Disseminated neuroblastoma (stage IV and IV-S) in the first year of life. Outcome related to age and stage

Abstract

Background. Infants (age 0–11 months) with disseminated neuroblastoma are known to have a better prognosis than older children with the disease, but there is little information regarding factors that influence the outcome of the disease in these patients. Methods. The authors report a series of 110 infants with disseminated neuroblastoma with disease diagnosed between March 1976 and February 1991 in 21 institutions participating in the Italian Cooperative Group on Neuroblastoma (ICGNB). Of the 110 infants, 34 had Stage IV disease, and 76 had Stage IV‐S disease. Results. The 5‐year survival probability was 77% for all patients, 71% for those with Stage IV disease, and 81% for those with Stage IV‐S disease. Of the 34 infants with Stage IV disease, the 9 who were 5 months or younger at the time of disease diagnosis are all alive (1 with active disease) at 7–143 months after diagnosis, whereas of the 25 infants who were 6–11 months of age at the time of disease diagnosis, 10 have died. Of the 76 infants with Stage IV‐S disease, 12/64 who were 5 months of age or younger at the time of disease diagnosis died (mostly of massive hepatomegaly); 9 of these deaths occurred in infants with disease diagnosed before they were 2 months old, whereas 1 death occurred in the 12 infants with disease diagnosed when they were 6–11 months old. Four infants with Stage IV‐S disease achieved complete disease remission and subsequently had relapse of disease. High levels of serum LDH and low urinary excretion of vanillylmandelic acid were associated with worse prognosis. Conclusions. The authors suggest that infants older than 6 months of age who have Stage IV disease require aggressive therapy. For infants with disease diagnosed before they are 2 months old, Stage IV‐S disease may have a worse prognosis than Stage IV disease.

Detection of Neuroblastoma Cells in Bone Marrow and Peripheral Blood by Different Techniques

“…The spread of tumor cells to bone marrow (BM) is a grim prognostic indicator for patients with neuroblastoma (NB), with the exception of infants (1). The search for BM infiltration is therefore of utmost importance for both staging and therapeutic purposes.…”

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

“…This study confirms that stage 4s NB is associated with an excellent survival rate. Indeed, the OS is 88% and comparable to previous studies (Suarez et al, 1991;De Bernardi et al, 1992;Strother et al, 1995;Van Noesel et al, 1997;Katzenstein et al, 1998;Nickerson et al, 2000). We confirm that over onethird of patients have clinically favourable tumours without organ dysfunction despite widespread disease, and that for these patients careful observation is sufficient with a survival rate of 100%.…”

“…These observations suggest that in patients with rapidly expanding intra-abdominal disease, earlier and more intensive treatment may be necessary. As already reported, very young infants had a worse prognosis (De Bernardi et al, 1992;Katzenstein et al, 1998;Nickerson et al, 2000). In previous studies, infants less than 2 months at diagnosis were found to fare worse, although in some other studies the threshold was 1 or 3 months old.…”

Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

“…Approximately half of the children diagnosed with neuroblastoma have metastatic disease on diagnosis and, except for infants (De Bernardi et al, 1992), have a low probability of cure . The remaining children present with localised disease and have an overall better prognosis, mainly depending on the degree of tumour resection (Cecchetto et al, 1983;von Schweinitz et al, 2000).…”