title: Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate.
abstract: BACKGROUND
  Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor of infancy and childhood. It is associated with the development of Kasabach-Merritt syndrome, a life-threatening consumptive thrombocytopenia. We report an interesting case of a massive periorbital congenital KHE in a neonate to raise awareness of this aggressive diagnosis.
  
  
  METHODS
  A male neonate presented with a large congenital mass of the lower eyelid. To prevent development of amblyopia, this mass was surgically excised on the sixth day of life.
  
  
  RESULTS
  Histologic investigation demonstrated spindle-shaped endothelial cells with surrounding crescentic vessels, which were GLUT-1 receptor-negative and D2-40 receptor-positive, consistent with KHE. Surgical excision of the periorbital KHE successfully cleared the neonate's visual axis. At 1 year of follow-up, there was no evidence of tumor recurrence, and visual development was progressing normally. A pleasing surgical result was achieved without periorbital distortion.
  
  
  CONCLUSIONS
  Pediatric vascular tumors have historically been wrought with diagnostic confusion. With recent advances in immunohistochemistry, this previously uncharacterized group of tumors has been differentiated into multiple distinct clinical entities. Accurate and timely diagnosis is paramount because these tumors vary greatly in their clinical behavior, prognosis, and recommended treatment. Surgical excision is preferred and necessary in a neonate with visual access obstruction to prevent amblyopia and irreversible blindness.
authors: Department of Plastic Surgery, Loma Linda University Children's Hospital, Loma Linda University, Loma Linda, California 92350, USA. mcmartin@llu.edu
authors: Mark C
authors: MC
authors: Martin
authors: Heidi
authors: H
authors: Harrington
authors: Wendy W
authors: WW
authors: Wong
mesh_terms: D003937:Diagnosis, Differential; D005142:Eyelid Neoplasms / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D006390:Hemangioendothelioma / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012514:Sarcoma, Kaposi / Q000151:congenital* / Q000473:pathology / Q000601:surgery