Primary cervical neuroblastoma in infants

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Abstract
We describe three cases of cervical neuroblastoma, each occurring in an infant less than one year old, and discuss the investigation and treatment of these patients. The prognosis of patients with advanced neuroblastoma is poor. By contrast, patients with stage I and II tumours often do well and it therefore seems appropriate for otolaryngologists to be familiar with the clinical features and management of localized cervical neuroblastoma. A history of upper aerodigestive compromise is typical, and in the evaluation of this complaint, the possibility of a cervical neuroblastoma must be borne in mind. A retropharyngeal firm, smooth mass is consistent with this diagnosis and supportive evidence can be obtained from ancillary studies. A biopsy is required for tissue diagnosis and, if possible, the entire tumour and sample local nodes should be excised. This will also relieve airway and pharyngeal obstruction. Complete tumour removal at the expense of major nerves and vessels is not necessary in view of the tendency to tumour regression. We conclude that prolonged disease-free survival can be achieved in infants by the use of limited surgery, with chemotherapy in some instances, and without the use of radiation therapy. Management decisions should be made jointly by paediatric otolaryngology and paediatric oncology teams.