Chronic neuroblastoma: Indolent stage 4 disease in children Journal Article
| Authors: | Kushner, B. H.; Kramer, K.; Cheung, N. K. V. |
| Article Title: | Chronic neuroblastoma: Indolent stage 4 disease in children |
| Abstract: | 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-GD2 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-GD2 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. © 2001 American Cancer Society. |
| Keywords: | cancer chemotherapy; cancer survival; child; clinical article; controlled study; preschool child; treatment outcome; child, preschool; unclassified drug; disease course; mortality; cancer recurrence; cisplatin; doxorubicin; fluorouracil; multimodality cancer therapy; pathophysiology; cancer radiotherapy; combined modality therapy; chemotherapy; cytarabine; cancer staging; carboplatin; dacarbazine; cancer immunotherapy; metastasis; neoplasm recurrence, local; etoposide; alkylating agent; cyclophosphamide; melphalan; vincristine; cancer mortality; ifosfamide; monoclonal antibody; chronic disease; cause of death; cancer regression; iodine 131; infant; neuroblastoma; tumor recurrence; neoplasm metastasis; (3 iodobenzyl)guanidine; remission; remission induction; biologic therapy; toxicity; onset age; retinoic acid; myeloablative agent; monoclonal antibody gd2; humans; human; male; female; priority journal; article; indolent disease |
| Journal Title: | Cancer |
| Volume: | 95 |
| Issue: | 6 |
| ISSN: | 0008-543X |
| Publisher: | Wiley Blackwell |
| Date Published: | 2002-09-15 |
| Start Page: | 1366 |
| End Page: | 1375 |
| Language: | English |
| DOI: | 10.1002/cncr.10800 |
| PUBMED: | 12216106 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Export Date: 14 November 2014 -- Source: Scopus |
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