title: Widening spectrum of liver angiosarcoma in children.
abstract: OBJECTIVE
  Liver hemangiomas are vascular tumors, which occur in the first months of life and carry risks of initial complications, but are considered to be benign histologically and to regress with time. Histologic studies suggest that a subtype, type 2 hemangioendothelioma, is akin to angiosarcoma and may have a severe long-term prognosis. We report 5 girls with type 2 hemangioendothelioma of the liver.
  
  
  RESULTS
  Three children initially presented with classical infantile multinodular hemangioma, including cardiac and pulmonary complications and regression of tumors at age 1½ to 2½ years. All 3 experienced tumor relapse at ages 2½ to 3, leading to death at ages 2½ to 5. Tumor histology showed type 2 hemangioendothelioma. The other 2 children presented with liver tumors at ages 2 and 3 years. In 1, initial biopsy of a single tumor showed benign type 1 hemangioendothelioma, but surgical resection was followed by relapse in the remaining liver, lung metastases, and death. Whole tumor histology showed both type 1 and 2 lesions. In the other child, tumor biopsy showed type 2 lesions. She underwent liver transplantation and is alive without tumor recurrence 3 years later.
  
  
  CONCLUSIONS
  Careful follow-up is necessary to detect late recurrence in infants with multinodular liver hemangiomas. Vascular liver tumors occurring after infancy are likely to be malignant. The high risk of relapse in the remaining liver suggests that if no metastases are detected, liver transplantation is preferable to surgical tumor resection in both situations.
authors.affiliation: Service d'Hépatologie Pédiatrique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, and Université Paris Sud 11, Paris, France. oanez.ackermann@bct.aphp.fr
authors.forename: Oanez
authors.initials: O
authors.lastname: Ackermann
authors.forename: Monique
authors.initials: M
authors.lastname: Fabre
authors.forename: Stephanie
authors.initials: S
authors.lastname: Franchi
authors.forename: Daniele
authors.initials: D
authors.lastname: Pariente
authors.forename: Dominique
authors.initials: D
authors.lastname: Debray
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.forename: Frederic
authors.initials: F
authors.lastname: Gauthier
authors.forename: Olivier
authors.initials: O
authors.lastname: Bernard
mesh_terms: D000008:Abdominal Neoplasms / Q000473:pathology* / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006390:Hemangioendothelioma / Q000473:pathology* / Q000601:surgery; D006391:Hemangioma / Q000473:pathology / Q000601:surgery; D006394:Hemangiosarcoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology / Q000601:surgery; D008113:Liver Neoplasms / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation; D011379:Prognosis