Refractory Kaposiform Hemangioendothelioma Associated with the Chromosomal Translocation t(13;16)(q14;p13.3)

被引:13
|
作者
Zhou, Shengmei [1 ,2 ]
Wang, Larry [1 ,2 ]
Panossian, Andre [2 ,3 ]
Anselmo, Dean [2 ,3 ]
Wu, Samuel [1 ,2 ]
Venkatramani, Rajkumar [4 ]
机构
[1] Childrens Hosp Los Angeles, Dept Pathol & Lab Med, Los Angeles, CA 90027 USA
[2] Univ Southern Calif, Keck Sch Med, Los Angeles, CA 90033 USA
[3] Childrens Hosp Los Angeles, Dept Surg, Div Plast & Maxillofacial Surg, Los Angeles, CA 90027 USA
[4] Baylor Coll Med, Texas Childrens Canc Ctr, Dept Pediat, Houston, TX 77030 USA
关键词
chromosomal translocation; kaposiform hemangioendothelioma; sirolimus; EPITHELIOID HEMANGIOENDOTHELIOMA; SIROLIMUS; LYMPHANGIOMATOSIS; CHILDHOOD; GENE;
D O I
10.2350/15-09-1707-CR.1
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
We report a 7-year-old male with a history of recurrent kaposiform hemangioendothelioma (KHE) in the sacral area and multiple thoracic vertebral lesions. Tumor karyotyping revealed the balanced translocation t(13;16)(q14;p13.3). He had mildly decreased platelet counts but never experienced any episodes of Kasabach-Merritt phenomenon. He was treated with vincristine for 1 year but improvement was minor. Significant clinical improvement was seen with sirolimus therapy. To the best of our knowledge, this is the first report to demonstrate that KHE is associated with a clonal karyotypic abnormality.
引用
收藏
页码:417 / 420
页数:4
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