Intraventricular meningioma with anaplastic transformation and metastasis via the cerebrospinal fluid

We report a surgical case of intraventricular meningioma that arose in the trigone of the right lateral ventricle of a 61‐year‐old woman. Local recurrence and metastasis (dissemination) via the CSF to the fourth ventricle developed about 52 months postoperatively. Further subarachnoid dissemination in the pontine base and spinal cord occurred 6 months later. The tumor exhibited a histopathological appearance of transitional meningioma without cellular atypism in the original specimen, but in the metastatic nodules in the fourth ventricle and spinal subarachnoid space the histopathology was that of typical anaplastic meningioma. Only four cases of anaplastic intraventricular meningioma that developed metastasis via the CSF have been reported. This report presents the fifth case, which is also the second case in which progression from ordinary low‐grade meningioma to anaplastic meningioma was demonstrated histopathologically.     

Hickam's dictum: Angiosarcoma‐to‐meningioma metastasis

Tumor‐to‐tumor metastasis is a seldom reported phenomenon whereby a neoplasm seeds within another histologically distinct tumor, with only 84 cases documented in the literature. We hereby describe the case of a 95‐year‐old woman who died of widespread metastases identified as a primary hepatic angiosarcoma on autopsy, and the interesting finding of a seeding foci within a dural meningioma. Although meningiomas are the most common intracranial neoplasms to harbor such a phenomenon, this is to our knowledge the first case where an angiosarcoma was identified as the donor tumor.     

Aggressive rhabdoid meningioma with osseous,papillary and chordoma‐like appearance

Meningiomas are the most common primary intracranial tumors. They are usually benign and slowly growing; however, they may show histologically malignant features categorizing them into grade II or III of World Health Organization (WHO) classification. Rhabdoid meningioma (RM) is an uncommon meningioma variant categorized as WHO grade III. The clinical course of RM is determined by local recurrences, invasion of adjacent brain and/or dura, widespread leptomeningeal dissemination, remote metastases and fatal clinical outcome. Herein we report a case with recurrent aggressive left occipital parasagittal region RM in which the patient initially declined radiation treatment. The tumor was resected four times in 5 years. Histopathological examination revealed a rhabdoid meningioma with metaplastic, papillary and chordoid differentiation. Six months after her fourth operation the patient died of progressive disease. RM is a rare subtype of malignant meningioma and the role of different adjuvant therapeutic options are still unknown. Clinical presentation, radiological features and pathologic findings of this uncommon tumor are discussed.     

Malignant meningioma with adenocarcinoma‐like metaplasia: Demonstration of intestinal phenotype

Meningiomas show a diverse histopathologic appearance, often referred to as metaplastic changes; however, adenocarcinoma‐like metaplasia is an extremely rare condition. Here, we present a novel case. A dura‐based bulky mass located in the right frontotemporal region was identified radiologically in an 83‐year‐old woman. The tumor, yellow to ash‐gray in color, was subtotally removed. Histopathological examination revealed robust adenocarcinoma‐like structures within a conventional meningothelial neoplasm. Meningioma elements showed a WHO grade I to III histology. Morphological and immunophenotypic transition between meningothelial and columnar epithelial cells was confirmed on detailed observation. It was of note that the adenocarcinomatous components shared an immunophenotype with intestinal epithelium, expressing CDX2, MUC2 and cytokeratin 20. The present case could be differentiated from secretory meningioma based on distinct cellular atypia, lack of intracytoplasmic lumina and pseudosammoma bodies, and the intact status of the KLF4 gene. In addition, the morphological and immunophenotypic transition excluded the possibility of metastatic carcinoma within meningioma. This is the first reported case of meningioma with adenocarcinoma‐like metaplasia harboring an intestinal immunophenotype.     

Concurrent spinal nerve root schwannoma and meningioma mimicking single‐component schwannoma

We present a first case of concurrent tumors consisting of schwannoma and meningioma arising at the same spinal level in a patient without neurofibromatosis. A 49‐year‐old man without clinical evidence of neurofibromatosis presented with a 5‐month history of right neck pain. MRI demonstrated an extradural tumor involving the right‐sided C2 nerve root with a small intradural component. T1‐ and T2‐weighted and contrast‐enhanced MRI could not differentiate the intradural tumor as different from the extradural tumor. Total removal of the tumors was performed. No contiguity of the extradural tumor with the intradural tumor was seen. The intradural tumor attached strongly to the dura mater around the C2 nerve root exits. Intraoperative pathological diagnosis confirmed the extradural tumor as schwannoma and the intradural tumor as meningioma. We therefore thoroughly coagulated the dura mater adjacent to the intradural tumor and resected the dura mater around the nerve root exits together with the tumor. Pathological examination revealed that the resection edge of the extradural component consisted of a spinal nerve with thickened epineurium and was free of neoplastic cells. No schwannoma component was evident in the intradural tumor. No obvious transition thus existed between the extra‐ and intradural tumors. Distinguishing these tumors prior to surgery is critical for determining an optimal surgical plan, as schwannoma and meningioma require different surgical procedures. We therefore recommend a careful review of preoperative imaging with the possibility of concurrent tumors in mind.     

Tumor‐to‐tumor metastasis from pituitary carcinoma to radiation‐induced meningioma

Radiation‐induced meningioma and pituitary carcinoma are both uncommon. Tumor‐to‐tumor metastasis (TTM) from pituitary carcinoma to meningioma, to our knowledge, has not been previously reported. A 67‐year old man presented with a previous history of transcranial subtotal resection of pituitary adenoma, at the age of 36, followed by radiotherapy. The follow‐up was uneventful for the following 31 years. The patient presented with worsening sight and numbness of the right arm. Three separate lesions were found on MRI. Histological examinations revealed pituitary carcinomas and TTM from pituitary carcinoma to meningioma. A constant surveillance is necessary for patients with pituitary tumor, especially those followed by radiotherapy.     

Ki‐67 proliferative index predicts clinical outcome in patients with atypical or anaplastic meningioma

Meningiomas represent the second most common central nervous system neoplasms in adults and account for 26% of all primary brain tumors. Although most are benign, between 5% and 15% of meningiomas are atypical (grade II) whereas 1–2% are anaplastic meningiomas (grade III). Although histological grade is the most relevant prognostic factor, there are some unusual cases in which establishing a diagnosis of high‐grade meningioma following 2000 World Health Organization (WHO) histological criteria is extremely difficult. Therefore, the aim of the present study was to evaluate the predictive value of Ki‐67 labeling index and its contribution to current WHO classification in predicting tumor recurrence and overall survival in patients with high‐grade meningiomas. A total of 28 patients (with 16 atypical meningiomas and 12 anaplastic meningiomas) were evaluated for demographic, clinical, radiological and therapeutic variables, and for Ki‐67 immunohistochemistry. Median Ki‐67 labeling index in the whole series was 7.0 (0.5–31.5) with no differences with respect to the histological grade (P = 0.87). In the univariate analysis, Ki‐67 labeling index and postoperative Karnofsky performance status were identified as significant prognostic factors of tumor recurrence and overall survival. The multivariate analysis demonstrated that Ki‐67 labeling index is the only independent predictor of both tumor recurrence and overall survival. More importantly, this predictive value was maintained in both patients with atypical and patients with anaplastic meningioma.     

Co‐existing fibrous dysplasia and atypical lymphoplasmacyte‐rich meningioma

We report an unusual and extremely rare case of coexisting fibrous dysplasia of the sphenoid sinus with atypical lymphoplasmacyte rich meningioma (World Health Organization Grade II), right frontal lobe in a 25‐year‐old male. The patient presented with history of generalized tonic clonic seizures with a loss of vision in the right eye. Radiologically it was seen that the sphenoid sinus lesion was sclerotic and was extending and causing compression of the optic nerve. Magnetic resonance imaging revealed mass lesion in the right frontal lobe, iso to hypointense on T₁ weighted images and hyperintense on T2 weighted images with uniform contrast enhancement. Clinically and radiologically the possibility of fibrous dysplasia of the sphenoid bone and sinus with metastases in the frontal lobe from a different primary was considered. On histopathology the frontal lobe lesion showed a tumor composed of cells with vesicular nuclei, focal nuclear pleomorphism, a mitotic rate of = 4/10 high power field and a single focus of necrosis. The cells were arranged in fascicles and had a prominent lymphoplasmacytic infiltrate. In contrast, the sphenoid sinus lesion was composed of narrow, curved bony trabeculae with a characteristic fishhook configuration without osteoblastic rimming and interspersed with fibrous tissue of variable cellularity. Although no definite causative factors are described for the coexistence of these tumors, however, a common yet undetermined denominator in the origin has been proposed in the literature.     

Low‐grade follicular lymphoma in the dura: Rare mimic of meningioma

Lymphomas rarely present as a localized mass within the dura. We report a case of a 72‐year‐old woman, normally fit and well, who presented with a sudden episode of grand mal seizure. Imaging studies showed an extra‐axial mass arising from the dura, with radiologic features characteristic of a meningioma. The mass was surgically excised, and the histologic features were of a grade 1 follicular lymphoma. Analysis using fluorescent in situ hybridization (FISH) demonstrated t(14:18) translocation. A bone marrow aspirate showed focal infiltration of lumbar vertebra by low‐grade follicular lymphoma. Intracranial meningeal involvement by non‐Hodgkin lymphoma usually occurs in the form of diffuse leptomeningeal spread. It is seen in high‐grade lymphomas, often in association with widespread systemic disease. The majority of cases of dural lymphomas reported in the literature have been primary mucosa‐associated lymphoid tissue (MALT)‐type lymphomas. Most of these presented as localized dural masses mimicking meningiomas. Low‐grade follicular lymphoma, either primary or secondarily involving the dura, appears to be an exceedingly rare occurrence.     

Follicular lymphoma of the dura associated with meningioma: A case report and review of the literature

A 70‐year‐old immunocompetent woman who had been radiologically diagnosed with a left parasagittal meningioma 5 years previously developed a palpable subgaleal mass and underwent neurosurgery. Histologically the tumor was composed of thickened fibrotic dural tissue, infiltrated by a follicular lymphoma, of grade IIIB. A coexistent transitional meningioma had been infiltrated by the neoplastic lymphoid tissue. Two months after surgery, the patient developed evidence of extracranial dissemination of the lymphoma to the cervical lymph nodes and was treated with polychemotherapy plus Rituximab and intrathecal methotrextate. The patient is alive and recurrence‐free at 1‐year follow up. A review of the literature indicates that lymphomas developing primarily in the meninges, have an indolent course and tend to be localized in areas rich in meningothelial cells.     

Synchronous intravascular large B‐cell lymphoma within meningioma

Intravascular large B‐cell lymphoma is a rare and aggressive lymphoma with a dismal prognosis. Synchronous intravascular large B‐cell lymphoma within meningioma has not previously been documented. We report a case of a 73‐year‐old woman of Asian descent who presented with fever of unknown origin with generalized weakness. CT scan and MRI of the head revealed a dural‐based mass lesion consistent with meningioma in the left frontal cerebral convexity. Surgery was performed to remove the tumor and histopathology showed a meningioma within which was a synchronous intravascular large B‐cell lymphoma. The hematology and oncology services were consulted and palliative treatment was initiated due to the patient's poor Eastern Cooperative Oncology Group performance status. The patient died within 30 days post‐surgery. To the best of our knowledge, this case represents the first report of synchronous intravascular large B‐cell lymphoma within a meningioma.     

Immunocytochemical analysis of glucose transporter protein‐1 (GLUT‐1) in typical,brain invasive,atypical and anaplastic meningioma

Glucose transporter‐1 (GLUT‐1) is one of the major isoforms of the family of glucose transporter proteins that facilitates the import of glucose in human cells to fuel anaerobic metabolism. The present study was meant to determine the extent of the anaerobic/hypoxic state of the intratumoral microenvironment by staining for GLUT‐1 in intracranial non‐embolized typical (WHO grade I; n = 40), brain invasive and atypical (each WHO grade II; n = 38) and anaplastic meningiomas (WHO grade III, n = 6). In addition, GLUT‐1 staining levels were compared with the various histological criteria used for diagnosing WHO grade II and III meningiomas, namely, brain invasion, increased mitotic activity and atypical cytoarchitectural change, defined by the presence of at least three out of hypercellularity, sheet‐like growth, prominent nucleoli, small cell change and “spontaneous” necrosis. The level of tumor hypoxia was assessed by converting the extent and intensity of the stainings by multiplication in an immunoreactive score (IRS) and statistically evaluated. The results were as follows. (1) While GLUT‐1 expression was found to be mainly weak in WHO grade I meningiomas (IRS = 1–4) and to be consistently strong in WHO grade III meningiomas (IRS = 6–12), in WHO grade II meningiomas GLUT‐1 expression was variable (IRS = 1–9). (2) Histologically typical, but brain invasive meningiomas (WHO grade II) showed no or similarly low levels of GLUT‐1 expression as observed in WHO grade I meningiomas (IRS = 0–4). (3) GLUT‐1 expression was observed in the form of a patchy, multifocal staining reaction in 76% of stained WHO grade I‐III meningiomas, while diffuse staining (in 11%) and combined multifocal and areas of diffuse staining (in 13%) were only detected in WHO grades II and III meningiomas, except for uniform staining in angiomatous WHO grade I meningioma. (4) “Spontaneous” necrosis and small cell change typically occurred away from the intratumoral capillary network embedded within the pattern of GLUT‐1 staining. Taken together, GLUT‐1 staining cannot be applied as a substitute for histologic grading in order to predict tumor behavior. However, assessment of tumor hypoxia in association with “spontaneous” necrosis and foci of small cell change may substantially contribute to the neuropathologic diagnosis of WHO grades II and III meningioma.     

Tumor‐to‐tumor metastasis to the central nervous system

Tumor‐to‐tumor metastasis is a well‐recognized phenomenon. Meningioma is the most common intracranial host tumor, with the breast and lung being the most common primary sites. We report herein two such cases of metastasis from pulmonary adenocarcinoma and malignant melanoma (MM) of vulva, respectively. Case 1: a 69‐year‐old female smoker who had a history of right upper lobectomy of lung for adenocarcinoma presented with a headache and altered mental status, and was found to have a left temporal contrast‐enhancing mass with massive surrounding edema on MRI. The resection specimen revealed foci of metastatic adenocarcinoma within a microcystic meningioma. Case 2: a 75‐year‐old woman with a history of radical vulvectomy for MM died of widespread systemic metastasis of MM. At autopsy, a 2.5 × 2 × 2 cm firm nodule attached to the falx was incidentally found, with focal black discoloration at the periphery of the mass. Histologic examination showed a fibroblastic meningioma with a focus of metastatic MM. Case 1 is the first case report describing a microcystic variant of meningioma harboring metastatic carcinoma. Although MM is one of the most common metastatic brain tumors, MM‐to‐meningioma metastasis is reportedly extremely rare, but can occur.     

Lymphoplasmacyte‐rich meningioma: A convexity mass with regional enhancement in the adjacent brain parenchyma

Lymphoplasmacyte‐rich meningioma (LPM) is a rare, benign variant of meningioma, characterized by massive inflammatory cell infiltration and a variable proportion of meningothelial tumorous elements. Here we report the clinicopathological features of an LPM located at the right frontal convexity in a 37‐year‐old woman. The patient had suffered an initial generalized tonic‐clonic seizure when she was 32 weeks pregnant. The lesion exhibited low intensity on T1‐weighted MRI and high intensity on T2‐weighted images, with surrounding parenchymal edema. The mass exhibited gadolinium enhancement with dural tail signs. Moreover, multiple foci of linear enhancement spreading through the sulci and into the nearby brain parenchyma were evident. At 1 month after parturition, en bloc removal of the mass, the attached dura mater and adjacent brain tissue was performed. Histologically, the mass located in the subdural space was composed of a mixture of B‐ and T‐lymphocytes and plasma cells. Within the mass, multiple small lobules of meningothelial cells showing immunoreactivity for epithelial membrane antigen and vimentin were observed. The inflammatory cells had also infiltrated the subarachnoid and Virchow‐Robin spaces, and the dura mater. The cerebral cortex showed ischemic changes, but no tumor cell invasion. On the basis of these histological features, the lesion appeared to be LPM with an inconspicuous meningothelial component and extensive inflammatory infiltration. This case appears to provide useful information on the pathogenesis of this variant.     

恶性脑膜瘤的临床特点及诊疗体会

目的：总结恶性脑膜瘤的临床特点及诊疗体会。方法回顾性分析解放军总医院收治的27例经病理证实为恶性脑膜瘤（ WHOⅢ级）患者的临床表现、诊治经过、术后辅助治疗及预后等资料,窦镰旁恶性脑膜瘤7例,大脑凸面恶性脑膜瘤8例,蝶骨嵴恶性脑膜瘤5例,岩斜区等其他部位恶性脑膜瘤7例,其中肿瘤直径＞5 cm 19例。主要影像学特点为磁共振增强扫描呈明显不规则强化,周围水肿带明显。结果27例恶性脑膜瘤病例中,间变型恶性脑膜瘤18例,横纹肌样恶性脑膜瘤7例,乳头型恶性脑膜瘤2例。患者均行显微手术治疗,无死亡病例。按照Simpson标准,SimpsonⅠ级切除者11例,SimpsonⅡ级切除者6例,SimpsonⅢ级切除者6例,SimpsonⅣ级切除者4例。随访平均37．2个月,Ⅰ～Ⅱ级切除者7例（41．2％）出现复发,Ⅲ～Ⅳ级切除者患者均复发（100％）。其中,术后放疗者13例,未行放疗者14例,复发率分别为61．5％及78．6％。结论恶性脑膜瘤易复发,治疗首选显微外科全切除肿瘤,并处理硬膜及受累颅骨,术后应辅以放疗。     

侧脑室三角区肿瘤显微手术治疗的初步临床分析

目的探讨不同手术入路显微镜下行侧脑室三角区肿瘤的手术治疗方法。方法对47例肿瘤位于侧脑室三角区并经显微手术治疗的患者相关资料进行回顾性分析,详细分析不同手术入路及术中操作技巧。结果 42例患者颅内肿瘤显微镜下全切,5例因肿瘤较大、界限不清部分切除,其中,2例胶质瘤和1例星型母细胞瘤部分切除+去骨瓣减压术,1例间变性室管膜瘤因肿瘤生长至对侧,部分切除术后复发;1例胶质肉瘤,出现迟发性术区出血,急诊行手术清除术区脑内血肿,第二天死亡。1例同侧术后出现硬膜外血肿,血肿量不多,未再次手术,经积极治疗逐渐吸收。5例患者术后合并颅内感染,行腰大池脑脊液持续引流,感染痊愈,预后良好。出院时GOS评分:39例预后评分5分,4例预后评分4分,1例评分3分,1例预后评分2分,2例评分1分。结论对于不同类型、不同生长方向的侧脑室三角区肿瘤,在影像导航系统指引下,选择合适的手术入路、不断提高手术技巧,可明显减少患者的功能损伤及手术并发症的出现,改善患者的预后。     

侧脑室内胶质瘤与脑膜瘤的碰撞瘤一例并文献复习

目的 报告1例罕见的侧脑室内胶质瘤与脑膜瘤的碰撞瘤,并探讨其发生原因.方法 39岁男性,6年前曾行侧脑室内胶质瘤切除术;此次以头痛及头晕发病,MRI示胶质瘤原位复发,且同时合并另一占位性病变,予以手术切除.术后病理证实为胶质瘤与脑膜瘤的碰撞瘤;本文同时也对这种碰撞瘤进行文献复习.结果 术后患者恢复满意,并行伽玛刀放射治疗,随访1年时患者状态良好.本文在复习侧脑室内胶质瘤与脑膜瘤的碰撞瘤发生原因后,总结了几种假设.结论 复发的胶质瘤诱发脉络丛内的蛛网膜细胞恶性转化可能是导致脑膜瘤的原因,同时胶质瘤的刺激也可能诱导了脑膜瘤的生长.     

Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1‐TFE3 fusion

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well‐studied subtype of sarcoma. A 39‐year‐old man presented with seizures due to a left temporal meningeal‐enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase‐resistant periodic acid‐Schiff revealed very rare granular and rod‐like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT‐PCR and sequencing analysis demonstrated ASPCR1‐TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded.     

Chordoid meningioma of the foramen magnum in a child: a case report and review of the literature

We report a 3-year-6-month-old boy with chordoid meningioma in the foramen magnum. Chordoid meningioma represents between 0.5 and 1.0% of all meningiomas, and it is frequently located in the supratentorial region. The patient started with an episode of instability, falls, and headache; after which, he developed cuadriparesis, cervical pain, and neck stiffness, which slightly improved after medical treatment, so he was referred to our hospital. Physical examination revealed left hemiparesis and cervical muscle spasm with left torticollis. Magnetic resonance imaging (MRI) demonstrated an intradural–extramedullary well-circumscribed, homogeneous enhancing mass, in the anterior part of the foramen magnum with cervical extension. The operative technique was the extreme-lateral craniocervical retrocondylar approach with total removal and dural coagulation. Histopathological examination demonstrated a chordoid meningioma, with meningotelial basophilic mucoid chordoma-like component in 80% of the tissue. The immunohistochemical stains were positive to epithelial membrane antigen, vimentin, and S-100 protein. At 10 months follow-up, the patient showed improvement in his neurological deficit with physical rehabilitation, and motor response in his extremities are now normal; neck stiffness with cervical spasm disappeared postoperatively. MRI showed no residual tumor. To our knowledge, this is the first report of a chordoid meningioma on the foramen magnum in a child.     

Correlative evidence that tumor cell‐derived caveolin‐1 mediates angiogenesis in meningiomas

Since it has recently been reported that caveolin‐1 (cav‐1) may favor the progression of prostatic and renal cancers by stimulating tumor neoangiogenesis, we thought it of interest to analyze the correlation between cav‐1 expression and tumor microvessel density (MVD) in meningiomas. In the present study we quantified cav‐1 expression by immunohistochemistry and used CD105 immunohistochemical staining to measure MVD. Sixty‐two formalin‐fixed, paraffin‐embedded, surgically resected meningiomas were submitted to the analysis. On the basis of cav‐1 immuno‐expression, cases were subdivided into meningiomas displaying a low (n = 34) and a high (n = 28) cav‐1 immuno‐expression, respectively. Mann–Whitney test showed that a significantly higher MVD was present in the cases with a high cav‐1 expression than in those with a low expression (mean 24.44 vs. 41.28 microvessels/mm²) (P = 0.0001). Moreover, Spearman test revealed a significant positive correlation between cav‐1 rate of expression and MVD counts in the meningiomas of our series (r = 0.390; P = 0.0023). Therefore, our study demonstrates the existence of an association between cav‐1 expression and neoangiogenesis in meningiomas, suggesting that cav‐1 may mediate the progression of these tumors by stimulating the angiogenic process. Besides, it is known that the progression of meningiomas is paralleled by an increase in MVD. The clarification of cav‐1 role in the neoangiogensis of meningiomas may open new insights about the possibility of novel therapeutic strategies in these neoplasias.