Survival from locally invasive or widespread neuroblastoma without cytotoxic therapy.

Abstract

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.

Clinical Categories of Neuroblastoma Are Associated with Different Patterns of Loss of Heterozygosity on Chromosome Arm 1p

“…Each of these groups has distinct clinical behavior with significant differences in survival rates. 34 Hyperdiploid tumors have a relatively benign biological behavior, with a tendency to regress spontaneously (stage 4s), or mature (stages 1, 2, and 3) and do not require cytotoxic treatment to achieve cure rates exceeding 95%. 34 The diploid group is primarily composed of tumors that are clinically aggressive with poor prognosis despite multimodal therapy.…”

“…34 Hyperdiploid tumors have a relatively benign biological behavior, with a tendency to regress spontaneously (stage 4s), or mature (stages 1, 2, and 3) and do not require cytotoxic treatment to achieve cure rates exceeding 95%. 34 The diploid group is primarily composed of tumors that are clinically aggressive with poor prognosis despite multimodal therapy. 18,19 Different mechanisms of 1p LOH, either CIN or MIN, may be responsible for the different pattern of loss for each genetic subgroup of NB, although we did not detect any relationship between DNA index and LOH.…”

“…Most patients in this group do not require cytotoxic therapy and achieve an overall survival rate of Ͼ95%. 34 The diploid group of tumors, mostly INSS stage 4, presented a high incidence of LOH with large deletions at both 1p36 and 1p22. This group of tumors has MYCN oncogene amplification in a subset of cases (36%).…”

Localised and unresectable neuroblastoma in infants: excellent outcome with low-dose primary chemotherapy

“…The usefulness of neoadjuvant treatment may be questioned in such a subgroup of patients. A recent report suggests that apart from surgical excision adjuvant treatment is not necessary, providing no N-myc amplification has been found (Kushner et al, 1996). However, this is a single-centre study that included only four patients with true INSS stage 3 disease and no dumb-bell tumour, and such results may be difficult to achieve in a multicentre setting.…”

Molecular Genetics of Neuroblastoma and the Implications for Clinical Management: A Review of the MSKCC Experience

“…In marked contrast, most patients with non-stage 4 NB are expected to survive even without medical treatment. More importantly, for these patients, cytotoxic therapy may not alter the natural history of NB, and locally recurrent or residual tumor post-surgery does not generally evolve into the lethal metastatic form of NB [7].…”