A Case of Kaposiform Hemangio-Lymphangiomatosis.
- Author:
Jun Eun PARK
1
;
Jong Jin SEO
;
Hyung Nam MOON
;
Thad T GHIM
Author Information
1. Department of Pediatrics, University of Dankook College of Medicine, Chonan, Korea. pedpje@hitel.net
- Publication Type:Case Report
- Keywords:
Kaposiform;
Hemangio-lymphangiomatosis;
Hemangioma;
Lymphangioma;
Interferon-alpha2b
- MeSH:
Hemangioma;
Humans;
Lung Diseases, Interstitial;
Lymphangioma;
Mediastinum;
Neoplasm Metastasis;
Respiratory Insufficiency;
Sarcoma, Kaposi;
Thymus Gland;
Vascular Neoplasms
- From:Korean Journal of Pediatric Hematology-Oncology
2000;7(2):287-292
- CountryRepublic of Korea
- Language:Korean
-
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.
