CNS & pituitary tumors
Gliomas, glioneuronal tumors, and neuronal tumors
Neuronal and mixed neuronal-glial tumors
Paraganglioma
Author: Eman Abdelzaher, M.D., Ph.D.
Last author update: 1 February 2014
Last staff update: 9 August 2023
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Paraganglioma CNS
Table of Contents
Definition / general | Sites | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Cytology description | Immunohistochemistry & special stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Abdelzaher E. Paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorparaganglioma.html. Accessed September 30th, 2023.
Definition / general
- Low grade neuroendocrine neoplasm arising in paraganglionic tissue
- Corresponds to WHO grade 1
- Preferred diagnosis is now cauda equina neuroendocrine tumor
- 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
- 48 year old woman with paraganglioma of cauda equina (Neurol India 2009;57:833)
- 49 year old man with paraganglioma of filum terminale (World J Surg Oncol 2009;7:95)
- Paraganglioma of the cauda equina region - 3 cases (Surg Neurol Int 2011;2:96)
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
Images hosted on other servers:
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
Cytology description
- Uniform cells with salt and pepper nuclei
- No fibrillar background
Immunohistochemistry & special stains
- Reticulin stain accentuates the nesting pattern
- Chief cells: chromogranin A+, synaptophysin+, variable S100 and cytokeratin
- Sustentacular cells: S100+, often GFAP+ (Brain Pathol 2005;15:169)
Electron microscopy description
- Chief cells: dense core (neurosecretory) granules
Differential diagnosis
- Jugulotympanic paraganglioma:
- Meningioma: whorls, psammoma bodies, EMA+
- Metastatic carcinoma: anaplasia, more bone destruction, EMA+
- Spinal paraganglioma:
- Ependymoma: diffusely GFAP+
- Myxopapillary ependymoma: pseudopapillae, perivascular collars of mucin, GFAP+
- Schwannoma: biphasic Antoni A and B areas, chromogranin A and synaptophysin negative
