CASE 10822 Published on 10.04.2013

Ganglioneuroblastoma in a 6-year-old


Chest imaging

Case Type

Clinical Cases


Rossi P, Raffo L, Sarti E, Aringhieri G, Vitali S, Giusti S, Caramella D, Bartolozzi C.

Ospedale Santa Chiara,
Via Roma 67 56125 Pisa, Italy;

6 years, male

Area of Interest Nuclear medicine, Lung, Spine, Thoracic wall, Head and neck, Musculoskeletal spine, Mediastinum, Oncology, Paediatric ; Imaging Technique SPECT, Ultrasound, Ultrasound-Colour Doppler, CT, MR, MR-Diffusion/Perfusion, Conventional radiography
Clinical History
A 6-year-old boy came to our department after the sudden development of a hard and slightly mobile swollen area below the right clavicle. The child was asymptomatic.
Imaging Findings
An ultrasound demonstrated the presence of a solid, inhomogeneous swollen area with increased vascularisation, localised in the right subclavicular fossa (fig. 1).
CT scan showed (fig. 2):
-a right apical, paravertebral rounded mass with well-defined margins, parenchymal density with calcifications inside, with neither infiltration of nearby structures nor bone erosion;
-a connection between the tumour and the intervertebral foramina (IF), that was confirmed in a subsequent MRI (fig. 3): tumour pedicle was extending through C7-T1 IF, with no evidence of bone marrow oedema.
The tumour at MRI displaced the parietal pleura, extending between it and the chest wall, had a cerebroid aspect and showed inhomogeneous contrast enhancement.
Patient underwent surgery to remove the tumour and histological analysis of the surgical specimen revealed the presence of a ganglioneuroblastoma (areas of ganglioneuroma mixed with undifferentiated neuroblastoma areas).
After surgery the patient began chemotherapy with cyclophosphamide-adriamycin.
I123 scintigraphy (fig. 4) showed no metastasis.
Two-month follow-up chest radiograph demonstrated no evidence of recurrence (fig. 5).
Neuroblastic tumours (NTs) are the second most common extracranial malignant tumours of childhood and the most common solid tumours of infancy [1]. NTs (ganglioneuroma, ganglioneuroblastoma and neuroblastoma) represent 80% of the tumours under 5 years of age. They are rare above 10 years old, median age at diagnosis is 22 months, affecting mostly Caucasian boys, most NT are sporadic; few are autosomal dominant and in this case, NT may affect both adrenal glands and have mulitple locations [2]. NTs originate from embryonic cells involved in the development of the sympathetic nervous system due to a block in their differentiation process.
NT mainly affect adrenal glands (40%), but may develop anywhere there is sympathetic tissue (abdomen, mediastinum, neck).
NTs differ from each other depending on the cellular maturation and the stroma amount [3]. Neuroblastoma (NB) is the most serious but also the most common type of NTs.
Ganglioneuroma is mainly asymptomatic and the only symptoms are those related to compression. NB creates a mass, symptoms are absent or non-specific (fever, fatigue, weight loss). Diagnosis is difficult until metastatic manifestations appear, such as pain, respiratory symptoms or gastrointestinal disorders.
NB metastasises through both lymphatic and blood vessels, may metastasise everywhere, especially to the liver. In the newborn multiple locations to the skin may give a bluish coloration "blueberry muffin baby".
Imaging findings may help diagnosis, especially concerning the location of the mass, describing the magnitude and relationships, identifying metastases: US is a first level examination, MRI is better to determine spinal tumours, CT for staging, nuclear medicine for recurrences. The 90% of NB produces catecholamines and their presence in the urine may direct the diagnosis, which becomes certain after histological examination. NB consists of poorly defined small cells with dark nuclei, less cytoplasm and solid growth; immunohistochemical examination is positive for NSE, CD56, synaptophysin. Ganglioneuroblastoma consists of ganglion cells with abundant eosinophilic cytoplasm, nucleoli and "vesicular" nuclei, mixed with undifferentiated neuroblasts. To establish the diagnosis of a ganglioneuroblastoma the presence of schwannian stroma, consisting of Schwann cells, fibroblasts, neurites is required [4, 5].
NB course is variable, depending on clinical, histopathological, molecular factors. Age and stage are the most important factors, the younger the child the better the prognosis. N-MYC gene amplification and deletion of 1p are other factors of an aggressive disease [6]. NB represents 15% of all deaths from cancer in children, treatment of choice is surgery and chemotherapy (cisplatin, etoposide, doxorubicin); new treatment with stem cell transplant is being studied [7].
Differential Diagnosis List
Primary ganglioneuroblastoma in the right subclavicular fossa
Extracranial meningioma
Final Diagnosis
Primary ganglioneuroblastoma in the right subclavicular fossa
Case information
DOI: 10.1594/EURORAD/CASE.10822
ISSN: 1563-4086