Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma

Our plain-English summary of Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma, by L. Ferreiro, E. San-José, J. Suárez-Antelo, et al (The Clinical Respiratory Journal, 2017).

The study

• Authors: L. Ferreiro, E. San-José, J. Suárez-Antelo, I. Abdulkader, A. Martínez-de-Alegría, F.J. González-Barcala, L. Valdés
• Journal: The Clinical Respiratory Journal
• Published: 2017;11(6):1079-1085
• Type of article: Case report
• Cited by: 6 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Pleural epithelioid haemangioendothelioma (EHE) is a rare tumour that originates in the vascular endothelium with an intermediate degree of malignancy between haemangioma and angiosarcoma. Smoking and asbestos exposure are unproven risk factors and diagnosis is usually confirmed by thoracoscopy, since pleural fluid (PF) cytology is often not conclusive. Immunohistochemistry can also help to confirm the diagnosis. We report an 85-year-old patient with bilateral pleural EHE diagnosed by thoracoscopy, who debuted with a spontaneous bilateral haemothorax, the second described so far, and we conducted a thorough review of the literature to describe the clinical, radiological and prognostic features, as well as the PF, of this rare tumour.

© 2016 John Wiley & Sons Ltd.

In plain English

This article was a case report plus a review of published cases of primary pleural epithelioid haemangioendothelioma (EHE). EHE is a rare tumour arising from blood-vessel lining cells, here in the pleura. The patient was an 85-year-old man who smoked (40 pack-years) and reported no history of asbestos exposure. He had left-sided chest pain, worsening breathlessness and a dry cough. The fluid around his left lung was blood (a haemothorax), and 2300 mL was drained. The tube came out after 72 hours; two days later, fluid appeared on the right side and was also a haemothorax. Fluid cytology was negative on both sides. Biopsies taken by thoracoscopy led to a diagnosis of bilateral pleural EHE. He received only symptomatic treatment and died 60 days after diagnosis. The authors described him as the second reported pleural EHE case that first appeared as spontaneous bilateral haemothorax. Of the 35 cases reviewed, including this one, 25 were men and 32 had pleural effusion. Of 28 cases, 24 died between several days and 29 months after diagnosis. The authors concluded that there is no standardized treatment and the prognosis is poor.

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 with a review of previously published cases
• Who was studied: One 85-year-old man who smoked (40 pack-years), plus 34 previously documented cases of primary pleural EHE found through a PubMed literature search
• Exposure: The patient reported no history of asbestos exposure. He was a smoker (40 pack-years).
• Main results:
  • In the patient, the left pleural fluid was a haemothorax. 2300 mL was drained through a chest tube.
  • The left chest tube was removed at 72 hours. Two days later, the patient became anaemic and fluid appeared on the right side.
  • In the patient, the right-sided fluid was also a haemothorax.
  • In the patient, pleural fluid cytology was negative on both the left and the right side.
  • In the patient, the pleural biopsy was positive for vimentin, cytokeratin and CD31 and negative for calretinin. The final diagnosis was bilateral pleural EHE.
  • The patient received only symptomatic treatment and died 60 days after diagnosis.
  • The literature search found only 34 documented cases of primary pleural EHE.
  • Among the reviewed cases (including this one), 25 of 35 were men (71.4%), and pleural effusion was the usual imaging finding (32 of 35, 91.4%).
  • Among reviewed cases with outcome data, 24 of 28 (85.7%) died between several days and 29 months after diagnosis.
• The authors' conclusion: The authors concluded that pleural EHE has no standardized treatment and a poor prognosis. They also concluded that when a bilateral haemothorax occurs without prior chest trauma, clotting disorders or vascular lesions, malignant pleural disease is highly probable, and that diagnosing pleural EHE usually requires thoracoscopy.
• Limitations the authors state:
  • For the three reviewed patients reported as surviving (at 14, 18 and 27 months), the authors noted that follow-up time was short.

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: Bilateral, Epithelioid, Asbestos Exposure, Thoracoscopy, Cytology, Immunohistochemistry.

Related studies

• 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)
• Simultaneous bilateral spontaneous hydropneumothorax: a rare presentation of bilateral malignant pleural mesothelioma — H.E. Fayed, V.K. Woodcock, et al. (2013)
• Pleural fluid cytological yield and visceral pleural invasion in patients with epithelioid malignant pleural mesothelioma — V. Pinelli, S. Laroumagne, et al. (2012)
• Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features — E.J. Crotty, H.P. McAdams, et al. (2000)
• CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers — Z. Yusıflı, K. Kösemehmetoğlu (2014)

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