Spinal paraganglioma

Spinal paragangliomas are tumours of neuroendocrine origin that rarely involve the spine (usually the filum terminale and cauda equina). Paragangliomas overall are most commonly located within the adrenal gland (pheochromocytomas) or in the head and neck. 

For a general discussion of paragangliomas please refer to parent article: paraganglioma

The age range at presentation for spinal paragangliomas varies from 13-70 years. The mean age at presentation is 47 years. Males are more commonly affected than females.

Presentation is either with local mass effects of neuroendocrine symptoms. 

The most common presenting symptoms are lower back pain and sciatica form mass effect.

Cauda equina paragangliomas frequently actively secrete neuropeptides, particularly 5-hydroxytryptamine and somatostatin, although symptoms related to this chemical production are usually absent. 

Additionally these lesions may be a rare source of superficial siderosis, and thus present with sensory neural impairment, cranial nerve dysfunction and myelopathy 4.

All paragangliomas consist of nests (zellballen) of chief cells. They are highly vascular WHO grade I neoplasms. The majority (75%) of paragangliomas are encapsulated.

Plain film

Plain films are unlikely to demonstrate any abnormality, although vertebral scalloping or rarely calcification may be seen 4

Angiography

Conventional angiography demonstrates an intense early blush that persists into the late arterial and early venous phases.

CT

Spinal paragangliomas appear as soft tissue masses inferior to the conus. If contrast is administered they enhance vividly (as do paragangliomas elsewhere). 

Large lesions may rarely demonstrate osseous erosion or remodelling of the adjacent vertebrae (vertebral scalloping). Rarely these lesions calcify 4

MRI

Similarly, on MRI these masses usually appear as well-circumscribed masses, inferior to the conus. 

With some lesions, the characteristic “salt-and-pepper” appearance of neck and skull base paragangliomas may also be seen. Associated syringohydromyelia has been reported in some cases.

Reported signal characteristics include:

  • T1: isointense
  • T2
    • hyperintense
    • flow voids are typically seen along the surface of and within the tumour nodule
    • haemorrhage is common, leading to a ‘cap sign
  • T1 C+ (Gd): intense enhancement is virtually always seen

Surgical resection is the treatment of choice, sometimes with preoperative embolization to reduce intra-operative blood loss. 

Post-resection recurrence rate is less than 5%.

The differential diagnosis is essentially that of other intradural extramedullary tumours, particularly those located in the lumbar region. 

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Article Information

rID: 19243
Tag: spine
Synonyms or Alternate Spellings:
  • Paraganglioma of the filum terminale
  • Spinal paragangliomas
  • Paraganglioma of spine
  • Paraganglioma of the spine

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