Treatment and outcomes of high-risk neuroblastoma in Southeast Asia: a single-institution experience and review of the literature

INTRODUCTION@#In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor.@*METHODS@#A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors.@*RESULTS@#There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%.@*CONCLUSION@#Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.

Neuropsychiatric symptoms in Chinese children with systemic lupus erythematosus (Ⅱ) / 中华风湿病学杂志

Objective To investigate the relationship between NP involvement and clinical factors and the potential predictors for NPSLE in children. Methods Sixty-two Chinese children with SLE diagnosed between 1990 and 2006 were retrospectively reviewed. Clinical characteristics and potential predictors for NPSLE were analyzed in patients with NP vs those without NP, early-onset NP vs late-onset NP, SLE-onset vs NP-onset in late-onset NP group. Results Ratio of lupus nephritis(LN) at SLE onset was less common in patients with NP disorders than those without NP; the mean age for the early-onset group was significantly younger and the SLEDAI score was higher than those of late-onset group. There was no difference in all the clinical and serological factors for SLE-onset vs NP-onset in the late-onset NPSLE group. Conclusion NP development is negatively associated with renal involvement at SLE diagnosis. Early-onset NPSLE usually happens in young patients with high disease activity scores. There are no clinical factors that can predict the development of NPSLE.

Neuropsychiatric symptoms in Chinese children with systemic lupus erythematosus / 中华风湿病学杂志

Objective To define the clinical characteristics of NPSLE in Chinese children by analyzing their demographic data,clinical manifestations,laboratory parameters and treatment.Methods Sixtytwo children with SLE who were diagnosed between 1990 and 2006 were retrospectively reviewed.The demographic data,clinical manifestations,laboratory parameters,imaging and treatment were analyzed.The syndromes were compared with those reported in other studies.Results Nineteen (31%) SLE patients with 21 NP events were identified.The most common NP manifestations were cognitive dysfunction (48%),seizure disorder (43%) and headache (29%).The abnormality percentage of SPECT was 80%.86% patients received MP pulses therapy.Most (74%) patients recovered without long-term neurological complications.Conclusi NP symptoms are common in Chinese children with SLE,however,the prese-ntations are different from that reported in other studies.SPECT and MR are sensitive tests for NPSLE.The prognosis of NPSLE in our series is favorable.