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Congenital Ewing Sarcoma Presenting as a Rapidly Growing Neck Mass in a Newborn
Hong De Sa1, John Pearl, Anna Maria Hibbs
1University Hospitals Rainbow Babies and Children's Hospital, Cleveland, Ohio (Drs De Sa, Hibbs, Pereda, Suzuki, Curtis, and Stearns); and Case Western Reserve University School of Medicine, Cleveland, Ohio (Mr Pearl).
Background:
Ewing sarcoma (EWS) is an aggressive soft-tissue and bone malignancy. Congenital EWS is extremely rare, and its presenting features can be unique from that of EWS occurring in older children.
Clinical Findings:
A full-term female infant with a neck mass present at birth was admitted to a level I nursery with an otherwise well appearance and normal vital signs. After consultation with a neonatologist, she was transferred to a neonatal intensive care unit where she developed sudden respiratory collapse from rapid growth of the mass causing airway obstruction, leading to emergent intubation. Ultrasound and MRI scans of the neck mass demonstrated cystic and vascular components, and a timely biopsy revealed small round blue cells with diffuse CD99 expression and chromosomal translocation 11;22.
Primary Diagnosis:
Ewing sarcoma.
Interventions:
An accelerated workup for EWS was done due to the patient's critical status. On day of life (DOL) 8, she was started on treatment of EWS as per the current standard-of-care AEWS0031. On DOL 24, she underwent tracheostomy placement.
Outcomes:
The patient completed 14 total cycles of chemotherapy and is more than 12 months old. Her tracheostomy was decannulated at 6 months of age.
Practice Recommendations:
The rarity of EWS in neonates and its presentation as a neck mass make this disease difficult to recognize unless clinicians have a high index of suspicion. The aims of this case report are to increase awareness of malignancy as a potential cause of neck masses in neonates and to prompt nurses and physicians to prepare for airway stabilization at appropriate levels of care if a neck mass is present at birth.
