Widening Spectrum of Liver Angiosarcoma in Children

Ackermann, Oanez*; Fabre, Monique; Franchi, Stephanie; Pariente, Daniele; Debray, Dominique*; Jacquemin, Emmanuel*; Gauthier, Frederic§; Bernard, Olivier*

Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e318230146c
Original Articles: Hepatology and Nutrition
Abstract

Objectives: 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.

Methods and 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.

Author Information

*Service d’Hépatologie Pédiatrique

Service d’Anatomopathologie

Service de Radiologie Pédiatrique

§Service de Chirurgie Pédiatrique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, and Université Paris Sud 11, Paris, France.

Address correspondence and reprint requests to Oanez Ackermann, Service d’Hépatologie Pédiatrique, Hôpital Bicêtre, 78 Rue du Général Leclerc, 94275 Le Kremlin Bicêtre Cedex, Paris, France (e-mail: oanez.ackermann@bct.aphp.fr).

Received 11 February, 2011

Accepted 26 July, 2011

The authors report no conflicts of interest.

Copyright 2011 by ESPGHAN and NASPGHAN