Pyrogenic cytokine interleukin-6 expression by a chordoid meningioma in an adult with a systemic inflammatory syndrome. Case report and review of the literature

Chordoid meningioma is a rare meningothelial tumor characterized by chordoma-like histological features with lymphoplasmacellular infiltration. This tumor is often seen in children, but not in adults, with a systemic inflammatory syndrome (iron-resistant microcytic anemia and/or dysgammaglobulinemia) and very rarely with a persistent moderate hyperthermia. In the present report the authors describe a temporal chordoid meningioma in a 30-year-old woman who presented with fever, headache, and a serological inflammatory syndrome. The clinical symptomatology, chiefly the fever, disappeared immediately after removal of the tumor. To the authors' knowledge, only one similar patient with such clinical presentation and response to surgery has been mentioned in the literature. Interestingly, at immunohistochemical examination, the neoplasm showed focal positivity for the pyrogenic cytokine interleukin-6. The capacity of the tumor to produce this pyrogenic cytokine could explain both the patient's clinical presentation and her response to the surgical management.     

A case of chordoid meningioma]

Chordoid meningioma is a very rare variant, especially in adults. We report an adult case of chordoid meningioma. A 52-year-old man was admitted to our hospital due to right hemiparesis. MRI revealed a left temporal convexity mass that showed two structures; the cerebral layer was shown as an isointensity area on T1WI and T2WI, and the dural layer seemed relatively hypointense on T1WI and hyperintense on T2WI. The tumor was well enhanced with gadolinium, especially on the dural side. A left external carotid arteriogram showed tumor stains with feeding vessels from the left middle meningeal artery. The tumor was totally removed via fronto-temporal craniotomy. Histological examination of the surgical specimen revealed two different structural components, meningothelial meningioma on the cerebral side, and chordoid meningioma on the dural side, consisting of clustering spindled cells and partly vacuolated ones in the mucoid stroma. In immunohistochemical examination, tumor cells showed positive staining for vimentin and epithelial membrane antigen (EMA), and negative for cytokeratin. From the above findings, this case was diagnosed as chordoid meningioma in an adult, a very rare variant of meningioma.     

Intraparenchymal meningioma in a child

A 10-year-old boy presented with an intraparenchymal meningioma, which had no attachment to the dura, manifesting as grand-mal seizure. Neurological examination showed no abnormalities. Magnetic resonance (MR) imaging revealed a round, well demarcated mass in the left frontal lobe, which was homogeneously enhanced. The tumor appeared to be intraaxial and caused marked peritumoral white matter edema. At operation, the mass was totally embedded in the frontal lobe and gross total resection was accomplished. The histological diagnosis was meningothelial meningioma with chordoid components in World Health Organization grade I. His postoperative course was uneventful and postoperative MR imaging revealed no residual tumor. Intraparenchymal meningioma should be considered in the differential diagnosis of an intraaxial lesion in a child.     

Early malignant transformation of a petroclival meningothelial meningioma

Although some authors have reported the malignant transformation of meningiomas, there has been no previous report describing that a meningothelial meningioma transformed into an atypical meningioma within 1 year. This report documents a 57-year-old woman who presented with right hearing disturbance. Magnetic resonance imaging revealed a right petroclival meningioma. The tumor was subtotally removed and was diagnosed to be a meningothelial meningioma. Seven months after surgery, a recurrence of the tumor was confirmed. The diagnosis of this recurrent tumor was an atypical meningioma. The MIB-1 index and the percent of p53 protein-positive cells in the primary tumor were 4.6% and 35.4%, respectively, whereas those of the recurrent tumor were 34.7% and 33.1%, respectively. A chromosomal DNA copy number loss was observed on 1p, 6q, 10, 14q, and −22q detected in both the primary and the recurrent tumors. These results suggest that the present case had a potentially malignant tumor in the early stage, although it had the histological features of benign meningiomas. An evaluation of the MIB-1 index, as well as the expression of p53 and chromosomal aberrations, may be useful for predicting the malignant transformation of meningiomas.     

Chordoid meningioma--case report

A 69-year-old female presented with a de novo lesion detected incidentally. Computed tomography demonstrated an isodense mass in the left parietal convexity with peritumoral edema, with homogeneous enhancement by contrast medium. Magnetic resonance imaging showed the left parietal convexity tumor as isointense on T(1)-weighted imaging and homogeneously hyperintense on T(2)-weighted imaging, with homogeneous enhancement and dural tail sign after intravenous administration of gadolinium-diethylenetriaminepenta-acetic acid. The tumor was totally removed. The histological diagnosis was chordoid meningioma. Combined immunohistochemical staining was helpful to differentiate chordoid meningioma from other chordoid neoplasm.     

Rhabdoid papillary meningioma.

We have studied an uncommon case of rhabdoid papillary meningioma in a 15-year-old boy with a dura-based mass arising in the left posterior fossa. The patient exhibited prominent extracranial extension during the past 6 years, consisting of a mixture of both perivascular pseudopapillary growth and rhabdoid cytologic features of neoplastic meningothelial cells. The meningothelial features were evidenced by the focal whorl formation of tumor cells, coexpression of epithelial membrane antigen and vimentin, and ultrastructural findings of interdigitated cytoplasmic process and intercellular junction. However, the regional and histologic resemblances to ependymoma were further complicated by unexpected focal expression of glial fibrillary acidic protein, neurofilament, and alpha-smooth muscle actin of the tumor cells. The rhabdoid morphology was characterized by sheets of tumor cells with eccentric nuclei and abundant eosinophilic cytoplasm with often recognizable intracytoplasmic hyaline inclusions. These inclusions revealed ultrastructural paranuclear whorls of intermediate filaments, ruling out the other forms of intracytoplasmic eosinophilic inclusions resembling rhabdoid morphology. Diagnosis of an unusual rhabdoid papillary meningioma with aggressive behavior is resoluble by immunohistochemical and ultrastructural analyses.     

Lipomatous meningioma: report of 2 cases and review of the literature

BACKGROUND: Lipomatous meningioma is a rare but, most of the time, benign tumor. Its pathogenesis is still debated: it is usually considered to be part of the metaplastic meningioma, but several authors recently suggested that fat accumulation inside the tumor was related to metabolic disorders of the meningothelial cells. CASES DESCRIPTION: We report 2 cases of lipomatous meningioma. Both patients were women older than 60 years. One patient suffered from headache and seizures, the other one presented with behavioral disturbance. Radiological features depended on the amount of fat accumulation within the tumor. Surgical treatment allowed complete resection in both cases without any complications. Both meningiomas were of transitional-type and were apparently composed of 2 populations of cells: meningothelial cells and lipid-laden cells resembling mature adipocytes. Immunohistochemical study showed that lipid-laden cells expressed EMA, CD99, and progesteron receptor, favoring a meningothelial differentiation rather than an adipocytic lineage. CONCLUSION: Our study strongly suggests that lipomatous meningioma results from an accumulation of lipid inside meningothelial cells rather than a true metaplasia.     

A case of vacuolated meningioma

A rare case of falx meningioma with extensive vacuolization was reported. A 57-year-old woman was admitted to Okayama University Hospital because of numbness in her left lower limb. A plain CT scan showed a hypodense mass in the right frontal region with marked enhancement after contrast medium injection. MRI on T1 weighted images showed low intense mass with the so-called "peritumoral band" of low intensity at the periphery of the mass. Angiogram showed a mass effect and a tumor stain fed by the right pericallosal artery and left middle meningeal artery. The mass was excised en bloc. Microscopic examination demonstrated tumor cells with varied sized vacuoles, which were mostly fat-negative, except for small islands with typical meningothelial cells in the periphery of the tumor. Ultrastructurally, there were two kinds of vacuole in the tumor cells. The smaller, round vacuoles were in the cytoplasm, while the larger ones were in the extracellular space. The cell processes had developed junctional complex, and numerous cytoplasmic filaments were evident. With these findings, this mass was diagnosed as vacuolated meningioma. It is important especially in frozen section not to misdiagnose the tumor as liposarcoma, chordoma, metastatic brain tumor and so on.     

Progression of Intracranial Meningioma during Luteinizing Hormone-Releasing Hormone Agonist Treatment for Prostate Cancer: Case Report

The authors describe a male patient who developed a large intracranial meningioma during the hormone therapy for pre-existing prostate cancer. A 70-year-old man received a brain check-up, and no intracranial abnormality was detected. Five months later, prostate cancer was diagnosed, and he underwent prostatectomy. Leuprorelin acetate, a luteinizing hormone-releasing hormone (LH-RH) agonist, was subsequently administered to the patient once a month for 3 years. After that he presented with a large parasagittal mass, which was excised. The tumor was histologically diagnosed as meningothelial meningioma, and LH-RH receptors were verified immunohistochemically in the cytoplasm of the tumor cells. Leuprorelin acetate may accelerate the rapid growth of meningioma in this patient.     

Third ventricular meningioma--case report

A 63-year-old man presented with headache. Magnetic resonance imaging showed a mass lesion homogeneously enhanced with gadolinium, which occluded the route from the third ventricle to the aqueduct. The patient underwent surgery for removal of the tumor via the right frontal transcortical-transventricular approach to the third ventricle via the transchoroidal route. Intraoperative diagnosis was meningioma. Total removal of the tumor was achieved in piecemeal fashion (Simpson grade 1). The histological diagnosis was meningothelial meningioma. The patient was discharged without neurological deficits. Third ventricle is a rare and difficult site to remove tumor totally. However, total removal was needed in this case of benign meningioma, so the operative strategy and the differential diagnosis before operation is considered to be very important.     

Exacerbation of radiation induced meningioma due to hemorrhage after cerebral angiography: a case report

We report the case of a 34-year-old woman who exhibited acute deterioration in her condition after cerebral angiography for evaluation of a large meningioma. She had undergone surgery and irradiation for a glioma in the right occipital lobe 23 years before this episode. She experienced incapacity at work. On CT and MRI, a large meningioma was detected on the left frontal convexity; this tumor was thought to be radiation-induced. Cerebral angiography was performed to assess the vascularization of the tumor. Her condition began to deteriorate 2.5 h after the cerebral angiography. CT revealed an increase in the mass of the tumor, and a high density area in the tumor. We immediately removed the tumor. Histopathological examination revealed the tumor to be a meningothelial meningioma. New hemorrhagic foci were identified in the tumor. In addition, macrophages containing hemosiderin were detected, and some of the tumor vessels exhibited hyaline degeneration. We suspected that angiography triggered bleeding in the meningioma, which was already predisposed to hemorrhage.     

Meningioma associated with subdural hematoma--case report.

The case of a 49-year-old female with a left parietal convexity meningioma associated with an acute subdural hematoma is described. She was admitted because of sudden onset of severe headache accompanied by nausea and vomiting. She was also confused, and 6 hours after admission she developed lethargy, right hemiplegia, and left mydriasis with no pupillary reaction to light. Computed tomography disclosed a round, extra-axial mass in the left parietal region; it was heterogeneously enhanced. Emergency craniotomy, performed after carotid angiography, revealed a tumor with a massive underlying subdural hematoma. The histological diagnosis was meningotheliomatous meningioma, and there were many meningothelial cells within the hematoma.     

Castleman disease of the spine mimicking a nerve sheath tumor. Case report

Castleman disease is a rare lymphoproliferative disease of unknown cause. In most cases, afflicted patients present with a mediastinal mass although the disease may manifest in numerous other sites, including intracranially and rarely intraspinally. The authors report on the case of a 19-year-old woman who presented with a large paraspinal mass emanating from the T7-8 neural foramen. The morphological and signal characteristics revealed on magnetic resonance imaging were similar to those of nerve sheath tumors. The patient underwent open biopsy sampling of the lesion, and results of a pathological evaluation revealed hyaline-vascular type Castleman disease. She underwent radiotherapy and remains symptom-free with a radiographically stable lesion 1 year later. Although the disease has been reported to mimic a meningioma when encountered in intracranial locations, to the authors' knowledge, this is the first case of the disorder mimicking a nerve sheath tumor. When the diagnosis of Castleman disease is made, good results can be obtained with partial resection and radiotherapy.     

A case of suprasellar hemangioblastoma with thoracic meningioma

Supratentorial hemangioblastoma is encountered very rarely, with only about 110 cases reported. This report concerns a case of a suprasellar hemangioblastoma associated with thoracic meningioma. A 62-year-old man was admitted with visual disturbance of the left eye. T2-weighted magnetic resonance imaging (MRI) showed a high signal intensity mass in the suprasellar region without perifocal edema. T1-weighted MRI with gadolinium showed a homogeneously enhanced mass. The angiogram revealed remarkable tumor staining originating from the right and left internal carotid arteries (ICA) and the left external carotid artery (ECA). On the basis of the pre-operative diagnosis of tuberculum sellae meningioma, total tumor removal was performed. Photomicrographs of the specimen showed numerous capillaries divided by stroma cells. Positive immunostaining for factor VIII related antigen (F VIII RAg) was observed in endothelial cells, but immunostaining for epithelial membrane antigen (EMA) was not observed in any cells. A diagnosis of suprasellar hemangioblastoma was made on the basis of these findings. The patient suffered paraparesis on the postoperative seventh day. Since MRI showed a thoracic tumor, a second total tumor removal was performed. The tumor was diagnosed as a meningothelial meningioma. The patient was discharged without evidence of new neurological deficits. Our experience shows that, when a mass is vascularized in the cerebral hemisphere, the possibility of hemangioblastoma needs to be taken into consideration.     

Primary intraorbital ectopic meningioma

We report a case of intraorbital meningioma. Operative findings and histopathological examination revealed the tumoc to be meningothelial meningloma and to be located entirely outside the optic dura. This case demonstrates the occurrence of primary intraorbital ectopic meningioma, and the tumor was removed through a modified Dolenc approach. The primary intraorbital ectopic meningioma is discussed and the surgical approach to the orbital apex region is reviewed.     

Primary Intraorbital Ectopic Meningioma

We report a case of intraorbital meningioma. Operative findings and histopathological examination revealed the tumoc to be meningothelial meningloma and to be located entirely outside the optic dura. This case demonstrates the occurrence of primary intraorbital ectopic meningioma, and the tumor was removed through a modified Dolenc approach. The primary intraorbital ectopic meningioma is discussed and the surgical approach to the orbital apex region is reviewed.     

Melanocytic colonization of a meningothelial meningioma: histopathological and ultrastructural findings with immunohistochemical and genetic correlation: case report

OBJECTIVE AND IMPORTANCE: Melanocytic colonization of nonpigmented extracranial tumors has been reported in adenocarcinomas, squamous cell carcinomas, skin appendage tumors, and dermatofibrosarcoma protuberans. To our knowledge, melanocytic colonization of a meningioma has not previously been described. CLINICAL PRESENTATION: We report an unusual case of a 70-year-old African-American woman who presented with a large frontoparietal meningioma that extended through the calvarium. INTERVENTION: Craniotomy with gross total resection of the tumor was performed. Histochemistry, immunocytochemistry, ultrastructural analysis, and molecular genetic study via fluorescence in situ hybridization confirmed melanocytic colonization of a meningothelial meningioma. CONCLUSION: With the inclusion of meningothelial meningioma, the spectrum of tumors affected by melanocytic colonization continues to expand.     

Tumor-to-tumor metastases: Latent renal cell carcinoma discovered after elective surgical resection of a convexity meningioma

BackgroundTumor-to-tumor metastases are extremely rarely reported lesions, which usually involve an indolent lesion hosting a more aggressive neoplasm. We present an unusual initial manifestation of a previously unknown clear cell renal cell carcinoma as a tumor-to-tumor metastasis in a typical meningothelial meningioma.Case reportA 73-year old patient with transient left slight monoparesis was addressed to our Neurosurgical Department after being evaluated by his general practitioner and passing a cerebral MRI which revealed a right frontotemporal mass attached to the meninge. At presentation, no deficits were identified; therefore an elective surgery was proposed. Histological analysis revealed a typical meningothelial meningioma containing a metastatic clear cell renal cell carcinoma. Additional thoraco-abdominal computer tomography identified a 6 cm diameter lesion within the right kidney with radiological features highly suggestive of a primary clear cell renal cell carcinoma.ConclusionOur case highlights the need for a specialized neuropathological approach to clinical and imagistic indolent meningiomas, as they may require important differential diagnosis that can highly impact the treatment and follow-up of brain tumor patients.