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Case Report Open Access

Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature

  • Authors:
    • Ari M. Abdullah
    • Fahmi H. Kakamad
    • Soran H. Tahir
    • Rawa M. Ali
    • Marwan N. Hassan
    • Rebaz Haji Ali
    • Hiwa O. Abdullah
    • Hadeel A. Yasseen
    • Harem K. Ahmed
    • Bander A. Abdalla
    • Sarhang Sedeeq Abdalla
  • View Affiliations / Copyright

    Affiliations: Department of Scientific Affairs, Smart Health Tower, Sulaymaniyah 46001, Iraq
    Copyright: © Abdullah et al. This is an open access article distributed under the terms of Creative Commons Attribution License [CC BY 4.0].
  • Article Number: 14
    |
    Published online on: January 19, 2026
       https://doi.org/10.3892/wasj.2026.429
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Abstract

Due to its rarity and nonspecific symptoms, pulmonary epithelioid hemangioendothelioma (PEH) is often prone to misdiagnosis or delayed diagnosis until it progresses to advanced stages. The present case report describes the case of a patient with symptomatic PEH identified by computed tomography (CT) angiography. A 31‑year‑old male worker presented with a 1‑month history of left‑sided chest pain. He had a history of 10 years of smoking. The patient initially sought evaluation by a cardiologist. A cardiac workup revealed normal coronary vessels. However, a contrast‑enhanced chest CT scan demonstrated multiple well‑defined bilateral pulmonary nodules. The patient underwent video‑assisted thoracoscopic surgery for obtaining biopsy of bilateral lung nodules, pleura and mediastinal lymph nodes. A histopathological examination confirmed the diagnosis of PEH. Immunohistochemical staining supported the diagnosis with ERG positivity, and TTF1 and AE1/AE3 negativity in the tumor cells. In addition, a total of 12 cases of PEH were selected from the literature and these cases were reviewed. The ages of these patients ranged from 35 to 70 years, with a slightly equal sex distribution. Of note, one‑third of the cases were incidental findings. The majority of the cases had bilateral lung lesions on imaging, some of which exhibited calcifications, necrosis, or pleural effusion. Metastases were observed in 4 cases, affecting bones, lymph nodes, liver, spleen and brain. Immunohistochemistry demonstrated positivity for vascular markers in all cases. A total of 6 patients succumbed due to disease progression and related complications. On the whole, the present case report demonstrates that PEH may present with non‑specific symptoms and may often be diagnosed incidentally during general checkups.

View Figures

Figure 1

Chest computed tomography scan with IV
contrast. (A) Lung window on high-resolution computed tomography.
(B) Soft tissue window with IV contrast illustrating multiple
bilateral pulmonary nodules (green arrows), one of them containing
central coarse calcification (blue arrow).

Figure 2

(A) Section illustrating lung
parenchymal tissue (black arrow) with a hyalinized nodule on the
left side (blue arrow) that is composed of slit-like spaces (red
arrows); hematoxylin and eosin staining; magnification, x4). (B)
Section illustrating epithelioid cells (black arrows) located
within a hyalinized stroma (black stars) with the presence of
vascular spaces that are lined by endothelial cells (blue arrows);
hematoxylin and eosin staining; magnification, x10). (C) ERG
immunohistochemistry illustrating a diffuse, strong nuclear
staining pattern within the nodule.
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Copy and paste a formatted citation
Spandidos Publications style
Abdullah AM, Kakamad FH, Tahir SH, Ali RM, Hassan MN, Ali RH, Abdullah HO, Yasseen HA, Ahmed HK, Abdalla BA, Abdalla BA, et al: <p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>. World Acad Sci J 8: 14, 2026.
APA
Abdullah, A.M., Kakamad, F.H., Tahir, S.H., Ali, R.M., Hassan, M.N., Ali, R.H. ... Abdalla, S.S. (2026). <p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>. World Academy of Sciences Journal, 8, 14. https://doi.org/10.3892/wasj.2026.429
MLA
Abdullah, A. M., Kakamad, F. H., Tahir, S. H., Ali, R. M., Hassan, M. N., Ali, R. H., Abdullah, H. O., Yasseen, H. A., Ahmed, H. K., Abdalla, B. A., Abdalla, S. S."<p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>". World Academy of Sciences Journal 8.2 (2026): 14.
Chicago
Abdullah, A. M., Kakamad, F. H., Tahir, S. H., Ali, R. M., Hassan, M. N., Ali, R. H., Abdullah, H. O., Yasseen, H. A., Ahmed, H. K., Abdalla, B. A., Abdalla, S. S."<p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>". World Academy of Sciences Journal 8, no. 2 (2026): 14. https://doi.org/10.3892/wasj.2026.429
Copy and paste a formatted citation
x
Spandidos Publications style
Abdullah AM, Kakamad FH, Tahir SH, Ali RM, Hassan MN, Ali RH, Abdullah HO, Yasseen HA, Ahmed HK, Abdalla BA, Abdalla BA, et al: <p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>. World Acad Sci J 8: 14, 2026.
APA
Abdullah, A.M., Kakamad, F.H., Tahir, S.H., Ali, R.M., Hassan, M.N., Ali, R.H. ... Abdalla, S.S. (2026). <p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>. World Academy of Sciences Journal, 8, 14. https://doi.org/10.3892/wasj.2026.429
MLA
Abdullah, A. M., Kakamad, F. H., Tahir, S. H., Ali, R. M., Hassan, M. N., Ali, R. H., Abdullah, H. O., Yasseen, H. A., Ahmed, H. K., Abdalla, B. A., Abdalla, S. S."<p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>". World Academy of Sciences Journal 8.2 (2026): 14.
Chicago
Abdullah, A. M., Kakamad, F. H., Tahir, S. H., Ali, R. M., Hassan, M. N., Ali, R. H., Abdullah, H. O., Yasseen, H. A., Ahmed, H. K., Abdalla, B. A., Abdalla, S. S."<p>Pulmonary epithelioid hemangioendothelioma: A case report and brief review of the literature</p>". World Academy of Sciences Journal 8, no. 2 (2026): 14. https://doi.org/10.3892/wasj.2026.429
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