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DOI: 10.1055/s-2007-989270
© Georg Thieme Verlag KG Stuttgart · New York
Surgery of Liver Tumors in Children in the Last 15 Years
Publication History
received May 14, 2007
accepted May 23, 2007
Publication Date:
11 December 2007 (online)
![](https://www.thieme-connect.de/media/ejps/200706/lookinside/thumbnails/10.1055-s-2007-989270-1.jpg)
Abstract
Aim: Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years. Patients and Methods: A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 ± 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves. Results: Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results. Conclusions: Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.
Key words
liver - tumor - children - transplantation
References
- 1 Aronson D C, Schnater J M, Staalman C R. et al . Predictive value of the pretreatment extent of disease system in hepatoblastoma: results from the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL‐1 study. J Clin Oncol. 2005; 23 1245-1252
- 2 Austin M T, Leys C M, Feurer I D. et al . Liver transplantation for childhood hepatic malignancy: a review of the United Network for Organ Sharing (UNOS) database. J Pediatr Surg. 2006; 41 182-186
- 3 Bakshi P, Srinivasan R, Rao K L. et al . Fine needle aspiration biopsy in pediatric space-occupying lesions of liver: a retrospective study evaluating its role and diagnostic efficacy. J Pediatr Surg. 2006; 41 1903-1908
- 4 Barnhart D C, Hirschl R B, Garver K A. et al . Conservative management of mesenchymal hamartoma of the liver. J Pediatr Surg. 1997; 32 1495-1498
- 5 Belhadj K, Reyes F, Farcet J P. et al . Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood. 2003; 102 4261-4269
- 6 Brown J, Perilongo G, Shafford E. et al . Pretreatment prognostic factors for children with hepatoblastoma - results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1. Eur J Cancer. 2000; 36 1418-1425
- 7 Christison-Lagay E R, Burrows P E, Alomari A. et al . Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg. 2007; 42 62-67 67-68
- 8 Davies J Q, de la Hall P M, Kaschula R O. et al . Hepatoblastoma - evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases. J Pediatr Surg. 2004; 39 1321-1327
- 9 Domm J A, Thompson M, Kuttesch J F. et al . Allogeneic bone marrow transplantation for chemotherapy-refractory hepatosplenic gammadelta T-cell lymphoma: case report and review of the literature. J Pediatr Hematol Oncol. 2005; 27 607-610
- 10 Douglass E C. Hepatic malignancies in childhood and adolescence (hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma). Cancer Treat Res. 1997; 92 201-212
- 11 Evans A E, Land V J, Newton W A. et al . Combination chemotherapy (vincristine, adriamycin, cyclophosphamide, and 5-fluorouracil) in the treatment of children with malignant hepatoma. Cancer. 1982; 50 821-826
- 12 Helmberger T K, Ros P R, Mergo P J. et al . Pediatric liver neoplasms: a radiologic-pathologic correlation. Eur Radiol. 1999; 9 1339-1347
- 13 Hernandez F, Navarro M, Encinas J L. et al . The role of GLUT1 immunostaining in the diagnosis and classification of liver vascular tumors in children. J Pediatr Surg. 2005; 40 801-804
- 14 Katzenstein H M, Krailo M D, Malogolowkin M H. et al . Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer. 2003; 97 2006-2012
- 15 Lauwers G Y, Grant L D, Donnelly W H. et al . Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Am J Surg Pathol. 1997; 21 1248-1254
- 16 Otte J B, Pritchard J, Aronson D C. et al . Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL‐1 and review of the world experience. Pediatr Blood Cancer. 2004; 42 74-83
- 17 Perilongo G, Shafford E, Plaschkes J. SIOPEL trials using preoperative chemotherapy in hepatoblastoma. Lancet Oncol. 2000; 1 94-100
- 18 Reynolds M. Pediatric liver tumors. Semin Surg Oncol. 1999; 16 159-172
- 19 Sari N, Yalcin B, Akyuz C. et al . Infantile hepatic hemangioendothelioma with elevated serum alpha-fetoprotein. Pediatr Hematol Oncol. 2006; 23 639-647
- 20 Shafford E A, Pritchard J. Hepatoblastoma - a bit of a success story?. Eur J Cancer [Am]. 1994; 30 1050-1051
- 21 Stocker J T. Hepatic tumors in children. Clin Liver Dis. 2001; 5 259-281
Dr. Manuel Lopez Santamaría
Department of Pediatric Surgery
Hospital Infantil La Paz
Paseo de la Castellana 261
28046 Madrid
Spain
Email: mlopez.hulp@salud.madrid.org