Uncommon Variant Of Meningioma Research Articles

An Atypical Meningioma Presenting at an Unusual Site

Meningiomas are tumours that originate from the arachnoid cells (meningocytes) and the majority are benign grade I tumours according to World Health Organisation (WHO). Chordoid meningioma is an uncommon variant of meningioma and corresponds to grade II tumour in WHO classification of tumours of the Nervous System 2016, because of its more aggressive behaviour and increased likelihood of recurrence. These meningiomas may appear extracranially (i.e. head and neck region, sinonasal tract, ear, temporal bone, scalp, etc) in only 2% cases. The histopathological and immunohistochemical evaluation is usually diagnostic. Here, the authors present a case of Chordoid meningioma in a 32-year-old patient who presented with complaints of nasal obstruction clinically suspected to be due to nasal polyp.

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.

Pineal chordoid meningioma complicated by repetitive hemorrhage during pregnancy: Case report and literature review

Chordoid meningioma is an uncommon variant of meningioma, and is very rarely found in the pineal region. We report a case of pineal region chordoid meningioma occurring in a young woman complicated by repetitive hemorrhages in the setting of pregnancy. A 23-year-old woman, 28 weeks pregnant, was transferred to our hospital for further management of a multi-septated, hemorrhagic pineal region mass and hydrocephalus. MRI revealed a heterogeneous T2-hyperintense lesion measuring 1.7 × 1.7 cm in the pineal gland. Resection of the tumor through an occipital transtentorial approach was performed. Histopathologic examination of the lesion confirmed the diagnosis of chordoid meningioma demonstrating cords and clusters of eosinophilic cells with rare cytoplasmic vacuolation arranged in a mucinous stroma. Additionally, there was abundant lymphoplasmacytic infiltration within the tumor. The details of this case are presented with a review of the literature.

Clear cell meningioma with histologically aggressive appearance and clinically aggressive behavior: A case report

Clear cell meningioma (CCM) is an uncommon variant of meningioma, corresponding to WHO grade II. We present a case of CCM with histologically aggressive appearance and clinically aggressive behavior. The tumor demonstrated rapid regrowth and brain metastasis. The histological progression from the ordinal CCM to the atypical area and higher MIB-1 index was observed. We assume that the short time of recurrence and metastasis may result from atypical histological features in our case. If the CCM has a histologically aggressive appearance as in our case, we suggest that postoperative adjuvant radiotherapy should be performed despite total resection of the tumor.

Clinicopathologic analysis of rhabdoid meningioma

Rhabdoid meningioma is an uncommon variant of meningioma, and was classified separately for the first time in the 2000 World Health Organization’s classification of tumors of the nervous system. Because it often shows malignant histological features and follows an aggressive clinical course, it has been classified as a grade III neoplasm. We describe the clinicopathologic features of 13 patients with this rare tumor. From 13 patients (seven male, six female), 19 specimens of rhabdoid meningioma were obtained between 2001 and 2009. The mean age of patients was 50.4 years at their first operation. The mean postoperative follow-up period was 35.7 months. Five patients experienced tumor recurrence, and two patients died from the disease. The mean time to first recurrence was 36.1 months. The recurrence-free survival rates at 1 and 5 years were 62% and 23%, respectively. Immunohistochemically, all tumors were positive for vimentin and epithelial membrane antigen. MIB-1 labeling indices were higher following tumor recurrence. Close follow-up and aggressive treatment of these tumors is warranted.

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

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.

Chordoid meningioma: a report of ten cases

Chordoid meningioma is a rare variant of meningioma with histological features resembling those of chordoma. This tumor has a great risk of recurrence and aggressive growth (WHO grade II). This study was done to document the clinical and pathological features of ten patients with chordoid meningioma who submitted to surgery at the National Institute of Neurology and Neurosurgery in Mexico City. Clinical, histological and immunohistochemical features were examined. The age range was from 30 to 67 years old (mean, 34.2 years). Seven patients were female and three male. The duration of symptoms varied from 3.5 months to 5 years (mean, 14.1 months). No systemic symptoms were noted. The tumor was localized in eight cases in the supratentorial compartments. Histologically, the tumors were characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Two of the ten tumors showed metaplasic changes, and seven showed brain invasion. Tumor cells demonstrated CK7, EMA and focal S-100 protein and Ep-CAM. Cytokeratin AE1/AE3, GFAP and synaptophysin were negative. The MIB-1 proliferative index was from 6 to 9% (mean 7.8). PCNA Li was 6 to 20% (mean, 14), and microvascular density was 6-16 (mean, 14.5). The mean rate of the MIB-1 labeling index in recurrences was 7.1% versus 6.33% for no tumor recurrence. Chordoid meningioma, World Health Organization grade II, is an uncommon variant of meningioma with a propensity for aggressive behavior and increased likelihood of recurrence. Chordoid meningiomas are predominantly tumors of young adults with a predilection for the supratentorial location. Intraventricular location and absence of systemic manifestations, despite the presence of abundant B-lymphocytes, mast cells and low MIB-1 LI, are some of the interesting findings in the present series that need further study. Hence, a larger number of cases with adequate follow-up data need to be studied further to establish the clinical relevance of this variant.

Clear cell meningioma with anaplastic features: Case report and review of literature

Clear cell meningioma (CCM) is an uncommon variant of meningioma, corresponding to WHO grade II. We present two cases of CCMs with anaplastic features in the intracranial and intraspinal region. The first case is a 65-year-old male who gradually developed changes in behavior over a period of 1 year. The second case is a 35-year-old female who presented with a 7-month history of posterior cervicothoracic pain and dysuria for 1 week. Magnetic resonance imaging revealed an intracranial lesion in the right frontal lobe in the male patient, and an intradural extramedullary lesion at C7 in the female patient. On histological examination, both tumors partly exhibited unusual anaplastic appearances with nuclear pleomorphism, high mitotic activity and necrosis, distinct from classical CCMs. Tumor cells were immunoreactive to epithelial membrane antigen (EMA) and vimentin, with a high MIB-1 index up to 40%. Total excision was performed. The male patient was found to have developed local recurrence and lateral ventricle metastasis 3 months after surgery. A diagnosis of CCM with anaplastic features was made (WHO grade III). Based on its aggressive behavior, we recommend postoperative adjuvant radiotherapy or chemotherapy even if total excision of the tumor has been performed, and MRI scans every 3–6 months during the first period of follow-up.

Distinguishing Chordoid Meningiomas From Their Histologic Mimics

Chordoid meningioma, World Health Organization grade II, is an uncommon variant of meningioma with a propensity for aggressive behavior and increased likelihood of recurrence. As such, recognition of this entity is important in cases that show similar morphologic overlap with other chondroid/myxoid neoplasms that can arise within or near the central nervous system. A formal comparison of the immunohistochemical features of chordoid meningioma versus tumors with significant histologic overlap has not been previously reported. In this study, immunohistochemical staining was performed with antibodies against D2-40, S100, pankeratin, epithelial membrane antigen (EMA), brachyury, and glial fibrillary acidic protein (GFAP) in 4 cases of chordoid glioma, 6 skeletal myxoid chondrosarcomas, 10 chordoid meningiomas, 16 extraskeletal myxoid chondrosarcoma, 18 chordomas, 22 low-grade chondrosarcomas, and 27 enchondromas. Staining extent and intensity were evaluated semiquantitatively and mean values for each parameter were calculated. Immunostaining with D2-40 showed positivity in 100% of skeletal myxoid chondrosarcomas, 96% of enchondromas, 95% of low-grade chondrosarcomas, 80% of chordoid meningiomas, and 75% of chordoid gliomas. Staining with S100 demonstrated diffuse, strong positivity in all (100%) chordoid gliomas, skeletal myxoid chondrosarcomas, low-grade chondrosarcomas, and enchondromas, 94% of chordomas, and 81% of extraskeletal myxoid chondrosarcomas, with focal, moderate staining in 40% of chordoid meningiomas. Pankeratin highlighted 100% of chordoid gliomas and chordomas, 38% of extraskeletal myxoid chondrosarcomas, and 20% of chordoid meningiomas. EMA staining was positive in 100% of chordoid gliomas, 94% of chordomas, 90% of chordoid meningiomas, and 25% of extraskeletal myxoid chondrosarcomas. Brachyury was positive only in the chordomas (100%), whereas GFAP was positive only in the chordoid gliomas (100%). EMA was the most effective antibody for differentiating chordoid meningioma from skeletal myxoid chondrosarcoma, low-grade chondrosarcoma, and enchondroma, whereas D2-40 was the most effective antibody for differentiating chordoid meningioma from extraskeletal myxoid chondrosarcoma and chordoma. Our findings demonstrate that in conjunction with clinical and radiographic findings, immunohistochemical evaluation with a panel of D2-40, EMA, brachyury, and GFAP is most useful in distinguishing chordoid meningioma from chordoid glioma, skeletal myxoid chondrosarcoma, extraskeletal myxoid chondrosarcoma, chordoma, low-grade chondrosarcoma, and enchondroma. A lack of strong, diffuse S100 reactivity may also be useful in excluding chordoid meningioma. Among the neoplasms evaluated, brachyury and GFAP proved to be both sensitive and specific markers for chordoma and chordoid glioma, respectively. Of note, this study is the first to characterize the D2-40 immunoprofile in extraskeletal myxoid chondrosarcoma, results that could be of utility in differential diagnostic assessment.

Oncocytic Meningioma: Report of a Case With Progression After Radiosurgery

Oncocytic meningioma is an uncommon variant of meningioma, characterized histologically by cells rich in mitochondria. This subtype of meningioma needs to be distinguished from other types of meningioma because of its more aggressive behavior. A case of oncocytic meningioma showing rapid clinical progression with diffuse intracranial dissemination after radiosurgery is described. Resistance to radiotherapy has been previously reported in oncocytic tumors of various body sites. This new case is a further example of the greater aggressiveness of oncocytic meningioma, suggesting that radiation therapy might worsen the course of disease.

Clear cell Meningioma, an uncommon variant of meningioma: a clinicopathologic study of nine cases

Clear cell meningioma (CCM) is an uncommon variant of meningioma, which affect younger patients, occur more often in spinal or cerebello pontine locations and shows a higher recurrence rate. Only few case reports have been described in the literature. The study has been undertaken to document the clinicopathological features of nine cases of CCM, operated at All India Institute of Medical Sciences during 1998 to December 2005. Clinical information was retrieved from the records of our Neurosurgery Department. The cases were stained with H&E, periodic Acid Schiff (PAS) with and without diastase. Immunohistochemistry for pancytokeratin, epithelial membrane antigen, vimentin, glial fibrillary acidic protein, and MIB-1 was done in all cases. During a period of 8 years, nine cases of CCM were diagnosed. Age ranged from 10 to 65 years (median age 26.0 years) with female predominance. Most common location was posterior fossa (CP angle). Clinically most of the patients presented with history of headache and features of cranial nerve palsies. The duration of symptoms varied from 3 to 60 months (mean 16.7 and median of 4 months). Radiologically lesions showed homogenous enhancement and were isointense to brain parenchyma. Histopathologic examination revealed tumor cells to be arranged in sheets with clear cytoplasm and monomorphic nuclei. MIB-1 labeling index (LI) ranged from 2 to 12% with a mean of 9%. Follow up varied from 3 to 84 months (median 36 months) and recurrence was noted in two patients after 2 and 3 years of surgery, respectively, despite their low MIB-1 labeling indices. CCM is a rare variant of meningioma with poor outcome. Less than 50 cases have been described in the literature. Low rate of recurrence and recurrence despite their low MIB-1 LI are some of the features, which needs to be documented. Hence, larger number of cases with adequate follow-up data need to be studied further to establish the clinical significance of this variant.

CT and MRI of lipomeningioma: case report and review of the literature

A case of lipomeningioma, an uncommon variant of meningioma, is described. Typical radiological features of meningioma, such as broad implantation in the dura, strong contrast enhancement and dural tail were associated with characteristics pointing to fatty content: negative density (−80 HU) on CT as well as short T1 and T2 on MRI.