Cystic ganglioneuroblastoma – incidental finding in a child

Clinical History

A 9-year-old child was admitted to the emergency department for fever and cough for 5 days. Pneumonic consolidations were not identified in chest X-rays, however, as an incidental finding, a calcified lesion was observed in the left hypochondrium, so ultrasound and then MRI was performed to better characterise it.

Imaging Findings

The chest AP X-ray showed a calcified lesion in the left hyponchondrium (Fig 1).
The ultrasound showed a left adrenal mass, predominantly hypoechoic, with some echogenic content (Fig 2).
In MRI, the lesion had an internal component that was mostly cystic, with haemorrhagic zones conditioning fluid-fluid level (Fig 3). There was also calcification of its wall, already described in previous studies.
After intravenous administration of contrast media, nodular uptake of the most caudal region of the lesion was observed (Fig 4). Axial diffusion-weighted image showed high-signal-intensity solid nodules due to restricted diffusion (Fig 5).

Discussion

Ganglioneuroblastoma is a rare tumour, originating in ganglion cells of the sympathetic nervous system. More than 90% have been observed in children under 5 years of age and are considered a variant of neuroblastoma. Histology may show a nodular or diffuse pattern, the nodular pattern having a higher incidence of metastasis. The site of more frequent origin is in the adrenal glands, as in our case, has also been observed in the extraadrenal retroperitoneum, the posterior mediastinum and less frequent in the neck [1].
Sometimes the clinical presentation is of abdominal pain or distention due to the mass effect of the tumour.
As an adrenal incidentaloma, it is difficult to establish differences from other adrenal masses. For example in CT it may have several appearances, from a solid or predominantly cystic mass to small solid nodules, in relation to the number of tumoural ganglion cells. MRI is superior especially in the detection of metastasis and the ganglioneuroblastoma appears heterogeneous, with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. On dynamic MRI, neuroblastomas and ganglioneuroblastomas have early enhancement, in contrast to ganglioneuroma [2]. However, it is difficult to reach a preoperative diagnosis and the majority are diagnosed based on the postoperative histological analysis.
However, in the case of a predominantly cystic adrenal incidentaloma, in a child or young patient, the most frequent cystic adrenal gland tumours are neuroblastoma, ganglioneuroma, ganglioneuroblastoma, pheochromocytoma, teratoma, and the most exceptional carcinoma. We must also take into account that cystic lesions of the adrenal glands can be grouped into three subgroups: with pure cystic component, parasitic cysts and cystic component with solid nodule, the latter being associated with haemorrhage and necrosis.
In general, purely cystic lesions correspond to simple cysts, pseudocysts, lymphangiomas, or haemangiomas.
Cysts with haemorrhage are associated with perinatal stress, whereas the traumatic aetiology is not accepted. Hydatid cysts in the adrenal glands are very rare, it is not a typical location [3].
The treatment is surgical and sometimes requires chemotherapy and radiation, although there is no consensus. Patients have not been followed for long periods of time and survival data are limited.

Differential Diagnosis List

Final Diagnosis

Cystic nodular ganglioneuroblastoma- stroma rich.

URL:	https://eurorad.org/case/15018
DOI:	10.1594/EURORAD/CASE.15018
ISSN:	1563-4086