Retrospective analysis of biological factors in a recurrent IV-S neuroblastoma with intermediate-grade malignancy

A clinical stage of IV-S neuroblastoma is known to include heterogeneous tumors. The authors report a case of IV-S neuroblastoma that relapsed after regression 4 years and 10 months after diagnosis. Multiagent chemotherapy was necessary to control the disease, and the patient has been doing well with no evidence of disease for more than 5 years. Although Shimada's classification showed favorable characteristics, N-myc protein was positive and DNA ploidy was diploidy in the tumor at diagnosis. Discrepancy in DNA ploidy was observed and aneuploidy was shown in tumor specimens at recurrence. Experience in this case showed the following important clinical features: (1) IV-S neuroblastoma of intermediate-grade malignancy does exist, and patients with such a disease may be cured by aggressive treatment; (2) Identifying IV-S patients with an unfavorable clinical course is possible by examining biological prognostic factors.