Korean J Pediatr Hematol Oncol.
2000 Oct;7(2):287-292.
A Case of Kaposiform Hemangio-Lymphangiomatosis
- Affiliations
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- 1Department of Pediatrics, University of Dankook College of Medicine, Chonan, Korea. pedpje@hitel.net
- 2Department of Pediatrics, University of Ulsan College of Medicine, Seoul, Korea.
Abstract
- Kaposiform hemangio-lymphangiomatosis is an extremely rare and locally aggressive vascular neoplasm. This neoplasm histologically shows dilated vascular spaces lined by flat endothelium-like cells and areas of spindle cells forming slit-like vascular spaces similar to those described in Kaposi's sarcoma. We report a case of this neoplasm which originated from the mediastinum infiltrating other adjacent soft tissues such as thymus and pulmonary interstitium without the evidence of distant metastasis. In spite of interferon-alpha2b therapy and excision of this neoplasm, the patient expired due to respiratory failure caused by aggravating interstitial pneumonia and progression of the neoplasm.
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