Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms

被引:28
作者
Iacobas, Ionela [1 ]
Simon, Mitchell L. [2 ]
Amir, Tali [3 ]
Gribbin, Christopher E. [2 ]
McPartland, Thomas G. [4 ]
Kaufman, Matthew R. [5 ]
Calderwood, Stanley [1 ]
Nosher, John L. [2 ]
机构
[1] Drexel Univ, Coll Med Pediat Hematol Oncol, St Peters Univ Hosp, New Brunswick, NJ 08901 USA
[2] UMDNJ Robert Wood Johnson Med Sch, Dept Radiol, New Brunswick, NJ 08903 USA
[3] UMDNJ Robert Wood Johnson Med Sch, New Brunswick, NJ 08903 USA
[4] St Peters Univ Hosp, Pediat Orthoped Surg, New Brunswick, NJ 08901 USA
[5] Drexel Univ, Coll Med, St Peters Univ Hosp, Dept Plast Surg, New Brunswick, NJ 08901 USA
关键词
Kaposiform hemangioendothelioma; Sirolimus/Rapamycin; Retroperitoneal; Vascular tumor; De-vascularization;
D O I
10.1016/j.clinimag.2015.01.003
中图分类号
R8 [特种医学]; R445 [影像诊断学];
学科分类号
1002 ; 100207 ; 1009 ;
摘要
Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement. (C) 2015 Elsevier Inc. All rights reserved.
引用
收藏
页码:529 / 532
页数:4
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