Rare Subtype Of Meningioma Research Articles

Intracranial Papillary Meningioma

Papillary meningioma (PM) is an uncommon meningioma subtype, and the clinical characteristics remain unclear. To determine the clinical characteristics and prognosis of PM. The clinical data of 30 PM patients were collected, the samples were reexamined, and the patients' prognoses were based on clinical observations and calculated according to the Kaplan-Meier method. The 30 patients included 16 males and 14 females (median: 34.0 years upon initial diagnosis). Of the 48 intracranial operations in the 30 patients, total removal was attained in 34 surgeries, and subtotal removal in 14 surgeries. Radiotherapy was provided in 20 patients. In 40 specimens with follow-up, 29 attained the positive aggressive factors. Six tumors showed positive progestogen receptor (PR) combined with negative Bcl-2. The median follow-up period was 39.0 months. Tumor recurrence occurred in 18 patients (median: 17.0 months); the recurrence rates following total removal and subtotal removal were 57.1% and 100%, respectively. Fourteen patients died of the recurrence. In the univariate analyses, positive aggressive factors (P = .021), positive PR combined with negative Bcl-2 immunoreactivity (P = .011), the extent of resection (P = .001), and radiotherapy (P = .002) were significantly related to progression-free survival. The MIB-1 labeling index was not significantly related to progression-free survival (P = .88). PM is a rare subtype of meningioma with a tendency of recurrence. The extent of resection is an important prognosis factor. The presence of positive histopathological index increases the recurrence risk. Positive PR combined with negative Bcl-2 immunoreaction might predict a good prognosis. Postoperative radiotherapy may play a vital role in prolonging the time to tumor recurrence.

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 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.

Rhabdoid Meningioma-An Uncommon and Aggressive Variant

Rhabdoid meningioma is a rare subtype of meningiomas accounting for 1-3% of all intracranial meningiomas and classified as WHO Grade III tumor. It has an aggressive course and needs to be treated by both surgery and radiotherapy. Here we present a rare case of rhabdoid meningioma in a 24 year old lady with complaints of focal seizures, paresis and diplopia. CT & MRI showed a mass attached to dura in left posterosuperior frontoparietal region with peripheral edema. Histopathology showed large tumor cells arranged in sheets, whorls and papillary pattern. The patient was treated by surgery followed by radiation. This is reported to aware the surgical pathologists to keep this subtype as a differential diagnosis while interpreting any meningioma as it has an aggressive course.

Intracranial clear cell meningioma: a clinicopathologic study of 15 cases

Clear cell meningioma (CCM) is a rare histological variant of meningioma. CCM has a high recurrence rate and aggressiveness. In this study, we reviewed our experience in the treatment of the lesion. Here we present a series of 15 patients with intracranial CCM. The clinical data were retrieved from the records of our Neurosurgery Department and the patients' prognoses were attained by clinic service and telephone. Immunohistochemistry for epithelial membrane antigen (EMA), vimentin, glial fibrillary acidic protein (GFAP), CD10, and S-100 was done, and the MIB-1 labeling index was calculated in all cases. The 15 patients included eight males and seven females; the mean age was 34.8 years. The most frequent initial symptoms were headache and hearing loss. The most common location was the cerebellopontine angle (CPA) zone. Eleven patients had total removal and four patients underwent subtotal removal. Histological features of atypia were present in different proportions, from 6.7% to 100%, and six cases accorded with atypia. Three tumors showed brain invasion. EMA and vimentin were 100% positive, and CD10 was 100% negative. GFAP was 87% negative and S-100 was 93% negative. The mean follow-up period was 36.7 months. Three patients with brain invasion all recurred and five cases with atypia recurred. In 11 patients with total removal, six patients recurred. In four patients with subtotal removal, three patients recurred. Kaplan-Maier analysis showed that incomplete surgical resection was significantly associated with recurrence (p = 0.001). The MIB-1 labeling index for recurrence was 5.7 ± 2.7% versus 2.8 ± 1.5% for no recurrence (p = 0.036). CCM is a rare subtype of meningioma, with a tendency to present in younger patients and a propensity to recur. Immunohistochemistry plays a vital role in differentiating CCM from other tumors. Brain invasion, atypia and MIB-1 labeling index are likely to predict the recurrence. The extent of resection might be connected with the prognosis.

Giant chordoid meningioma symptomatic immediately after pregnancy: report of a rare case

Chordoid meningioma is a very rare subtype of meningioma with less than 90 cases reported in the literature. Meningiomas related with a pregnancy are also very rare. Herein, we present a pregnancy-associated case of a chordoid meningioma and briefly discuss possible mechanisms. Approximately 10 hours after a Caesarean section, a 37-year-old woman became stuporous with a Glasgow coma scale score of 9 - 10 and the right pupil became dilated without reaction to light. An emergency computed tomography scan showed a right frontotemporal lesion measuring 8 x 7 x 6 cm which enhanced moderately and homogenously after intravenous contrast injection. The patient's hemoglobin level was slightly lower than normal level but there was no dysgammaglobulinemia or lymphoid hyperplasia. The patient was immediately taken to the operating theater and the tumor was gross totally removed. The postoperative period was uneventful; and histopathological diagnosis was chordoid meningioma. The exact mechanism causing a pregnancy-associated increase in the size of meningiomas and the amount of peritumoral edema has not been clearly elucidated. As for this case of chordoid meningioma associated with pregnancy, we think mucin accumulation in tumor could be involved in an increase in the tumor size while delivery procedures with the common anaesthetic and sedative drugs may also have enhanced the peritumoral edema by causing a relative decrease in the cerebral blood flow.

Supratentorial clear cell meningioma in a child: A rare tumor at unusual location

Clear cell meningioma is a rare subtype of meningioma seen mainly in pediatric patients. Supratentorial location is an unusual site of occurrence and its natural history and prognosis are not well described in the literature. We present a unique case of a left-sided frontoparietal tumor in a 15-year-old child who was managed successfully with gross total surgical excision and is recurrence-free at 18 months follow-up. A favorable clinical behavior and a longer symptom-free interval can be expected after gross total removal. The patients should be followed after successful surgery and other modalities of therapy should be used for recurrence.

Méningiome en plaque frontal : forme rare d’une tumeur commune !

En plaque meningioma are a morphological subgroup of meningioma defined by a carpet or sheet-like lesion that infiltrates the dura and sometimes invades the bone. We present an exceptional case due to its localization and evolution. A 41-year-old female patient consulted for progressive swelling of the left frontoparietal region. CT-scan revealed thickening of the cranial medulla, without cerebral lesion. The tumor was removed. Histological analysis confirmed en plaque meningioma which recurred 7 years after. En plaque meningioma are a rare subtype of meningioma which rarely affects the skull vault. Relapse is frequent.

Fractionated stereotactic radiation therapy in the management of primary optic nerve sheath meningiomas

We analysed our long-term results after fractionated stereotactic radiotherapy (FSRT) in patients with primary optic nerve sheath (pONSM) meningioma, as a rare subtype of meningiomas. Between 01/1995 and 12/2007, 32 patients with pONSM were treated with FSRT. Fifteen patients received radiotherapy as primary treatment, four after biopsy, and six patients after surgical resection. Seven patients were irradiated for recurrent disease. Seventeen lesions were histologically proven and determined as WHO grade I pONSM. Median target volume was 15.7 cc, median total dose 54.9 Gy. Twenty-nine patients showed clinical symptoms before radiotherapy like reduced vision, unilateral loss of vision, or an exophthalmia. Median follow-up was 4.5 years. Overall local tumor control was 100%. Twenty-six patients had stable disease based on CT/MRI, while 6/32 had a partial remission of tumor volume. 97% of our patients showed stable vision or an improvement of visual acuity. Eleven patients (38%) showed an improvement of pre-existing clinical symptoms like double vision, exophthalmia and visual acuity. Only one patient showed an impairment of vision with progressive concentric decreasing of the visual field on the side of the irradiated ONSM. These data demonstrate that FSRT is an effective and safe treatment modality for local control in patients with pONSM with minimal risk of significant late toxicity.

Microcystic meningiomas: radiological characteristics of 16 cases

As a rare subtype of meningioma, only a few reports deal with radiological characteristics of microcystic meningiomas and the problem remains controversial. The authors have analyzed the radiological findings of a series of microcystic meningiomas with a special focus on magnetic resonance images (MRI) and conventional angiography. Sixteen patients of histologically proven microcystic meningiomas were included. Analysis of preoperative MRI including signal intensity characteristics, enhancement patterns and peritumoural edema were performed and correlated with angiographic and histological findings. Peritumoural edema was graded using edema index (EI) which was defined as the ratio of VE/VT. The tumours were uniformly visualized as a high-signal mass lesion in T2-weighted images and as a low-signal mass lesion in T1-weighted images regardless of tumour vascularity shown by angiography. T2-weighted images revealed that peritumoural brain edema was severe in 11, moderate in 1, mild in 2 and negligible in 2 patients and this was closely related to the co-existence of irregular tumour marginal enhancement. However, other features failed to distinguish these lesions from other subtypes of meningioma. The cases presented demonstrate that characteristic MRI findings suggestive of microcystic meningiomas are; (1) low signal intensity mass in T1- and high signal intensity mass in T2-weighted images; (2) high incidence of peritumoural edema.

Secretory meningioma: immunohistochemical findings and evaluation of mast cell infiltration

Secretory meningiomas constitute a relatively rare subtype of meningiomas, accounting for only 1.1% at our institution, with a 6:1 predominance of female patients. This study aimed to obtain more information about the immunohistochemical characteristics of this histological entity, and to analyse the effects of histological factors such as the presence of mast cells on the radiological evidence of surrounding tumour oedema that frequently occurred in this subtype of meningioma. Fourteen cases of secretory meningioma were examined. Relevant clinical information was obtained from the patient files. Peritumoural oedema was determined either by CT or MRI scans and graded as small, moderate and severe. In order to perform the quantitative evaluation of mast cells in secretory meningiomas in a comparison with other meningiomas, 14 non-secretory meningiomas were randomly selected and used as a control group. The immunohistochemical staining of carcinoembryonic antigen was positive within the secretory droplets and the cells surrounding them in all cases. Ki 67 (MIB 1) proliferative index mean values were 2.4%, indicating low expression in all secretory meningiomas. Moreover, from our statistical analysis, there is no clear-cut pattern of various types of cytokeratins emerging in secretory meningiomas. The secretory meningiomas were characterized by a significantly increased number of mast cells as compared with non-secretory meningiomas of different grades. As the present clinical findings and laboratory results could not confirm a correlation between mast cell density and radiological evidence of oedema, further studies of mediators are warranted.

Prognostic value of proliferation index and expression of the RNA component of human telomerase (hTR) in papillary meningiomas.

Papillary meningioma is a rare subtype of meningioma that often behaves aggressively. In order to characterize factors that may influence this behavior, we chose to compare MIB-1 labeling index (LI) and telomerase RNA localization (hTR) in papillary meningiomas, meningiomas, and atypical meningiomas. LI is now often used to supplement histologic grade in the evaluation of these lesions. More recent studies indicate that increased expression of hTR is detected in many neoplastic cells, and may play an essential role in cell immortalization. The study group consisted of five papillary meningiomas (and a recurrence in one case), 11 conventional meningiomas, and eight atypical meningiomas. Conventional meningiomas showed either negative or 1 + hTR. Atypical meningiomas showed 1 + hTR. Papillary meningiomas showed the highest hTR (five of six, including recurrence, 2-3+ and one 1+). Generally, the LI was very low for conventional meningiomas (< 2%). The LI of atypical meningiomas ranged from 3-19%, mean 12%, and from 5.5-17.5%, mean 11.75% for papillary meningiomas. LI differentiated between meningiomas, and papillary or atypical meningiomas. hTR further delineated papillary (moderate to high) from atypical meningiomas (low). The combined variable of LI and hTR expression could be a useful independent prognostic indicator in patients with papillary meningioma.