小児の希少がんである肝芽腫に関する当院での経験

Hepatoblastoma is a common malignant liver tumor in children with a peak incidence in the first two years. Its previous mortality was reported to be 24%, but refinements in risk stratification, advances in chemotherapy and application of protocol-based therapy, including surgery, have resulted in improving long-term outcomes. We describe our experience of treating children with hepatoblastoma who underwent surgery using the new treatment guidelines between January 2013 and December 2020. During the aforementioned period, 6 patients with hepatoblastoma (4 male and 2 female) were treated in our unit with median age at presentation being 15 (range 6-36) months. One patient was PRETEXT stage I, three were stage II, and two were stage III. All of them received chemotherapy prior to surgical resection as per the SIOPEL-3 and SIOPEL-4 protocols varying between 2 and 4 cycles, following which size reduction in the tumors was noted and the surgical procedures included right hepatectomy in three patients, non-anatomical hepatic resection in one, left lateral hepatectomy in one, and left hepatectomy in one. The mean duration of surgery was 205 (range 115-300) min, and the mean blood loss was 50 (range 20-150) ml. There was no operative mortality, following which all patients then had adjuvant chemotherapy and are now alive with a median follow-up of 40.6 (18-104) months. The management of hepatoblastoma has changed significantly following the incorporation of recent updates from international groups into clinical practice. With the use of risk stratification, preoperative and adjuvant chemotherapy, and surgical excision, the overall and event-free survival has improved, reiterating the value of a multidisciplinary approach toward the treatment of this disease.