Ultrastructural examination is essential for diagnosis of papillary meningioma. Mr imaging features of clearcell meningioma with diffuse. Clear cell meningiomas in spinal canal and cerebellopontine angle korean j radiol 32, june 2002 127 ab c fig. Most meningiomas are lowgrade, indolent tumors, but the more aggressive histologic subtypes, of which clear cell meningioma is one example, are optimally treated with.
Clear cell meningioma masquerading as trigeminal schwannoma. Clear cell meningioma, an uncommon variant of meningioma. The subtypes include choroid and clear cell meningioma. We now report a case of meningioangiomatosisassociated meningioma with atypical and clear cell variant. A clinicopathologic study of a potentially aggressive variant of meningioma. Hyalinization of the stroma and perivascular regions may result in extensive areas of fibrosis in older tumors. Until now 17 intracranial clear cell meningioma cases had been reported in. The study has been undertaken to document the clinicopathological features of nine cases of ccm, operated. The recurrence rate has been reported to be as high as 60%.
Clear cell meningiomas have been reported to occur at a younger age group mean age of 30 years and are more frequently located within the spinal canal see spinal meningioma and posterior fossa. This is the first case documenting arachnoid origin of. The remainder of the neuraxis was subsequently imaged with no additional. By world health organization criteria, 46 histologic features of a meningioma that warrant grade ii designation include chordoid, clear cell, or atypical histology, the latter depicted as having. We describe a granular cell tumour developing in clear cell meningioma of the falx. This variant has been reported in sites such as spinalintradural lumbar and thoracic, cerebellopontine angle, and supratentorial. Pdf clearcell meningioma ccm, an unusual subset of meningioma has. Granular and clear cells showed immunoreactivity for vimentin, epithelial membrane antigen and progesterone receptors. Supratentorial location is an unusual site of occurrence and its natural history and prognosis are not well described in the literature. Pdf pathology and molecular genetics of meningioma. Clear cell meningioma ccm, first reported by manivel and sung 1 in 1990, is a rare histological subtype of meningioma, accounting for histopathology of meningioma the following 51 files are in this category, out of 51 total. A diagnostic marker for the differential diagnosis. Atypical meningioma by histomorphology either a or b.
Grade ii meningiomas atypical are defined by one or more of the following four criteria. Clear cell meningioma ccm, a newly described subtype is categorized as who grade ii tumor. Clear cell meningioma ccm is a rare histological variant of meningioma. The clear cell meningioma is rare and a recently described histologic variant of meningioma. Grade iii anaplastic meningiomas are malignant cancerous. Histologic typing of tumors of the central nervous system. Meningioma cell proliferation was found to be inhibited by the progesterone receptor antagonist mifepristone ru486 in vitro, suggesting a treatment modality. Conclusions these results expand the spectrum of pathogenic variants in smarce1 and show that mutation screening can help to facilitate meningioma classification. Clear cell meningioma ccm is a rare variant of meningioma with an aggressive clinical course and usually occurs in the cerebello pontine angle cpa or cauda equina. Sporadic meningioangiomatosisassociated atypical meningioma. 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. Cystic meningioma should be included in the differential diagnosis of a cpa mass with atypical radiologic features, such as a large cyst and enhanced mural nodule. Meningiomas are the most common intracranial primary neoplasm in adults.
Sensations gradually returned to normal and the patient was discharged on the 10 th day. Prior to the acceptance of ccm as a distinct variant of meningioma, intraparenchymal tumors with clear cell morphology were often reported as metastatic renal cell carcinoma, oligodendroglioma, hemangioblastoma, and clear cell ependymoma, especially so when diagnoses were based solely on histomorphology. Classification of meningiomasadvances and controversies. Intermediate mitotic rate 4 mitoses10 hpf for whatever hpf means, see hpfitis. E870 41 year old woman with clear cell meningioma of the cauda equina j spinal disord tech 2005. Clear cell and chordoid variants of meningioma are associated with higher recurrence rates even in the absence of the above criteria. We present 12 cases which were diagnosed with ccm in a single institution between 1997 and 2014, and investigate their smarce1 mutation status. Distinguishing chordoid meningiomas from their histologic.
This means the tumors have a higher chance of coming back after being removed. Histopathology was suggestive of clear cell meningioma ccm. This tumor consists of sheets of polygonal cells with clear cytoplasm due to the accumulation of glycogen. Meningioangiomatosis is a rare hamartomatous lesion or meningiovascular malformation in brain. Precontrast axial ct scan shows a huge, irregular, mixed solid and cystic mass in the left cerebellopontine angle. Histomorphological spectrum of meningioma with variants. Clear cell meningioma is a rare disease entity showing a more aggressive nature, clinically, than those of other subtypes of meningioma. Media in category histopathology of meningioma the following 51 files are in this category, out of 51 total. Only 17 cases have been previously reported in children. Until now 17 intracranial clear cell meningioma cases had been reported in the english language literature.
Clearcell and chordoid variants of meningioma are associated with higher recurrence rates even in the absence of the above criteria. By summarizing the related literature, we found that the most common pathological subtype of cpa cystic meningioma is the clear cell subtype, which belongs to who grade ii. It occurs in younger persons and commonly in the spinal canal. Clear cell meningioma is a variant of meningiomas classified as who grade ii for its. A detailed knowledge of meningiomas imaging characteristics and behavior aids in correctly identifying these lesions and guiding treatment decisions. Clearcell meningioma asian journal of neurosurgery. Duralbased metastatic clear cell tumors with lowgrade morphology such as metastatic renal cell carcinoma and prostatic carcinoma may closely mimic clear cell meningioma. A case report on cystic meningioma in cerebellopontine angle. We present a case of clear cell meningioma in a 17yearold man in whom initial mr imaging showed localized leptomeningeal.
The most interesting aspect of clear cell meningioma is the high recurrence rate and agressiveness. An unusual case of clear cell meningioma deb p, datta sg j. Excludes clear cell, chordoid, papillary, and rhabdoid subtypes. Only few case reports have been described in the literature. Cytoplasmic staining for brachyury encountered in the stromal cells of this tumor, but not in the clear cell of metastatic ccrc or in the neoplastic cells of angiomatous or clear cell meningioma, may be of relevant help for the differential diagnosis of these entities. Meningioma pathology, genetics, and biology journal of. Cells were polygonal to spindle with moderate to abundant clear.
Clearcell meningioma is composed of sheets of polygonal cells with clear, glycogenrich cytoplasm. Since clear cell meningioma has only recently been recognized as a morphologic entity, its pathobiology has not been studied. We present a rare case of recurrent supratentorial ccm in a 58yearold male. The study has been undertaken to document the clinicopathological features of nine cases of ccm, operated at all india institute of medical. Mar 25, 2012 grade ii meningiomas atypical are defined by one or more of the following four criteria. Although it has bland cytologic features, it has a higher rate of recurrence than does conventional meningioma.
Smarce1 mutations in pediatric clear cell meningioma j neurosurg pediatr volume16 september 2015 297 the radiological findings, the tumor differential included nerve sheath tumor, myxopapillary ependymoma, meningioma, lymphoma, and germ cell tumor. An unusual case of clear cell meningioma article pdf available in journal of cancer research and therapeutics 54. Clear cell meningioma ccm is a rare variant of meningioma. Histology revealed tumor cells arranged mostly in diffuse sheets and at places in whorls. Smarce1 mutations in pediatric clear cell meningioma. The intensity of fgf2 expression in cytoplasm are shown in figure 2. The subtypes include papillary and rhabdoid meningioma. Nov 28, 2016 as a result, these two tumours were re. Although the spectrum of clinical and molecular genetic issues regarding meningiomas remains undefined, novel genetic alterations that are associated with tumor morphology, malignancy, or location have recently been discovered. The histopathological spectrum of human meningiomas.
Diffuse cytokeratin positivity is present in these tumors, and this contrasts with a mostly negative pattern in classic meningiomas. Recently, smarce1 mutations have been shown to cause spinal and cranial ccms. We present a case of clear cell meningioma in a 17yearold man in. Clear cell meningioma ccm, first reported by manivel and sung 1 in 1990, is a rare histological subtype of meningioma, accounting for clear cell meningioma is a rare disease entity showing a more aggressive nature, clinically, than those of other subtypes of meningioma. Clearcell meningioma ccm is an uncommon, aggressive variant of meningioma, usually affecting younger females and having predilection for infratentorial locations. Clear cell meningioma is a rare distinctive cytological subtype of meningioma with high recurrence rate and increased mortality, which necessitate frequent followup after surgical excision. Fourteen examples occurring in seven females and six males, ages 9 to 82 years mean 29 years, were examined.
Histomorphological spectrum of meningioma with variants and. Clearcell meningioma is a rare disease entity showing a more aggressive nature, clinically, than those of other subtypes of meningioma. Meningiomas account for over one third of all intracranial neoplasms, but differentiating them from certain mimics can at times be tricky. We now report a case of meningioangiomatosisassociated meningioma with atypical and clear. Clear cell meningioma is a rare subtype of meningioma seen mainly in pediatric patients. The dura is opened, and the meningioma can be seen extending en plaque over the surface of the brain. Clearcell meningioma ccm, an unusual subset of meningioma. Feb 11, 2010 prior to the acceptance of ccm as a distinct variant of meningioma, intraparenchymal tumors with clear cell morphology were often reported as metastatic renal cell carcinoma, oligodendroglioma, hemangioblastoma, and clear cell ependymoma, especially so when diagnoses were based solely on histomorphology. Clear cell meningioma is characterized by patternless sheets of clear polygonal cells.
The contribution of the mayo clinic studies the extreme vagueness and subjectivity of the criteria for atypical and anaplastic meningiomas in this scheme is clear. Smarce1 mutation screening in classification of clear cell. Jul 14, 2005 clear cell meningioma ccm is a rare variant of meningioma. In extremely rare condition, meningioma may occur together with meningioangiomatosis, and only 19 cases have been described in english literature until now. This is the first case documenting arachnoid origin of neoplastic granular cells in meningioma. No convincing predilection for either gender has been reported 1 clinical presentation. Little is known about tumour infiltrating lymphocytes in meningioma which often colocalize with t cells, enhancing t cell responses by producing antibodies, stimulatory cytokines, and chemokines and serving as local antigen presenting cells apcs. Apr 21, 2014 duralbased metastatic clear cell tumors.
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