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Case Reports
. 2024 Mar 12;19(1):118.
doi: 10.1186/s13019-024-02602-4.

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

Parviz Mardani  1 , Reza Shahriarirad  1   2 , Mohammad Nekooeian  3   4 , Mohammad Hossein Anbardar  5 , Bizhan Ziaian  1 , Hooman Kamran  1   6 , Nazanin Ayare  6 , Masoud Vafabin  1 , Damoun Fouladi  7   8
Affiliations
Case Reports

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

Parviz Mardani et al. J Cardiothorac Surg. .

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.

Keywords: Epithelioid hemangioendothelioma; Pleural tumors; Thoracic cancer; Vascular tumors.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Computed tomography scan of lungs demonstrating left-sided pleural effusion, and minimal pleural thickening, without accompanying parenchymal abnormalities
Fig. 2
Fig. 2
Histopathological evaluation of pleural epithelioid hemangioendothelioma. Microscopic sections show solid nests of epithelioid cells with intracytoplasmic lumina formation (H&E x 200); Also, positive reactivity of tumor cells for CD31 and CD34, and a high Ki67 index (CD31 × 400; CD34 × 400; Ki67 × 100)
Fig. 3
Fig. 3
Post-operative chest radiography of a 39-year-old female patient with a diagnosis of pleural epithelioid hemangioendothelioma following extrapleural pneumonectomy
See this image and copyright information in PMC

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References

    1. Bagan P, Hassan M, Barthes FLP, Peyrard S, Souilamas R, Danel C, Riquet M. Prognostic factors and Surgical indications of Pulmonary Epithelioid Hemangioendothelioma: a review of the literature. Ann Thorac Surg. 2006;82(6):2010–3. doi: 10.1016/j.athoracsur.2006.06.068. - DOI - PubMed
    1. Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT): an analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer. 1983;51(3):452–64. doi: 10.1002/1097-0142(19830201)51:3<452::AID-CNCR2820510317>3.0.CO;2-M. - DOI - PubMed
    1. Crotty EJ, McAdams HP, Erasmus JJ, Sporn TA, Roggli VL. Epithelioid Hemangioendothelioma of the Pleura. Am J Roentgenol. 2000;175(6):1545–9. doi: 10.2214/ajr.175.6.1751545. - DOI - PubMed
    1. Bocchino M, Barra E, Lassandro F, Ranieri F, Muto R, Rea G. Primary pleural haemangioendothelioma in an Italian female patient: a case report and review of the literature. Monaldi Arch Chest Dis 2016, 73(3). - PubMed
    1. Lee YJ, Chung MJ, Jeong KC, Hahn CH, Hong KP, Kim Y-J, Kim YT. Pleural epithelioid hemangioendothelioma. Yonsei Med J. 2008;49(6):1036–40. doi: 10.3349/ymj.2008.49.6.1036. - DOI - PMC - PubMed

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