Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon

J Pediatr Surg. 2016 Jun;51(6):1047-50. doi: 10.1016/j.jpedsurg.2016.03.014. Epub 2016 Apr 9.

Abstract

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.

Keywords: Kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; Neonatal kaposiform hemangioendothelioma; Splenic mass; Vascular tumor.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Hemangioendothelioma / diagnosis*
  • Hemangioendothelioma / surgery
  • Humans
  • Infant, Newborn
  • Kasabach-Merritt Syndrome / diagnosis*
  • Kasabach-Merritt Syndrome / surgery
  • Magnetic Resonance Imaging
  • Male
  • Radiography
  • Sarcoma, Kaposi / diagnosis*
  • Sarcoma, Kaposi / surgery
  • Spleen / diagnostic imaging*
  • Splenectomy
  • Splenic Neoplasms / diagnosis*
  • Splenic Neoplasms / surgery

Supplementary concepts

  • Kaposiform Hemangioendothelioma