Survival from locally invasive or widespread neuroblastoma without cytotoxic therapy Journal Article

Authors: Kushner, B. H.; Cheung, N. K. V.; LaQuaglia, M. P.; Ambros, P. F.; Ambros, I. M.; Bonilla, M. A.; Gerald, W. L.; Ladanyi, M.; Gilbert, F.; Rosenfield, N. S.; Yeh, S. D.
Article Title: Survival from locally invasive or widespread neuroblastoma without cytotoxic therapy
Abstract: Purpose: To test the hypothesis that cytotoxic therapy is not needed at diagnosis to assure the survival of most patients with non-stage 4 neuroblastoma. Methods: Patients with non-stage 4 disease received no cytotoxic therapy in the absence of N-myc amplification. The International Neuroblastoma Staging System (INSS) was used. Results: Of 84 consecutive patients with previously untreated, newly diagnosed neuroblastoma, 31 (37%) had non-stage 4 disease. All 31 patients initially received no cytotoxic therapy because none of them had N-myc amplification. Nine stage 1 patients are relapse-free. This report focuses on the 22 patients with locally invasive or distant disease: two stage 2A with gross residual tumor postsurgery, 11 stage 2B with ipsilateral or midline lymph node involvement, four stage 3, and five stage 4S. Eight of the 22 patients were older than 1 year. Postsurgery, 13 patients had visible residual disease, and two others had markedly increased urinary catecholamine levels for more than 1 year. Recurrent or enlarging tumors regressed spontaneously (n = 2) or were excised 5 to 39 months after diagnosis (n = 4). One of the latter had chromosome 1p deletions (common in poor-risk neuroblastoma) that were not detected in the patient's original tumor resected 23 months earlier-findings consistent with clonal evolution or multifocal disease. The patient received chemotherapy. All 22 patients are alive 24 to 98 months (median, 64) from diagnosis. Conclusion: Our results suggest that non-stage 4 patients without N-myc amplification can be spared cytotoxic therapy because (1) residual postsurgical or recurrent biologically favorable neuroblastoma rarely evolves into lethal stage 4 disease; and (2) neuroblastoma in lymph nodes has no prognostic significance. These findings are remarkable because no other cancer includes subtypes that are curable without therapy to ablate residual disease.
Keywords: adolescent; cancer survival; child; preschool child; aged; child, preschool; major clinical study; cancer staging; lymph node metastasis; neoplasm staging; cancer invasion; cancer regression; infant; neuroblastoma; neoplasm, residual; neoplasm invasiveness; neoplasm regression, spontaneous; humans; prognosis; human; male; female; priority journal; article
Journal Title: Journal of Clinical Oncology
Volume: 14
Issue: 2
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 1996-02-01
Start Page: 373
End Page: 381
Language: English
PUBMED: 8636746
PROVIDER: scopus
DOI: 10.1200/JCO.1996.14.2.373
Notes: Article -- Export Date: 22 November 2017 -- Source: Scopus
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MSK Authors
  1. Samuel D J Yeh
    54 Yeh
  2. Brian Kushner
    190 Kushner
  3. Nai-Kong Cheung
    439 Cheung
  4. Nancy S Rosen
    27 Rosen
  5. William L Gerald
    367 Gerald
  6. Marc Ladanyi
    864 Ladanyi