A rare subtype of meningioma: Case series of anaplastic meningioma and review of the literature.

RATIONALE: Anaplastic meningioma, a rare subtype of meningioma, has malignant morphological characteristics and a World Health Organization (WHO) grade of III. PATIENT CONCERNS: In this report, we present findings from 6 cases of anaplastic meningioma. DIAGNOSES: Pathological examination of the tumors, including hematoxylin and eosin staining and immunohistochemical staining, was performed. Of the six cases of anaplastic meningioma, two were recurrent tumors from original seminoma with a WHO grade of I. Histologically, three cases had carcinoma-like morphology, one case had sarcoma-like morphology, and two had two kinds of tissue structures: carcinoma-like tumor cell nests and areas with spindle tumor cells. Necrosis was detected in most cases (5/6). Ki67 index was high and varied from 20% to 70%. INTERVENTIONS: All the patients received surgery. 3 patients received adjuvant radiotherapy. 1 patient received chemotherapy. OUTCOMES: 4 patients had no recurrence at follow-up of 19, 30, 46 and 54 months after the last surgery. 1 patient had recurrence 3 months after the last surgery. 1 patient died 12 days after the last surgery. LESSONS: This malignant subtype can be secondary to a WHO grade I meningioma after a long quiescent period. Necrosis was common in the tumor tissues, and Ki67 index was usually high. For patients with a history of meningioma, including benign cases, regular physical examination is important for early detection of tumor recurrence and malignant transformation.

Secretory meningiomas: immunohistochemical pattern of lectin and ultrastructure of pseudopsammoma bodies.

Secretory meningioma is an infrequent histological subtype of benign, WHO grade I meningioma, that is characterized by focal epithelial and secretory transformation of meningothelial cells. The leading histopathological feature of neoplastic tissue is the presence of eosinophilic hyaline inclusions, defined as "pseudopsammoma bodies". These inclusions are mostly intracytoplasmic, different in size and often multiple. They are stained with periodic acid-Schiff (PAS) and are immunopositive for epithelial and secretory markers. The aim of this study was to determine the pattern of lectin bindings and ultrastructural features of secretory meningiomas. The examination was performed on 8 cases of secretory meningiomas that occurred in women and were mostly associated with prominent peritumoural oedema. Histologically, the tumours exhibited numerous eosinophilic, PAS positive pseudopsammoma bodies. Immunohistochemical studies revealed a strong, ring-like cytokeratin expression around the pseudopsammoma bodies. The inclusions were CEA and EMA positive but negative for vimentin. The immunolabeling with four lectins (PNA, SBA, Con A and DBA) was studied. The majority of pseudopsammoma bodies and surrounding tumour cells were strongly labelled with PNA and SBA. Immunolabelling with Con A showed irregular staining with high intensity in small inclusions. Immunostaining with DBA was seldom positive in inclusions and negative in the tumour cell cytoplasm. Ultrastructure of pseudopsammoma bodies exhibited advanced heterogeneity. The size of inclusions and the content of intracytoplasmic lumina varied greatly. Some pseudopsammoma bodies seemed to be located extracellularly and lacked the obvious lumina. Our ultrastructural study and lectin binding pattern support the unique epithelial and secretory transformation of neoplastic cells connected with their altered glycosylation.

The rim pattern of meningioma on 3D FLAIR imaging: correlation with tumor-brain adhesion and histological grading.

PURPOSE: We evaluated the rim patterns of intracranial meningiomas on nonenhanced (NE) and contrast-enhanced (CE) 3-dimensional fluid-attenuated inversion recovery (3D FLAIR) imaging on 3-tesla magnetic resonance (MR) imaging to clarify the associated imaging and pathological findings and the value of the rims in predicting tumor cleavability. METHODS: Thirty-two patients with meningioma underwent tumor excision. We classified the rim patterns on 3D FLAIR, subdivided into "rim-NE," a rim with relatively low signal intensity on NE 3D FLAIR and "rim-CE," a rim with relatively high signal intensity on CE 3D FLAIR, into 4 grades by their extent from 0 (no rim visible) to 3 (rim visible over most of the tumor-brain interface) and correlated them with tumor size, grade of peritumoral brain edema, presence/absence of pial supply, grade of tumor-brain adhesion, and histological findings. RESULTS: On NE 3D FLAIR, "rim-NE" was graded 0 in 5 patients, one in 5, 2 in 6, and 3 in 16. On CE 3D FLAIR, "rim-CE" was graded 0 in one patient, one in 13, 2 in 4, and 3 in 14. The grade of "rim-NE" correlated negatively with the grade of brain edema (P = 0.023) and positively with surgical tumor-brain cleavability (P < 0.001). It also correlated with the amount of connective tissue at the tumor-brain interface histologically (P = 0.041). Furthermore, a lower grade of "rim-NE" was more often seen in atypical than benign meningioma (P = 0.003). Although "rim-CE" was more prominent in tumors with pial supply on digital subtraction angiography (DSA) (P = 0.002), it was not useful in predicting tumor-brain adhesion or histological tumor grading. CONCLUSION: The rim pattern of meningioma on NE 3D FLAIR can predict surgical cleavability and histological tumor grading. A higher grade of rim pattern on CE 3D FLAIR suggests prominent pial supply to the tumor but has no added value in predicting tumor-brain adhesion and histological tumor grading.

A case of clear cell meningioma with tyrosine-rich crystals.

We present a case of clear cell meningioma with unusual clinical and pathologic features. The patient was a 54-year-old man who underwent laminectomy and durotomy for an intradural tumor in the lumbar spinal canal. Sections showed a predominance of dense collagenous tissue with irregularly shaped and irregularly sized magenta-colored extracellular deposits. On electron microscopy, these deposits were osmiophilic and "petaloid." The final diagnosis of clear cell meningioma rested on relatively inconspicuous intervening nests of glycogen-containing clear cells that were positive for epithelial membrane antigen. The unusual extracellular deposits seen in this case have previously been characterized as tyrosine-rich crystals of the type most commonly seen in salivary gland tumors. Recognition of this tumor as a clear cell meningioma, despite misleading clinical features and initially challenging histologic findings, is not only a matter of diagnostic accuracy but also imparts important prognostic information.

Ultrastructure of meningiomas: autophagy is involved in the pathogenesis of "intranuclear vacuoles".

We report here common ultrastructural findings in a short list of meningiomas. At the lower power magnification, a tumour consisted of elongated or round cells and innumerable cellular processes connected with diverse intercellular junctions. Nuclei presented no specific features, nucleoli were infrequently seen and heterochromatin was clumped beneath the nuclear membranes. In a case of clear cell meningioma, cells were of watery cytoplasm. Occasionally, immobile cilia, completely ensheathed by the cytoplasm and anchored by blepharoplasts were seen; as we did not encounter those rare cilia in cross-sections, no further insight into their inner microtubular-doublet structure was possible. The cytoplasm of the cells and the processes were filled with the intermediate filaments. In the intercellular space, collagen fibrils and electron-dense material was occasionally observed. The majority of the tumour samples were filled with processes. Several types of junctional complexes were observed. The most frequent were desmosomes and in the proper plane of section their whole pentalaminar structure was readily discernible. However, robust tonofilaments, as seen in epithelial neoplasms, were not observed. Those desmosomal junctions were either completely symmetric or asymmetric, but the exact symmetry could not be judged without the assistance of a goniometer. Some junctional complexes were more elaborate, with desmosomal junctions separated by a tight apposition of membranes, which suggests tight junctions. "Intranuclear vacuoles" well-visible even at low power were defined as indentation of the cytoplasm into the nucleus. Within these vacuoles, autophagic vacuoles and lysosomal bodies were seen, suggesting an active macroautophagy process. In 2 cases, severe lipidization of meningioma cell cytoplasm was observed. In a case of anaplastic meningioma, a mitotic figure was found. In another case, empty rectangular spaces in the cytoplasm, suggestive of pre-existing crystalloid structures, were seen.

Pediatric rhabdoid meningioma: a morphological, immunohistochemical, ultrastructural and molecular case study.

Rhabdoid meningioma is an uncommon meningioma variant categorized as WHO grade III. The majority of cases occur in adulthood. Herein, we describe a right fronto-temporal rhabdoid meningioma affecting a 3-year-old boy. The lesion measured approximately 4 cm in diameter and incorporated the ipsilateral middle cerebral artery. Sub-total surgical excision of the mass was performed. Histologically, the tumor was mainly composed of globoid plump cells with inclusion-like eosinophilic cytoplasm, peripheral nuclei, prominent nucleoli and occasional intra-nuclear cytoplasmic pseudo-inclusion. The cells appeared in many areas loosely arranged and focally disclosed a papillary architecture. At immunohistochemistry, the tumor cells were EMA, vimentin, HHF35, PgR, INI-1 and p53 positive. The proliferative index (Mib-1) was 15% in the most positive areas. Ultrastructurally, tumoral cells showed an abundant cytoplasm, which was filled with numerous intermediate filaments. Desmosomal junctions were seen. RT-PCR revealed the presence of NF2 gene expression. Molecular study did not indicate alterations of the INI-1 gene, whereas it showed the presence of Pro72Arg in exon 4 at heterozygous state in the TP53 gene. Morphologic features along with immunohistochemical, ultrastructural and molecular results were consistent with the diagnosis of rhabdoid meningioma. The patient was treated with chemotherapy. The lesion remained stable after 33 months of follow-up. Rhabdoid meningiomas rarely occur in children. Owing to its rarity, each new case should be recorded to produce a better clinical, pathological, molecular, prognostic and therapeutic characterization of this lesion.

Ultrastructural profile of microcystic meningioma.

OBJECTIVE: Microcystic meningioma was originally classified as a subtype of meningioma by the World Health Organization classification of brain tumours in 1993, and accounts for 1.6% of intracranial meningiomas. This subtype is a variety of meningioma in which micro- and macro-cysts are diffuse. The morphologic characteristics are well defined, while the histogenetic mechanism that give rise to these patterns remain unclear. MATERIALS AND METHODS: The authors present an electron microscopic study of an unusual case of fronto-temporal microcystic meningioma, manifesting as history of headache, right paresis and dysphasia in a 73-year-old female. Computer tomography revealed a large hypodense mass in the left fronto-temporal region, with slight contrast enhancement. RESULTS: Ultrastructural observation showed complex alterations among small vessels and intratumoral capillaries in a background of severe modification in vessel permeability. CONCLUSIONS: This electronic microscopy study documented that growth of the cyst was due not only to accumulation of in extracellular fluid, but also to cytolysis consequent to ingravescent hydropic degeneration.

Secretory meningioma of the middle ear: a light microscopic, immunohistochemical and ultrastructural study of one case.

A 66-year-old woman was referred with left hearing loss. A probable diagnosis of left secretory otitis media with effusion was formulated. A left myringotomy was performed to remove hyperplastic hard tissue from the tympanic cavity. A high resolution CT scan of the temporal bone disclosed a soft-tissue mass completely involving the mastoid and tympanic cavity, surrounding the ossicular chain which appeared spared with no signs of infiltration. The histopathologic, immunohistochemical and ultrastructural response was secretory meningioma, a rare variant of conventional meningothelial meningioma in atypical sites.

A report of supratentorial leptomeningeal hemangioblastoma and a literature review.

Hemangioblastomas of the CNS are solid or cystic vascular-rich tumors, most common in the cerebellum, less frequent in the brainstem or spinal cord, and rare in supratentorial locations with meningeal involvement. We document a case in a 58-year-old man who presented with about 2 months history of motor weakness and speech dysfunction. The tumor was a heterogeneously enhanced dural-based tumor with high vascularity and perifocal edema in the left frontal lobe. The tumor was completely removed followed by embolization and preoperative radiotherapy. Histologic examination revealed a hemangioblastoma with features resembling angiomatous meningioma. Immunohistochemistry for epithelial membrane antigen (EMA) and S100 may be helpful to make differential diagnosis. Electron microscopic investigation is essential to differentiate between meningiomas and other leptomeningeal tumors.

Microcystic meningioma in a dolphin (Delphinus delphis): immunohistochemical and ultrastructural study.

A wild common dolphin was found stranded on the French Atlantic coast. At necropsy, an intracranial grey- to tan-coloured mass (7 x 5 x 4 cm) was found at the right cerebellopontine angle, compressing the right cerebellar hemisphere, the brainstem and the occipital lobe of the right cerebral hemisphere. Microscopically, the tumour was composed of small lobules of polygonal to elongated neoplastic cells with multifocal areas of stellate and vacuolated cells. Neoplastic cells strongly expressed vimentin, S-100 protein and neuron-specific enolase. They were rarely positive for cytokeratin. Ultrastructurally, the neoplastic cells displayed all the diagnostic features of meningiomas and in some areas showed long cytoplasmic processes delimiting extracellular spaces. The immunohistochemical and ultrastructural features were consistent with the histopathological diagnosis of a microcystic meningioma. This is the first report of a meningioma in dolphins or in any other cetacean species.

Meningeal melanocytoma.

BACKGROUND: Meningeal melanocytoma was first described over 30 years ago as a benign tumour derived from melanocytes. Since then, data suggest that its mode of presentation is variable without a clear predilection for any particular site in the neuroaxis. Although classified as a benign tumour, this tumour shows a marked tendency towards reduced survival following subtotal resection and transformation over time in a limited number to malignant melanoma. Incomplete resection of these tumours without postoperative radiotherapy has only a 42% 5-year survival rate. Its classification as a benign tumour should be revised, given the published 5-year survival data. ILLUSTRATIVE CASE: We report a fatal case of meningeal melanocytoma in the cerebello-pontine angle in a 10-year-old child. This case exemplifies the vascular nature of these lesions even with minimal vascular blush on angiography. An updated literature search is presented, the results of which highlight the need for close follow-up and adjuvant treatment following subtotal resection.

Intracerebral schwannoma clinically and radiologically mimicking meningioma.

A case of intracerebral schwannoma (ICS) occurring in a 33-year-old woman is presented. The patient's history of headache, numbness, tingling and the recent development of weakness of the right upper extremity with right facial droop began during pregnancy. Magnetic resonance imaging (MRI) showed a 4 x 2 x 2 cm heterogeneous, gadolinium-enhanced mass at the left frontoparietal junction, with peritumoral edema and a dural-based attachment. During her pregnancy, the mass increased in size. The surgically resected specimen consisted of lobulated, somewhat gelatinous soft tissue. Microscopically, the tumor demonstrated classic biphasic Antoni type A and B patterns, admixed with degenerative changes. Immunohistochemically, the neoplastic cells were positive for S-100 protein (diffuse and strong), CD34 (primarily in Antoni B areas), glial fibrillary acidic protein (GFAP; weak and diffuse) and calretinin (mainly in Antoni A areas), while none was positive for CD31, estrogen and progesterone receptors, bcl-2, or epithelial membrane antigen (EMA). Ultrastructurally, basal laminae and Luse bodies were identified. The differential diagnosis includes fibrous meningioma, solitary fibrous tumor, and ICS. Twenty-seven cases of ICS were reviewed in which the histological diagnosis was confirmed immunohistochemically or ultrastructually, and the cases were summarized (including the present case). A combined use of immunostains (S-100 protein, EMA, CD34, and maybe calretinin) is of great help in distinguishing ICS from its histological mimickers.

Primary chordoid meningioma of lung.

Primary meningiomas of the lung are rare. Most pulmonary meningiomas are typical syncytial or transitional meningiomas with smaller numbers of fibrous-type tumors. Herein, we report an unusual pulmonary tumor with the microscopic, immunohistochemical, and ultrastructural characteristics of a chordoid meningioma. The tumor was composed of cords and fascicles of small- to medium-sized spindle and epithelioid cells with eosinophilic cytoplasm and round nuclei with finely dispersed chromatin. The tumor cells were surrounded by an abundant mucoid, vacuolated stroma. The periphery of the tumor was enveloped by a significant lymphoplasmacytic infiltrate. The neoplastic cells were positive for vimentin and epithelial membrane antigen only. The unusual morphology of the tumor caused significant diagnostic difficulties. The differential diagnosis included inflammatory myofibroblastic tumor, spindle cell myoepithelioma, and extraskeletal myxoid chondrosarcoma. To the best of our knowledge, this is possibly the first description of an extracranial or intrapulmonary chordoid meningioma.

Microscopic anatomy of the brain-meningioma interface.

We analyzed the relation between meningioma and the brain in 50 surgical cases. So-called capsule formation was seen in 20 meningiomas, of which 13 were categorized as thin and 7 as thick. In 21 meningiomas the arachnoid membrane was intact, and 10 meningiomas had no underlying arachnoid membrane. The other 19 tumors showed partial disruption of the arachnoid membrane. The degree of arachnoid disruption correlated with the tumor grade, perifocal edema, pial blood supply on angiography, and tumor size. The existence of brain invasion correlated with the tumor grade and partially with tumor size. In case of invasive tumor, GFAP-positive cells were found deep in the tumor, usually in contact with blood vessels. The axons in gliotic brain often showed degenerative changes such as ballooning or varicose swelling. Meningiomas were usually demarcated by a basement membrane that was collagen type 4 (Col4)-positive. However, atypical and anaplastic meningiomas usually lacked Col4 staining at the interface. In two benign meningiomas that looked like an invasive growth, Col4 staining was seen above the brain. A pia mater-like structure covered the tumor surface in both cases. We could not demonstrate a relation between the expression of matrix metalloproteinase (MMP)-2 or MMP-9 and arachnoid disruption or brain invasion.

Meningioma with eosinophilic granular inclusions.

Rare meningiomas have been described that contain eosinophilic inclusions that have a granular or granulofilamentous ultrastructure. We describe a 66-year-old woman who developed a planum sphenoidale meningioma. Histologically, the tumor was composed of meningothelial cells arranged in fascicles and whorls, typical of a well-differentiated meningioma. Many tumor cells contained round intracytoplasmic eosinophilic inclusions that were periodic acid Schiff-negative and red on Masson trichrome. The inclusions were immunopositive for vimentin, and were immunonegative for epithelial membrane antigen, smooth muscle actin, desmin and type IV collagen. Ultrastructural examination showed the inclusions were composed of round to oval, well-demarcated, non-membrane-bound, osmiophilic granular material. The inclusions within this tumor had histochemical, immunohistochemical and ultrastructural properties not described in other reported meningiomas with eosinophilic granular or granulofilamentous inclusions.

Sclerosing meningioma: immunohistochemical analysis of five cases.

Sclerosing meningioma is a rare morphologic subtype of meningioma and may be mistaken for atypical or malignant meningioma and astrocytoma or schwannoma because of marked collagen deposits and a sparse population of cells with little resemblance to meningothelial cells. Authors describe the histopathologic and immunophenotypic features of five cases of sclerosing meningioma. Histologically, all the cases consisted of paucicellular collagenous tissue containing spindle cells with or without small foci of meningothelial cell proliferation. The morphology and immunohistochemical profile of the spindle cells were different from those of conventional meningothelial cells. The meningothelial cells showed a typical immunoreactivity of conventional meningiomas, while the spindle cells displayed a strong expression of vimentin. The Ki-67 labelling index was uniformly low in all cases, and none of cases expressed p53 protein. In summary, the recognition of meningothelial cells in massively sclerotic lesions is helpful for a correct diagnosis. In the cases with a total absence of meningothelial cells, however, the vague collagenous whorls are more diagnostic rather than immunohistochemistry. Considering association with clear cell meningioma, prospective and retrospective long-term follow-up is necessary for deciding whether reminiscent clear cell meningiomas should be separated from sclerosing meningioma or not.