During development, mice with mutations of stem cell factor (SCF) or its receptor c-kit exhibit defects in melanogenesis, as well as hematopoiesis and gonadogenesis. Because melanocytes derive from neural crest cells, the role of SCF and c-kit was investigated in the neural crest-derived childhood tumor neuroblastoma. Using reverse transcription-polymerase chain reaction analysis, simultaneous ...

The genetic etiology of sporadic neuroblastoma is still largely obscure. In a genome-wide association study, we identified single-nucleotide polymorphisms (SNP) associated with neuroblastoma at the CASC15, BARD1, LMO1, DUSP12, HSD17B12, HACE1, and LIN28B gene loci, but these explain only a small fraction of neuroblastoma heritability. Other neuroblastoma susceptibility genes are likely hidden a...

A cloned suspension culture of mouse C1300 neuroblastoma cells bound, at 2degrees, sheep erythrocytes passively coated with nerve growth factor, with the formation of rosettes. When grown in tissue culture dishes to which they could attach, neuroblastoma cells rapidly transformed, within 48 hr emitting cytoplasmic processes some of which were several mm long. Most of the attached neuroblastoma ...

The presence of acetylcholinesterase in the tumour cells of neuroblastoma has been shown by enzyme histochemistry. For comparison, some other tumours likely to be found in children and commonly presenting histologically as small cell tumours have also been studied. Acetylcholinesterase activity was seen in rhabdomyosarcoma, but, compared with neuroblastoma, the activity was focal and sparse. On...

Neuroblastoma cell lines can be models for neuroblastoma responsiveness to chemotherapy-induced cell death as well as for the sensitivity of untransformed neuronal cells to the toxic side-effects of chemotherapy. Both phenomena are relevant to neuroblastoma cells exposed to bortezomib, a proteasome inhibitor in clinical use. It has been shown previously that neuroblastoma cell lines are prone t...

N euroblastoma is the most frequently occurring extracranial tumour type in children. It arises from the undifferentiated neural crest derived cells destined to become the sympathetic nervous system, and primary tumours typically occur in the adrenal medulla and paraspinal location in the abdomen or chest. At diagnosis, most children of .1 year of age have metastases, commonly in lymph nodes, b...

The quinoxaline anti-tumor agent (R+)XK469 mediates its effects by topoisomerase IIB inhibition. This report describes a 14-year old with relapsed neuroblastoma who experienced disease stabilization for 14 months while receiving (R+)XK469 monotherapy. Due to this favorable response, laboratory studies were undertaken to determine efficacy in the preclinical setting. (R+)XK469 inhibited prolifer...

Metastatic neuroblastoma is an aggressive malignancy with a poor prognosis. Recent findings have shown that sorafenib decreases cell viability and increases apoptosis in human neuroblastoma cell lines. We report an experience of compassionate use of sorafenib in children with treatment-refractory neuroblastoma. Sorafenib showed transient anti-tumor activity in all four patients without adverse ...

PURPOSE Epigenetic aberrations have been shown to play an important role in the pathogenesis of most cancers. To investigate the clinical significance of epigenetic changes in neuroblastoma, we evaluated the relationship between clinicopathologic variables and the pattern of gene methylation in neuroblastoma cell lines and tumors. EXPERIMENTAL DESIGN Methylation-specific PCR was used to evalu...

Despite intensive research efforts and therapeutic advances over the last few decades, the pediatric neural crest tumor, neuroblastoma, continues to be responsible for over 15% of pediatric cancer deaths. Novel therapeutic options are needed for this tumor. Recently, investigators have shown that mice with syngeneic murine gliomas treated with an engineered, neuroattenuated oncolytic herpes sim...