Epithelioid angiosarcoma: A rare cause of pericarditis and pleural effusion

Our plain-English summary of Epithelioid angiosarcoma: A rare cause of pericarditis and pleural effusion, by U. Durani, A. Gallo de Moraes, J. Beachey, et al (Respiratory Medicine Case Reports, 2018).

The study

• Authors: U. Durani, A. Gallo de Moraes, J. Beachey, D. Nelson, S. Robinson, N.S. Anavekar
• Journal: Respiratory Medicine Case Reports
• Published: 2018;24:77-80
• Type of article: Case report
• Cited by: 12 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Angiosarcomas are rare cancers accounting for less than 2% of all soft tissue sarcomas. We report the case of an unusual presentation of pleural epithelioid angiosarcoma in a patient with constrictive pericarditis and recurrent pleural effusion. A 62 year old smoker presented with acute chest pain. ECG showed diffuse elevation of ST segments in the precordial leads. After extensive evaluation, he was diagnosed with viral pericarditis and treated with colchicine. Two weeks later the patient presented to the emergency department with a large right pleural effusion. Evaluation of the pleural fluid obtained from a thoracentesis revealed an exudative effusion with negative microbial studies and no evidence of malignant cells. His pleural effusion re-accumulated rapidly, requiring repeated thoracenteses over several weeks. Medical thoracoscopy was performed and pleural biopsy revealed primary pleural epithelioid angiosarcoma. Staging PET scan revealed malignant enhancement of right pleura, pericardium, right iliac bone and right shoulder. He died suddenly within 6 weeks of diagnosis, prior to initiating palliative chemotherapy. Pleural angiosarcoma should be considered in the differential diagnosis of recurrent pleural effusions of unknown etiology. Negative cytology does not rule out the diagnosis; excisional biopsy is required. Reported risk factors include asbestos exposure, prior chest radiation, active smoking and history of complicated pleural tuberculosis. Pleural epithelioid angiosarcomas carry a very poor prognosis, with the majority of patients dying within months of diagnosis.

In plain English

This case report described a single patient, a 62-year-old African-American male smoker who worked as a custodian at a dairy factory. He first came to the emergency department with sharp chest pain and was presumptively diagnosed with viral myopericarditis, an inflammation of the heart and its lining. Two weeks later he returned with a large right-sided pleural effusion, which is fluid around the lung. The fluid kept coming back and needed repeated drainage. Tests of the fluid found no cancer cells. A pleural biopsy taken during thoracoscopy showed high-grade pleural epithelioid angiosarcoma, a rare cancer of blood-vessel cells. A staging PET scan showed activity in the pericardium, the right pleural fluid, the right iliac bone, left shoulder soft tissue and nodules in both lungs, which the authors called consistent with metastatic cancer. Hours after the scan he went into cardiac arrest and died, before planned palliative chemotherapy began. Autopsy was declined. The authors wrote that his only potential risk factor was active smoking. Asbestos exposure and prior chest radiation, other possible risk factors cited from earlier reports, were not evident in this case. The authors concluded that this cancer should be considered in recurrent unexplained exudative pleural effusions.

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 (single patient)
• Who was studied: One 62-year-old African-American male smoker who worked as a custodian at a dairy factory and had latent tuberculosis treated ten years earlier
• Exposure: Not a study of asbestos exposure. The authors identified active smoking as the patient's only potential risk factor. Asbestos exposure and prior chest radiation, described as other possible risk factors, were not evident in this case. His job was custodian at a dairy factory.
• Main results:
  • Thoracentesis removed 2700 mL of fluid, which was exudative. Cytology found no malignant cells, and repeat cytology remained negative.
  • A thoracoscopic pleural biopsy showed high-grade pleural epithelioid angiosarcoma.
  • Immunoperoxidase studies of the tumor showed reactivity to CD31, FLI-1, AE1/3, OSCAR, WT-1 and D2-40.
  • The staging PET scan showed malignant FDG avidity of the pericardium (SUV max 16.1) and FDG activity in the right pleural effusion, the right iliac bone, left shoulder soft tissue and pulmonary nodules in both lungs. The authors described the scan as consistent with metastatic cancer.
  • A few hours after the PET scan, the patient went into shock and cardiac arrest and died. Autopsy was declined.
  • The patient died before cancer treatment. Had he survived, the plan was palliative chemotherapy with weekly paclitaxel.
• The authors' conclusion: The authors concluded that, though rare, pleural epithelioid angiosarcoma should be considered in patients with recurrent unexplained exudative pleural effusions, and that thoracoscopic or open pleural biopsy should be considered for diagnosis.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleural Effusion, Sarcoma, Thoracentesis, Medical Thoracoscopy, Pleural Biopsy, Staging, PET Scan.

Related studies

• Primary pleural epithelioid angiosarcoma. A case report and review of the literature — E. Dainese, B. Pozzi, et al. (2010)
• Epithelioid angiosarcoma of the pleura: a case report — M.S. Roh, J.Y. Seo, et al. (2001)
• Asbestos induced pericardial effusion and constrictive pericarditis — D. Davies, M.I. Andrews, et al. (1991)
• Malignant deciduoid mesothelioma: a rare variant of epithelioid mesothelioma — L. Taylor, D. Cooper, et al. (2019)
• Biphasic malignant mesothelioma with epithelioid and sarcomatoid components (dedifferentiated mesothelioma) and intrapulmonary growth: a rare entity mimicking desquamative interstitial pneumonia — J. Laforga, A. Gonzalez Garcia (2020)
• Malignant pleural neoplasm with both differentiation of epithelioid mesothelioma and squamous-cell carcinoma, a rare phenomena — A. Re, D. Shersher, et al. (2021)

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