Pleural epitheliod hemangioendothelioma: What started as a liver fluke and ended up being almost mistaken for malignant mesothelioma

Our plain-English summary of Pleural epitheliod hemangioendothelioma: What started as a liver fluke and ended up being almost mistaken for malignant mesothelioma, by O.H. Jamy, B. Huber, S. Giri (Annals of Thoracic Medicine, 2015).

The study

• Authors: O.H. Jamy, B. Huber, S. Giri
• Journal: Annals of Thoracic Medicine
• Published: 2015;10(4):289-91
• Type of article: Case report
• Cited by: 2 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Epitheliod hemangioendothelioma (EHE) is a rare tumor of vascular origin. The pleural variant has only been reported around 20 times in English literature. It commonly occurs in older men and carries a poor prognosis with average survival lasting from a few weeks to months. Pleural EHE (PEHE) can be a diagnostic challenge due to its rarity as well as similarities to other pleural and vascular tumors. There is currently no standard treatment for EHE. Due to the rarity of this disease, reaching a final diagnosis is challenging. It's clinical, radiological, and pathological resemblance to malignant mesothelioma can cause a delay in diagnosis. Special stains such as CD31, CD34, and factor VIII related antigen can help differentiate between the two. Ordering appropriate stains in a timely manner can help avoid misdiagnosing PEHE.

In plain English

This was a case report of one patient, a 24-year-old Middle Eastern man who had moved to the US from Iraqi Kurdistan at age two. The authors described it as the youngest reported case of pleural epitheliod hemangioendothelioma (PEHE), a rare tumor of vascular origin. The patient first had abdominal pain, and tests suggested an Echinococcus infection of the liver. A right-sided pleural effusion (fluid around the lung) followed. During chest surgery, significant pleural thickening suggested mesothelioma, and frozen sections initially favored mesothelioma. Final stains were strongly positive for CD31 and negative for several other markers, and PEHE was diagnosed. The patient did not tolerate carboplatin and paclitaxel and died of respiratory failure within 2 months. Citing earlier reports, the authors noted that PEHE has been related to asbestos and radiation exposure. This patient had no such history in the US and was unsure about asbestos in Kurdistan. The patient had visited Kurdistan four times, for 3-4 weeks each, which the authors judged too short to be associated with asbestos-related pleural disease. The authors concluded that PEHE should be considered among pleural tumors to avoid initial misdiagnosis.

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 discussion of earlier published reports
• Who was studied: One 24-year-old Middle Eastern male who moved to the US with his family from Iraqi Kurdistan at age two
• Exposure: No history of radiation or asbestos exposure while living in the US. The patient was unsure about asbestos exposure in Kurdistan, which he visited 4 times for 3-4 weeks each. The authors judged that period not long enough to be associated with asbestos-related pleural disease. (The authors cited earlier literature relating PEHE to asbestos and radiation exposure.)
• Main results:
  • Initial studies suggested an Echinococcus infection of the liver.
  • Thoracentesis of the right-sided pleural effusion showed a Klebsiella infection, with negative cytology.
  • At video-assisted thoracic surgery, significant pleural thickening suggested mesothelioma, and frozen sections initially favored mesothelioma.
  • Final stains were strongly positive for CD31 and negative for MOC-31, Ber-Ep4, p63, OCT-4, and alpha-fetoprotein, and PEHE was diagnosed.
  • The patient did not tolerate carboplatin and paclitaxel and died of respiratory failure within 2 months.
• The authors' conclusion: The authors concluded that PEHE should be added to the list of possible diagnoses for pleural tumors and distinguished from other pleural and vascular tumors, to avoid an initial misdiagnosis.

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Prognosis, CD34.

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