Management Of Cervicomediastinal Neuroblastoma Presenting With Life-threatening Tracheal Obstruction In Infancy.
Komuro, H., & Hoshino, N. (2013). Management of cervicomediastinal neuroblastoma presenting with life-threatening tracheal obstruction in infancy. Journal of pediatric hematology/oncology, 35(8), e323–e325. https://doi.org/10.1097/MPH.0b013e31827b4747
View Original Source →Abstract
The authors report on 3 infants below 6 months of age at diagnosis, with cervicomediastinal neuroblastoma who presented with life-threatening tracheal obstruction as an oncologic emergency. These neuroblastomas were characterized by favorable biology and chemoresistance. All initially grew rapidly before spontaneously regressing. Nerve injuries occurred in all patients as a result of tumor location. Maintenance of the airway until the expected spontaneous regression was a critical component in the management of these patients.
Case Details
Disease Location
Left cervicomediastinal tumor
Personal Characteristics
1 day old infant with respiratory distress
Clinical Characteristics
Diagnosed at 1 day of age, intubated for respiratory failure. Found to have mets to skin, liver, lymph nodes and bm. Favorable histology on biopsy, increased u vma, hva. Initially not given any chemo but due to massive liver mets, a single low dose radiation treatment (1.5 gy) to the liver effectively suppressed the growth of the liver metastasis. Low-dose radiation to the growing local tumor was applied in 3 fractions of 1.5 gy over 41 to 60 days of age with no further effect. The tumor started to regress spontaneously at 90 days of age and the airway compression disappeared by 100 days. The patient was extubated soon after plication of the left hemidiaphragm at 135 days of age. The urinary excretion of vma and hva normalized at 320 days of age and the patient was doing well with no evidence of tumor recurrence.
Remission Characteristics
Initially not given any chemo but due to massive liver mets, a single low dose radiation treatment (1.5 gy) to the liver effectively suppressed the growth of the liver metastasis. Low-dose radiation to the growing local tumor was applied in 3 fractions of 1.5 gy over 41 to 60 days of age with no further effect. The tumor started to regress spontaneously at 90 days of age and the airway compression disappeared by 100 days. The patient was extubated soon after plication of the left hemidiaphragm at 135 days of age. The urinary excretion of vma and hva normalized at 320 days of age and the patient was doing well with no evidence of tumor recurrence.
Treatment & Mechanisms
Clinical Treatment
Low dose radiation