CNS tumor
Neuronal and mixed neuronal - glial tumors
Paraganglioma

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 31 August 2017, last major update February 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Paraganglioma CNS

Cite this page: Abdelzaher, E. Paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorparaganglioma.html. Accessed October 23rd, 2017.
Definition / general
  • Low grade neuroendocrine neoplasm arising in paraganglionic tissue
  • Corresponds to WHO grade I
  • Terminology is generally based on anatomic site: e.g. carotid body paraganglioma, jugulotympanic paraganglioma, spinal paraganglioma
  • "Pheochromocytoma" is used exclusively for histologically identical tumors arising from the adrenal medulla
  • Incidence:
    • Spinal paraganglioma: 3.5% of tumors in the cauda equina / filum terminale region
    • Jugulotympanic paraganglioma: #2 most common tumor of the temporal bone
  • Adults (4th - 6th decades)
  • Spinal paraganglioma: slightly > in males
  • Jugulotympanic paraganglioma: strong female predilection
Sites
  • Spinal:
    • Intradural tumors in cauda equina / filum terminale region
    • Rarely involves other spinal levels
  • Intracranial:
    • Usually extension from jugulotympanic paraganglioma
    • Rarely, purely intracranial; e.g. sellar / suprasellar, posterior fossa
Clinical features
  • Spinal paraganglioma: low back pain, sciatica
  • Jugulotympanic paraganglioma with intracranial extension: tinnitus, hearing loss
Radiology description
  • Spinal paraganglioma:
    • MRI: discrete avidly enhancing "sausage shaped" mass
    • CT: may show scalloping of the vertebral bodies
  • Jugulotympanic paraganglioma:
    • MRI: salt and pepper pattern on T2
    • CT: a typical "moth eaten" erosion of temporal bone
Radiology images

Images hosted on other servers:

MRI

Irregular mass at the jugular foramen

Sagittal T1 weighted image with gadolinium

Enhancing well defined lesion at L4-L5

Sagittal T2 and T1 weighted images

Prognostic factors
  • The vast majority of spinal paraganglioma and jugulotympanic paraganglioma are clinically benign
  • Prognosis depends on tumor location:
    • Spinal paraganglioma: excellent prognosis, 4% local recurrence following excision, rarely metastasizes (J Neurosurg 1991;75:320)
    • Jugulotympanic paraganglioma: 50% local recurrence, 5% metastasis
Case reports
Treatment
  • Therapeutic options include surgery, radiation (conventional or radiosurgery) and vascular embolization
  • Spinal paraganglioma:
    • Most can be resected in toto
    • Surgical excision is curative
  • Jugulotympanic paraganglioma:
    • Less often resected intact
    • More therapeutically challenging because of difficult anatomy, tumoral hypervascularity and advanced stage at diagnosis
Clinical images

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Cauda equina paragangliomas

Lesion, after durotomy

Gross description
  • Spinal paraganglioma:
    • Encapsulated oval mass with typically prominent superficial blood vessels
    • Soft with typical red brown cut surface
    • Attached to filum terminal or less likely nerve root
    • Variable cystic change
  • Jugulotympanic paraganglioma:
    • Usually not received intact
    • Soft with typical red brown cut surface
Gross images

Images hosted on other servers:

Lesion after the en block removal

Microscopic (histologic) description
  • Organoid pattern:
    • Zellballen: nests of chief cells surrounded by bipolar sustentacular cells with delicate intervening capillary network
  • Chief cells have round to oval nuclei with salt and pepper chromatin pattern and no / mild endocrine atypia
  • Sustentacular cells are spindled and inconspicuous (visible by IHC)
  • Low level mitotic activity and hemorrhagic necrosis can be seen but have no prognostic impact
  • Variable fibrosis
  • 50% mature ganglion cells (gangliocytic paraganglioma)
  • Less common features:
    • Carcinoid-like architecture
    • Melanotic paraganglioma
    • Oncocytic paraganglioma
Microscopic (histologic) images

Images hosted on other servers:

H&E, synaptophysin

Various images

Chief cells are chromogranin+

Jugulotympanic paraganglioma: S100+

S100+

Paraganglioma of lumbar cord: synaptophysin+

Cytology description
  • Uniform cells with salt and pepper nuclei
  • No fibrillar background
Immunohistochemistry
Electron microscopy description
  • Chief cells: dense core (neurosecretory) granules
Differential diagnosis