Ganglioneuroblastoma

A ganglioneuroblastoma is a transitional tumour of the sympathetic nervous system which lies on the intermediate spectrum of disease between a well-differentiated, low-grade, ganglioneuroma and a an undifferentiated, hight-grade, neuroblastoma⁶.

Epidemiology

They are seen more commonly in children younger than 10 years. There is no definite gender predilection reported at the time of writingin children, but it may be more common in males when presenting (rarely) in adults ^1-6.

Pathology

The tumour containsarises from primitive neuroectodermal cells located within the adrenal glands and sympathetic ganglia ⁶. They contain elements of both malignant neuroblastoma and benign ganglioneuroma. They

Macroscopically, these tumours may be encapsulated.

Location

They are commonly attached to a nerve trunk or arise form the adrenal gland.

Radiographic features

Imaging features can be variable and can range from a solid, well-marginated, oblong paraspinous mass with homogeneous enhancement or as an irregular, cystic, poorly-marginated, locally-invasive mass with evidence of metastatic disease ².

Plain radiograph

Non-specific but may hint to an initial diagnosis in larger lesions. May show a posterior retroperitoneal, mediastinal, or neck mass.

Ultrasound

Not a typical imaging modality of choice but may show a heterogeneously echogenic lesion if able to be identified. Anechoic areas may be present corresponding to regions of haemorrhage and/or necrosis 

CT

Variable and can range from homogeneous (especially small lesions) to heterogenous (large lesions).

MRI

Maybe be more useful in assessing intraspinal extension. 

• T1: usually low signal intensity; haemorrhagic areas may manifest as areas of high signal
• T2: usually high signal intensity but heterogenous; cystic areas may be high signal.
• T1 C+: may show variable enhancement

Calcification may be difficult to detect at MR imaging; signal voids may be apparent. 

See alsoDifferential diagnosis

• neuroblastic

  The features of a ganglioneuroblastoma are, almost by definition, difficult to distinguish from ganglioneuromas and neuroblastoma as well as form other peripheral nerve sheath tumours