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

Clin Imaging. 2015 May-Jun;39(3):529-32. doi: 10.1016/j.clinimag.2015.01.003. Epub 2015 Jan 14.

Abstract

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.

Keywords: De-vascularization; Kaposiform hemangioendothelioma; Retroperitoneal; Sirolimus/Rapamycin; Vascular tumor.

Publication types

  • Case Reports

MeSH terms

  • Antibiotics, Antineoplastic / therapeutic use*
  • Child, Preschool
  • Hemangioendothelioma / blood supply*
  • Hemangioendothelioma / diagnosis
  • Hemangioendothelioma / drug therapy*
  • Humans
  • Kasabach-Merritt Syndrome / blood supply*
  • Kasabach-Merritt Syndrome / diagnosis
  • Kasabach-Merritt Syndrome / drug therapy*
  • Male
  • Multimodal Imaging
  • Neovascularization, Pathologic
  • Retroperitoneal Neoplasms / blood supply*
  • Retroperitoneal Neoplasms / diagnosis
  • Retroperitoneal Neoplasms / drug therapy*
  • Sarcoma, Kaposi / blood supply*
  • Sarcoma, Kaposi / diagnosis
  • Sarcoma, Kaposi / drug therapy*
  • Sirolimus / therapeutic use*

Substances

  • Antibiotics, Antineoplastic
  • Sirolimus

Supplementary concepts

  • Kaposiform Hemangioendothelioma