Pleural epithelioid hemangioendothelioma in a 39-Year-old female: a case report

Our plain-English summary of Pleural epithelioid hemangioendothelioma in a 39-Year-old female: a case report, by P. Mardani, R. Shahriarirad, M. Nekooeian, et al (Journal of Cardiothoracic Surgery, 2024).

The study

• Authors: P. Mardani, R. Shahriarirad, M. Nekooeian, M.H. Anbardar, B. Ziaian, H. Kamran, N. Ayare, M. Vafabin, D. Fouladi
• Journal: Journal of Cardiothoracic Surgery
• Published: 2024;19(1):118
• Type of article: Case report
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin which can be primarily be seen in various tissues. EHE originating from the pleura is an even more uncommon subtype which may mimic mesothelioma and pleural carcinomatosis. The prognosis of pleural EHE is poor and there is no consensus on the optimal therapeutic approach.

Case Presentation: A 39-year-old middle-eastern female presented with progressive dyspnea and left shoulder discomfort. Chest computed tomography scan revealed a left side pleural effusion and pleural thickening. Pleuroscopy was done and biopsies were taken which were positive for CD31, CD34, CK, factor 8-R-antigen, and vimentin. Patient was diagnosed with pleural epithelioid hemangioendothelioma (PEHE) and chemotherapy was started and underwent extrapleural pneumonectomy 7 months later. Unfortunately, the patient passed away 10 months after diagnosis due to disease complications.

Conclusions: Once PEHE is suspected in histology it can be confirmed with immunohistochemistry. Chemotherapy, surgery or a combination of both is currently used as the treatment but the standard treatment remains a question.

© 2024. The Author(s).

In plain English

This article was a case report from Shiraz University of Medical Sciences in Iran. It described one patient with pleural epithelioid hemangioendothelioma (PEHE), a rare cancer of blood-vessel origin arising in the lining around the lung. The authors noted that it can look like mesothelioma. In January 2021, a 39-year-old middle-eastern woman came to the hospital with worsening shortness of breath and left shoulder discomfort. A CT scan showed fluid around the left lung and minimal pleural thickening, and the fluid contained malignant cells. Biopsies taken during pleuroscopy confirmed PEHE through staining tests that were positive for CD31, CD34, CK, factor 8-R-antigen and vimentin. The authors reported that she had no exposure to smoke or asbestos. They wrote that PEHE has been related to asbestos or radiation, but that there is not enough evidence to prove it. She received MAID chemotherapy and seven months later had an extrapleural pneumonectomy. She died of complications of the disease, a 10-month survival. The authors concluded that immunohistochemistry can confirm PEHE once it is suspected, but that the standard treatment remains a question.

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), with a table of previously published surgically treated PEHE cases
• Who was studied: one 39-year-old middle-eastern woman treated at the authors' hospital in Shiraz, Iran, starting January 2021
• Exposure: The authors reported that the patient had no exposure to smoke or asbestos. No fiber type or asbestos exposure was studied.
• Main results:
  • Chest CT showed a left-sided pleural effusion and minimal pleural thickening, without lung parenchymal abnormalities.
  • Pleural fluid analysis showed malignant cells.
  • Pleuroscopy found multiple creamy nodular lesions on the visceral and parietal pleura.
  • Biopsy histopathology and immunohistochemistry confirmed PEHE, positive for CD31, CD34, CK, factor 8-R-antigen and vimentin.
  • After MAID chemotherapy, the patient underwent extrapleural pneumonectomy seven months later because of involvement of the adjacent lung, severe adhesions and thick pleura.
  • In November 2021 the patient developed peritoneal metastasis, pericardial effusion and ascites, and died of complications of the disease.
  • The MAID chemotherapy followed by surgery resulted in a 10-month survival for this patient.
• The authors' conclusion: The authors concluded that once EHE is suspected through histology, it can be confirmed with immunohistochemistry, but that, based on available data, the standard treatment for PEHE remains a question.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Pleural plaques and thickening.

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleura, Mesothelioma, Prognosis, Dyspnea, Pleural Effusion, Pleural Thickening, CD34.

Related studies

• Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features — E.J. Crotty, H.P. McAdams, et al. (2000)
• Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma — L. Ferreiro, E. San-José, et al. (2017)
• CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers — Z. Yusıflı, K. Kösemehmetoğlu (2014)
• Malignant pleural mesothelioma mimicking the intrapulmonary growth pattern of epithelioid hemangioendothelioma — Y. Muramatsu, K. Isobe, et al. (2014)
• Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review — M. Al-Shraim, B. Mahboub, et al. (2005)
• Primary epithelioid haemangioendothelioma of the peritoneum: an unusual mimic of diffuse malignant mesothelioma — R.L. Attanoos, N.S. Dallimore, et al. (1997)

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