 |
| About Bioline | All Journals | Testimonials | Membership | News |
|
||||||
|
||||||
Indian Journal of Cancer, Vol. 47, No. 3, July-September, 2010, pp. 314-316 Original Article Hepatoblastoma: Experience from a single center Singh T, Satheesh CT, Appaji L1 , Aruna Kumari BS1 , Padma M1 , Kumar MV2 , Mukherjee G3 Departments of Medical Oncology, 1 Pediatrics Oncology, 2 Surgical Oncology and 3 Pathology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India Code Number: cn10075 PMID: 20587909 DOI: 10.4103/0019-509X.64724 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 Introduction 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 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]
Results 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 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
Copyright 2010 - Indian Journal of Cancer The following images related to this document are available:Photo images[cn10075t1.jpg] |
| |||||||||
![[Table - 1]](/showimage?cn/photo/cn10075t1.jpg){kind=link}