Clinical features, treatment and prognosis of 12 patients with primary hepatoblastoma combined with central nervous system metastasis

Abstract

Objective To investigate the clinical characteristics, treatment and prognosis of hepatoblastoma (HB) patients with the central nervous system (CNS) metastasis. Methods The clinical data of 12 patients with CNS metastasis who were admitted to Beijing Tongren Hospital, Capital Medical University from January 2011 to June 2016 were retrospectively analyzed.The clinical features, treatment and prognosis of HB patients with CNS metastasis were summarized. Results (1) Clinical features: all 12 patients were diagnosed as stage-Ⅳ patients according to posto-perative Children ′s Oncology Group (COG ) stage system.The primary positions of extrahepatic metastatic characteristics were as follows: lung metastasis in 12 cases, bone metastasis in 3 cases, and adrenal metastasis in 2 cases, right atrial metastasis in 1 case, and abdominal lymph node metastasis in 1 case.Fetal and epithelial mixed type was the most common type in pathologic Pathologic classification of HB (50%, 6/12 cases). CNS metastasis was detected in all 12 cases during the course of treatment.Brain metastasis were found in 11 cases of CNS imaging features, and 1 case had spinal metastatic tumors.(2)The treatment for CNS metastasis: all cases were given chemotherapy-based comprehensive treatment after being diagnosed as CNS metastasis of HB.Ten cases of CNS metastasis were treated by maintenance chemotherapy and 2 cases received chemotherapy plus intracranial tumor resection.(3)The prognosis of HB with CNS metastasis: the follow-up time was from 7 months to 54 months in 12 HB cases with CNS metastasis, and the median follow-up time was 20 months.The survival time of HB was 1-15 months, and the median survival time was 3.5 months after the diagnosis of CNS metastasis.Two cases of CNS metastasis were given intracranial tumor resection and chemotherapy and were followed up to June 2016, and survival time was 15 months and 5 months respectively.The survival time of other 10 cases without intracranial surgery was 1-6 months, and the median survival time was 3 months.By drawing survival function curve and applying Log-Rank test for CNS transfer children, the survival time of patients given intracranial tumor resection and chemotherapy was longer than those without surgery (P<0.05). Conclusions The occurrence of CNS metastasis occurred in HB patients in phase-Ⅳ patients with pulmonary metastasis.The most common site of CNS metastasis is brain metastasis, which is hematogenous metastatic pathway, and 1 case of spinal cord metastasis was considered as the local invasion and metastasis.For HB cases with CNS metastasis, the survival time is short and the prognosis is poor, but the survival time might be prolonged for the phase-Ⅳ patients with intracranial tumor resection. Key words: Hepatoblastoma; Metastasis; Prognosis