Jason King, MD
Fellow, Emergency Medicine & Clinical Instructor
Wayne State University
Other Contributors
Helene Tigchelaar, MD Professor, Pediatrics Wayne State University
The Case
A 4 year old male with history of asthma, eczema, and multiple food allergies presents to the Emergency Dept with a cough, congestion, and difficulty in breathing. He has had 5 – 6 episodes of watery, nonbloody, light green emesis. His appetite and activity has been decreased today. He has had no fever, rash, pain, diarrhea, or constipation. The mother says the patient was fine the day prior.
Physical Exam
HR 120 BP 96/47 RR 32 T 36.8 C Sat 96% on RA
General Mild to Moderate Respiratory distress with tachypnea
Respiratory Expiratory wheeze bilaterally
Cardiac Mild tachycardia, Regular rate and Rhythm, no murmur
The remainder of the Physical exam, including neurologic exam was normal. The patient was then treated with 3 inhaled albuterol + ipratropium nebs and oral prednisolone in the Emergency Dept and chest and abdominal x-rays were ordered because his respiratory status didn’t change significantly.
What is the diagnosis?
A. Bronchial Foreign Body
B. Vascular Ring
C. Community Acquired Pneumonia
D. Paraspinal Mass
E. Congenital Heart Disease
D. Paraspinal Mass
The chest X-Ray shows a paraspinal mass. He actually just had an asthma exacerbation too – the mass was an incidental finding. It is seen best on the lateral view as the crescentic opacity anterior to the vertebrae.
Annotated lateral Chest X-Ray – Courtesy Jason King
With this incidental finding of a paraspinal mass on x-ray, a Thoracic CT and then MRI were done, with the likely diagnosis of neuroblastoma, ganglioneuroblastoma, or ganglioneuroma. This led to a full oncologic work up, including MIBG, lab work with HVA/VMA, and bone marrow biopsy. A thorascopic excision of the paraspinal mass was successfully done, leaving a chest tube in place. Pathology revealed that the mass was a ganglioneuroblastoma. MIBG, HVA/VMA, and bone marrow biopsy were all negative and patient has not been started on chemotherapy.
Ganglioneuroblastoma is part of the spectrum of neuroblastictumors, including neuroblastomas, ganglioneuromas, and ganglioneuroblastomas. They are differentiated by the proportions of neuroblasts and Schwannian cells and have an intermediate malignant risk. Treatment varies and may include surgery, radiation, and/or chemotherapy. With low risk tumors, surgery and observation is the main treatment. Relapses are typically salvaged with surgery and chemotherapy.
This case highlights the importance of looking at the entire chest X-Ray and using a systematic approach. Other examples of “missed” findings on chest X-Rays include broken ribs in non-accidental trauma, pneumomediastinum and small pneumothoracies, foreign bodies and more.
References
Brodeur GM, Pritchard J, Berthold F, et al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J Clin Oncol 1993; 11:1466.
Perez CA, Matthay KK, Atkinson JB, et al. Biologic variables in the outcome of stages I and II neuroblastoma treated with surgery as primary therapy: a children’s cancer group study. J Clin Oncol 2000; 18:18.
Shimada H, Ambros IM, Dehner LP, et al. The International Neuroblastoma Pathology Classification (the Shimada system). Cancer 1999; 86:364.
