Paraspinal ganglioneuroma

Paraspinal ganglioneuromas are extremely rare. Like neuroblastomas and ganglioneuroblastomas, ganglioneuromas are derived from the primordial neural crest cells that form the sympathetic nervous system.

Please refer to ganglioneuroma for a more general discussion including epidemiology, clinical presentation, and treatment and prognosis.

Pathology

Paraspinal ganglioneuromas most commonly arise in the paravertebral sympathetic chains of the posterior mediastinum (41.5%) or retroperitoneum (37.5%). Less common sites include the adrenal gland (21%) and neck (8%) ³. Paravertebral ganglioneuromas frequently extend through the neural foramina to involve the epidural space of the spinal canal ¹¹. Intradural extramedullary ganglioneuromas have been reported but are extremely rare ¹⁰.

Radiographic features

Plain radiograph

• may show a posterior mediastinal mass, sometimes causing rib spreading and foraminal erosion
• a retroperitoneal mass may be identified
• scoliosis may be present

CT

• homogeneous low attenuation mass
• calcifications are seen in 42-60% ³
  • calcification is typically fine and speckled but may be coarse
• slight to moderate enhancement which may be heterogeneous or homogeneous

MRI

Ganglioneuromas are well-circumscribed masses. They may appear encapsulated, although a true capsule is infrequent ³. Signal characteristics include:

• T1: low signal intensity
  • may have a whorled appearance ¹
• T2: heterogenous high signal intensity
• T1 C+ (Gd): enhancement varies from mild to marked

Differential diagnosis

• spinal neuroblastoma and ganglioneuroblastoma
  • often cannot be differentiated from ganglioneuroma based on imaging characteristics
  • metastases usually indicate neuroblastoma or ganglioneuroblastoma
  • compared with ganglioneuromas, tumor calcification in neuroblastomas is more often amorphous and of a rough pattern
• spinal schwannoma and neurofibroma
  • centered on neural foramen
  • primarily seen in middle-aged adults