Chordoid Meningioma Research Articles

Notochordal tumors : Benign notochordal tumors and chordomas

Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with apredominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with aloss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/-, epithelial membrane antigen (EMA)+/-, S100 protein+/-, brachyury+. This profile helps to distinguish these tumors from other lesions such as chondrosarcoma, chordoid meningioma, and metastases of carcinoma.

Innate immunity may play a role in growth and relapse of chordoid meningioma.

Chordoid meningioma (CM) is a rare subtype of meningioma, which represents only 0.5% of all meningiomas. It is classified as Grade II according to the World Health Organization classification because of its tendency to relapse. Pathological and clinical characteristics have been studied in order to forecast the future evolution of the lesions. However, information about infiltration of macrophagic elements and mast cells is very scarce. The authors analyzed the immunohistochemical patterns of three cases and a relapse of CM, in order to verify whether infiltrating macrophages are in a polarized state and what would be the proportion between such elements and mastocytes. Results suggest that macrophages in CMs are mainly in a non-polarized or M2 state and their abundance might be associated with a major potential of relapse; additionally, there is an inverse correlation between the number of mast cells and macrophages. Further studies are requested in order to confirm these intriguing data.

Rare Suprasellar Chordoid Meningioma with INI1 Gene Mutation

Background: Chordoid Meningioma is a rare brain tumour characterized genetically by loss of genetic material from chromosome 22q at cytogenetic level resulting in mutation of NF2 gene.Objectives and case report: In the present report, we described a rare case of suprasellar chordoid meningioma, which presented in a 32-year-old-woman. Her only complain was throbbing headache. Neurological examination showed left temporal hemianopia, decreased visual acuity (3/6), and no physical abnormalities related to Castleman syndrome were noted. Cranial magnetic resonance (MR) images demonstrated a 28x15 mm mass in the sellar region, which showed iso-to low intensity that enhanced vividly after gadolinium with upwards displacement of the Optic chiasm.Total surgical excision of the tumour was performed and subsequent histological examination of the tumour showed typical histology pattern of chordoid meningioma grade II according to the WHO classification system of meningiomas.Genomic DNA was extracted and mutation analysis for INI1 gene using primer of exon 4, 5, 7, and 9 showed mutation involving exon 9. DNA sequencing showedheterozygosity C­­­­­­­­­­­­­­­­­­-T mutation in exon 9 of INI1 gene leading to change of amino acid serine to phenylalanine at (codon 63). The details of this case are presented with a review of the literature.

Validation of nuclear STAT6 immunostaining as a diagnostic marker of meningeal solitary fibrous tumor (SFT)/hemangiopericytoma.

NAB2-STAT6 gene fusion is a molecular characteristic of solitary fibrous tumors (SFT) and hemangiopericytoma, underscoring their definition as one diagnostic entity. NAB2-STAT6 fusion is associated with nuclear relocation of STAT6 protein that can be detected by immunohistochemistry. We evaluated the diagnostic value of STAT6 expression in meningeal tumors. 77 meningeal tumors (17/77 (22.0%) SFT/hemangiopericytoma, 11/77 meningothelial meningioma, 10/77 atypical meningioma 8/77 chordoid meningioma, 9/77 fibroblastic meningioma, 10/77 transitional meningioma, 3/77 rhabdoid meningioma and 9/77 anaplastic meningioma) were included. STAT6 immunohistochemistry was performed on FFPE specimens using a fully automated slide-staining system and anti-STAT6 antibody SC-20:sc621. Two independent observers analyzed all specimens blinded to histological diagnoses, and a third observer was consulted in case of discordancy. STAT6 immunohistochemistry yielded an exclusively nuclear immunostaining signal. 16/17 (94%) SFT/hemangiopericytoma specimens presented with clear-cut, wide-spread, and moderate to strong staining in tumor cell nuclei and were rated as STAT6-positive. In only 1 SFT case with weak and focal nuclear STAT6 immunostaining signal, STAT6 expression was rated discordant (observer 1: STAT6-negative, observers 2 and 3: STAT6-positive). All non-SFT/hemangiopericytoma cases were unanimously rated as STAT6-negative. In 76/77 (98.7%) cases the evaluation of STAT6 immunostaining results was in agreement among observers. STAT6 immunohistochemistry is a robust method to verify diagnosis of SFT/hemangiopericytoma and should therefore be included in the diagnostic work-up of meningeal tumors. In singular cases, weak and focal STAT6 expression may lead to false-negative evaluation and may prompt further molecular work-up. .

Clinical Features, Treatment, and Prognostic Factors of Chordoid Meningioma: Radiological and Pathological Features in 60 Cases of Chordoid Meningioma

As one subtype of meningiomas, chordoid meningiomas (CMs) are rarely described. In this study, the authors aimed to analyze the incidence, clinical features, treatment, and prognostic factors of CMs. Among 9873 cases of meningioma surgically treated between 2003 and 2013 at Beijing Tiantan Hospital, cases of CM were chosen for analysis and were reviewed retrospectively. To ascertain which factors were related with progression-free survival (PFS), statistical analysis was performed. Sixty cases of pathologically confirmed and surgically treated CMs were identified. They accounted for 0.61 % of the entire series of meningioma. Simpson Grade I, II, III, and IV resection was performed in 26, 19, 9, and 6 patients, respectively. Fifty-four patients were followed up for 25-141 months after operation. Of these patients, 19 received postoperative adjuvant radiotherapy. Seventeen patients experienced tumor progression, and median PFS was 54 months. The 3- and 5-year PFS rates were 89.7% and 57.1%, respectively. Two patients died of tumor recurrence and 1 died of unrelated reason. Univariate analysis demonstrated tumor shape and extent of resection were significantly associated with prognosis. Multivariate analysis revealed only extent of resection was an independent prognosis factor for PFS. CM is a rare subtype of World Health Organization grade II meningioma. It more frequently involved the adult patients and was without sex predilection. The confirmed diagnosis relied on pathological validation. Total resection whenever possible is recommended to prolong PFS of patients. The value of adjuvant radiotherapy for CM need to be further studied.

MNGO-21GENOTYPING OF MENINGIOMA BY TARGETED AMPLICON SEQUENCING USING MiSeq

BACKGROUND: Meningiomas are the most common primary brain tumors, accounting for about 27% of all intracranial tumors. Loss of Neurofibromin 2 (NF2) is found in about half of sporadic meningiomas, and recently, mutations in TRAF7, KLF4, AKT1, and SMO are reported in non-NF2 meningioma. To establish practical genotyping methods, we performed targeted amplicon sequencing with 45 meningioma specimens and evaluated the association with clinicopathological features. METHODS: Frozen samples of 45 meningiomas were fixed with PAXgene Tissue System (QIAGEN) and embedded in paraffin (PFPE). Amplicon sequencing panel targeted NF2, TRAF7, KLF4, AKT1 and SMO were designed by GeneRead Mix-n-Match panel and Custom panel (QIAGEN). Genomic DNA was extracted from PFPE tissues and libraries were prepared using GeneRead DNAseq Targeted Panels V2 (QIAGEN). The libraries were sequenced by the MiSeq (Illumina). The raw read data obtained from amplicon sequencing were processed by GeneRead Panel Variant Calling service for analysis of mutations and copy number alterations (CNA). RESULTS: Thirty three cases (73%) harbored NF2 loss, in which 20 cases (61%) simultaneously carried NF2 mutation. Mutations in TRAF7, KLF4, AKT1, and SMO were observed in 6 (13 %), 4 (9 %), 2 (4 %) and 0 (0%) cases, respectively. Altogether, 40 of 45 case (89 %) carried at least one or more gene alteration including mutations in 5 genes listed above and NF2 loss. Result of genotyping was correlated with histological subtypes as follows. All of nine fibrous meningiomas carried NF2 loss, while all of 4 meningiomas with secretory component carried both TRAF7 and KLF4 mutations. A case of chordoid meningioma harbored only AKT1-E17K mutation. CONCLUSIONS: Here we established the clinical sequence system for meningioma by targeted amplicon sequencing with desktop sequencer in which we demonstrated favorable results for CNAs as well as mutations.

Erratum to: Predictors of recurrence in the management of chordoid meningioma.

Erratum to: Predictors of recurrence in the management of chordoid meningioma.

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

As an uncommon subtype of meningioma, chordoid meningioma (CM) of the spinal canal is extremely rare. There have been only two reported cases of intraspinal CM in the literature, and this lesion has not been previously reported in the pediatric age group. To the best of our knowledge, the present study reports the first case of spinal chordoid meningioma in a pediatric patient. A 12-year-old female presented with a 3-month history of progressive numbness and weakness in the right-side limbs, and intermittent pain in the neck and right shoulder. Spinal magnetic resonance imaging (MRI) revealed an intraspinal lesion at the C2-3 level with irregularly heterogeneous enhancement. The patient underwent a C2-3 laminotomy. Due to adhesion to the dura and proximity to the vertebral artery, the tumor was partially removed intraoperatively. The post-operative course was uneventful and the symptoms were apparently relieved. The patient experienced recurrence 5 years subsequent to surgery. MRI revealed an extradural regrown tumor at the C2-5 level. Partial removal combined with radiotherapy was performed. However, the patient experienced progression of tetraplegia and succumbed to severe pneumonia and respiratory failure 5 months subsequent to the second surgery. In the present study, the clinicoradiological findings and treatment outcome of this rare entity are discussed, in addition to a review of the relevant literature. Spinal CMs should be included in the differential diagnosis of intraspinal tumors of the pediatric spine. Multidisciplinary treatment, consisting of total surgical removal and adjuvant radiotherapy, should be considered due to the aggressive nature of this abnormality and the risk of long-term recurrence.

Predictors of recurrence in the management of chordoid meningioma

Management of chordoid meningiomas (CMs) is complicated by high rates of recurrence, particularly following subtotal resection. Optimal management is not established given the paucity of published experience. To identify prognostic factors for recurrence following resection, the authors conducted the largest systematic review of CMs to date. A comprehensive search on MEDLINE (OVID and Pubmed), Scopus, Embase, and Web of Science utilizing the search terms "chordoid" AND "meningioma" was performed to identify all reports of pathologically confirmed intracranial CMs. A total of 221 patients were included, comprising 120 females and 101 males. Mean age, MIB-1/Ki67, and tumor size was 45.5 years, 4.3% (range 0.1-26.6%), and 4.1 cm (range 0.8-10 cm), respectively. 5-, and 10- year progression free survival was 67.5 and 54.4%, respectively. Gross total resection (GTR) and subtotal resection was achieved in 172 and 48 patients, respectively. Adjuvant radiotherapy (RT) was given to 30 patients. Multivariate analysis found GTR was strongly correlated with decreased recurrence rates (HR 0.04, p = <0.0001), while higher MIB-1 labeling index (≥5 vs <5%) was associated with increased recurrence (HR 7.08; p = 0.016). Adjuvant RT, age, gender, and tumor location were not associated with recurrence. GTR resection is the strongest predictor of tumor control, and should be the goal to minimize local progression. Additionally, higher MIB-1 labeling was associated with increased rates of tumor recurrence. Tumors that are subtotally resected or demonstrate higher MIB-1 are at greater recurrence and warrant consideration for RT and close long term follow up.

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

Meningiomas are very commonly diagnosed intracranial primary neoplasms, of which the chordoid subtype is seldom encountered. Our aim was to retrospectively review preoperative MR imaging of intracranial chordoid meningiomas, a rare WHO grade II variant, in an effort to determine if there exist distinguishing MR imaging characteristics that can aid in differentiating this atypical variety from other meningioma subtypes. Ten cases of WHO grade II chordoid meningioma were diagnosed at our institution over an 11-year span, 8 of which had preoperative MR imaging available for review and were included in our analysis. Chordoid meningioma MR imaging characteristics, including ADC values and normalized ADC ratios, were compared with those of 80 consecutive cases of WHO grade I meningioma, 21 consecutive cases of nonchordoid WHO grade II meningioma, and 1 case of WHO grade III meningioma. Preoperative MR imaging revealed no significant differences in size, location, signal characteristics, or contrast enhancement between chordoid meningiomas and other meningiomas. There were, however, clear differences in the ADC values and normalized ADC ratios, with a mean absolute ADC value of 1.62 ± 0.33 × 10(-3) mm(2)/s and a mean normalized ADC ratio of 2.22 ± 0.47 × 10(-3) mm(2)/s in chordoid meningiomas compared with mean ADC and normalized ADC values, respectively, of 0.88 ± 0.13 × 10(-3) mm(2)/s and 1.17 ± 0.16 × 10(-3) mm(2)/s in benign WHO grade I meningiomas, 0.84 ± 0.11 × 10(-3) mm(2)/s and 1.11 ± 0.15 × 10(-3) mm(2)/s in nonchordoid WHO grade II meningiomas, and 0.57 × 10(-3) mm(2)/s and 0.75 × 10(-3) mm(2)/s in the 1 WHO grade III meningioma. Chordoid meningiomas have statistically significant elevations of ADC and normalized ADC values when compared with all other WHO grade I, II, and III subtypes, which enables reliable preoperative prediction of this atypical histopathologic diagnosis.

Intraparenchymal chordoid Meningioma After Radiotherapy for Hodgkin Lymphoma: A Case Report and Review of the Literatur

Objective: Hodgkin lymphoma can be treated by radiotherapy or chemotherapy alone or combined. Meningiomas account for 1-4.2% of all primary intracranial tumors in children, and chordoid meningioma is a very rare subtype. In this study, we investigated a case of an intraparenchymal chordoid meningioma that developed during the early stage in a patient with Hodgkin lymphoma who had been treated with radiotherapy. Case: A 10-year-old male patient was diagnosed with Hodgkin lymphoma and was treated with a combination of radiotherapy and chemotherapy. He presented at our emergency service 6 years later. He had a fever and was suffering from discomfort and insignificant left hemiparesis (4/5). Contrast-enhanced cranial magnetic resonance imaging (MRI) showed a mass in the right temporoparietal region. The intracranial lesion was surgically excised. The tumor was identified as a WHO grade 2 chordoid meningioma by the pathological examination. The Ki-67 proliferation index was found to be 20-25%. Conclusion: Surgeons must remember that radiation-associated meningiomas may occur in the early stage of the treatment as well as in the late stage. Young patients with grade 2 chordoid meningiomas must be followed-up in case of recurrence, and tumors with high Ki-67 indexes are highly expected to relapse.

Chordoid meningioma in a pediatric patient with tuberous sclerosis complex.

Meningeal tumors are rare in childhood, comprising less than 4.2% of all pediatric primary intracranial tumors.1 Fourteen histological subtypes of meningiomas exist in the World Health Organization (WHO) classification system.2 Chordoid meningioma (CM) is a rare meningiomal variant characterized histologically by features that mimic chordoma and other chordoid neoplasms.2 Chordoid meningiomal tumors tend to exhibit local recurrence and aggressive behavior, although they typically show morphologically benign features. In a tuberous sclerosis complex (TSC) patient, hamartomas can occur in multiple organs and cause diverse clinical symptoms. Cortical tubers, subependymal nodules (SENs) and subependymal giant cell astrocytomas (SEGAs) are representative lesions of TSC and can be detected by magnetic resonance imaging (MRI) of the brain.3 SEGA lesions have been found to occur in approximately 6.1% to 18.5% of TSC patients, and account for 90% of all intracranial tumors associated with TSC.4 SEGA lesions are commonly located in the foramen of Monro or the periventricular region,5 and can cause loss of vision, papillary edemas, intracranial calcification, and hydrocephalus.6 The primary method for treating SEGA lesions consists of surgical resection of the tumors; complete and early surgical removal of tumors has been shown to be the most important prognostic factor.6 Childhood CM has been shown to account for only ~0.5-1% of all meningiomas, with intraventricular occurrence of CM reported in only 27 children to date.2,7 Also, CM has not been reported in any TSC patients to date. Here we report a case of intraventricular CM, which appeared highly similar to SEGA in brain MRI scans, in a child with TSC.

Chordoid meningioma - a case report: clinicopathological features and differential diagnosis of an uncommon tumor

Meningiomas are tumors that originate from the arachnoid cell and the majority are benign and grade I tumors according to World Health Organization. Chordoid meningioma is an uncommon variant of meningioma and corresponds to grade II tumor in the World Health Organization Classification of Tumors of the Nervous System 2007 because of its more aggressive behavior and increased likelihood of recurrence. A 75-year-old female was referred to the neurosurgery department complaining of headache, syncope, and seizure. Radiological examination revealed a mass lesion in the neighbourhood of the frontal lobe that destructed bone and was associated with peritumoral edema. The patient underwent surgery. The tumor was totally excised with the dura beneath. Histopathological examination showed that the tumor was composed of clusters and cords of small polygonal cells with fine chromatin and eosinophilic vacuolated cytoplasm embedded in a myxoid matrix, and also focal whorls of spindle-shaped cells. Two mitoses were seen in 10 high power fields. Vascular proliferation was observed in some tumoral areas. Bone invasion was present. Immunohistochemical analysis of the tumor cells revealed widespread strong membranous and cytoplasmic expression of epithelial membrane antigen. The Ki67 labeling index was 6-8%. All of these findings were consistent with a diagnosis of chordoid meningioma, the neoplasm was identified as grade II based on the World Health Organization Classification, 2007. In this report we present a case of chordoid meningioma without classical radiological findings of meningioma with areas of vascular proliferation that mimicked glial tumors at histopathologic examination.

Clinical features and treatment outcome of chordoid meningiomas in a single institute.

ObjectiveMeningioma is the second most common primary central nervous system neoplasm. In contrast, chordoid meningioma is rare; due to the paucity of cases, little is known about its clinical features or treatment outcomes. The objectives of this study were to describe the clinical characteristics and outcomes for patients with chordoid meningioma.MethodsIn total, 16 patients, with newly diagnosed chordoid meningioma who underwent surgical excision between 1999 and 2012 were included. We retrospectively evaluated the medical records, radiological findings, and pathological findings. The median follow-up period was 56.5 (range, 3-170) months. The MIB-1 labeling index ranged from 1 to 26.60% (median, 5.04).ResultsSimpson grade I, II, and III resections were performed in four, nine, and three patients, respectively. The overall recurrence rate was 37.5%. Overall progression-free survival (PFS) after resection was 94.7 months (95% CI=62.9-126.6). Of the 4 patients with Simpson grade I resection, recurrence occurred in one patient. Among the Simpson grade II and III resection groups, eight patients underwent adjuvant radiation therapy and they showed significantly longer PFS (121 months, 95% CI=82.1-159.9) than the patients who underwent surgery alone (40.5 months, 95% CI=9.6-71.3) by the log-rank test (p<0.05).ConclusionChordoid meningiomas are difficult to manage and have a high rate of recurrence. Complete resection of the tumor is a key determinant of better outcomes. Adjuvant radiation therapy is recommended, eparticulary when Simpson grade I resection was not achieved.

Optic nerve glioma: A great mimicker

Background:Arachnoid proliferation, although rare, is known to occur in association with optic gliomas. However, chondroid and chordoid metaplasia has not been reported previously.Case Description:A 27-year-old male presented with progressive, painless loss of vision in right eye, associated with vomiting and headache for one and a half months. Computed tomography (CT) scan revealed a contrast enhancing mass arising from planum sphenoidale. Perioperative findings showed the tumor adherent to the right optic nerve and attached to basal dura and falx. A clinical impression of an intradural, optic nerve sheath meningioma was made. Histopathological examination revealed a glial tumor with adjacent areas displaying marked fibroblastic and arachnoid cell proliferation with chondroid as well as chordoid differentiation along with myxoid change and dense collagenisation. Reticulin stain, immunochemistry with glial fibrillary acid protein (GFAP), epithelial membrane antigen (EMA), and S-100 helped to arrive at the final diagnosis of optic glioma displaying exuberant arachnoid proliferation with cartilaginous metaplasia.Conclusion:We report a case of optic nerve glioma displaying extensive arachnoid proliferation, chordoid, and cartilaginous metaplasia, which mimicked chondrosarcoma or chordoid meningioma, posing a diagnostic dilemma. A clinical feedback, simple reticulin stain, and GFAP staining is of immense value in such cases to arrive at the correct diagnosis.

A case report of chordoid meningioma with contiguous extracranial invasion

Tumors of the rhinopharynx or cavum are rare, with an incidence of 0.5 to 2 cases per 100,000 inhabitants. Are more common in males, and the mean peak age is between 40 and 50 years old. They are very aggressivewith a tendency to earlymetastasis. The authors describe the case of a male patient, 47 years old, ex-smoker, presents with backache, fever (37.5 °C–38 °C), irritative cough and asthenia, for nearly two months. These complaints were initially interpreted as mechanical or degenerative back pain and tracheobronchitis. Medicated by the attending physician with antibiotics without improvement, was asked complementary exams: chest radiograph had no changes and simplify blood tests:without anemia or leukocytosis, c-reactive protein: 18 mg/dl, velocity sedimentation—70ms, ferritin—817 ng/ml. He was admitted in Internal Medicine for study. As first diagnosis most likely was spondylodiscitis we order other tests, including culture and serology tests, which proved negative. The CT of the lumbar spine revealed lytic lesions in the vertebral bodies, sacrum and iliac bone suggestive of secondary deposits. After this, was asked thoracoabdominal-pelvic CT that revealed two hepatic nodules and a 9 mm nodular thickening of the wall of the rectum. Colonoscopy revealed no changes and biopsies of the rectum were made. Anatomopathologic results were inconclusive. Other tests without significant changes. Meanwhile he is re-hospitalized for pneumonia and cervical pain. CT of the cervical spine showed an osteolytic lesion of C7 and C3–C4 tissue mass. For suspected neoplasm of nasopharynxwas asked soft tissues CT of the head and neckwith identification of amass at the cavum left side, and a nodule in left pulmonary apex. Spine MRI with multiple changes. Requested the collaboration of Otorhinolaringology for excisional surgical biopsy and histological study revealed the presence of squamous cell carcinoma (WHO type 1). Was diagnosed a nasopharyngeal carcinoma with bone, liver and lung metastasis. Without surgery indication, he was proposed to chemotherapy (cisplatin e paclitaxel), however died in 3 months after first hospitalization, 1 month after chemotherapy. We believe that this is an important case, not only for being uncommon, but also for not having manifested the most common form which is usually local manifestations, but with a back pain that is a much common complaint, which made the diagnosis much more difficult. In addition, alerts us to the aggressiveness of this tumor and the need for the rapid diagnosis and aggressive treatment.

The radiological and histopathological differential diagnosis of chordoid neoplasms in skull base

Background Chordoid neoplasms refer to tumors appearing to have histological features of embryonic notochord, which is characterized by cords and lobules of neoplastic cells arranged within myxoid matrix. Because of radiological and histological similarities with myxoid matrix and overlapping immunohistochemical profile, chordoma, chordoid meningioma, chordoid glioma, and rare extraskeletal myxoid chondrosarcoma enter in the radiological and histological differential diagnosis at the site of skull base. However, there is always a great challenge for histopathologists to make an accurate diagnosis when encountering a chordoid neoplasm within or near the central nervous system. The aim of this study is to investigate and summarize the radiological, histological features and immunohistochemical profiles of chordoid neoplasms in skull base, and to find a judicious panel of immunostains to unquestionably help in diagnostically challenging cases. Methods A total of 23 cases of chordoid neoplasms in skull base, including 10 chordomas, 5 chordoid meningiomas, 3 chordoid gliomas and 5 extraskeletal myxoid chondrosarcomas, were collected from the First Affiliated Hospital, Sun Yat-sen University and Guangdong Tongjiang Hospital. MRI examination was performed on the patients before surgical treatment. Microscopical examination and immunohistochemical staining study using vimentin (Vim), pan-cytokeratin (PCK), epithelial membrane antigen (EMA), S-100 protein (S-100), glial fibrillary acidic protein (GFAP), D2-40, Galectin-3, CD3, CD20, Ki-67 were performed on the samples of cases. The clinicopathological data of the patients was also analyzed retrospectively. Results Most of chordomas were localized in the clivus with heterogeneous hyperintensity on T2WI scanning. The breakage of clivus was observed in most cases. Histologically, the tumor cells of chordoma exhibited bland nuclear features and some contained abundant vacuolated cytoplasm (the so-called physaliferous cells) within myxoid matrix. Areas of cartilage presented in individual cases. The tumor cells of chordoma were diffusely immuno-positive for PCK and EMA. Chordoid meningioma is characterized by a homogeneous contrast-enhancing mass with dural tail sign. The epithelioid cells were arranged in cords and clusters within a myxoid matrix, which was highly reminiscent of chordoma. The ratio of myxoid component and conventional meningioma was different in tumors. EMA was detectable in all chordoid meningiomas, and 4/5 cases were positive for D2-40. That was a finding which was helpful in distinguishing chordoid meningioma from chordoma. Chordoid gliomas were localized in the third ventricle with homogeneous enhancement on MRI examination. The tumors were composed of clusters and cords of epithelioid tumor cells within a variably mucinous stroma containing lymphoplasmacytic infiltratation. The most distinctive immunohistochemical feature of chordoid gliomas is their strong diffuse reactivity for GFAP. EMA and PCK positive expression can also be seen focally in individual cases. Although extraskeletal myxoid chondrosarcomas share the similar radiological and histological appearance with chordoma, all chondrosarcomas in the present study are completely negative for epithelial markers except for immuno-positivity of S-100 focally. In addition, Galectin-3 can be detected in most of chordoid neoplasms in skull base. Conclusion The specific localization and MRI features of tumors are useful diagnostic clues for the differential diagnosis of chordoid neoplasms in skull base. However, combining with histological features, a panel of selected immunostains, including PCK, EMA, GFAP, S-100 and D2-40, is helpful in making an accurate diagnosis for those diagnostically challenging cases which usually appear to have atypical radiological features or in an unusual site. Ki-67 index and Galectin⁃3 are not recommended to use as diagnostic markers for chordoid neoplasms of skull base because of their less neoplastic specificity.

Clinical features and treatment of intracranial chordoid meningioma: a report of 30 cases

To discuss the clinical characteristics and prognosis of chordoid meningioma (CM). Tumour samples of CM from 30 patients were re-examined. The postoperative outcomes were analyzed on the basis of clinical observations. The survival probabilities were calculated using the Kaplan-Meier method. Thirty-two operations were performed in 30 cases, including 27 operations for total removal and five operations for subtotal removal. The median follow-up period was 34.0 months. Tumour recurred in five patients, with a median recurrence time of 32.0 months. No systemic manifestations of Castleman's syndrome were found. The majority (80%) of tumours were found in the supratentorial compartments. The MIB-1 labelling index (MIB-1 LI) varied from 1% to 10%. In univariate analyses, the presence of aggressive factors (P = 0.001) and the extent of resection (P = 0.037) were related to progression-free survival (PFS). The MIB-1 LI (P = 0.50) and postoperative radiotherapy (P = 0.62) were not related to PFS. Chordoid meningioma is a rare subtype of meningioma, and is often found supratentorially. There is an absence of association with Castleman's syndrome. Aggressive factors and the extent of resection are helpful in predicting recurrence. It might be more pertinent to downgrade CM to grade I, unless it shows aggressive factors.

Chordoid meningioma: a retrospective series of seven consecutive cases

Chordoid meningioma is a rare variant of meningioma characterized by a more aggressive behavior. The present study documents the histological, radiological and clinical features of seven cases treated at the Policlinico Umberto I of Rome from 1999 to 2010. There were five males and two females. Most of the cases were located in the supratentorial space, especially the convexity. Surgical gross total resection was achieved in four cases. Of the remaining three cases, two relapsed and underwent further surgeries and adjuvant treatment. The MIB-1 index had a mean value of 7.5 (range 0.3-25.8). Tumors were composed of epithelioid cells or plump to spindle cells, forming cords, cribriforms or nests, in a mucoid matrix. All tumors showed diffuse positive immunoreactivity to vimentin and epithelial membrane antigen. Surgery is the first line of treatment for this kind of lesion. Gross total resection guaranteed a survival free from recurrences in our series. On the other hand, radiation therapy must be considered in patients submitted to a subtotal resection.

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.