Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Our plain-English summary of Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature, by J. Shao, J. Zhang (Oncology Letters, 2014).

The study

• Authors: J. Shao, J. Zhang
• Journal: Oncology Letters
• Published: 2014;8(6):2517-2522
• Cited by: 22 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25-54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

In plain English

This article described four patients diagnosed with pulmonary epithelioid hemangioendothelioma (PEH) at Shanghai Chest Hospital, Shanghai Jiao Tong University, between 2006 and 2013, and reviewed earlier published reports. PEH is a rare, low-grade malignant tumor of blood-vessel origin in the lung. The patients were a 54-year-old man and three women aged 54, 46 and 30. Three were first identified during physical examination, and one first showed a right pleural effusion. All four had multiple lung nodules or lesions and underwent lobectomy or pulmonary wedge resection. Tumor cells in all four cases tested positive for CD31, factor VIII and vimentin, and three cases tested positive for CD34. All four tested negative for markers including CK, CK7, calretinin and desmin. One patient was stable after seven years. One patient, with persistent pleural effusion, received chemotherapy and died after three years. The other two had been followed for six and five months. The authors wrote that PEH involving the pleura must be distinguished from malignant pleural mesothelioma. The article did not discuss asbestos. The authors concluded that PEH is a rare, diverse tumor with varying prognoses, and they called for larger studies.

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 series of four patients (retrospective analysis) with a review of the literature
• Who was studied: Four patients with pulmonary epithelioid hemangioendothelioma diagnosed at Shanghai Chest Hospital (Shanghai Jiao Tong University), China, 2006 to 2013: one 54-year-old male and females aged 54, 46 and 30. Two were surgical patients at the hospital and two were consultation patients from other hospitals.
• Main results:
  • Of the four patients, three were initially identified during physical examination and one showed a right pleural effusion as the first sign.
  • All four cases showed multiple lung nodules or lesions.
  • Cases 3 and 4 had lobectomy; cases 1 and 2 had pulmonary wedge resection.
  • Tumor cells in all four cases were positive for CD31, F8 (factor VIII) and vimentin. CD34 was positive in three cases, and EMA was focally positive in one case.
  • Tumor cells in all four cases were negative for TTF1, CK, CK7, calretinin and desmin.
  • Case 1 (54-year-old male) was stable with no significant progression at seven-year follow-up.
  • Case 2 (54-year-old female) had persistent pleural effusion, received chemotherapy at another hospital, and died of the disease after three years.
  • The authors stated that PEH invading the pleura, or with a pleural effusion, must be distinguished from malignant pleural mesothelioma, which they described as negative for CD31 and CD34.
• The authors' conclusion: The authors concluded that PEH is a rare but diverse form of malignant vascular tumor with varying patient prognoses, and that larger studies are needed.
• Limitations the authors state:
  • The authors stated that studies with a larger sample size are required.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Differential Diagnosis, Prognosis, Life Expectancy, Pleura, Eosinophilic, Interstitial, Necrosis.

Related studies

• Cytologic features, immunocytochemical findings, and DNA ploidy in four rare cases of epithelioid hemangioendothelioma involving effusions — Y. Chen, A. Khanna, et al. (2018)
• Clinicopathological characteristics of primary peritoneal epithelioid mesothelioma of clear cell type: A case report — X.M. Du, Y.P. Wei, et al. (2021)
• 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)
• Primary pleural epithelioid hemangioendothelioma mimicking as a posterior mediastinal tumor — J. Wethasinghe, J. Sood, et al. (2015)
• Malignant pleural mesothelioma mimicking the intrapulmonary growth pattern of epithelioid hemangioendothelioma — Y. Muramatsu, K. Isobe, et al. (2014)

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