Figure 1
Chest X-ray
Bulky homogeneous well-defined opacity in the right posterior mediastinum.
ECR 2002 COD #13: Thoracic ganglioneuroma
SectionPaediatric radiology
Case TypeClinical Cases
AuthorsLuisa Lobo, MD, Jose Fonseca-Santos, MD
Connected authors
4 years, male
Clinical History
Impaired breath sounds in the lower third of the right hemithorax were detected on routine examination in this patient, who had a history of asthma, but was currently asymptomatic. Laboratory data were normal.
Imaging Findings
Impaired breath sounds in the lower third of the right hemithorax were detected on routine examination in this patient, who had a history of asthma, but was currently asymptomatic. Laboratory data were normal.
On chest X-ray a bulky rounded well-defined homogeneous opacity was seen in the posterior mediastinum, with no calcifications within it. Chest ultrasound showed the round solid mass in the right hemithorax to be hypoechoic and homogeneous, well defined, and measuring 8cm in diameter. On chest CT (without IV contrast, because of the patient's asthmatic status) the mass was homogeneous with low attenuation and without calcifications. T1-weighted MR imaging showed the mass to have low-signal intensity. The borders were well defined and no spinal abnormalities were detected.
Discussion
Most posterior mediastinal masses in children are neurogenic tumours arising from ganglion cells of the sympathetic chain, the so-called "neuroblastoma-ganglioneuroma complex", with ganglioneuroma representing the benign mature form of the spectrum (2,4,5).
Other posterior mediastinal lesions include neurofibroma, schwannoma, lymphoma, teratoma, neuroenteric cysts, lateral meningocele, Bochdalek hernia, paraspinal extension of inflammatory spinal disease, and extramedullary haematopoesis (4,5).
Ganglioneuroma is a rare benign encapsulated tumour arising from mature ganglion cells of the sympathetic chain, typically occurring in older children and adolescents, in contrast with malignant neuroblastoma, which occurs most frequently in young children (1,2,4). Patients are usually asymptomatic as the tumour grows slowly and is often endocrinally inactive (3). The development of symptoms depends on the location and size of the tumour. Most frequently ganglioneuromas occur in the posterior mediastinum, followed by the retroperitoneum and the neck (1).
Ganglioneuromas have an oval or crescent shape, with sharply delineated borders, usually with little mass-effect relative to the size of the tumour, which can be an important imaging feature in differentiating from other more malignant forms (1).
Chest X-ray allows detection of most mediastinal masses. On ultrasound ganglioneuromas are usually homogeneous and hypoechoic; on pre-contrast CT they are hypodense and on post-contrast CT they show a moderate inhomogeneous enhancement. About 20% exhibit calcifications, usually discrete and punctate rather than amorphous and coarse as in neuroblastomas (1,2).
On MRI, ganglioneuromas are hypointense on T1-weighted images and hyperintense on T2-weighted images (1,2). Intraspinal extension can occur, and MR is the preferred imaging modality for assessing spinal involvement (1,3,4).
Ganglioneuroma is surgically treated and the prognosis is excellent.
Differential Diagnosis List
Ganglioneuroma
Final Diagnosis
Ganglioneuroma
References
[1]
Ichikawa T, Ohtomo K, Araki T, Fujimoto H, Nemoto K, Nanbu A, Onoue M, Aoki K. Ganglioneuroma: computed tomography and magnetic resonance features.
Br J Radiol 1996 Feb;69(818):114-21. (PMID: 8785637)
[2]
Lee JY, Lee KS, Han J, Yoon HK, Kim TS, Han BK, Kim J, Shim YM.
Spectrum of neurogenic tumors in the thorax: CT and pathologic findings.
J Comput Assist Tomogr 1999 Mar;23(3):399-406. (PMID: 10348446)
[3]
Lucas K, Gula MJ, Knisely AS, Virgi MA, Wollman M, Blatt J. Catecholamine metabolites in ganglioneuromas.
Med Pediatr Oncol 1994;22:240-3. (PMID: 8107654)
[4]
Merten DF.
Diagnostic imaging of mediastinal masses in children.
AJR 1992 Apr;158(4):825-32. (PMID: 1546601)
[5]
Swischuk LE.
Respiratory system.
In Swischuk LE (ed) Imaging of the newborn, infant and young child.
Williams and Wilkins, Baltimore, pp 143-4 (1997).
Case information
| URL: | https://www.eurorad.org/case/1395 |
| DOI: | 10.1594/EURORAD/CASE.1395 |
| ISSN: | 1563-4086 |
Chest X-ray
Bulky homogeneous well-defined opacity in the right posterior mediastinum.
