Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features

Our plain-English summary of Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features, by E.J. Crotty, H.P. McAdams, J.J. Erasmus, et al (AJR. American Journal of Roentgenology, 2000).

The study

• Authors: E.J. Crotty, H.P. McAdams, J.J. Erasmus, T.A. Sporn, V.L. Roggli
• Journal: AJR. American Journal of Roentgenology
• Published: 2000;175(6):1545-9
• Cited by: 61 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Objective: The purpose of our study was to describe the clinical and radiologic features of epithelioid hemangioendothelioma of the pleura.

Conclusion: Pleural epithelioid hemangioendothelioma is an uncommon malignancy that typically affects older men, who present with chest pain and dyspnea. This lesion manifests on chest radiographs and CT scans with unilateral pleural fluid and nodular pleural thickening and appears similar to diffuse pleural carcinomatosis or mesothelioma.

In plain English

This retrospective case series came from Duke University Medical Center. It described four patients with epithelioid hemangioendothelioma of the pleura, a rare malignant tumor of blood-vessel origin. They came from the institution's pathology database over a 2-year period. The Methods section described all as men aged 55 to 71 (mean 62.3 years), but one figure caption describes a 51-year-old man. Two thoracic radiologists reviewed chest X-rays, CT scans and, for one patient, MR images. All had symptoms, commonly chest pain and dyspnea (shortness of breath). Three had a history of prior asbestos exposure, and three had a history of smoking. The article did not describe the exposure further or analyze any link to the tumor. All tumors were right-sided. All patients had small to moderate pleural effusions (fluid around the lung), and CT showed smooth and nodular pleural thickening in all of them. Three died, on average 10 months (range 1 to 19 months) after symptoms began. The fourth was lost to follow-up. The authors concluded that on imaging the tumor resembles diffuse pleural carcinomatosis or mesothelioma, and that it often becomes widely metastatic with a poor prognosis.

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: retrospective case series (review of imaging and records of four patients)
• Who was studied: Four men with pathologically proved pleural epithelioid hemangioendothelioma, identified from the pathology database at Duke University Medical Center over a 2-year period. The Methods give their ages as 55 to 71 (mean 62.3 years), but the Figure 3 caption describes a 51-year-old man.
• Exposure: Three of the four patients had a history of prior asbestos exposure (no fiber type, product, job or dose given). Three had a history of smoking.
• Main results:
  • All four patients were symptomatic at presentation; chest pain and dyspnea were the common complaints
  • On chest radiographs, all four patients had right-sided pleural effusions, ranging from small to moderate in size
  • On CT, smooth and nodular pleural thickening was seen in all four patients
  • Two patients had multiple pulmonary nodules at presentation, suggestive of metastatic disease; these nodules measured 3 to 12 mm and enlarged on later scans
  • Tumors in all patients were negative for cytokeratin and showed immunoreactivity to some or all of the vascular-endothelial markers CD31, CD34 and factor VIII
  • Three of the four patients died of progressive disease, on average 10 months (range 1 to 19 months) after symptom onset; the fourth was lost to follow-up
  • In the patient who died of liver failure, postmortem examination found metastases to the lung, mediastinal lymph nodes, liver and retroperitoneal lymph nodes
• The authors' conclusion: The authors concluded that pleural epithelioid hemangioendothelioma is an uncommon malignancy that typically affects older men. They stated that on chest radiographs and CT it appears as unilateral pleural fluid with nodular pleural thickening, similar to diffuse pleural carcinomatosis or mesothelioma, and that it often becomes widely metastatic with a poor prognosis.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleura, Dyspnea, Chest X-Ray, CT Scan, Pleural Thickening, Mesothelioma.

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