Tuberculosis and Respiratory Diseases
- 제40권3호
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- Pages.301-307
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- 1993
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- 1738-3536(pISSN)
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- 2005-6184(eISSN)
폐, 종격동 및 뇌를 침범한 방추세포형 혈관내피종 1예
A Case of Spindle Cell Hemangioendothelioma Involving the Lung, Mediastinum and Brain
- 김환태 (인제대학교 의과대학부속 백병원 내과학교실) ;
- 김인호 (인제대학교 의과대학부속 백병원 내과학교실) ;
- 이봉춘 (인제대학교 의과대학부속 백병원 내과학교실) ;
- 강창일 (인제대학교 의과대학부속 백병원 내과학교실) ;
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윤혜경
(인제대학교 의과대학부속 백병원 병리학교실)
- Kim, Hwan-Tae (Department of Internal Medicine, College of Medicine, In-Je University) ;
- Kim, In-Ho (Department of Internal Medicine, College of Medicine, In-Je University) ;
- Lee, Bong-Choon (Department of Internal Medicine, College of Medicine, In-Je University) ;
- Kang, Chang-Il (Department of Internal Medicine, College of Medicine, In-Je University) ;
-
Yoon, Hye-Kyoung
(Department of Anatomical Pathology, College of Medicine, In-Je University)
- 발행 : 1993.06.30
초록
Intravascular bronchioloalveolar tumor is now recognized as a pulmonary form of hemangioendothelioma(HE). HE is an unusual tumor of adult life which is characterized by proliferation of an "epithelioid" or "spindle" endothelial cell. In the lung it usually presents as multiple bilateral slowly growing nodules less than 2 cm in diameter. The aetiology and pathogenesis of this disease are unknown. Spindle cell HE occurs at any age, but approximately one half of patient are 25 years of age or younger and males are affected twice more frequently than females. On light microscopic examination, the tumor show mild cellular atypia, nearly absent mitoses and electron-microscopic studies reveal evidence of endothelial cell differentiation. Intracytoplasmic localization of Factor VIII-related antigen is demonstrated on immunohistochemical study, which confirmed the endothelial origin of the tumor. No effective therapy is yet known for HE, but survival of this tumor can be quite long. However, one half of the patient have died, usually of progressive pulmonary insufficiency. This 19-yr-old male complained of Rt. chest pain and intermittent hemoptysis. Simple chest film and chest CT scan showed the Rt. pleural effusion, variable sized bilateral pulmonary nodules, irregular large heterogenous tumor with well enhancement and extensive necrosis in the anterior mediastinum. The mediastinal mass was biopsied and diagnosed as spindle cell HE by light microscopic finding and immunohistochemical studies.
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