Paraganglioma

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Paraganglioma
ParagangliomaMRI.jpg
Sagittal MRI of cauda equina paraganglioma
Tumor Class Chemodectoma
Cell of origin Neural Crest
WHO Grade I
Survival Curable with surgery
Treatment Surgical resection
Histologic Features
Radiographic Features

Contents

Definition

Paraganglioma is a neuroendocrine neoplasm, usually encapsulated and benign, arising in specialized neural crest cells associated with autonomic ganglia. In the CNS, paragangliomas are almost exclusively located in the cauda equina. Paraganglioma corresponds to WHO Grade I.

Epidemiology

Cauda equina paragangliomas generally occur in adults. The mean age at presentation is 47 years, with a slight male predominance (1.4:1).

Localization

In the CNS, paragangliomas are almost exclusively located in the cauda equina..

Clinical presentation

Patients with cauda equina paragangliomas present with low back pain and signs of cauda equina compression.

Imaging

MRI demonstrates as isointense, homogeneously enhancing mass lesion intimately associated with the cauda equina.

Macroscopy

Cauda equina paragangliomas are intradural lesions attached to the filum terminale or to a caudal nerve root. These lesions may occasionally penetrate dura and invade bone.

Histopathology

Cauda equina paragangliomas is a well-differentiated tumor resembling paraganglia, composed of chief (type I) cells arranged in nests or lobules (zellballen) and surrounded by a single layer of sustenacular (type II) cells. Type I cells stain for neuron-specific enolase, synaptophysin and chromogranin. Type II cells are uniformly S-100 positive.
Photomicrograph of paraganglioma

Molecular genetics

The molecular genetics of cauda equina paragangliomas have not been elucidated.

Prognosis

The majority of cauda equina paragangliomas are slow-growing, benign tumors that may be cured by surgical resection. It is estimated, however, that 4% of these lesions will recur following gross total resection. CSF seeding has been documented.

  • i. Paragangliomas can be distinguished from ependymoma (which can both occur in the fillum terminale) based on their immunoreactivity for chromogranin and synaptophysin and the arrangment of cells in nests or cords surrounded by reticulin
  • ii. Histologically see nests of small, uniform cells are surrounded by a vascular stroma; cells within the nests are immunoreactive for chromogranin-A; S-100 antibodies immunolabel the cells at the periphery of the nests
  • iii. Occur most often in either the temporal bone (CN 9 or 10) or carotid body (present as painless mass in the jaw under the sternocleidomastoid)
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