Chordoid meningioma: a clinicopathologic study of 42 cases

The term chordoid meningiomas was first used by Kepes et al. in 1987 to describe a meningeal tumor in young patients associated with microcytic anemia and/of dysgammaglobulinemia. Such tumors were composed of spindle or epithelioid cells disposed in chordoma-like clusters and cords in a myxoid matrix and often featured a prominent lymphoplasmacellular infiltrate. Our study includes 42 chordoid meningiomas that represented 0.5% of all meningiomas operated at Mayo Clinic during the interval 1975 to 1997. The male to female ratio was 1:1 and the age range was 12 to 77 years (mean, 47.4 yrs). Only two (5.2%) occurred in children. The majority (88%) were large and supratentorial. No manifestation of systemic disease was noted. Chordoid elements comprised 10% to 100% of the tumors: 34 (81%) were more than 50% chordoid. Thirty-seven tumors (88%) were classified as typical and five as atypical. Lymphoplasmacytic infiltrates varied, being moderate in 10 cases (23.8%), mild in 15 (35.7%), and absent in 17 (40.5%). In 14 (42%) of the 33 cases with available follow up, one or more recurrences were noted. All but one recurrent tumor had been subtotally resected. In 86% of recurrent tumors, the primary lesion was more than 50% chordoid in pattern and contained little or no inflammatory infiltrate. In our experience, chordoid meningiomas are primarily tumors of adults, lack sex predilection, are unassociated with systemic manifestations, and uniformly recur when subtotally excised.     

Osteomatous meningioma report of two cases

Summary Two rare cases of osteomatous meningioma of the spinal cord are described. The capacity of these neoplasms to produce bone trabeculae represents one of the many potentialities of differentiation of meningioma cells, which may come from neural crest cells. This histogenetic derivation is sustained by the association of a meningioma with a schwannoma in the second case.From a histological diagnostic point of view, osteomatous meningioma must be distinguished carefully from a bone- infiltrating meningioma.     

Chordoid meningioma with polyclonal gammopathy. Case report

The authors present a case of chordoid meningioma in a 55-year-old woman who manifested headache and personality change. Magnetic resonance imaging of the brain and cerebral angiography demonstrated a mass in the right frontal lobe that resembled a typical convexity meningioma. However, the pathological diagnosis was chordoid meningioma, a rare subtype of this tumor that usually occurs in adolescence and is known to be associated with Castleman syndrome. A meningothelial meningiomatous pattern suggestive of a meningothelial origin was focally present, and cytokeratin-positive squamoid cells were noted in the tumor. The lesion lacked dense infiltration of lymphocytes and plasma cells. Polyclonal gammopathy was the only sign of Castleman syndrome and hypochromic microcytic anemia was absent in this case. Polyclonal gammopathy resolved completely 6 months after total removal of the mass.     

蝶骨翼脑膜瘤81例临床回顾分析

目的 总结蝶骨翼脑膜瘤的诊断、分型及手术治疗的经验和体会。方法 回顾性分析了经显微手术和病理证实的蝶骨翼脑膜瘤81例，其中内侧型43例，外侧型36例，扁平型2例，巨大型7例，大型63例，小型11例。采用改良翼点入路53例；额下翼点联合入路7例；经颧弓翼点入路15例；额颢眶颧入路6例。结果 Simpson Ⅰ级切除45例，Ⅱ级切除17例，Ⅲa级切除9例，Ⅲb级6例，Ⅳa级3例。术后本组患者颅高压、视力下降及眼球突出等临床症状均有不同程度的改善和恢复。术后随访9个月～6年，复发5例，3例行二次于术。结论 充分全面的术前评估，正确选择手术入路，熟练掌握术区显微解剖及显微手术操作，可以提高肿瘤全切率，减少术后并发症，以降低术后致残率和复发率。     

Chordoid glioma of the third ventricle

Background  Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle with both glial and chordoid features. It was first described by Brat in 1998. Since there is no detailed information available on the outcome after surgery and adjuvant treatment, we reviewed the literature. Methods  A literature search through PUBMED revealed 50 cases of chordoid glioma. Most reports were found in pathology journals. Information on the postoperative course was sometimes very limited. We reviewed the available literature and studied in detail the presenting symptoms, mortality and postoperative complications in relation to the extent of resective surgery, as well as the importance of adjuvant treatment. Conclusions  Mortality in the immediate postoperative period is 32% and is higher after gross total resection as compared to subtotal resection. Non-fatal postoperative complications are hypothalamic disorders and mental alterations. Gross total resection is the treatment of choice since no recurrence has been reported after macroscopically complete resection, but this is often difficult because of the location and adherence to the hypothalamus. The role of postoperative radiotherapy is uncertain. There is some indication that radiosurgery with or without conventional irradiation is superior to conventional radiation alone. Planned subtotal resection followed by stereotactic radiosurgery can be a safe and effective alternative in a patient in whom gross total resection is considered to be too risky. There is no report on the use of chemotherapy in the treatment of chordoid gliomas. More information about the optimal treatment strategy is needed, and more reports are also needed.     

A Case of Subcortical Meningioma

Subcortical meningioma, which has been reported as meningioma without dural attachment, a cerebral subcortical lesion, is extremely rare. Very few findings of radiological examination of subcortical meningioma have been described. Pre-operative differentiation of this lesion is generally difficult. We characterize the peritum oural oedema adjacent to the meningioma in this region, and we suggest that radiological findings of peritum oural oedema contribute to differential diagnosis of subcortical meningiomas.     

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.     

Thoracic spine intra- and extradural dumbbell-shaped meningioma: Case report and extensive review of the literature with 21 cases

Introduction

Spinal dumbbell-shaped meningioma is a rare condition usually mistaken preoperatively for schwannoma. The present study reported a case of dumbbell-shaped meningioma, with an extensive review of literature.

Chordoid glioma: report of two rare examples with unusual features

Summary Background. Chordoid glioma, a rare tumour of the third ventricle, represents a distinct clinico-pathologic entity. Thirty nine examples have been described in the literature, mostly in females and in the third ventricle. The clinical presentation is variable but they tend to occur mostly in adults. There is only one report of a chordoid glioma in a 12 year old child. Finding. This paper describes two examples of chordoid glioma in a seven year old female child and a 70 year old male respectively. Radiologically, the paediatric chordoid glioma was located in the juxtaventricular region in the occipital horn of the lateral ventricle and was of mixed density whereas the adult patient had a typical third ventricle location with homogenous contrast enhancement. Gross total surgical removal was achieved in both but the adult patient died post-operatively due to intra ventricular bleeding and bacterial meningitis. The younger patient is doing well at the last follow up two years post-operatively. Microscopically, both showed the classic morphology of chordoid glioma. Ultrastructural examination was suggestive of ependymal differentiation. Conclusion. The younger age and unusual location are some of the rare features which need documentation and have not been described earlier. We propose that chordoid glioma is a variant of an ependymoma (WHO grade II) which arises from tanycytes and should be included in the WHO classification of brain tumors. Correspondence: Dr. Mehar Chand Sharma, M.D., Associate Professor, Department of Pathology, AIIMS, New Delhi 110029, India.     

Intracranial/Extracranial meningioma arising in the hypoglossal canal: case report.

A case of a patient with a posterior fossa meningioma extending through the hypoglossal canal to the cervical region as described in this article has not been previously described in the literature. Investigations and surgical management are outlined and pathological classifications are discussed. A literature review including recent reports of extracranial meningiomas is presented. Extracranial meningiomas are exceedingly rare and a high index of suspicion is necessary to make the diagnosis.     

Fractionated stereotactic radiotherapy for optic nerve sheath meningioma: eight cases

Optic nerve sheath meningioma (ONSM) accounts for one-third of primary optic nerve tumors, and 2% of all meningiomas. ONSM must be distinguished from other meningiomas, in particular from cavernous meningiomas because of the different prognosis and treatment. The most frequent clinical sign is a progressive or sudden unilateral visual loss. Treatment of ONSM is still subject to discussion. This report covers a series of eight ONSM patients treated with fractionated stereotactic radiotherapy. MATERIAL AND METHODS: Between 2000 and 2006, we managed eight patients with ONSM. The average patient age was 47 years. There were five women and three men. The most frequent clinical signs were visual loss (100%), proptosis (35%), diplopia (25%). One patient was initially treated with surgery. All patient have been treated by fractionated stereotactic radiotherapy. 45 Gy in 25 fractions were delivered on the meningioma area at a rate of 5 fractions of 1.8 Gy per week. We used a Brainlab framework associated with a thermo-formed mask. A computed tomography then magnetic resonance imaging was obtained for each patient. The data was merged and planning took place on a Brainlab dosimetric console. The treatment was performed with a head-only Varion linear accelerator, with a Brainlab multi-blade collimator. RESULTS: The average follow-up was 27 months. Each patient had a complete radiological and ophthalmologic exam every 3 months during the first year, then every 6 months thereafter. Tumor control rate was 100%. Vision was re-established in five patients and three patients had improvement, including one patient during treatment. 100% of proptosis and diplopias regressed. No side effect was reported. CONCLUSION: This is still a preliminary study, but the results suggest that fractionated stereotactic radiotherapy may emerge as a primary treatment for ONSM, delaying surgery, which has proven functionally disappointing.     

Spinal interdural meningioma developing between two layers of the dura mater. Case report

Meningiomas seldom occur in children. Here a case is reported of spinal cord compression from an interdural meningioma developing between two layers of the spinal dura mater in a young child.     

Hydroxyurea treatment for unresectable meningioma

BACKGROUND: Management of unresectable progressive meningioma remains controversial and constitutes a major challenge since therapeutic options including chemotherapy and hormone modulation are limited. Recent data have suggested that hydroxyurea treatment may have an antitumoral effect. The purpose of this prospective phase II study was to evaluate the efficacy of hydroxyurea treatment for unresectable progressive meningioma. METHODS: From 1997 to 1999, consecutive patients presenting unresectable meningioma with clinically and/or neuroradiologically documented progression were considered for entry into this protocol. Previous radiotherapy was not a mandatory inclusion criteria. Treatment consisted of continuous oral administration of hydroxyurea at a dose of 20 mg/kg per day. Follow-up assessment included physical examination, computed tomography (CT), and magnetic resonance imaging (MRI) performed every three months, as well as regular blood testing. The primary endpoint was documentation of objective response by MRI or CT. RESULTS: The intent-to-treat population was 43 patients with at least 18 months follow-up. Median age was 60.4 years. Twenty-eight patients had undergone surgery following initial diagnosis. The meningioma was located in the skull base in 67% of patients. Histology was benign in 18 and atypical in 10. The eligible population included 36 patients with documented progressive disease at the time of inclusion; with progression documented clinically in 29 (67.5%) and/or radiologically in 20 (46%). In 7 patients, clinical or radiological progression could not be confirmed. The intent-to-treat analysis at median 26 months follow-up revealed objective response to hydroxyurea in only 3 patients (7%) including one on the basis of improvement in visual symptoms and two on MRI analysis. Progressive disease was observed clinically or radiologically in 26 patients (60.5%). Of the eligible population (n=36), 2 achieved an objective response and 13 (36%) exhibited stabilization under hydroxyurea therapy, while 21 (58%) progressed under treatment. Overall tolerance was good but anemia (grade I-II) and asthenia (grade I-II) were observed in 28% and 23.5% respectively. Treatment was discontinued in 3 patients because of chronic skin toxicity in one and anemia and asthenia in two. CONCLUSION: Hydroxyurea treatment is of marginal efficacy for meningioma and must not be considered as an alternative if radiotherapy or surgery is feasible. New efficient medical treatments are still required for progressive meningiomas.     

Intraparenchymal meningioma: an unusual radiologic and anatomic presentation of a meningioma. Case report

The authors describe a case of a completely intrinsic brain tumor which was resected totally and which proved to be a meningioma. In correlation with this anatomic diversity, the MRI findings also significantly differed from that of the known radiologic features of classical meningioma.     

Glycogen-rich meningioma. Case report and short review

A case of meningioma with cytoplasm rich in glycogen granules is described as an atypical type of meningotheliomatous meningioma.     

Intrasellar meningioma

Two purely subdiaphragmatic intrasellar meningiomias are described. The rarity of this unusual intrasellar tumor is stressed, and the differential diagnosis of intrasellar meningioma versus pituitary adenoma is discussed. The good results of the rhinoseptal route for surgery are emphasized.     

Intramedullary clear cell meningioma

Summary Intramedullary clear cell meningioma (CCM), which is more aggressive than other meningioma variants, is extremely rare. To date, only one case of such a spinal tumour has been documented. We report the first case of an intramedullary CCM originating in the thoracic region of the spinal cord.