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Management Of Cervicomediastinal Neuroblastoma Presenting With Life-threatening Tracheal Obstruction In Infancy.

Komuro & Hoshino, 2013Neuroblastoma

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

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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

6- months-old with cyanosis, respiratory distress and horner's triad.

Clinical Characteristics

Intubated for respiratory distress, CT confirmed mass and also had bone marrow mets. Biopsy: favorable histology - hyperdiploid, no myc-n, high trka expression, intermediate mki. 3 cycles of chemo given despite which tumor continued to grow, increased u. Vma, hva. Emergency subtotal resection done at 8 months, post op phrenic n palsy. No chemotherapy post op, tumor regressed and calcified at 21 month although there was progression of ln mets

Remission Characteristics

Cycles of chemo given despite which tumor continued to grow, increased u. Vma, hva. Emergency subtotal resection done at 8 months, post op phrenic n palsy. No chemotherapy post op, tumor regressed and calcified at 21 month although there was progression of ln mets.

Treatment & Mechanisms

Clinical Treatment

Chemotherapy, emergent subtotal resection