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Year : 2022  |  Volume : 25  |  Issue : 1  |  Page : 1-4

Chordoid Meningioma. Case Report and Review of the Literature

1 Department of Neurosurgery, Institute of Psychiatry and Neurology, Sobieskiego, Warsaw, Poland
2 Department of Neuropathology, Institute of Psychiatry and Neurology, Sobieskiego, Warsaw, Poland

Date of Submission02-Dec-2020
Date of Acceptance31-Oct-2021
Date of Web Publication19-Jan-2022

Correspondence Address:
Dr. M Prokopienko
Department of Neurosurgery, Institute of Psychiatry and Neurology, Warsaw, Poland, Sobieskiego 9 Street, 02-957 Warsaw
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_667_20

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Chordoid meningioma is an uncommon histopathological type of meningioma, frequently associated with Castleman's syndrome. Histologically, chordoid meningiomas are similar to chordomas. Because of their high proliferative index, they present aggressive biological behavior and high risk of postoperative recurrence. We report a case of chordoid meningioma in an adult patient without Castleman's syndrome manifestation. As its chordoid feature is related with a rapid recurrence after incomplete removal, meticulous histopathological examination is crucial for the adequate postoperative treatment plan.

Keywords: Castleman's disease, chordoid meningioma, mitotic index

How to cite this article:
Prokopienko M, Wierzba-Bobrowicz T, Grajkowska W, Stępień T, Sobstyl M. Chordoid Meningioma. Case Report and Review of the Literature. Niger J Clin Pract 2022;25:1-4

How to cite this URL:
Prokopienko M, Wierzba-Bobrowicz T, Grajkowska W, Stępień T, Sobstyl M. Chordoid Meningioma. Case Report and Review of the Literature. Niger J Clin Pract [serial online] 2022 [cited 2022 Dec 3];25:1-4. Available from:

   Introduction Top

Meningioma is the most common benign intracranial tumor, representing 13%–36% of all primary central nervous system neoplasms. Among meningiomas, 90% are benign.[1],[2] Starting from 2007, all meningiomas with cortical invasion became considered as grade II.[3] This increased the rate of these meningiomas from 5% to 20%–35%.[3] Among grade II group, besides atypical and clear cell meningiomas, the rare type tumors constitutes chordoid meningiomas (CMs). CMs represent approximately less than 1% of intracranial meningiomas.[4],[5],[6] The term CM was initially presented by Kepes et al.[7] who defined it as having a chordoma-like histological appearance with a clustering of tumor cells. We report a 71-year-old patient with CM in parasagittal region infiltrating the superior sagittal sinus and motor cortex of the left hemisphere.

   Case Report Top

A 71-year-old nonsmoking right-handed woman, presented with 2 months history of left leg paresis gradual progression. The patient was able to walk with the crutches. No sensory deficit was found. Cranial magnetic resonance imaging (MRI) revealed a 3 × 4 × 4 cm mass in the left occipital region with superior sagittal sinus infiltration [Figure 1]a and [Figure 1]b.
Figure 1: (a and b) MRI scans showing choroid meningioma in axial and sagittal plane before surgery. (c and d) – MRI scans showing tumor remnants in the superior sagittal sinus 1 month after surgery, before Gamma Knife therapy. (e and f) – MRI scans 6 months after surgery, recently after Gamma Knife therapy

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Two weeks after the initial presentation, she underwent left frontoparietal craniotomy. Considering the superior sagittal sinus infiltration by the tumor, advanced age of the patient, a subtotal tumor resection was undertaken. Small tumor remnants within the sinus cavity were coagulated thoroughly. The infiltration of the arachnoid by the tumor increased the cerebral cortex injury. Left lower-limb paresis progression was observed immediately after the procedure, and it subsequently regressed during the rehabilitation. The patient was able to walk with crutches 2 months after surgery. Repeat MRI carried out 1 month after surgery confirmed tumor remnants within the superior sagittal sinus cavity [Figure 1]c, [Figure 1]d. The patient was reviewed by oncologist and stereotactic radiotherapy (Cyberknife) was applied to irradiate the remnants of the tumor within the superior sagittal sinus. No tumor tissue progression was observed 6 months after surgery [Figure 1]e and [Figure 1]f.

The sample tissue from neurosurgical procedure was fixed in 10% buffered formalin and paraffin embedded. The specimens were stained with hematoxylin-eosin and mucicarmine methods. Immunohistochemical studies were performed with antibodies to epithelial membrane antigen (EMA) (1:200, Leica clone GP1,4), cytokeratin, CK (1:50, DACO clone AE1/AE3), glial fibrillary acid protein, GFAP (1:300, Bio-Rad, clone 1B4), and Ki-67 (1:75, Invitrogen, clone SP6). The sections were examined and photographed.

Microscopic examination

Neuropathological examination revealed that samples of the tumor were composed of cords of round, ovoid, and epithelial cells, embedded in a prominent myxoid background [Figure 2]a,[Figure 2]b, and [Figure 2]d. Sometimes, mild nuclear atypia was focally present. The conventional meningothelial or transitional morphology was visible in small fields [Figure 2]c. Immunohistochemical staining showed positive staining for epithelial membrane antigen (EMA) [Figure 2]e. The Ki-67 labeling index was about 8% [Figure 2]f. The tumor cells were negative for glial fibrillary acid protein (GFAP) and cytokeratin (CK). Based on these findings, a final diagnosis of CM (WHO GII) was made.
Figure 2: Neuropathological features of choroid meningioma with hemorrhage. (a) Cords of tumor cells and trabeculae of round, ovoid, and epithelial cells. H&E. (b) The tumor cells were embeded in a prominent myxoid backround. H&E. (c) Typical meningothelial areas with hemorrhage. H&E. (d) Tumor cells embeded in mucinous stroma. Mucicarmine. (e) Cords of tumor cells with intense EMA immunoreactivity. (f) Ki-67 labeling index shows nuclear immunoreactivity

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The tumor removal was subtotal. As the patient age did not encourage us to conduct more aggressive surgical treatment, she was referred for a radiation therapy.

   Discussion Top

CMs are very rare tumors, as they represent only 0.5% of all meningiomas. Less than 300 cases have been reported worldwide to date. Kepes et al.,[7] who first described these lesions, reported their association with Castleman's syndrome. Castleman's disease is a rare disorder that involves an overgrowth of cells in your body's lymph nodes described by Benjamin Castleman in 1956.[8] The most common form of the disorder affects a single lymph node, usually in the chest or abdomen. Couce et al.[5] concluded that the systemic disease associated with CMs was restricted to children. No manifestation of systemic disease in adults was noted in his material.

Histologically, CMs are similar to chordomas. They contain trabeculae, or cords of eosinophilic vacuolated cells in a myxoid matrix.[9] High mitotic rate, increased cellularity, small cell change, sheet like growth, macro nucleoli, and focal necrosis are present.[3],[10] Loewenstern et al.[10] noticed moreover, that the mitotic index is a direct indicator of cell proliferation and predictor of tumor behavior. Aghi et al.[11] reported that prominent nucleoli were associated with higher risk of recurrence. In material of Lee et al.[12] focal necrosis was related with six-fold risk of recurrence. Baltus et al.[13] found, that detection of chromothripsis in CM can be related with considerable potential of recurrence after initial surgery. One of the most important regrowth patterns of the tumor is the high chordoid features percentage. It was first described by Couce et al.[5] Chordoid features greater than 50% as a significant tumor regression marker was emphasized also in later reports.[14],[15],[16],[17] In the study of Matyja et al.[18] significant chordoid component was concerned not only with high tumor recurrence rate, but also with more aggressive biological behavior. Hasegawa et al.[9] described relation with high mucoid quality of the tumor with its rapid spread and recurrence. In the differential diagnosis, correct identification of the vacuolated trabeculae of neoplastic cells in a myxoid background characteristic of CM is straightforward when accompanied by coexisting areas of typical meningioma.[10]

As the extent of resection is the most important prognostic factor in case of CM treatment, gross total resection (GTR) should be the goal for the surgical management of primary CM. It remains the strongest predictor of long-term rates of tumor control.[4],[5],[6],[7],[8],[9],[10],[11],[17],[18],[19],[20],[21],[22] As the recurrence remains high following subtotal resection, careful follow-up should be recommended in every case. The most illustrative reason for careful observation was presented by Couce et al.[5] In his long-term follow-up, all patients after a subtotal resection experienced tumor recurrence, whereas only one out of the 29 patients after a GTRs recurred.[5] Similar observations were noticed in the study by Zhu et al.[23] Although most of authors recommend adjuvant radiotherapy for atypical meningiomas in the management of residual disease following subtotal resection,[15],[24],[25] its efficacy remains unclear. In cases of incomplete resections, Liu et al.[26] justified its application as a method of salvage treatment. Conversely, in cases of complete tumor resections, most of the authors found no significant progression free survival rates increase after radiotherapy.[4],[15],[23],[24],[25],[26] Only few case studies advocated for the role of radiotherapy after GTR.[27],[28]

The MIB-LI (cell proliferation marker) is a histological marker for proliferative capacity, correlated with high rates of tumor control.[4] Higher MIB-LI has been noted to correlate with recurrent meningiomas in studies of some authors.[29],[30],[31],[32] Others did not share this opinion, founding no correlation with outcomes.[15],[32],[33]

Other factors concerned with unfavorable overall results were presented by Zhang et al.[34] In this large retrospective analysis, male gender, larger tumor size, surgical history, and bone invasion were defined as risk factors associated with poor outcome.

   Conclusions Top

CMs constitute a rare and diverse subtype of WHO grade II meningiomas. The review of the literature evidenced, that the most important factor leading to progression free survival is GTR. The role of adjuvant radiotherapy remains controversial. It should be indicated in patients with subtotal resection and high MIB-LI.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

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  [Figure 1], [Figure 2]


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