2020 Volume 56 Issue 3 Pages 262-272
Purpose: To discuss the treatment of neuroblastoma in infants, we retrospectively reviewed the cases of patients with unresectable, non-MYCN-amplified neuroblastoma by comparing those with clinical symptoms and those without them.
Methods: Eighteen consecutive patients with non-MYCN-amplified neuroblastoma were examined in this study. The neuroblastoma was detected in seven patients without symptoms preclinically and in 11 with symptoms, during the past 28 years. Their clinical and tumor characteristics, the reasons for tumor unresectability, risk classification, treatment, and outcome were reviewed.
Results: The reasons for the difficulty in tumor resection in seven patients without clinical symptoms included local invasions and lymph node and liver metastases, and the presence of IDRFs and IDRF-equivalent factors was the reason in three of the seven patients (43%). Excluding one patient who was observed without treatments, six patients were treated with standard protocols. All the seven patients showed NED. On the other hand, among the 11 patients diagnosed with symptoms, seven were in oncologic emergencies. The reasons for tumor unresectability were local invasions and metastases to lymph nodes, liver, and other distant organs, and the presence of IDRFs and IDRF-equivalent factors were the reasons in nine of the 11 patients (82%). Radiotherapy was conducted in addition to surgery and chemotherapy in four patients. While eight and two patients were in NED and AWD, respectively, one patient died of the disease. OS for all 18 patients was 94.4% and EFS was 83.0%.
Conclusion: The findings in infants with unresectable, non-MYCN-amplified neuroblastoma suggest that treatment options could include observation without treatments and chemotherapy following biopsy in patients who present no symptoms. In patients with clinical symptoms, chemotherapy should be promptly given following biopsy, and radiotherapy may be considered in case of oncologic emergencies.
