TY - JOUR
T1 - Metastatic progression in infants diagnosed with stage 4S neuroblastoma. A study of the Italian Neuroblastoma Registry
AU - Parodi, Stefano
AU - Sorrentino, Stefania
AU - Cataldo, Andrea Di
AU - Tondo, Annalisa
AU - Fagnani, Anna Maria
AU - Perri, Patrizia
AU - Gigliotti, Anna Rita
AU - Erminio, Giovanni
AU - Corrias, Maria Valeria
AU - De Bernardi, Bruno
N1 - Funding Information:
The authors are grateful to Ms Barbara Galleni for data management, Ms Sonia Scaramuccia for secretarial assistance, and to Drs Riccardo Haupt and Alberto Garaventa for helpful suggestions. The Authors are greatly indebted to the many physicians and nurses who participated in the care of these infants, and to pathologists and biologists that have analyzed the tumor specimens. Anna Rita Gigliotti and Giovanni Erminio received grants from Fondazione Italiana per la Lotta al Neuroblastoma.
Publisher Copyright:
© 2021 Wiley Periodicals LLC
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021
Y1 - 2021
N2 - Purpose: Stage 4S neuroblastoma, a tumor affecting infants, is characterized by the capacity to regress spontaneously and high cure rate. About a third of these infants undergo tumor progression requiring antitumor treatment and 10-15% eventually die. In case of metastatic progression, it may occur either at 4S sites (mainly liver) or sites characterizing stage 4 (mainly bone). Aim of this study was to estimate incidence, presenting features and outcome of infants who progressed to stage 4S or stage 4 sites. Patients: Of 280 Italian infants diagnosed with stage 4S neuroblastoma between 1979 and 2013 and registered in the Italian Neuroblastoma Registry, 268 were evaluable for this study, of whom 57 developed metastatic progression. Results: Progression to stage 4S sites occurred in 29/268 infants (10.8%) (Group A) and to stage 4 in 28/268 (10.4%) (Group B). No significant difference was observed between the two groups at the time of diagnosis. At the time of progression, Group A infants were younger (7.3 vs 14.4 months, P =.001) and had a shorter interval from diagnosis to progression (3.8 vs 9.6 months, P =.001). Survival after progression was worse for Group B infants (45% vs 69%, P =.058) and was associated with age at diagnosis lower than 2 months (P =.005) and adrenal primary tumor site (P =.008). Survival rates increased for both groups along the study period. Conclusions: Infants who progressed to stage 4 did worse, possibly in relation to older age at progression and longer interval between diagnosis and progression. Large prospective studies of these patients may lead to more effective treatments.
AB - Purpose: Stage 4S neuroblastoma, a tumor affecting infants, is characterized by the capacity to regress spontaneously and high cure rate. About a third of these infants undergo tumor progression requiring antitumor treatment and 10-15% eventually die. In case of metastatic progression, it may occur either at 4S sites (mainly liver) or sites characterizing stage 4 (mainly bone). Aim of this study was to estimate incidence, presenting features and outcome of infants who progressed to stage 4S or stage 4 sites. Patients: Of 280 Italian infants diagnosed with stage 4S neuroblastoma between 1979 and 2013 and registered in the Italian Neuroblastoma Registry, 268 were evaluable for this study, of whom 57 developed metastatic progression. Results: Progression to stage 4S sites occurred in 29/268 infants (10.8%) (Group A) and to stage 4 in 28/268 (10.4%) (Group B). No significant difference was observed between the two groups at the time of diagnosis. At the time of progression, Group A infants were younger (7.3 vs 14.4 months, P =.001) and had a shorter interval from diagnosis to progression (3.8 vs 9.6 months, P =.001). Survival after progression was worse for Group B infants (45% vs 69%, P =.058) and was associated with age at diagnosis lower than 2 months (P =.005) and adrenal primary tumor site (P =.008). Survival rates increased for both groups along the study period. Conclusions: Infants who progressed to stage 4 did worse, possibly in relation to older age at progression and longer interval between diagnosis and progression. Large prospective studies of these patients may lead to more effective treatments.
KW - metastatic progression
KW - neuroblastoma
KW - stage 4
KW - stage 4S
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U2 - 10.1002/pbc.28904
DO - 10.1002/pbc.28904
M3 - Article
AN - SCOPUS:85099435676
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
ER -