Pseudoglandular schwannoma of the cauda equina

Abstract

The authors present a case of pseudoglandular schwannoma with immunohistochemical findings consistent with epithelial metaplasia. Pseudoglandular schwannoma is a rare morphological variant of benign schwannoma characterized by the presence of glandlike structures lined with Schwann cells. To the best of the authors' knowledge, this is only the fifth case of pseudoglandular schwannoma reported in the literature. Clinical, imaging, and pathological findings are described. The pathological findings were consistent with a pseudoglandular schwannoma composed of typical Schwann cells arranged in an Antoni B pattern, with numerous large pseudocystic spaces. Serial immunohistochemical studies of tissue sections revealed that the cells lining the pseudoglandular spaces were not only diffusely reactive for S100 protein, but also demonstrated focal positivity for epithelial membrane antigen and cytokeratins AE and AE3. The particular immunohistochemical features of incompletely differentiated Schwann cells in the present case give support to the metaplastic theory of the origin of glandlike structures in benign peripheral nerve sheath tumors.

Schwannomas and Their Pathogenesis

“…The significance of inflammation in most schwannomas is unclear, but in vestibular schwannomas, the presence of CD163‐positive macrophages has been correlated with vascular density and tumor growth . Schwannomas may show areas with an epithelioid morphology (Figure H), and some subcutaneous examples are predominantly epithelioid , but true epithelial metaplasia is rare . A rare variant of schwannoma contains areas of neuroblastoma‐like rosettes, but this should not be confused with malignant neuroectodermal tumors .…”

A rare case of pseudoglandular schwannoma

“…Cells that form pseudoglandular structures undergo "epithelial metaplasia". In the case report by Ruggeri [5], the findings of focal epithelial membrane (EMA) and cytokeratin positivity in pseudoglandular areas support this theory. It also been reported that pseudoglandular schwannoma may progress to glandular schwannoma [6,7].…”