Chordoid meningioma: a clinicopathologic study of 11 cases at a single institution

Abstract

Chordoid meningioma is an uncommon variant of meningioma, which histologically bears a great resemblance to chordoma and often follows an aggressive clinical course. We examine clinicopathologic features of 11 cases of this rare tumor to further elucidate its behavior. Thirteen specimens of chordoid meningioma belonging to 11 patients were obtained at a single institution from 1995 to 2009. Correlations of histologic parameters, immunohistochemical study, and clinical features were assessed. This series included six men and five women with a mean age of 60.8 years at first surgery. Aside from one patient (case 5) who died of disease immediately after the first operation, the mean postoperative follow-up period for the other 10 patients was 41.4 months. Two patients each had a local tumor recurrence. The mean time to recurrence was 10.4 years. No systemic manifestations of Castleman syndrome, such as iron-refractory hypochromic/microcytic anemia and dysgammaglobulinemia, were found. Six tumors (46%) were classified as benign (grade I) and seven tumors (54%) atypical (grade II), if based solely on histologic grading irrespective of chordoid or clear cell components in our cases. Lymphoplasmacytic infiltrate was moderate in one tumor (7%), mild in eight tumors (62%), and absent in four tumors (31%). The inflammatory cells were predominantly T cells (CD3+), with only scarce B cells (CD20+). There was a wide range of MIB-1 labeling indices (0.3-25.8%, mean 7.5%), which increased following tumor recurrence. Our study demonstrates that chordoid meningiomas are not always associated with Castleman's Syndrome, and that this histologic category can be seen in the elderly as opposed to only in younger age groups.

A clinicopathological study of the significance of the proportion of choroid morphology in chordoid meningioma

“…In addition, most lymphocyte infiltrates in our meningiomas consisted of T cells. This differed from the report of Kepes [4][5][6][7][8][9][10][11].4%, mean = 5.2%) in their series and also reported a high rate of recurrence (39%). 1 Lilia et al reported a recurrence rate of 70% with a relatively narrow range in the MIB-1 labeling index (6.0-9.0%, mean = 7.3%).…”

“…So far, 130 patients with these tumors have been described in the English literature, largely in the form of isolated case reports, with the exception of five series (one series published in 2010 by our hospital analyzed 11 patients from 1995 to 2009). [1][2][3]9,11 Kepes et al published the first series of CM in 1988 with an emphasis on chordoma-like histology, young age, prominent peritumoral lymphoplasmacellular infiltrates, and association with systemic manifestations of Castleman syndrome. 3 They described occurrence in young individuals, chordoma-like histological appearance, peritumoral lymphoplasmacellular infiltrates with B cell predominance, and an association with the systemic manifestations of hematological conditions, including iron-refractory hypochromic/microcytic anemia and bone marrow plasmacytosis with dysgammaglobulinemia (Castleman's syndrome).…”

Chordoid Meningioma: Differentiating a Rare World Health Organization Grade II Tumor from Other Meningioma Histologic Subtypes Using MRI

“…A little more than 100 cases of chordoid meningioma have been described in the English-language literature, the majority of which are in the pathology and neurosurgery literature. [4][5][6][7][8][9] Attempts to distinguish benign from atypical and malignant meningiomas have been undertaken with variable results, and DWI and ADC values have provided the most reliable means of differentiation, 10,11 though no data analysis specifically examining the chordoid morphologic variant has been performed. To the best of our knowledge, only 3 case reports in which the MR imaging characteristics of chordoid meningiomas were described have been published in the radiology literature.…”

Spinal chordoid meningioma in a child: A case report and review of the literature

“…CM is a rare variant of meningioma that is characterized by epithelioid cord-like tumor cells in the myxoid stroma (4,9). CM has been recognized as an aggressive tumor of meningothelial origin and is considered to be a grade II lesion, in addition to clear cell and atypical meningioma, due to a high rate of recurrence, particularly following subtotal resection.…”