Primary malignant pericardial sarcomatoid mesothelioma: An autopsy report

Our plain-English summary of Primary malignant pericardial sarcomatoid mesothelioma: An autopsy report, by H. Muta, Y. Sugita, K. Ohshima, et al (Pathology International, 2017).

The study

• Authors: H. Muta, Y. Sugita, K. Ohshima, H. Otsubo
• Journal: Pathology International
• Published: 2017;67(6):311-315
• Type of article: Case report
• Cited by: 3 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Primary malignant pericardial sarcomatoid mesothelioma (PMPSM) is an extremely rare tumor with poor prognosis. We present an autopsy case in an 80-year-old man admitted for heart failure after one month of treatment at an outpatient clinic. He died three months after symptom onset. A complete autopsy revealed localization of the tumor to the pericardium without other lesions. Histologically, mainly spindle-shaped atypical cells with hyperchromatic nuclei and nucleoli were observed. Immunohistochemical markers for mesothelioma were positive for calretinin, cytokeratin AE1/AE3, and cytokeratin CAM5.2. Thus, we diagnosed primary sarcomatoid malignant mesothelioma of the pericardium. To our knowledge, only four PMPSM cases have been reported in the English literature in the past 30 years. Although PMPSM is rare, clinicians and pathologists should recognize it as a possible diagnosis of pericardial tumors. It is necessary to accumulate clinical and pathological diagnostic findings to establish early detection methods for this extremely rare disease.

© 2017 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

In plain English

This is a case report of one 80-year-old man in Japan, studied at autopsy, with a review of four earlier published cases. He came to hospital with heart failure symptoms. CT showed fluid and irregular thickening of the pericardium (the sac around the heart). A biopsy was postponed because he was so ill, and he died three months after symptoms began. At autopsy the tumor was confined to the pericardium, with no spread found in other organs. It was made mainly of spindle-shaped cells and stained positive for calretinin, cytokeratin AE1/AE3 and cytokeratin CAM5.2. The authors diagnosed primary sarcomatoid malignant mesothelioma of the pericardium. He had worked in agroforestry with no clear history of asbestos exposure, and no asbestos bodies were found in his lungs. Across the five cases, the two cytokeratin stains were positive in every case tested. The authors suggested this may be characteristic of the tumor and said more cases must be collected to develop early detection methods.

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 of one autopsy case, with a review of four previously reported cases of primary malignant pericardial sarcomatoid mesothelioma
• Who was studied: One 80-year-old man in Japan, previously treated for bronchial asthma, who presented with heart failure symptoms; compared with four previously published cases
• Exposure: The patient worked in the agroforestry industry; the article reports no clear history of asbestos exposure
• Main results:
  • In this patient, chest CT showed pericardial effusion with irregular thickening of the pericardium. Blood tests showed elevated cytokeratin-19 fragments (10.1 ng/mL) and hyaluronic acid (335 ng/mL).
  • In this patient, pericardial biopsy was postponed because of his severe condition, and he died three months after symptom onset
  • At autopsy, the tumor was localized to the pericardium. No tumor invasion or metastasis was seen in other organs, including the lung and pleura.
  • Under the microscope, the pericardial tumor showed a sarcoma-like pattern of mainly spindle-shaped atypical cells
  • In this patient, the tumor cells were positive for calretinin, cytokeratin AE1/AE3 and cytokeratin CAM5.2, focally positive for D2-40, and negative for WT-1
  • In this patient, the tumor cells were negative for CEA, TTF-1, S-100 protein, CD34 and aSMA
  • In this patient, the lungs showed prominent anthracosis and emphysematous changes, but no asbestos bodies
  • Across the five cases reviewed (this case plus four earlier ones), cytokeratin CAM5.2 was positive in 4 of 4 tested and cytokeratin AE1/AE3 in 3 of 3 tested. Calretinin was positive in 3 of 4 and WT1 in 0 of 2.
  • Of the four earlier cases, the authors stated that patients in two had not been exposed to asbestos. In the present case, asbestos exposure history was not clear.
• The authors' conclusion: The authors concluded that a high positive rate of cytokeratin AE1/AE3 and cytokeratin CAM5.2 appears to be a characteristic finding for this tumor. They also said that clinical and pathological findings from more cases must be accumulated to establish early detection methods.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Lung cancer, Mesothelioma.

Medical terms in this study, explained in our Lexicon: Pericardial, Sarcomatoid, Mesothelioma, Autopsy, Prognosis, Pericardium, Calretinin, CAM5.2.

Related studies

• Primary sarcomatoid malignant pericardial mesothelioma — D. Jiang, M. Kong, et al. (2013)
• Post-irradiation pericardial malignant mesothelioma: an autopsy case and review of the literature — M. Bendek, M. Ferenc, et al. (2010)
• An autopsy case of a malignant pericardial mesothelioma in a Japanese young man — M. Watanabe, H. Suzuki, et al. (1999)
• Sarcomatoid Type Primary Pericardial Mesothelioma with a Long-term Survival after the Onset of Cardiac Tamponade — C. Saisho, H. Ishii, et al. (2016)
• Primary pericardial mesothelioma: report of two cases and review of the literature — A.L. Sytman, R.N. MacAlpin (1971)
• Surgical pathology of primary cardiac and pericardial tumors — C. Basso, M. Valente, et al. (1997)

Browse more research in Pathology International, or return to medical research.