Chordoid Meningioma: A Clinical, Radiographic, and Pathologic Review of Seven Cases

Chordoid meningiomas are a rare subtype of meningiomas first described in young patients with Castleman’s syndrome. Clinically, chordoid meningiomas are classified as WHO grade II tumors because of their aggressive behavior and propensity for recurrence. Histologically, these tumors share features with chordomas. We have identified seven patients who underwent resection of chordoid meningiomas at UTSW from 2002 to 2011. All of the tumors in our series were supratentorial. No patients were found to harbor systemic manifestations consistent with Castleman’s syndrome. There were 6 female patients and 1 male patient. The MIB index varied from 0.5% to 12%. Clinical and radiographic follow-up ranged from 3 months to 9 years (mean, 3.9 years). We have found no evidence of recurrence in tumors with gross total resection, including resection of the involved dura. Our experience suggests that chordoid meningiomas are more common in women and are not always associated with Castleman’s syndrome. In addition, this variant of meningiomas is not confined to young adults. As with all meningiomas, the goal of surgery should be gross total resection of the lesion with the involved dura to avoid recurrence.