Surgical view of the treatment of patients with hepatoblastoma

Abstract

BACKGROUNDSurgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL‐1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy.METHODSBetween 1990 and 1994, 154 patients age < 16 years with HB were registered on SIOPEL‐1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/m2 intravenously over 24 hours and doxorubicin 60 mg/m2 intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO.RESULTSOne hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75%). Biopsy complications occurred in 7 of 96 patients (7%). Twenty‐two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18% and 5%, respectively. Complete macroscopic surgical resection was achieved in 106 patients (92%), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5‐year event free survival (EFS) rate for all 154 patients in the study was 66%, and the overall survival (OS) rate was 75%. For the 115 patients who were included in the surgical analysis that followed the exact protocol, the EFS and OS rates were 75% and 85%, respectively.CONCLUSIONSBiopsy is a safe procedure and should be performed routinely. Preoperative chemotherapy seems to make tumor resection easier. Reresection of a positive resection margin does not necessarily have to be performed, because postoperative chemotherapy showed good results. Resection of lung metastases can be curative if there is local control of the primary tumor; however, results showed that the patient's prognosis was worse. Surgical morbidity or mortality rates were not necessarily higher in large multicenter studies. More importantly, countries of lesser economic status also can contribute effectively to these trials. Cancer 2002;94:1111–20. © 2002 American Cancer Society.DOI 10.1002/cncr.10282

Surgical treatment of pulmonary metastases in pediatric solid tumors

“…In the International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL)-1 study, 22 patients had pulmonary metastases at diagnosis and 7 went on to thoracotomy for resection. Four of the seven patients undergoing thoracotomy were long-term survivors 63 . SIOPEL also studied 59 patients with relapsed hepatoblastoma.…”

“…Patients with metastases have a much lower survival rate (25–50%) compared to those without 63, 64 . While early case reports showed the potential for cure following metastasectomy, initial chemotherapy trials also showed the possibility of complete resolution of lung metastases with chemotherapy alone.…”

Novel Advances in Understanding of Molecular Pathogenesis of Hepatoblastoma: A Wnt/β-Catenin Perspective

“…The amount and type of adjuvant chemotherapy vary depending on the risk category. Fortunately, most hepatoblastoma tumors are chemosensitive, and more than 80% of tumors that were previously unresectable do eventually become amenable to surgery after initiation of chemotherapy 6 . In cases of PRETEXT stage I–III in which hepatoblastoma remains in close proximity to essential vascular structures, or in all cases of multifocal PRETEXT IV, liver transplantation becomes the only hope for cure.…”

Loss of EGFR-ASAP1 signaling in metastatic and unresectable hepatoblastoma

“…Tumor histology shows varying mixtures of the more differentiated fetal, less differentiated embryonal cells and undifferentiated small cells (SCU)3. Chemotherapy followed by local resection can be curative45. However, not all tumors are chemosensitive and liver transplantation is needed if the tumor cannot be resected surgically because of size, multifocality or proximity to or invasion of large central vessels6.…”

“…However, not all tumors are chemosensitive and liver transplantation is needed if the tumor cannot be resected surgically because of size, multifocality or proximity to or invasion of large central vessels6. Survival rates vary from 60–80% and chemotherapy sequelae such as deafness are common among survivors, suggesting room for improvement567. The pathognomonic overexpression of β-catenin in HBL is not prognostic.…”