Rare, malignant tumour arising from the embryological neural crest element of the peripheral sympathetic nervous system.
The most common extracranial solid tumour in children; the majority of patients are diagnosed by 5 years of age.
Diagnosis can usually be confirmed by urine catecholamines and imaging; however, biopsy of the primary site (or bone marrow for staging purposes) is required to establish biology (i.e., cytogenetics, pathology) and risk stratification.
Treatment depends primarily on risk classification and varies from observation alone for certain low-risk patients to intense multimodal therapy for high-risk patients.
Prognosis is excellent for patients with low-risk disease and poor for those with high-risk disease.
Relapsed or refractory (high-risk) disease is difficult to cure, and is associated with extremely low survival.
A malignant tumour arising from the embryological neural crest element of the peripheral sympathetic nervous system. It most commonly arises from the adrenal gland(s), but can form anywhere that sympathetic nervous tissue is present, including paraspinal sympathetic ganglia in the chest and abdomen.
The term neuroblastoma is commonly used to describe a spectrum of neuroblastic tumours including neuroblastomas (the most common type), ganglioneuroblastomas, and ganglioneuromas.
History and exam
- presence of risk factors
- age <5 years
- abdominal distention
- abdominal mass
- decreased appetite
- weight loss
- fussiness (infants)
- periorbital ecchymosis (panda eyes)
- subcutaneous skin nodules
- signs of spinal cord compression
- signs of Horner's syndrome
- signs of superior vena cava syndrome
- intractable secretory diarrhoea
- opsoclonus-myoclonus ataxia (OMA)
Neuroblastoma Research Program
Texas Children's Cancer Center and Center for Cell and Gene Therapy
Baylor College of Medicine Houston
JS declares that he has no competing interests.
Department of Pediatrics Section of Hematology/Oncology
Texas Children's Cancer Center
Baylor College of Medicine
JF declares that she has no competing interests.
Associate Professor and Consultant in Paediatric Oncology
University of Southampton
JCG declares that she has no competing interests.
Medical Director, Intra-Abdominal Solid Tumor Program
Children's Hospital at Montefiore
Assistant Professor, Departments of Pediatrics & Genetics
Albert Einstein College of Medicine
DW declares that he has no competing interests.
Senior Staff Specialist
Sydney Children’s Hospital
DZ declares that he has no competing interests.
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