Treatment of stage 4s neuroblastoma – report of 10 years' experience of the French Society of Paediatric Oncology (SFOP)

Schleiermacher, Gudrun; Rubie, H; Hartmann, Olivier; Bergeron, Christophe; Chastagner, Pascal; Méchinaud, Françoise; Michon, Jean

doi:10.1038/sj.bjc.6601154

Cited by 85 publications

(83 citation statements)

References 25 publications

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“…Certain tumors are found during the prenatal diagnosis prior to delivery (22). In the present study, patients < 6 months old were all at stage 4s, which was consistent with the results of previous studies (23,24). According to the COG classification, the majority of cases were in the low-and intermediate-risk groups (9 cases), and the rest were in the high-risk group (7 cases).…”

Section: Discussionsupporting

confidence: 80%

Clinical characteristics of infant neuroblastoma and a summary of treatment outcome

et al. 2016

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Abstract. Neuroblastoma (NB) is the most common malignant solid tumor in the peripheral nervous system in infants and young children, with a high degree of malignancy. The clinical characteristics and prognosis of NB in infants are unique. The present study retrospectively analyzed the prognosis of infant NB cases that underwent different treatments. In total, 16 infant NB cases (10 male and 6 female) who were treated between February 2007 and August 2013 in Beijing Tongren Hospital (Beijing, China), were enrolled in the study. They were diagnosed by pathology, medical imaging and serology methods. These 16 patients were subjected to comprehensive treatment, including chemotherapy, surgery, autologous peripheral blood stem cell transplantation (APBSCT) and radiation therapy. The age distribution and clinical stages were: 5 cases (31.25%) at ≤3 months (4 cases at stage 4s and 1 case at stage 4); 2 cases (12.5%) at 3-6 months (both at stage 4s); and 9 cases (56.25%) at 6-12 months (2 cases at stage 4s, 6 cases at stage 4 and 1 case at stage 3). Subsequent to treatment, nicotinic acid esterase (NSE) levels in the patient's serum significantly decreased. The NSE levels in 12 cases (75%) dropped to the reference value (0-15.2 ng/dl). All the NB infants at stages 4s and 3 were treated by surgery and chemotherapy (100%; 9/9). The 5 NB infants at stage 4 were treated by chemotherapy and surgery. For the 2 NB infants who experienced recurrence or whose condition was partially relieved after conventional therapy, APBSCT therapy was applied. At the last follow-up in September 2014, 13 cases (81.25%) presented with a complete response, 2 cases (12.50%) with a partial response and 1 case (6.25%) with recurrence after transplantation (progressive disease). In conclusion, infant NB cases are sensitive to chemotherapy, particularly the cases at stage 4s, which occur with a higher incidence, but with a better clinical response and prognosis after treatment. IntroductionNeuroblastoma (NB) is the most common malignant solid tumor from the peripheral nervous system. The disease originates from neural crest cells and accounts for 7-10% of malignant tumors among children (1-3). Several genetic abnormalities associated with NB have been characterized, including DNA content, gain of chromosome arm 17q or deletion of chromosome arms 1p and 11q (4-8). NB occurs frequently in infants, with a high degree of malignancy. According to the stage standard of the International Neuroblastoma Staging System (INSS) (9), NB is classified into stages 1-4, with a special stage 4s. Stage 4s cases occur in infants who are <1 year old. These cases are sensitive to chemotherapy, with a better prognosis than cases at stages 3 and 4. NB infants are mainly treated with surgery in conjunction with chemotherapy and radiotherapy. The response to the treatment also depends on the age of the patients and their sensitivity to chemotherapy. Previous studies have shown that the most robust prognostic factors are age, stage, histology and amplification of the N-Myc o...

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Section: Discussionsupporting

confidence: 80%

Clinical characteristics of infant neuroblastoma and a summary of treatment outcome

et al. 2016

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“…15 Elevated serum LDH may indicate increased tumor burden and has been identified as an independent poor prognostic factor. 3,9 In our evaluation of infants with stage 4S disease, we found that risk of an event was high for patients of very young age, that the age subgroup with the best outcome was approximately 19 to 38 days, and that risk of an event increased gradually after age 38 days. Although the definition of young age varies by study, multiple groups have reported that infants younger than 2 to 3 months old have poorer OS than older infants.…”

Section: 31mentioning

confidence: 93%

“…3,4 However, MYCN gene amplification has been shown to be an extremely important prognostic factor in infants with metastatic neuroblastoma in multiple cooperative studies, for infants with both stage 4S 3,9 and stage 4 disease. [28][29][30] Infants with either stage 4 or 4s neuroblastoma and MYCN gene amplification have a 2-year EFS of less than 30% despite intensive therapy, 28,30 whereas those without MYCN amplification may have a 2-year survival of greater than 90% with minimal or no chemotherapy.…”

Section: 27mentioning

confidence: 99%

“…The primary tumor must be localized (INSS 1 or 2), without infiltration across the midline or contralateral lymph node involvement. 2 Patients with 4S disease (7% to 10% of neuroblastoma) 3 have a more favorable outcome than other patients with metastatic neuroblastoma, often demonstrating spontaneous maturation and regression. Estimated survival rates of 70% to 90% have been reported, and these tumors are usually associated with favorable biologic features.…”

Section: Introductionmentioning

confidence: 99%

“…[4][5][6][7] Unfortunately, a subgroup of patients with stage 4S disease and unfavorable prognostic features-including MYCN amplification, chromosomal aberrations (loss of heterozygosity [LOH] 1p, aberration 11q, gain 17q), diploidy, and age younger than 2 months at diagnosis-has significantly worse outcomes. 3,4,[8][9][10][11][12][13][14] The upper age limit defining 4S disease has been debated as a result of recent reports demonstrating more favorable outcomes for patients with metastatic…”

Section: Introductionmentioning

confidence: 99%

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Prognostic Value of the Stage 4S Metastatic Pattern and Tumor Biology in Patients With Metastatic Neuroblastoma Diagnosed Between Birth and 18 Months of Age

Taggart

London

Schmidt

et al. 2011

JCO

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A B S T R A C T PurposePatients with neuroblastoma younger than 12 months of age with a 4S pattern of disease (metastases limited to liver, skin, bone marrow) have better outcomes than infants with stage 4 disease. The new International Neuroblastoma Risk Group (INRG) staging system extends age to 18 months for the 4S pattern. Our aim was to determine which prognostic features could be used for optimal risk classification among patients younger than 18 months with metastatic disease. MethodsEvent-free survival (EFS) and overall survival were analyzed by log-rank tests, Cox models, and survival tree regression for 656 infants with stage 4S neuroblastoma younger than 12 months of age and 1,019 patients with stage 4 disease younger than 18 months of age in the INRG database. ResultsUnfavorable biologic features were more frequent in infants with stage 4 disease than in infants with 4S tumors and higher overall in those age 12 to 18 months (although not different for stage 4 v 4S pattern). EFS was significantly better for infants younger than 12 months with 4S pattern than with stage 4 disease (P Ͻ .01) but similar for toddlers age 12 to 18 months with stage 4 versus 4S pattern. Among 717 patients with stage 4S pattern, patients age 12 to 18 months had worse EFS than those age younger than 12 months (P Ͻ .01). MYCN, 11q, mitosis-karyorrhexis index (MKI), ploidy, and lactate dehydrogenase were independently statistically significant predictors of EFS and more highly predictive than age or metastatic pattern. MYCN, 11q, MKI, histology, and 1p were combined in a survival tree for improved risk stratification. ConclusionTumor biology is more critical than age or metastatic pattern for prognosis of patients age younger than 18 months with metastatic neuroblastoma and should be considered for risk stratification.

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Antitumor Alkaloids in Clinical Use or in Clinical Trials

Cuendet

Pezzuto

2007

Modern Alkaloids

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Treatment of stage 4s neuroblastoma – report of 10 years' experience of the French Society of Paediatric Oncology (SFOP)

Cited by 85 publications

References 25 publications

Clinical characteristics of infant neuroblastoma and a summary of treatment outcome

Clinical characteristics of infant neuroblastoma and a summary of treatment outcome

Prognostic Value of the Stage 4S Metastatic Pattern and Tumor Biology in Patients With Metastatic Neuroblastoma Diagnosed Between Birth and 18 Months of Age

Antitumor Alkaloids in Clinical Use or in Clinical Trials

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