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ORIGINAL ARTICLE
Year : 2010  |  Volume : 47  |  Issue : 3  |  Page : 314-316
 

Hepatoblastoma: Experience from a single center


1 Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India
2 Department of Pediatrics Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India
3 Department of Surgical Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India
4 Department of Pathology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India

Date of Web Publication28-Jun-2010

Correspondence Address:
T Singh
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.64724

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 » Abstract 

Background: The cornerstones of successful treatment of hepatoblastoma (HB) include preoperative chemotherapy followed by complete anatomical resection of tumor, followed by chemotherapy. Advances in chemotherapy in the last 2 decades have been associated with a higher rate of tumor response and possibly a greater potential for resectability. Aims: We analyzed our single center experience with neoadjuvant chemotherapy (NACT) and surgery in HBs. Settings and Design: Our study included all children with HBs who received NACT and underwent surgical excision from January 1997 to July 2004. Materials and Methods: Patient characteristics, clinical features, clinical course, treatment modalities, and long-term outcome were analyzed. Results: There were 9 boys and 3 girls, aged 5-60 months (median age at tumor diagnosis was 24 months). All received NACT containing cisplatin and doxorubicin. Of the 12 children, 9 underwent hepatectomy and among them, 4 patients each had right and left hepatectomy and 1 patient underwent right extended hepatectomy. After surgery, all patients completed rest of the chemotherapy course (total 6 cycles). R0 resection was carried out in all the 9 cases with no life-threatening complications. Conclusions: Our experience of the 9 cases, although less in number, reaffirms the advantages of NACT followed by surgery. The prognosis for patients with resectable tumors is fairly good in combination with chemotherapy.


Keywords: Hepatectomy, hepatoblastoma, neoadjuvant chemotherapy


How to cite this article:
Singh T, Satheesh C T, Appaji L, Kumari BA, Padma M, Kumar M V, Mukherjee G. Hepatoblastoma: Experience from a single center. Indian J Cancer 2010;47:314-6

How to cite this URL:
Singh T, Satheesh C T, Appaji L, Kumari BA, Padma M, Kumar M V, Mukherjee G. Hepatoblastoma: Experience from a single center. Indian J Cancer [serial online] 2010 [cited 2019 Jul 24];47:314-6. Available from: http://www.indianjcancer.com/text.asp?2010/47/3/314/64724



 » Introduction Top


Primary hepatic malignancies in children account for 0.5-2% of all the solid tumors in childhood. Hepatoblastoma (HB) and hepatocellular carcinoma are the most common primary hepatic malignancies in childhood, making up 27.6% and 16.5% of pediatric tumors, respectively. [1] HB preferentially affects boys and occurs in infants or very young children with a median age of presentation of 16 months. Most cases of HBs are sporadic and they have rare but definite associations with specific predispositions and commonly arise within the setting of normal hepatic function. HBs are the most common liver tumors in children. The cornerstones of successful treatment include preoperative chemotherapy followed by complete anatomical resection of tumor, followed by adjuvant chemotherapy. Advances in chemotherapy in the last 2 decades have been associated with a higher rate of tumor response and possibly greater potential for resectability. [2] Preoperative neoadjuvant chemotherapy (NACT) with a single agent or a combination of agents increased resectability and survival as shown in the Society of Pediatric Oncology (SIOPEL-1) study. [3],[4],[5],[6]

We analyzed our single center experience with NACT and surgery in pediatric HBs.


 » Materials and Methods Top


Our study sample included all children with HBs who received NACT and underwent surgical excision from January 1997 to July 2004. The medical charts and radiographic images were reviewed retrospectively. HB was diagnosed on clincoradiologic grounds, alpha-feto-protein (AFP) levels, fine-needle aspiration cytology, and/or excision biopsy. Decisions regarding resectability were based on computed tomography (CT) evaluation. Patient characteristics, clinical features, clinical course, and chemotherapy are summarized in [Table 1].

NACT with cisplatin and doxorubicin (PLADO) chemotherapy consisted of cisplatin (PLA) on day 1 at a dose of 80 mg/m 2 , administered in a continuous 24-h infusion and Adriamycin (doxorubicin [DO]) at a dose of 30 mg/m 2 /d, administered as a continuous 24-h intravenous infusion on days 2 and 3 (total dose per course, 60 mg/m 2 ). [4] The children were given preoperative chemotherapy due to inoperability at presentation. After confirming good response to chemotherapy by CT scan, the patients were operated. Postsurgery patients received 3 further cycles of chemotherapy of same regimen.

For cancer staging, the Children's Cancer Group (CCG) and Pediatric Oncology Group (POG) staging system were used, namely [2],[7]

  1. complete microscopic resection;
  2. complete gross resection but positive microscopic margins;
  3. macroscopic residual tumors or unresectable localized disease; and
  4. metastatic disease.
Follow-up was with clinical examination, AFP levels, and abdominal sonography every 3 months for the first 2 years and then every 6 months for the next 3 years.


 » Results Top


There were 9 boys and 3 girls, aged 5-60 months (median age at tumor diagnosis was 24 months). All the patients presented with a palpable lump in the abdomen and a history of recurrent fever; 3 patients had history of weight loss. Anemia was present in 4 and none had jaundice. The initial AFP levels ranged from 1000 to 3,00,000 ng/mL. All patients were nonreactive for hepatitis B surface antigen, hepatitis C, and HIV.

A palpable mass in the abdomen was the presenting symptom in all the patients. Other presentations included pain in the abdomen, fever, and loss of weight.

Of the 12 children, 9 underwent hepatectomy (all were inoperable at presentation). Following were the surgeries undertaken for them:

Right hepatectomy: 4

Left hepatectomy: 4

Right extended hepatectomy (including segments IV, V, VI, VII, VIII, and I): 1

Following NACT, R0 resection was carried out. After the surgery, all patients completed rest of the chemotherapy course (total 6 cycles). All had R0 resection; 2 patients had an episode of neutropenia in the second cycle for which antibiotics were given and recovered. Overall, all the children tolerated chemotherapy well.

There were no intra- or postoperative complications and AFP levels returned to normal after completion of chemotherapy and surgery.


 » Discussion Top


Improvements in radiologic imaging, advances in chemotherapy, improved surgical techniques, and advances in liver transplantation have shown overall improvement in the outcome of children with HBs. The most important factor determining the outcome in children with HBs is a combination of complete surgical resection and chemotherapy. It has consistently resulted in improved resectability and survival. [8],[9],[10]

For surgically unresectable, nonmetastatic disease involving both lobes, transplantation is an emerging modality. [11] Otte et al. in their recent comprehensive publication on the global experience of liver transplantation, showed that primary liver transplant was associated with an 82% disease-free survival at 6 years as opposed to 30% for rescue liver transplants.[12] Transcatheter arterial chemoembolization or hepatic arterial chemoembolization involves giving chemotherapy and vascular occlusive agents via catheter into the artery supplying the tumor. This offers the advantage of higher tumor concentrations of chemotherapeutic drugs with lower systemic exposure. [13],[14],[15] The potential benefit of chemoembolization over systemic chemotherapy as initial therapy for unresectable tumors has not been determined.

Our experience of the 9 cases, although less in number, reaffirms the advantages of NACT followed by surgery. R0 resection was carried out in all the 9 cases with no life-threatening complications. The prognosis for patients with resectable tumors is good in combination with chemotherapy. However, new therapies are required for patients with unresectable or recurrent tumors as the outcome remains poor.

 
 » References Top

1.Stocker JT. Hepatic tumors in children. Clin Liver Dis 2001;5:259-81.  Back to cited text no. 1  [PUBMED]    
2.Schnater JM, Aronson DC, Plaschkes J, Perilongo G, Brown J, Otte JB, et al. Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL-1). Cancer 2002;94:1111-20.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Perilongo G, Shafford E, Plaschkes J; Liver Tumour Study Group of the International Society of Paediatric Oncology. SIOPEL trials using preoperative chemotherapy in hepatoblastoma. Lancet Oncol 2000;1:94-100.  Back to cited text no. 3  [PUBMED]    
4.Pritchard J, Brown J, Shafford E, Perilongo G, Brock P, Dicks-Mireaux C, et al. Cisplatin, doxorubicin, and delayed surgery for childhood hepatoblastoma: successful approach-results of the first prospective study of the International Society of Pediatric Oncology. J Clin Oncol 2000;18:3819-28.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Czauderna P, Otte JB, Aronson DC, Gauthier F, Mackinlay G, Roebuck D, et al. Guidelines for surgical treatment of the hepatoblastoma in the modern era-recommendations from the Childhood Liver Tumor Strategy Group of the International Society of Pediatric Oncology (SIOPEL). Eur J Cancer 2005;41:1031-6.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Fuchs J, Rydzynski J, Hecker H, Mildenberger H, Bόrger D, Harms D, et al. The influence of preoperative chemotherapy and surgical technique in the treatment of hepatoblastoma-a report from the German Cooperative Liver Tumor studies HB89 and HB94. Eur J Pediatr Surg 2002;12:255-61.  Back to cited text no. 6      
7.Reynolds M. Pediatric liver tumors. Semin Surg Oncol 1999;16:159-72.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Reynolds M. Conversion of unresectable to respectable hepatoblastoma and long term follow-up study. World J Surg 1995;19:814-6.  Back to cited text no. 8  [PUBMED]    
9.Schnater JM, Kφhler SE, Lamers WH, von Schweinitz D, Aronson DC. Where do we stand with hepatoblastoma? A review. Cancer 2003;98:668-78.  Back to cited text no. 9      
10.Bajpai M, Pal K, Agarwala S, Seth T, Gupta AK. Midterm results with hepatectomy after preoperative chemotherapy in hepatoblastoma. Pediatr Surg Int 2005;21:364-8.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Andrews W, Sommerauer J, Roden J, Andersen J, Conlin C, Moore P. 10 years experience of pediatric liver transplantation. J Pediatr Surg 1996;31:619-24.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Otte JB, Pritchard J, Aronson DC, Brown J, Czauderna P, Maibach R, 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.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Takayama T, Makuuchi M, Takayasu K, LeThai B, Ohyama H, Yamazaki S, et al. Resection after intraarterial chemotherapy of a hepatoblastoma originating in the caudate lobe. Surgery 1990;107:231-5.  Back to cited text no. 13  [PUBMED]    
14.Yokomori K, Hori T, Asoh S, Tuji A, Takemura T. Complete disappearance of unresectable hepatoblastoma by continuous infusion therapy through hepatic artery. J Pediatr Surg 1991;26:844-6.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]  
15.Kitahara S, Makuuchi M, Ishizone S, Terada M, Kawasaki S, Nakahata T, et al. Successful left trisegmentectomy for ruptured hepatoblastoma using intraoperative transarterial embolisation. J Pediatr Surg 1995;30:1709-12.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  



 
 
    Tables

  [Table 1]

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