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• W2012093906 abstract "Metastatic neuroblastoma is an unfavourable disease in which affected individuals have large invasive primary cancers and bone and bone marrow metastases. Children with neuroblastoma stage 4S comprise a small subgroup of infants with favourable outlook, who present with small primary tumours (similar to those with stage 1 and 2 disease) 1 Evans AE D'Angio GJ Randolph J A proposed staging for children with neuroblastoma: children's cancer study group A. Cancer. 1971; 27: 374-378 Crossref PubMed Scopus (853) Google Scholar that are not uncommonly located bilaterally in the adrenals. Metastases are restricted to the skin and liver, with limited involvement of bone marrow (<10%). 2 Brodeur GM Seeger RC Barrett A et al. International criteria for diagnosis, staging, and response to treatment in patients with neuroblastoma. J Clin Oncol. 1988; 6: 1874-1881 Crossref PubMed Scopus (516) Google Scholar Skin metastases—exceptional in almost all types of cancer (paediatric and adult)—appear as bluish subcutaneous nodules. Liver involvement, which is unprecedented in patients with stage 4 neuroblastoma, is widespread in individuals with neuroblastoma stage 4S, with tens to hundreds of separate nodules in all liver segments. The natural course of neuroblastoma stage 4S disease is an initial growth followed by gradual and simultaneous regression of tumour nodules, which takes months to resolve. The proportion of children with neuroblastoma stage 4S who survive is 70–90%, 3 van Noesel MM Hahlen K Hakvoort-Cammel FG Egeler RM Neuroblastoma 4S: a heterogeneous disease with variable risk factors and treatment strategies. Cancer. 1997; 80: 834-843 Crossref PubMed Scopus (62) Google Scholar but only 30–40% of those with stage 4 neuroblastoma survive. Molecularly, neuroblastoma stage 4S is characterised by near-triploidy and absence of genetic alterations, similar to stage 1 and 2 disease. 4 Vandesompele J Speleman F Van Roy N et al. Multicentre analysis of patterns of DNA gains and losses in 204 neuroblastoma tumors: how many genetic subgroups are there?. Med Pediatr Oncol. 2001; 36: 5-10 Crossref PubMed Scopus (76) Google Scholar , 5 Benard J Raguenez G Kauffmann A et al. MYCN-non-amplified metastatic neuroblastoma with good prognosis and spontaneous regression: a molecular portrait of stage 4S. Mol Oncol. 2008; 2: 261-271 Summary Full Text Full Text PDF PubMed Scopus (55) Google Scholar Structural genetic changes characteristic of stage 4 neuroblastoma (eg, segmental chromosomal alterations, MYCN amplification, and ALK mutations) are not present in children with neuroblastoma stage 4S, but if they are present, the tumour behaves as if it were stage 4 neuroblastoma. 3 van Noesel MM Hahlen K Hakvoort-Cammel FG Egeler RM Neuroblastoma 4S: a heterogeneous disease with variable risk factors and treatment strategies. Cancer. 1997; 80: 834-843 Crossref PubMed Scopus (62) Google Scholar Thus, neuroblastoma stage 4S has a unique combination of symptoms that do not fit a profile of classically invasive and metastasising tumour and contrast sharply with stage 4 neuroblastoma in most aspects." @default.
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• W2012093906 title "Neuroblastoma stage 4S: a multifocal stem-cell disease of the developing neural crest" @default.
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• W2012093906 doi "https://doi.org/10.1016/s1470-2045(12)70012-8" @default.
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