Neuroblastoma in adolescents

Abstract

BACKGROUNDNeuroblastoma (NB) occurs rarely during adolescence, and information is scarce on its characteristics and clinical course in this age group.METHODSPatients with NB who were included in the Italian Neuroblastoma Registry were considered for the current study. The clinical characteristics and survival of adolescents (age at diagnosis between 10 yrs and 18 yrs) were compared with those of children (ages 1–9 yrs). Infants (age < 1 yr) were excluded because of their well known favorable clinical course.RESULTSBetween 1116 children and 53 adolescents who were evaluated, no differences were documented with regard to the primary tumor site and the prevalence of advanced stage at diagnosis. If only patients with Stage IV NB were considered, then adolescents were less likely to be diagnosed with bone/bone marrow metastases (77%) compared with children (94%; P = 0.038), but adolescents were more likely to have metastases at unusual sites, such as the lung parenchyma or the central nervous system (23% vs. 7%, respectively; P = 0.005). With regard to biologic characteristics, adolescents did not differ significantly from children, although they always had a lower prevalence of unfavorable markers. In particular, MYCN amplification was documented in 21% of children and in 11% of adolescents (P = 0.173). At age 10 years, adolescents had a 20% overall survival rate and a 22% event‐free survival rate. Adolescents who had resectable disease had a 73% overall survival rate, which was worse compared with the rate among children with the same disease stage (89%), although the difference did not reach statistical significance (P = 0.159). No differences in survival were observed among patients with Stage IV NB, and adolescents had a probability of survival almost identical to that among children (6% vs. 16%, respectively; P = 0.481). However, when the analysis was restricted to events that occurred after patients developed a recurrence, even if the final outcome was poor for both groups, the difference was statistically significant (P = 0.022) mostly because of the more indolent disease course observed among the adolescents. This effect was even more evident for patients with Stage IV NB. When the 6‐year cut‐off point was used to separate children from adolescents, a significantly worse overall survival rate (P = 0.036) was documented for adolescents who had resectable disease (81% vs. 93% in children).CONCLUSIONSNB in adolescents had clinical and biologic characteristics similar to those observed among children. The clinical course of NB probably is correlated significantly with age at diagnosis, but information is scarce on the role of the biologic risk factors in this age group. The authors were able to identify a group of patients with a cut‐off age between 6 years and 10 years that had a more indolent course but a worse prognosis. Cancer 2006. © 2006 American Cancer Society.

Detection of GD2-positive cells in bone marrow samples and survival of patients with localised neuroblastoma

“…Data were retrieved from the Italian Neuroblastoma Registry (INBR) that collects information on clinical and biological characteristics of patients at diagnosis as treatment; follow-up is sought during protocol administration and then at least yearly after treatment discontinuation (Conte et al, 2006). Pathology data regarding the primary tumour were not considered for this study, since information according to the criteria proposed by the International Neuroblastoma Pathology Committee (INPC) was not available for patients diagnosed before 2003.…”

An Uncommon Presentation of Neuroblastoma

“…The most common symptoms are a hard, asymptomatic mass in the abdomen, or bone pain resulting from metastatic spread. The exception is that the bone marrow involvement occurs less frequently in adults, and there is a higher frequency of metastases at unusual sites such as lung or brain [4].…”

“…It seems that, in some studies, the treatment is similar to the protocol in children. Treatment options include surgical resection, chemotherapy or radiotherapy [8]. There are limitations in the longitudinal studies focusing on adult's neuroblastoma treatment due to the rare reports of this neoplasm in adults.…”

Recurrent Neuroblastoma

“…Only one tumor from the older age group had MYCN amplification. In a report of 1116 children and 53 adolescence (age 10 -18 years), adolescence were also found to have worse outcome than children (10-year OS 20% vs. 39%) and had an indolent course post-recurrence (Conte et al, 2006). Franks et al described 15 of the 16 NB patients aged between 13 and 33 years subsequently relapsed and 13 died.…”