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.
Highlights
Pulmonary epithelioid hemangioendothelioma (PEH) is the current term for a rare neoplasm originally described by Dail and Liebow in 1975 as intravascular sclerosing bronchioalveolar tumor (IVBAT) of the lung [1,2]
The present study describes four cases of PEH that were diagnosed at the Chest Hospital Affiliated to Shanghai Jiaotong University from 2006 to 2013
Case 3 was a 46‐year‐old female with no obvious symptoms; yet irregular clumps were found below the pleura of the right upper lung on a chest computed tomography (CT) during a routine physical examination (Fig. 1A)
Summary
Pulmonary epithelioid hemangioendothelioma (PEH) is the current term for a rare neoplasm originally described by Dail and Liebow in 1975 as intravascular sclerosing bronchioalveolar tumor (IVBAT) of the lung [1,2]. Four cases of PEH, which were diagnosed and treated at Shanghai Chest Hospital, Shanghai Jiao Tong University (Shanghai, China), were observed and analyzed with respect to the clinical manifestations, imaging findings, histopathological characteristics, immunohistochemical phenotypes and prognosis. Case 2 was a 54‐year‐old female who was admitted for chest tightness and fatigue, whereby X‐ray examination revealed a right pleural effusion. Case 3 was a 46‐year‐old female with no obvious symptoms; yet irregular clumps were found below the pleura of the right upper lung on a chest computed tomography (CT) during a routine physical examination (Fig. 1A). Case 4 was a 30‐year‐old female who was without obvious symptoms, and multiple nodules in both lungs were revealed on a chest CT during a routine physical examination (Fig. 1B). Cases 3 and 4 were six and five months into the postoperative follow‐up period at the time of writing, respectively. (Table I)
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