DISCUSSION


MRI of the chest and spine revealed a large (8.8 × 8.1-cm) homogeneous mass within the right hemithorax that entered the right bronchus with associated right lung atelectasis. The mass also extended through the nerve root foramen on the right at the T4/T5 level and entered the spinal canal. Ultrasound-guided biopsy showed a mature ganglioneuroma. 


Ganglioneuromas are a type of neu­ro­blastic tumor that arise from primitive sympathetic ganglion cells1 that originate during fetal development. Neuroblastic tumors, almost exclusively a disease of children, have unknown etiology. Maternal and preconceptual factors thought to play a role include opiate consumption,2 folate deficiency,3 and gestational diabetes mellitus.4 


Neuroblastomas arise within the sympathetic nervous system, most commonly in the adrenal gland, abdomen, thorax, and cervical spine. Diagnosis is made through tissue biopsy, radiologic imaging, and urine catecholamines. Ganglioneuromas, composed of Schwann cells,1 occur mostly in children aged 4 to 7 years. They can metastasize but are considered benign with excellent prognosis.5 Wheezing, a common symptom, occurs in up to 25% of children younger than 5 years.6 


Important aspects of the history in­clude age of onset and the course of illness. Distinguishing between intermittent and persistent wheezing helps differentiate asthma, foreign body aspiration, congenital or structural abnormalities, and bronchial constriction caused by a growing mass or lymph nodes. Certain clinical clues suggest a diagnosis other than asthma or foreign body. 


Physical examination must consist of digital inspection for cyanosis or clubbing, palpation, percussion, and auscultation. Percussion of the chest can help identify the position of the diaphragm and differences in resonances within lung fields. Auscultation identifies variations in air entry and any other related breath sounds. 


Significantly, this patient never re­ceived a chest radiograph prior to admission to help detect signs of cardiac failure, mediastinal masses, and vascular rings. Pulmonary function tests are important in determining the degree and location of airway obstruction and quantifying the response to bronchodilators and bronchoconstrictive stimuli. 


Treatment The patient underwent resection of the paraspinal thoracic mass. Postoperatively, residual tumor was found remaining within the spinal canal. In addition, there was a CSF leak into the thoracic cavity. The patient also exhibited severe right arm and leg weakness. She underwent laminectomy with total resection of the ganglioneuroma and repair of the CSF leak. As a result of a continuing CSF leak, the patient underwent thoracoscopy with mechanical pleurodesis. The leak of dura was closed using fibrin glue and DuraSeal sealant. 


Outcome The patient continued to improve during her hospital stay and was discharged fully ambulating and in good condition. She attends physical therapy twice a week. JAAPA


Tracey Reeve practices critical care medicine at Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. The author has indicated no relationships to disclose relating to the content of this article.


REFERENCES


1. Shimada H, Ambros IM, Dehner LP, et al. The International Neuroblastoma Pathology Classification (the Shimada system). Cancer. 1999;86(2):364-372.


2. Cook MN, Olshan AF, Guess HA, et al. Maternal medication use and neuroblastoma in offspring. Am J Epidemiol. 2004;
159(8):721-731. 


3. French AE, Grant R, Weitzman S, et al. Folic acid food fortification is associated with decline in neuroblastoma. Clin Pharmacol Ther. 2003;74(3):288-294. 


4. Chow EJ, Friedman DL, Mueller BA. Maternal and perinatal characteristics in relation to neuroblastoma. Cancer. 2007;
109(5):983-992.


5. De Bernardi B, Gambini C, Haupt R, et al. Retrospective study of childhood ganglioneuroma. J Clin Oncol. 2008;
26(10):1710-1716.


6. Fakhoury K. Approach to wheezing in children. UpToDate Web site. http://www.uptodate.com/patients/content/topic.
do?topicKey=~yUAVsd3TYJQRYr&selectedTitle=1~150&source=
search_result. Accessed July 1, 2010.


« Previous 1 2 Next »