BACKGROUND: Paragangliomas rarely involve the cauda equina region. In this location, these tumors can show misleading morphological features such as trabecular or papillary growth patterns and variable expression of cytokeratins. METHODS: We comparatively studied the immunohistochemical and ultrastructural patterns of 3 paragangliomas of the cauda equina (PCE) and of 8 paragangliomas from other sites. RESULTS: All the paragangliomas expressed neuroendocrine markers (neuron-specific enolase, chromogranin A, synaptophysin and neurofilament protein). In PCE, chief cells exhibited a strong positivity with a broad spectrum anti-cytokeratin antibody. The staining was diffuse in the cytoplasm or had a paranuclear dot-like disposition. In other sites, only one paraganglioma showed a focal expression of cytokeratins. At ultrastructural 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 epithelial, must be recognized in order to distinguish these tumors from, for example, a metastasis of a neuroendocrine carcinoma. PCE are slow-growing tumors and have mostly a favorable prognosis after complete surgical excision.
BACKGROUND:Paragangliomas rarely involve the cauda equina region. In this location, these tumors can show misleading morphological features such as trabecular or papillary growth patterns and variable expression of cytokeratins. METHODS: We comparatively studied the immunohistochemical and ultrastructural patterns of 3 paragangliomas of the cauda equina (PCE) and of 8 paragangliomas from other sites. RESULTS: All the paragangliomas expressed neuroendocrine markers (neuron-specific enolase, chromogranin A, synaptophysin and neurofilament protein). In PCE, chief cells exhibited a strong positivity with a broad spectrum anti-cytokeratin antibody. The staining was diffuse in the cytoplasm or had a paranuclear dot-like disposition. In other sites, only one paraganglioma showed a focal expression of cytokeratins. At ultrastructural 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 epithelial, must be recognized in order to distinguish these tumors from, for example, a metastasis of a neuroendocrine carcinoma. PCE are slow-growing tumors and have mostly a favorable prognosis after complete surgical excision.
Authors: Leonille Schweizer; Felix Thierfelder; Christian Thomas; Patrick Soschinski; Abigail Suwala; Damian Stichel; Annika K Wefers; Lars Wessels; Martin Misch; Hee-Yeong Kim; Ruben Jödicke; Daniel Teichmann; David Kaul; Johannes Kahn; Michael Bockmayr; Martin Hasselblatt; Alexander Younsi; Andreas Unterberg; Bettina Knie; Jan Walter; Diaa Al Safatli; Sven-Axel May; Andreas Jödicke; Georgios Ntoulias; Dag Moskopp; Peter Vajkoczy; Frank L Heppner; David Capper; Wolfgang Hartmann; Christian Hartmann; Andreas von Deimling; David E Reuss; Anne Schöler; Arend Koch Journal: Acta Neuropathol Date: 2020-09-14 Impact factor: 17.088