Background: Paragangliomas rarely involve the cauda equina region. In this
location, these tumors can show misleading morphological features such as t
rabecular or papillary growth patterns and variable expression of cytokerat
ins. Methods: We comparatively studied the immunohistochemical and ultrastr
uctural patterns of 3 paragangliomas of the cauda equina (PCE) and of 8 par
agangliomas from other sites. Results: All the paragangliomas expressed neu
roendocrine markers (neuron-specific enolase, chromogranin A, synaptophysin
and neurofilament protein). In PCE, chief cells exhibited a strong positiv
ity with a broad spectrum anti-cytokeratin antibody. The staining was diffu
se in the cytoplasm or had a paranuclear dot-like disposition. In other sit
es, only one paraganglioma showed a focal expression of cytokeratins. At ul
trastructural level, chief cells of PCE contained characteristic dense core
granules and intermediate-sized filaments sometimes grouped in paranuclear
whorls. Conclusion: The dual immunophenotype of PCE, paraganglionic and ep
ithelial, must be recognized in order to distinguish these tumors from, for
example, a metastasis of a neuroendocrine carcinoma. PCE an slow-growing t
umors and have mostly a favorable prognosis after complete surgical excisio
n.