A Case of Pulmonary Epithelioid Hemangioendothelioma-Review of the Literature-.:Review of the Literature

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Background. Pulmonary epithelioid hemangioendothelioma (PEH) is a rare pulmonary neoplasm, which was formerly known as intravascular bronchioloalveolar tumor, IVBAT. We report a case of PEH, with multiple small nodules throughout both lung fields, and review the published reports. Case. A 63-year-old woman whose chief complaint was back pain was found to have multiple small nodules in both lungs on chest radiograph, and was referred to our hospital. Clinical examination and routine laboratory tests were within normal limits. Tumor markers were not elevated. Diagnostic open lung biopsy was performed. Histologically, the center of the nodule was occupied by a hyalinized matrix and the periphery of the nodules showed intraalveolar or polypoid proliferation of the tumor cells. Immunohistologic staining showed that tumor cells were positive for endothelial markers, CD31, CD34 and Factor VIII-related antigen. These findings were consistent with a diagnosis of PEH. Conclusion. The reported clinical course of PEH was usually relatively slow but sometimes rapid. We have no effective therapeutic regimens for PEH. The patient remains asymptomatic about 1 year 5 months after the diagnosis without treatment. We will carefully follow up this case.
特定非営利活動法人日本肺癌学会の論文