RESULTS
Patient demographics:
There were 119 children >1 year of age with metastatic
neuroblastoma, forming 50.2% of the total non-infant NB patients. Mean
age of the patients was 3.5years (range 1-14 years) and male: female
ratio was 1.1:1. 89 patients consented for treatment, and are included
in the analysis.
Disease characteristics:
The most common site of primary tumour was suprarenal in 66 patients
(55.5%), followed by retroperitoneal (n=25,21%), thoracic/mediastinal
(n=7, 5.9%), cervical (n=8, 6.7%) and multifocal (n=3, 2.5%). Primary
tumour was undetected in 6 patients (5%). Commonest site of metastasis
was bone marrow (76.3%patients), followed by bone (15.9%), lymph nodes
(6.2%) and liver (1%). 36 patients (40.4%) had multiple metastatic
sites. Baseline LDH values were available for 79 patients, with mean
value of 3724 U/L (range 303 -16609 U/L). 58 patients (65%) had
LDH>750U/L and 21 patients (23.5%) had
LDH<750U/L.
Treatment and response:
Thirty-eight patients (42.6%) received Chemo A, out of which 30
patients (78.9%) had good response to chemotherapy and 6 patients
(15.7%) had poor response. 51 patients (57.3%) received Chemo B, out
of which 23 patients (45.09%) had good response and 24 patients
(47.05%) had poor response. In 6 patients, response assessment could
not be done because of early clinical progression or death. Surgery
could be attempted in 26 patients (29.2%), with excision in 16
patients, debulking in 6 patients and biopsy alone in 4 patients. Only 3
patients in this cohort received radiotherapy.12 patients received
metronomic maintenance chemotherapy after treatment completion (9
patients after Chemo A and 3 patients after Chemo B).
Table 1 shows the Chemotherapy regimen-based response and outcome
Relapse and death:
Overall 74 patients (86%) developed recurrence/progression of disease.
Out of the 53 patients who had good initial response to chemotherapy, 43
patients (81%) relapsed. The median time to recurrence/progression was
9 months (range 1-120months). 73 patients (85.9%) died, the median time
to death being 10 months (range1-123months). Cause of death was disease
progression in 71patients and toxicity-related deaths in 2 patients. 3
relapsed patients are lost for follow-up.
Survival:
At a median follow-up of 72 months (range 15-135months), there were 16
survivors. 5-year disease-free survival (DFS) was 17.6% and overall
survival (OS) was 18.4%.
Mean age of survivors was 2.3 years (range 1-9 years) and their mean LDH
at presentation was 728 U/L (range 303-1747 U/L). 11 patients out of 38
(28.9%) who received Chemo A and 5 patients out of 51(9.8%) who
received Chemo B survived. Out of the 12 patients who received
metronomic chemotherapy, 5 patients survived (41.6%). (Table 2)
Prognostic factors:
In univariate analysis, LDH >750 U/L, type of chemotherapy
regimen, response to initial chemotherapy, number of chemotherapy cycles
received and surgery were found to be statistically significant factors
for DFS and OS. Age, primary site and number of metastatic sites were
not statistically significant. On multivariate analysis,
LDH>750U/L, Chemo B regimen and poor response to
chemotherapy were statistically significant poor prognostic factors.
(figure 1.)
Details of prognostic factors are given in Table 3.