Chordoid meningioma: a clinicopathologic study of 42 cases

The term chordoid meningiomas was first used by Kepes et al. in 1987 to describe a meningeal tumor in young patients associated with microcytic anemia and/of dysgammaglobulinemia. Such tumors were composed of spindle or epithelioid cells disposed in chordoma-like clusters and cords in a myxoid matrix and often featured a prominent lymphoplasmacellular infiltrate. Our study includes 42 chordoid meningiomas that represented 0.5% of all meningiomas operated at Mayo Clinic during the interval 1975 to 1997. The male to female ratio was 1:1 and the age range was 12 to 77 years (mean, 47.4 yrs). Only two (5.2%) occurred in children. The majority (88%) were large and supratentorial. No manifestation of systemic disease was noted. Chordoid elements comprised 10% to 100% of the tumors: 34 (81%) were more than 50% chordoid. Thirty-seven tumors (88%) were classified as typical and five as atypical. Lymphoplasmacytic infiltrates varied, being moderate in 10 cases (23.8%), mild in 15 (35.7%), and absent in 17 (40.5%). In 14 (42%) of the 33 cases with available follow up, one or more recurrences were noted. All but one recurrent tumor had been subtotally resected. In 86% of recurrent tumors, the primary lesion was more than 50% chordoid in pattern and contained little or no inflammatory infiltrate. In our experience, chordoid meningiomas are primarily tumors of adults, lack sex predilection, are unassociated with systemic manifestations, and uniformly recur when subtotally excised.