Molecular Characterization of Pericardial Mesothelioma With Constrictive and Recurrent Pericarditis: TAZ Amplification and Hippo Pathway

Our plain-English summary of Molecular Characterization of Pericardial Mesothelioma With Constrictive and Recurrent Pericarditis: TAZ Amplification and Hippo Pathway, by R. Tudino, S. Smith, L. Cheng, et al (JACC. Case Reports, 2025).

The study

• Authors: R. Tudino, S. Smith, L. Cheng, K. French, C. Azzoli
• Journal: JACC. Case Reports
• Published: 2025;30(38):105759
• Type of article: Case report
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Malignant pericardial mesothelioma (MPM) is a rare, aggressive neoplasm with nonspecific clinical presentations. Although BAP1 loss and CDKN2A deletion are common in pleural mesothelioma, they are not universally present in MPM, complicating diagnosis.

Case Summary: A 73-year-old man presented with recurrent fever and chest pain from pericarditis. Positron emission tomography-computed tomography and transthoracic echocardiography revealed pericardial thickening and aortitis, concerning for inflammatory or infectious etiology. Initial histopathology was inconclusive. Diagnosis of MPM was confirmed via lymph node biopsy and molecular profiling, which revealed TAZ amplification. The patient was treated with IL-1 blockage and chemotherapy, with symptom improvement.

Discussion: In BAP1/CDKN2A-negative mesothelial proliferations, molecular testing for additional mutations, including TAZ amplification, may guide diagnostic certainty and novel therapeutic treatment.

Take-home Messages: MPM can closely mimic chronic inflammatory disease, and accurate diagnosis with comprehensive profiling is essential to guide targeted therapy. TAZ amplification suggests Hippo pathway as a driver of BAP-intact tumors and may serve as a predictive biomarker of mesothelial immunogenicity.

Published by Elsevier Inc.

In plain English

This article was a case report of one 73-year-old man with malignant pericardial mesothelioma, a cancer of the lining around the heart. He had 9 months of recurring fevers and chest pain from pericarditis that had not responded to nonsteroidal anti-inflammatory drugs and colchicine. Imaging suggested inflammation of the pericardium and aorta, and a positive tuberculosis blood test raised the possibility of tuberculosis. A pericardial biopsy showed atypical mesothelial cells. Expert review instead favored a benign reactive process, because the BAP1 marker was intact and a CDKN2A deletion was found in only 12% of cells, below the 20% threshold. He was treated for tuberculosis and vasculitis but remained symptomatic. A later lymph node biopsy confirmed metastatic mesothelioma, and genetic testing found amplification of the TAZ and DKC1 genes. After the IL-1 blocker anakinra, his symptoms resolved, and he then began palliative chemotherapy with carboplatin and pemetrexed. The article described his work in a chemical plant and later in plumbing and piping, but it did not mention asbestos exposure. The authors concluded that molecular profiling was important in rare mesothelioma subtypes.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report
• Who was studied: One 73-year-old man, born in Italy, with 9 months of recurrent fevers and chest pain, diagnosed with malignant pericardial mesothelioma
• Exposure: The article does not mention asbestos exposure for this patient. It reports work in a chemical plant with exposure to acetone and toluene, followed by work with plumbing and piping.
• Main results:
  • On the first pericardial biopsy, expert pathology review favored a benign reactive process because BAP1 staining was intact and there was no CDKN2A deletion by FISH
  • In this patient, CDKN2A deletion was present in 12% of cells, below the 20% threshold for a positive result
  • The patient received immunosuppressants and tuberculosis treatment but remained symptomatic
  • A lymph node biopsy confirmed metastatic mesothelioma
  • Genetic testing of the patient's tumor found amplification of the TAZ and DKC1 genes
  • After anakinra (an IL-1 receptor antagonist), the patient's symptoms resolved and inflammatory markers normalized; 2 weeks later, PET-CT showed reduced FDG uptake
  • The patient then started palliative chemotherapy; the article does not report the result of chemotherapy
• The authors' conclusion: The authors concluded that the case highlighted diagnostic challenges and pointed to TAZ amplification and Hippo pathway involvement, and that molecular profiling is important in rare mesothelioma subtypes to inform diagnosis and treatment.

Topics

Medical terms in this study, explained in our Lexicon: Pericardial Mesothelioma, Neoplasm, BAP1, CDKN2A, Pleural Mesothelioma, Pericardial, Etiology, Histopathology.

Related studies

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• Malignant primary pericardial mesothelioma presenting as effusive constrictive pericarditis: a case report study — A. Savarrakhsh, A. Vakilpour, et al. (2021)
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• Constrictive Pericarditis Caused by Primary Pericardial Mesothelioma: A Case Series — T.A. Meijs, J.F. Heidendael, et al. (2024)
• A Rare Cause of Constrictive Pericarditis: Primary Pericardial Mesothelioma — K. Ravindra, C. Vanchiere, et al. (2025)

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