Postsurgical staging
        Stage I
        Stage II
        Stage III
        Stage IV
Presurgical anatomic staging using imaging techniques
        PRETEXT stage 1
        PRETEXT stage 2
        PRETEXT stage 3
        PRETEXT stage 4

Postsurgical staging

A staging system based on postsurgical extent of tumor and surgical resectability has been used in the United States to group children with liver cancer. This staging system is used to determine treatment.[1-4] Children diagnosed with stage I and stage II hepatoblastoma have a cure rate of greater than 90% compared with 60% for stage III and approximately 20% for stage IV. In children diagnosed with hepatocellular carcinoma, those with stage I have a good outcome.[4] Stage II is too rarely seen to predict outcome, and stages III and IV are usually fatal.[5-7]

• No metastases, tumor completely resected.

• No metastases, tumor grossly resected with microscopic residual disease (i.e., positive margins); or tumor rupture, or tumor spill at the time of surgery.

• No distant metastases, tumor unresectable or resected with gross residual tumor, or positive lymph nodes.

• Distant metastases regardless of the extent of liver involvement.

The pretreatment extent of disease (PRETEXT) staging system for hepatoblastoma categorizes the primary tumor based on extent of liver involvement at diagnosis. The staging system was devised for use in an international hepatoblastoma treatment program in which only children with PRETEXT stage 1 hepatoblastoma undergo initial resection of tumor. All others are treated with chemotherapy prior to attempted resection of the primary tumor. The liver tumors are staged by interpretation of computerized tomography or ultrasound with or without additional imaging by magnetic resonance . The presence or absence of metastases is noted in addition to the PRETEXT stage, but does not alter the PRETEXT stage. The imaged liver is divided into 4 quadrants and involvement of each quadrant with tumor is determined. Stage increases and prognosis decreases as the number of quadrants radiologically involved with tumor increases from 1 to 4. Experienced radiologist review is important because it may be difficult to discriminate between real invasion beyond the anatomic border of a given sector and displacement of the anatomic border.[8-11] The 5-year overall survival in this international study, in which the study protocol called for treatment of children with preoperative doxorubicin and cisplatin chemotherapy, was 100%, 91%, 68%, and 57% for PRETEXT stages 1 to 4, respectively, including patients with metastasis.[12,13] The PRETEXT staging system has a moderate degree of interobserver variability, and the preoperative PRETEXT stage agrees with postoperative pathologic findings only 51% of the time, with overstaging in 37% of patients and understaging in 12% of patients.[10]

• Tumor involves only one quadrant; three adjoining liver quadrants are free of tumor.

• Tumor involves two adjoining quadrants; two adjoining quadrants are free of tumor.

• Tumor involves three adjoining quadrants or two nonadjoining quadrants; one quadrant or two nonadjoining quadrants are free of tumor.

• Tumor involves all four quadrants; there is no quadrant free of tumor.

References

1. Ortega JA, Krailo MD, Haas JE, et al.: Effective treatment of unresectable or metastatic hepatoblastoma with cisplatin and continuous infusion doxorubicin chemotherapy: a report from the Childrens Cancer Study Group. J Clin Oncol 9 (12): 2167-76, 1991.  [PUBMED Abstract]
   
   
2. Douglass EC, Reynolds M, Finegold M, et al.: Cisplatin, vincristine, and fluorouracil therapy for hepatoblastoma: a Pediatric Oncology Group study. J Clin Oncol 11 (1): 96-9, 1993.  [PUBMED Abstract]
   
   
3. Ortega JA, Douglass EC, Feusner JH, et al.: Randomized comparison of cisplatin/vincristine/fluorouracil and cisplatin/continuous infusion doxorubicin for treatment of pediatric hepatoblastoma: A report from the Children's Cancer Group and the Pediatric Oncology Group. J Clin Oncol 18 (14): 2665-75, 2000.  [PUBMED Abstract]
   
   
4. Douglass E, Ortega J, Feusner J, et al.: Hepatocellular carcinoma (HCA) in children and adolescents: results from the Pediatric Intergroup Hepatoma Study (CCG 8881/POG 8945). [Abstract] Proceedings of the American Society of Clinical Oncology 13: A-1439, 420, 1994. 
   
   
5. Giacomantonio M, Ein SH, Mancer K, et al.: Thirty years of experience with pediatric primary malignant liver tumors. J Pediatr Surg 19 (5): 523-6, 1984.  [PUBMED Abstract]
   
   
6. Katzenstein HM, Krailo MD, Malogolowkin MH, et al.: Hepatocellular carcinoma in children and adolescents: results from the Pediatric Oncology Group and the Children's Cancer Group intergroup study. J Clin Oncol 20 (12): 2789-97, 2002.  [PUBMED Abstract]
   
   
7. Czauderna P, Mackinlay G, Perilongo G, et al.: Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group. J Clin Oncol 20 (12): 2798-804, 2002.  [PUBMED Abstract]
   
   
8. Otte JB, Pritchard J, Aronson DC, 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 42 (1): 74-83, 2004.  [PUBMED Abstract]
   
   
9. Perilongo G, Shafford E, Maibach R, et al.: Risk-adapted treatment for childhood hepatoblastoma. final report of the second study of the International Society of Paediatric Oncology--SIOPEL 2. Eur J Cancer 40 (3): 411-21, 2004.  [PUBMED Abstract]
   
   
10. Aronson DC, Schnater JM, Staalman CR, 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 23 (6): 1245-52, 2005.  [PUBMED Abstract]
    
    
11. Roebuck DJ, Olsen Ø, Pariente D: Radiological staging in children with hepatoblastoma. Pediatr Radiol 36 (3): 176-82, 2006.  [PUBMED Abstract]
    
    
12. Pritchard J, Brown J, Shafford E, et al.: Cisplatin, doxorubicin, and delayed surgery for childhood hepatoblastoma: a successful approach--results of the first prospective study of the International Society of Pediatric Oncology. J Clin Oncol 18 (22): 3819-28, 2000.  [PUBMED Abstract]
    
    
13. 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 36 (11): 1418-25, 2000.  [PUBMED Abstract]
    
    

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