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Infantile hemangioendothelioma of the thymus with massive pleural effusion and Kasabach‐Merritt syndrome: Histopathological, flow cytometrical analysis of the tumor
Author(s) -
Hiraiwa Hisayuki,
Hamazaki Minoru,
Tsuruta Satoru,
Hattori Hiroyoshi,
Mimaya JunIchi,
Hasegawa Shirou,
Kohno Sumio,
Aoki Katsuhiko
Publication year - 1998
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1998.tb01999.x
Subject(s) - medicine , pathology , vimentin , pleural effusion , hemangioendothelioma , cd34 , flow cytometry , immunohistochemistry , stem cell , immunology , biology , genetics
Infantile hemangioendothelioma of the thymus is a rare disease. We describe a patient who developed a large anterior mediastinal mass, severe thrombocytopenia and massive pleural effusion at 1 month of age. Glucocorticosteroid and irradiation therapy had no effect on either the tumor size or clinical symptoms and the tumor was resected subtotally. Three months after the subtotal resection, the remaining tumor had almost disappeared and the symptoms had resolved. The patient has now been well for 1 year after surgery without evidence of recurrence. The tumor tissue was characterized by prominent vascular endothelial proliferation intermixed with a normal thymic structure, producing a picture consistent with that of an infantile hemangioendothelioma in the thymus, lmmunohistochemically, the tumor cells showed positive staining for vimentin, factor VIII and CD34. The DNA stemline and proliferative activity were examined by flow cytometry, which revealed a diploid stemline with a low growth fraction. DNA content and cell cycle analyses of the tumor tissue may be useful for predicting the biological behavior of the tumor.