Ganglioneuroblastoma

Last revised by Assoc Prof Frank Gaillard on 28 Jan 2022

A ganglioneuroblastoma is a transitional tumor of the sympathetic nervous system which lies on the intermediate spectrum of disease between a well-differentiated, low-grade, ganglioneuroma and an undifferentiated, hight-grade, neuroblastoma 6.

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

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

Macroscopically, these tumors may be encapsulated.

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

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.

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

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 hemorrhage and/or necrosis 

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

Maybe be more useful in assessing intraspinal extension. 

  • T1: usually low signal intensity; hemorrhagic 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. 

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

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Cases and figures

  • Figure 1: gross pathology
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  • Case 1
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  • Case 2
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