Jen-Yin Hou1, Ting-Chi Yeh1, Ting-Huan Huang1, Jin-Cherng Sheu2, Hsi-Che Liu3. 1. MacKay Medical College, New Taipei City, Taiwan; Division of Pediatric Hematology-Oncology, MacKay Memorial Hospital, Taipei, Taiwan; Division of Pediatric Hematology-Oncology, MacKay Children's Hospital, Taipei, Taiwan. 2. Division of Pediatric Surgery, MacKay Memorial Hospital, Taipei, Taiwan. 3. MacKay Medical College, New Taipei City, Taiwan; Division of Pediatric Hematology-Oncology, MacKay Memorial Hospital, Taipei, Taiwan; Division of Pediatric Hematology-Oncology, MacKay Children's Hospital, Taipei, Taiwan. Electronic address: hsiche@mmh.org.tw.
Abstract
BACKGROUND: Hepatoblastoma (HB) is the most common childhood primary hepatic malignancy. The overall survival rate in patients with HB has reached more than 80% over the past decades. The poor prognostic and high-risk HB have been defined, but the treatment and cure of refractory or relapsed HB is still an arduous task. METHODS: The complete records of HB in patients under the age of 18 at the MacKay Memorial Hospital between 1990 and 2019 were examined. RESULTS: The treatment results for 11 patients with refractory or relapsed HB are presented. The multi-modality treatment records were reviewed and the clinical characteristics associated with poor outcome included multifocal lesions, low α-fetoprotein, great vessel invasion and metastases. Delayed liver tumor surgery was carried out in eight cases. The median duration of follow-up for the 11 patients was 48.6 months (range 1.9 to 316.8 months). The 5-year and 10-year overall survival rate were 62.3% ± 15% (SE) and 49.9% ± 16.4% (SE), respectively. Most treatment-related toxicities were tolerable. The major concern during long term follow-up was irreversible high-frequency hearing loss. CONCLUSION: Patients with refractory/relapsed HB are still a thorny issue and more research is needed to improve the outcome.
BACKGROUND:Hepatoblastoma (HB) is the most common childhood primary hepatic malignancy. The overall survival rate in patients with HB has reached more than 80% over the past decades. The poor prognostic and high-risk HB have been defined, but the treatment and cure of refractory or relapsed HB is still an arduous task. METHODS: The complete records of HB in patients under the age of 18 at the MacKay Memorial Hospital between 1990 and 2019 were examined. RESULTS: The treatment results for 11 patients with refractory or relapsed HB are presented. The multi-modality treatment records were reviewed and the clinical characteristics associated with poor outcome included multifocal lesions, low α-fetoprotein, great vessel invasion and metastases. Delayed liver tumor surgery was carried out in eight cases. The median duration of follow-up for the 11 patients was 48.6 months (range 1.9 to 316.8 months). The 5-year and 10-year overall survival rate were 62.3% ± 15% (SE) and 49.9% ± 16.4% (SE), respectively. Most treatment-related toxicities were tolerable. The major concern during long term follow-up was irreversible high-frequency hearing loss. CONCLUSION:Patients with refractory/relapsed HB are still a thorny issue and more research is needed to improve the outcome.