Papillary Mesothelioma

Papillary mesothelioma is a very rare type of tumor. It’s also known as well-differentiated papillary mesothelioma. Most cases are typically benign (noncancerous), but doctors need to make sure they distinguish it from malignant (cancerous) mesothelioma. Learn more about this disease and how Mesothelioma Hope can help you find the treatments you need.

Fact-Checked and Updated by: Jenna Tozzi, RN

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What Is Papillary Mesothelioma?

Well-differentiated papillary mesothelioma (WDPM) is an uncommon subtype of epithelioid mesothelioma, making up just 0.3-5% of all mesothelioma cases.

It usually forms in the peritoneum (abdominal lining) or tunica vaginalis (testicular lining), but may also develop in the pleura (lung lining) or pericardium (heart lining).

Most cases of papillary mesothelioma aren’t actually cancerous, meaning patients can potentially live for decades.

That said, some patients with benign papillary mesothelioma may be misdiagnosed or develop cancer later on, which may impact their overall health outlook. If you think you have any type of mesothelioma, see a doctor immediately.

Key Facts on Papillary Mesothelioma

  • Also known as well-differentiated papillary mesothelial tumors (WDPMT)
  • There have been roughly 200 reported cases total since 1953.
  • Cells are typically round and form finger-like nodules known as papillae.
  • Many patients live 15+ years since their neoplasms (tumors) are benign.
  • Can be treated with surgery and chemotherapy.

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Can Papillary Mesothelioma Turn Malignant?

Yes. Many cases of papillary mesothelioma remain benign, but some patients may later develop malignant mesothelioma.

Researchers are still studying exactly why some cases of papillary mesothelioma have a malignant potential.

A 2022 report published in Modern Pathology proposed that papillary mesothelioma could be mistaken for mesothelioma in situ (where the cancer has only just started to form). This could explain why “benign” cases of papillary mesothelioma end up becoming cancer.

Who Is at Risk of Well-Differentiated Papillary Mesothelioma?

Papillary mesothelioma mainly develops in premenopausal women, while most malignant mesothelioma patients are men over the age of 70.

That said, another Modern Pathology report notes that men and women of any age can develop well-differentiated papillary mesothelioma.

Papillary Mesothelioma and Asbestos

The only known cause of malignant mesothelioma is exposure to a cancer-causing substance called asbestos. At this point, there haven’t been many papillary mesothelioma cases linked to asbestos.

“Asbestos exposure has been documented in some cases of WDPMT, but there are insufficient data to implicate causation owing to the rarity of this tumor.”
–World Health Organization (WHO)

Well-differentiated papillary mesothelioma tumors are quite rare, and doctors still don’t fully understand how they develop.

Most other epithelial mesothelioma subtypes, as well as cases of biphasic and sarcomatoid mesothelioma, are cancerous and directly connected to asbestos exposure.

Symptoms of Papillary Mesothelioma

While many patients don’t have symptoms, papillary mesothelioma can sometimes cause pain, fluid buildup, and a cough.

Which papillary mesothelioma symptoms (if any) develop depends on what part of the body the tumor has developed.

Here are possible symptoms by type:

  • Pleural mesothelioma: Patients may develop symptoms related to lung function and breathing, including shortness of breath, a dry cough, or pleural effusion (fluid buildup in the lung lining).
  • Peritoneal mesothelioma: Some patients with this type of papillary mesothelioma experience abdominal pain, weight loss, and peritoneal effusions (fluid buildup in the abdominal lining).
  • Testicular mesothelioma: Swelling and pain in the testicles may develop as well as a hydrocele (fluid buildup in the testicular lining).

Due to the lack of symptoms, up to 90% of papillary peritoneal mesothelioma cases are diagnosed during surgeries for unrelated health issues, according to Pathology Outlines.

Call (866) 608-8933 to speak with our Patient Advocates about possible symptoms of mesothelioma and learn about treatments that could help you.

Getting a Papillary Mesothelioma Diagnosis

Doctors can take several steps to confirm a papillary mesothelioma diagnosis.

These steps include:

  • Physical examination: Doctors can evaluate a patient’s symptoms and any history of asbestos exposure to help rule out other health issues.
  • Imaging scans: If a patient has symptoms of papillary mesothelioma, a computed tomography (CT) scan can help show possible signs of tumor growth (such as ascites).
  • Biopsies: A tissue or fluid biopsy allows pathologists to remove a sample of a tumor and see if mesothelial cells are present. It also helps determine the histological (cell) type by looking at mutations within the cells.
  • Staining: Immunohistochemical staining is key to help determine if a patient has malignant mesothelioma or benign papillary mesothelioma. An immunohistochemistry stain looks at genes in the tumor cells, such as BAP1, and doctors can use this information to make the right diagnosis.

After a diagnosis of well-differentiated papillary mesothelioma has been confirmed, doctors can recommend specific treatments.

Misdiagnosis of Well-Differentiated Papillary Mesothelioma

Well-differentiated papillary mesothelioma could be misdiagnosed because it is so rare.

One of the most notable misdiagnoses is mesothelioma in situ, where cancer has only just started to form. A 2022 Modern Pathology report notes that both conditions can look similar under a microscope.

Other differential diagnoses include:

  • Diffuse malignant mesothelioma: This type of cancer may have areas that could resemble papillary mesothelial tumors.
  • Other cancers: Papillary mesothelioma could be mistaken for serous ovarian tumors, carcinomas, or other types of cancer.
  • Papillary endosalpingiosis: Growths near the fallopian tube may look like tumors or lesions.
  • Reactive mesothelial hyperplasia: This occurs when a tissue or organ makes too many cells.

Misdiagnosis can lead to patients getting the wrong treatments, which could have a significantly negative affect on their health.

If you believe you’ve been misdiagnosed, you should get a second opinion. This allows another specialist to review your imaging scans and test results and either confirm your diagnosis or determine the correct one.

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Papillary Mesothelioma Prognosis

Many patients with well-differentiated papillary mesothelioma have a very favorable prognosis, or expected health outlook. Pathology Outlines reports that deaths from papillary mesothelioma are rare given that most cases are benign.

Papillary Mesothelioma Life Expectancy

The life expectancy of papillary mesothelioma patients is very favorable, with most patients living for many years after their diagnosis.

Here are notable life expectancy figures by type:

  • Pleural papillary mesothelioma patients have an average survival time of 74 months.
  • Peritoneal papillary mesothelioma patients lived 144 months.

The average life expectancy for malignant mesothelioma is 12-21 months.

That said, a case report from Modern Pathology notes that papillary mesothelioma can turn fatal if it becomes malignant. Doctors should regularly monitor patients to help catch a case of malignant papillary mesothelioma early on.

Papillary Mesothelioma Survival Rates

Survival rate measures the percentage of patients still alive after being diagnosed with an illness. The survival rates for well-differentiated papillary mesothelioma are encouraging, with most patients living 5 years or more.

Here are key points to know about survival rates:

  • The 5-year survival rate was 80% in a study of 45 peritoneal papillary mesothelioma patients treated with cytoreductive surgery and HIPEC.
  • Survival rates specific to malignant pleural mesothelioma haven’t been recorded, but a 2023 report published in Thorax noted that most patients achieve long-term survival.

Getting treatment from a specialist is the best way to improve your survival time after a papillary mesothelioma diagnosis.

Call (866) 608-8933 to learn about steps you can take to potentially improve your papillary mesothelioma prognosis.

Treatment for Papillary Mesothelioma

Doctors can treat papillary mesothelioma using a couple of different options, the most notable being surgery and chemotherapy. Learn about the mesothelioma treatments that could be used below.

Mesothelioma Surgery

Mesothelioma surgery may be recommended as the main treatment for well-differentiated papillary mesothelioma.

Types of surgery used to treat papillary mesothelioma include:

  • Local surgeries: Since most cases of papillary mesothelioma are benign, simply removing the tumor is often the best treatment, according to a 2022 report published in Modern Pathology.
  • Cytoreduction with HIPEC: Used for cases of peritoneal mesothelioma, this combines surgery with heated chemotherapy to remove and destroy tumors and cancer cells in the abdominal lining.
  • Extrapleural pneumonectomy (EPP): Treats papillary pleural mesothelioma by removing the tumors, the lung closest to them, and the lung lining.
  • Orchiectomy: Used to treat mesothelioma of the tunica vaginalis by removing tumors in the testicular lining and the testicle closest to them.
  • Pleurectomy with decortication (P/D): Also used to treat pleural mesothelioma, this surgery takes out the lung lining and cancer tumors. As opposed to an EPP, however, neither lung is removed.

A mesothelioma doctor can determine which surgical procedure will be best for your case.

Chemotherapy Drugs

Chemotherapy can help destroy well-differentiated papillary mesothelioma tumors. Chemotherapy drugs work by circulating through the body to kill harmful cells.

A 2023 study in the International Journal of Innovative Surgery noted that some papillary mesothelioma patients who received chemotherapy have lived up to 8 years without any evidence of the disease.

Multimodal Treatment

Doctors may use a combination of treatments as part of a multimodal therapy plan to treat cases of papillary mesothelioma that have started to spread.

In most cases, however, surgery is the best papillary mesothelioma treatment. Multimodal treatments are typically better for cases of malignant mesothelioma, where there’s a higher risk of the cancer spreading.

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Well-Differentiated Papillary Mesothelioma Survivors

Several papillary mesothelioma have gone on to become long-term survivors, living for many years thanks to medical care.

Here are some papillary mesothelioma survivors:

  • A woman lived 24 years with well-differentiated papillary mesothelioma, making her the longest-living survivor recorded. Her treatment plan included multiple surgeries and a heart transplant.
  • A U.S. Navy veteran diagnosed with malignant papillary mesothelioma in 2009 is still alive today, making him a 15-year survivor. His cancer is no longer spreading thanks to treatment.
  • A man with testicular papillary mesothelioma was still alive 6 years after treatment, as noted in a 2022 Modern Pathology report.

You may be able to survive well-differentiated papillary mesothelioma too with the right treatment plan and regular follow-up care.

Get Help for a Papillary Mesothelioma Diagnosis

If you or a loved one has papillary mesothelioma, it’s important to seek expert medical advice and support. With an accurate diagnosis and the right treatments, you could potentially live a full life.

At Mesothelioma Hope, we’re here to listen to your story and help you find the best course of action for your specific needs.

Our experienced team can:

  • Answer questions related to your diagnosis
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Call (866) 608-8933 or use our Free Doctor Match to take the first step toward finding hope.

Well-Differentiated Papillary Mesothelioma FAQs

What is the survival rate for malignant papillary mesothelioma?

Papillary peritoneal mesothelioma has a 5-year survival rate of 80%, as noted in a European Journal of Surgical Oncology report.

While specific data isn’t available, other types of papillary mesothelioma also have good long-term survival rates since most cases are benign (not cancerous) and can be treated with surgery.

What is the life expectancy of a papillary peritoneal mesothelioma patient?

Papillary peritoneal mesothelioma patients can live a long time with treatment. An Annals of Surgical Oncology report found that patients live 144 months (12 years) on average after a diagnosis.

Get our Free Mesothelioma Guide to find treatments that could help you live longer with well-differentiated papillary mesothelioma of the peritoneum.

Is mesothelioma always cancerous?

Most cases of mesothelioma are cancerous, but well-differentiated papillary mesothelioma is not. Papillary mesothelioma tumors are only malignant in rare cases.

Your doctors can determine if your tumors are benign or cancerous by ordering diagnostic tests like a biopsy.

Where does papillary mesothelioma form?

Well-differentiated papillary mesothelioma typically develops in the abdominal lining in women and in the testicular lining in men, according to a 2020 report in Translational Lung Cancer Research.

It can also sometimes form in the lining of the lungs or heart.

Jenna TozziWritten by:

Chief Patient Care Advisor

Jenna Tozzi, RN, is the Chief Patient Care Advisor for Mesothelioma Hope. With more than 15 years of experience as an adult and pediatric oncology nurse navigator, Jenna provides exceptional guidance and support to cancer patients and their loved ones. Jenna has been featured in Oncology Nursing News and is a member of the Academy of Oncology Nurse & Patient Navigators & the American Nurses Association.

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References
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