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Vol.43 No.2 contents Japanese/English

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Article in Japanese

- Case Report -

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

Hiroyuki Sakurai1, Kenji Suzuki1, Shun-ichi Watanabe1, Hisao Asamura1, Ryosuke Tsuchiya1, Arahumi Maeshima2
1Division of Thoracic Surgery, National Cancer Center Hospital, Japan, 2Division of Pathology, National Cancer Center Research Institute, Japan

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.
key words: Epithelioid hemangioendothelioma, Factor VIII-related antigen, Open lung biopsy, Intravascular bronchioloalveolar tumor (IVBAT), Pulmonary neoplasm

Received: October 31, 2002
Accepted: January 20, 2003

JJLC 43 (2): 143-147, 2003

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