Outcome of children older than one year with neuroblastoma

To assess the outcome of children older than one year with neuroblastoma treated at King Abdul-Aziz Medical City, Jeddah, Kingdom of Saudi Arabia. We retrospectively reviewed the files of 52 children older than one year with neuroblastoma [NBL] treated at our center between September 1987 and May 2003. Treatment consisted of OPEC chemotherapy regimen [vincristine, cisplatin, etoposide, and cyclophosphamide] or alternating OPEC/OJEC [carboplatin in place of cisplatin], surgical resection +/- radiotherapy [RT]. No patient received high dose therapy [HDT]. Thirty-four patients [65%] were stage 4, 12 [23%] stage 3, and 6 [11%] stage 2. Three stage 2 patients were treated with surgery only, all are alive in complete remission [CR]. All stage 3 and 4 patients were treated with chemotherapy and surgery +/- RT. After induction chemotherapy, CR was achieved in 17 patients [32%] and partial remission in 10 [19%]. Complete surgical resection was possible in 11 patients [22%]. Disease recurrence or progression occurred in 27 patients [51%]. With a median follow-up of 24 months [range 4-120], the 2-year event free survival was 10%, 82%, and 87% and the overall survival was 12%, 83%, and 100% for stage 4, 3, and 2. Children older than one year with localized NBL have good prognosis compared to those with stage 4. The use of HDT may improve the outcome in the latter group. Toxicity was significant, and adoption of risk-stratified treatment may help to reduce treatment complications

Stage 4S neuroblastoma, a disseminated tumor with excellent outcome

To review the clinical features and outcome of all cases of stage 4S neuroblastoma treated at our center. We retrospectively reviewed the files of all patients [n=75] with neuroblastoma treated at King Abdul-Aziz Medical City, Jeddah, Kingdom of Saudi Arabia between 1986 and 2005. We studied the clinical features and outcome of patients with stage 4S neuroblastoma. Six patients [8%] were confirmed to have stage 4S neuroblastoma. Three were boys with a median age at diagnosis of 4.5 months [range 28 days - 11 months]. Four patients required no intervention. The remaining 2 patients were treated with chemotherapy due to progressive hepatomegaly and respiratory distress. No patient required radiotherapy or surgical intervention. With a median follow up of 4 years [range 9 months - 15.5 years], all patients are alive and well. Two patients continue to have a residual abdominal mass, while complete resolution occurred in the others. Stage 4S neuroblastoma is a special tumor that carries excellent prognosis. Spontaneous regression may occur and intervention is only required in symptomatic patients

Congenital nephrotic syndrome

Congenital Nephrotic Syndrome is a rare disorder in which inheritance is probably autosomal recessive, The most striking characteristic of congenital nephrotic syndrome, in contrast to that occuring later during childhood, is the resistance to corticosteroid therapy and the almost invariable fatal outcome. It appears to have a peculiarly high incidence in Scandinavian countries, particularly Finland. Often it is familial; maternal antibodies may be responsible as in Rh- incompatibility, it appear before the child first year; sometimes during the first few weeks of life. The course is progressive and few patients survive the first year of life. Death is usually due to renal failure or secondary infection. In this report, an Iraqi family with congenital nephrotic syndrome was thoroughly studied in one of them renal biopsy was done in UK. that confirmed the diagnosis