Chronic neuroblastoma

BACKGROUND An indolent course is associated with neuroblastoma (NB) in adolescents and adults. In the current study, the authors analyzed this phenomenon in a large series of children with metastatic NB. METHODS The authors studied 38 patients who were diagnosed with NB in the first decade of life and had metastatic disease 5 years or more from diagnosis. RESULTS The median age at diagnosis was 3 years 10 months. MYCN was amplified in 2 of 28 patients tested. Of 30 patients with classic Stage 4 NB, 9 had a late first recurrence of disease (4.3–13 years from diagnosis). Of eight patients who had atypical cases at diagnosis (one isolated mandibular lesion, two Stage 4‐N, five non‐Stage 4), six had a late first distant recurrence of disease (4 years 11 months–38 years 8 months). Nineteen patients were off therapy continuously for 3 years or more before disease recurred a first or second time. Myeloablative therapy was used to consolidate a first or second response in 27 patients. High‐dose conventional therapy helped to achieve a second remission of disease in 9 of 20 patients assessable for response of first recurrence but achieved no major responses of second or third relapse in 10 of 11 patients. The combination of anti‐G D2 immunotherapy and/or cis ‐retinoic acid, targeted radiotherapy, and multiple cycles of chemotherapy with modest toxicity helped prolong survival. Twelve patients survive at 5 years 6 months+ to 19 years 4 months+ from diagnosis (median, 6 years 10 months+), including four with complete remission of disease; 10 received anti‐G D2 immunotherapy after recurrence. The other 26 patients died of disease ( n = 22) or toxicity ( n = 4) at 5 years–41 years 5 months from diagnosis (median, 6 years 5 months). CONCLUSIONS The concept of indolent or smoldering NB should not be limited to adolescents/adults. The expanding repertoire of anti‐NB treatments, including biologic therapies and chemotherapy regimens of modest toxicity, can convert childhood NB into a chronic disease with prolonged survival after recurrence. Cancer 2002;95:1366–75. © 2002 American Cancer Society. DOI 10.1002/cncr.10800