’s body temperature.
Known as the hot chemo approach (HIPEC), this chemotherapy works best for those with cancerous cells around the abdomen.
What Is the Prognosis for Papillary Mesothelioma?
The prognosis for papillary mesothelioma is generally positive. People with WDPM generally have a better prognosis than those with any other type of mesothelioma because it is usually benign.
According to the Journal of Medical Case Reports, several patients who underwent chemotherapy survived and have no trace of the cancer today.
In rare cases, some patients passed away within a few years after treatment. This is a striking difference between the survival rates of other types of mesothelioma, in which there is only a 5 to 50% chance that the patient will survive five years past diagnosis.
Since WDPM has a slow growth rate and typically doesn’t spread to other areas of the body, the outcome is favorable.
Even if cancer resurfaces, most people still have a strong chance of long-term survival, as papillary mesothelioma’s subtype is epithelial mesothelioma, which is the easiest to treat.
Talk to your medical team about the best treatment options and have all your questions about your diagnosis answered. A lawyer can also help if you believe you were exposed to asbestos in the past.
WDPM Legal Help
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Paul Danziger
Reviewer and EditorPaul Danziger grew up in Houston, Texas and earned a law degree from Northwestern University School of Law in Chicago. For over 25 years years he has focused on representing mesothelioma cancer victims and others hurt by asbestos exposure. Paul and his law firm have represented thousands of people diagnosed with mesothelioma, asbestosis, and lung cancer, recovering significant compensation for injured clients. Every client is extremely important to Paul and he will take every call from clients who want to speak with him. Paul and his law firm handle mesothelioma cases throughout the United States.
References
- Butnor, K.J., Sporn, T.A., Hammar, S.P., and Roggli, V.L. (2001, October). Well-Differentiated Papillary Mesothelioma. Am. J. Surg. Pathol. 25(10), 1304-9.
Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/11688466 - Burrig, K.F., Pfitzer, P., and Hort, W. (1990, September). Well-Differentiated Papillary Mesothelioma of the Peritoneum: A Borderline Mesothelioma. Virchows Archiv A. 417, 443-7.
Retrieved from: http://link.springer.com/article/10.1007%2FBF01606033 - MedlinePlus. (2022, February 28). Abdominal Exploration.
Retrieved from: http://www.nlm.nih.gov/medlineplus/ency/article/002928.htm - Clarke, J.M. and Helft, P. (2010, October 29). Long-Term Survival of a Woman With Well Differentiated Papillary Mesothelioma of the Peritoneum: A Case Report and Review of the Literature.
Retrieved from: http://www.jmedicalcasereports.com/content/4/1/346 - Malpica, A., Sant’Ambrogio, S., Deavers, M.T., and Silva, E.G. (2012, January). Well-Differentiated Papillary Mesothelioma of the Female Peritoneum: A Clinicopathologic Study of 26 Cases. Am. J. Surg. Pathol. 36(1), 117-27.
Retrieved from: https://pubmed.ncbi.nlm.nih.gov/22024662/ - Shrestha, R., Nabavi, N., Volik, S., Anderson, S., Haegert, A., McConeghy, B., Sar, F., Brahmbhatt, S., Bell, R., Le Bihan, S., Wang, Y., Collins, C., and Churg, A. (2020, June). Well-Differentiated Papillary Mesothelioma of the Peritoneum Is Genetically Distinct from Malignant Mesothelioma. Cancers (Basel). 12(16), 1568. doi: 10.3390/cancers12061568.
Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7352777/