Malignant epithelioid vascular tumors of the pleura: report of a series and literature review

Our plain-English summary of Malignant epithelioid vascular tumors of the pleura: report of a series and literature review, by P.J. Zhang, V.A. Livolsi, J.J. Brooks (Human Pathology, 2000).

The study

• Authors: P.J. Zhang, V.A. Livolsi, J.J. Brooks
• Journal: Human Pathology
• Published: 2000;31(1):29-34
• Type of article: Case report, Review
• Cited by: 69 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Primary malignant vascular tumors of the pleura are rare. The significance and difficulty of distinction between pleural epithelioid hemangioendothelioma (EHE) and angiosarcoma have not yet been addressed. A new series of pleural angiosarcoma is reported, and the relevant literature is reviewed. Five cases were identified from files of the authors' institutions and personal consultation cases (J.J.B.). Twenty-six cases of primary malignant vascular tumors of the pleura were identified in the literature. In a total of 31 cases, 22 were from the West and 9 from Japan. Patients were 22 to 79 years old (average, 57), and the male/female ratio was 9:1. Prior chronic pyothorax was identified only in cases reported from Japan. History of exposure to radiation or asbestos was noted in a few Western cases. The most common presentation was pleural thickening and effusion. Almost all of the patients died of disease shortly after diagnosis. A spectrum of histology ranging from characteristic high-grade epithelioid to relatively low-grade EHE-like features was observed in our cases and can be found in previous reports. Most cases showed variable spotty cytokeratin immunoreactivity. Endothelial markers (factor 8, CD34, or CD31) were invariably positive. Pleural angiosarcomas are often epithelioid and can be easily mistaken for mesothelioma or carcinoma clinically and histologically. Awareness of this rare tumor should prompt the use of endothelial markers when faced with a questionable mesothelioma. When cytokeratin is negative, or focal with strong vimentin reactivity, a vascular tumor should be suspected and confirmed with vascular markers. Because of their invariably aggressive behavior, all epithelioid vascular tumors of the pleura should be considered highly malignant regardless of the presence of EHE-like histological features.

In plain English

This article reported 5 new cases of epithelioid angiosarcoma of the pleura, a rare cancer of vessel-lining cells in the membrane around the lungs. It also reviewed 26 previously published cases of primary malignant vascular tumors of the pleura. The 5 new patients came from two U.S. medical centers and one author's consultation files. They were 45 to 66 years old. All had diffuse pleural thickening and effusion, which led to a clinical diagnosis of mesothelioma in most, and all died within 6 months of diagnosis. In laboratory staining, all 5 tumors reacted for cytokeratin (patchily in 4) and for the vessel marker CD31. Of the 31 cases overall, 22 were from Western countries and 9 from Japan. All reported Japanese patients had chronic pyothorax caused by tuberculosis. Among the 22 Western cases, the authors said asbestos exposure might be responsible in 3 (14%) and radiation in 2. One of the three asbestos histories was recorded with a question mark, and no fiber types were named. The authors concluded that these tumors are easily mistaken for mesothelioma and should all be considered highly malignant.

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 series of 5 patients combined with a literature review of published cases
• Who was studied: 5 new cases from Roswell Park Cancer Institute (1970 to 1998), University of Pennsylvania Medical Center (1989 to 1998) and one author's personal consults, plus 26 cases from the English-language literature; 31 cases in total, 22 from the West and 9 from Japan
• Exposure: Possible causes recorded in case histories: asbestos exposure (in 3 published Western cases, no fiber type stated), prior radiation, and tuberculosis-related chronic pyothorax (Japanese cases). None of the authors' own 5 cases was described as having asbestos exposure.
• Main results:
  • The authors' 5 patients were 45 to 66 years old, with a mean of 57.2 years.
  • All 5 of the authors' patients presented with diffuse pleural thickening and effusion, and most were clinically diagnosed as mesothelioma.
  • All 5 of the authors' patients died of the disease within 6 months of diagnosis.
  • Positive staining in the authors' cases: cytokeratin 5 of 5, CEA 1 of 4, CD31 5 of 5, CD34 4 of 5, factor VIII 4 of 5.
  • Among the 22 Western cases, the authors judged prior radiation and asbestos exposure might be responsible in a few: radiation in 2 cases and asbestos in 3 cases (14%).
  • One of the published asbestos histories was recorded with a question mark.
  • All reported Japanese patients had chronic pyothorax caused by lung or pleural tuberculosis.
  • In the 26 published cases, ages ranged from 22 to 79 years (mean 57), and all but 2 patients were male.
  • Only 1 published patient, with a tumor diagnosed as epithelioid hemangioendothelioma (EHE), was reported alive with disease, at 6-month follow-up.
• The authors' conclusion: The authors concluded that all diffuse epithelioid vascular tumors of the pleura should be considered highly malignant, even when they resemble the lower-grade EHE, because of their aggressive behavior.
• Limitations the authors state:
  • The authors could not find any published pleural EHE case with enough follow-up to show a lower-grade course.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleura, Empyema, Pleural Thickening, Histology, CD34, Mesothelioma, Carcinoma.

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• Malignant vascular tumours of the pleura in "asbestos" workers and endothelial differentiation in malignant mesothelioma — R.L. Attanoos, S.K. Suvarna, et al. (2000)
• Pseudomesotheliomatous angiosarcoma: a pleuropulmonary lesion simulating malignant pleural mesothelioma — G. Falconieri, R. Bussani, et al. (1997)
• Malignant mesothelioma of the pleura with desmoplastic histology: a case series and literature review — K. Hashimoto, Y. Okuma, et al. (2016)
• Expression of calretinin, thrombomodulin, keratin 5, and mesothelin in lung carcinomas of different types: an immunohistochemical analysis of 596 tumors in comparison with epithelioid mesotheliomas of the pleura — M. Miettinen, M. Sarlomo-Rikala (2003)

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