"Chordoid" Meningioma with Systemic Manifestations of Castleman Syndrome: A case report

ABSTRACT

Chordoid meningioma is a recently established meningeal tumor and is characterized by a chordoma like histologic appearance, peritumoral lymphoplasma cell infiltrates causing systemic manifestations similar to Castleman syndrome and having a good prognosis. We experienced a case of chordoid meningioma in a 25 year-old woman. The patient preoperatively manifested iron-resistant hypochromic microcytic anemia, polyclonal gammopathy with beta-gamma bridging and detected a huge mass in the right temporo-parietal convexity of the brain. Microscopically, the mass was composed of nests and cords of cuboid, partly vacuolated cells in a mucoid matrix, simulating chordoma. The tumor was surrounded by masses of lymphoplasma cells around vessels, many of the plasma cells contained Russell bodies. Ultrastructural findings showed intranuclear cytoplasmic invaginations, microvilli protruding from cytoplasmic surfaces and well formed desmosomes. Some portions of tumor cell surface were covered by stretches of basal lamina.