Papillary meningioma: Diagnosis by intraoperative crush smear cytology

Papillary meningioma is a rare, aggressive variant of meningioma. It accounts for less than 1% of all meningiomas. It is categorized as WHO grade III due to high rate of recurrence and metastasis. Histopathologic features of papillary meningioma are well described, but cytomorphology is rarely been described. We report a case of papillary meningioma, diagnosed on intraoperative crush cytology and later confirmed on histopathology. Papillary meningioma possesses characteristic cytological features. Along with the cellular meningothelial features, radial arrangement of cells around thin and thick walled hyalinised blood vessels gives the diagnostic clue. In addition, knowledge of clinical and radiological features is extremely helpful to arrive at the correct diagnosis and to differentiate it from other papillary lesions involving central nervous system. Due to aggressive clinical course and poor prognosis of papillary meningioma, a timely recognition of the diagnosis is desirable and helpful for rationalizing approaches to therapy.     

Cytologic features of pigmented atypical meningioma mimicking melanoma on intraoperative crush preparations

Pigmented tumors rarely arise in the meninges, and when they do, these are mainly melanocytomas or melanomas. We describe the cytologic findings of atypical meningioma with intratumoral hemosiderin pigment mistaken for spindle cell melanoma in a 33‐year‐old male patient during intraoperative consultation. Preoperative radiologic images revealed a cystic meningeal mass with intratumoral hemorrhage. The crush preparation demonstrated cellular smears of syncytial clusters as well as fascicles of large pleomorphic spindle cells with discrete cytoplasmic brown pigment. Detection of cytoplasmic brown pigment and a preponderance of large spindle cells with nuclear pleomorphism led to a diagnosis of spindle cell melanoma on intraoperative cytology. Histopathologic examination displayed high cellularity, nuclear pleomorphism with prominent nucleoli, and foci of spontaneous necrosis. In addition, there were areas showing classic meningotheliomatous meningioma features. Altogether, the histologic findings were consistent with atypical meningioma. The cytoplasmic pigment in the tumor cells was confirmed to be hemosiderin using special stains and immunohistochemistry. To the best of our knowledge, this is the first case report describing cytomorphology of atypical pigmented meningioma. We discuss the differential diagnosis in intraoperative cytology and a possible mechanism related to intratumoral hemosiderin deposition in meningiomas. Diagn. Cytopathol. 2015;43:149–152. © 2014 Wiley Periodicals, Inc.     

Fine-needle aspiration cytology of a primary ectopic meningioma

Meningiomas are benign tumors derived from arachnoid cells. Most commonly an intracranial lesion, meningiomas may be found extracranially in various anatomic sites. A 23-yr-old white female presented with left-sided palpable mass located submucosally in the floor of the mouth. CT scan revealed no evidence of mass elsewhere in the head and neck region. Fine-needle aspiration cytology (FNAC) showed loose and cohesive cellular fragments with lobular growth pattern and uniform round or ovoid cells. The diagnosis of low-grade salivary gland neoplasm, not further classified, was made. The tumor was locally excised. The differential diagnoses of an extracranial meningioma and pleomorphic adenoma were discussed at the frozen section. Based on light microscopic, immunohistochemical, and electron microscopic (EM) findings, the final diagnosis of an ectopic meningioma was rendered. Ectopic meningiomas may pose a diagnostic challenge to clinicians and cytopathologists. It is easily forgotten in the list of differential diagnosis at an ectopic site. Primary ectopic meningioma in a region containing salivary gland(s) may mimic benign and low-grade malignant salivary gland tumors in FNAC.     

Clear cell meningioma with anaplastic features: Case report and review of literature

Clear cell meningioma (CCM) is an uncommon variant of meningioma, corresponding to WHO grade II. We present two cases of CCMs with anaplastic features in the intracranial and intraspinal region. The first case is a 65-year-old male who gradually developed changes in behavior over a period of 1 year. The second case is a 35-year-old female who presented with a 7-month history of posterior cervicothoracic pain and dysuria for 1 week. Magnetic resonance imaging revealed an intracranial lesion in the right frontal lobe in the male patient, and an intradural extramedullary lesion at C7 in the female patient. On histological examination, both tumors partly exhibited unusual anaplastic appearances with nuclear pleomorphism, high mitotic activity and necrosis, distinct from classical CCMs. Tumor cells were immunoreactive to epithelial membrane antigen (EMA) and vimentin, with a high MIB-1 index up to 40%. Total excision was performed. The male patient was found to have developed local recurrence and lateral ventricle metastasis 3 months after surgery. A diagnosis of CCM with anaplastic features was made (WHO grade III). Based on its aggressive behavior, we recommend postoperative adjuvant radiotherapy or chemotherapy even if total excision of the tumor has been performed, and MRI scans every 3–6 months during the first period of follow-up.     

“Benign” metastatic meningioma: clinico-pathological analysis of one case metastasising to the lung and overview on the concepts of either primitive or metastatic meningiomas of the lung

Lung “metastases” of benign meningiomas are rarely described events of biological and clinical interest. We, here, report of a 70-year-old healthy woman found by CT scan to have multiple lesions, the two largest in the right lung on routine examination. Anamnesis revealed that the patient underwent a surgical resection of cerebral meningioma 12 years before. The larger lung lesion was a 3-cm node located in the right lung and was removed by wedge resection. Macroscopically, it showed well-defined borders, whitish colour and firm consistency; histologically, it was uniformly composed by spindle meningothelial cells arranged in fascicules including psammoma bodies. The morphological and immunohistochemical features of this lesion, together with the similarity with the original cerebral tumour and its indolent evolution, led to a final diagnosis of “benign” meningioma metastatic to the lung. Lung metastatic meningiomas may be a diagnostic challenge because of their unusual site of presentation and the possible confusion with primitive lung meningiomas or primary mesenchymal lung lesions. They represent a typical example of “benign” tumours that may implant to the lung similar to other tumours, definitely considered benign but reported to rarely present unusual secondary localization.     

Giant cell‐rich osteosarcoma of the parotid gland: An exceptionally rare entity at an unusual site

Giant cell‐rich osteosarcoma is a rare histologic variant of conventional osteosarcoma that affects mainly the extremities. Extraskeletal giant cell‐rich osteosarcoma is therefore exceedingly rare. Here, we report the first case of this uncommon tumor involving the parotid gland in a 62‐year‐old male who presented with initial right jaw swelling. Radiologic work‐up revealed a 6.2 cm mass involving the right parotid gland. Fine‐needle aspiration cytology showed numerous multinucleated giant cells in a background of dyshesive epithelioid cells and rare clusters of spindle stromal cells, suspicious for malignancy. The subsequent excisional biopsy showed histopathologic features diagnostic for giant cell‐rich osteosarcoma. Diagn. Cytopathol. 2016;44:1107–1111. © 2016 Wiley Periodicals, Inc.     

Emergence of a high-grade sarcoma in a recurrent meningioma: malignant progression or collision tumor?

Anaplastic meningiomas that resemble sarcomas often reveal clues to their meningothelial differentiation or develop in a plausible setting that confirms their meningothelial origin. Malignant mesenchymal neoplasms without obvious evidence of meningothelial differentiation or origin are more likely to be true primary or metastatic sarcomas. Because of their clinical and biological differences, it is important to distinguish anaplastic meningioma from a sarcoma. We present a 67-year-old woman with multiple meningiomas, who developed a high-grade spindle cell tumor 6 months after the resection of a World Health Organization grade I meningioma. It was not clear whether this tumor represented a malignant transformation of meningioma or a primary sarcoma. Malignant transformation of a meningioma is exceptional within this short period and a coexisting sarcoma and meningioma are equally uncommon. Even though these malignant neoplasms are rare in general, they appear to be more prevalent in patients with multiple meningiomas including those with neurofibromatosis type 2.     

Metaplastisches Meningeom mit kartilaginärer Differenzierung

A meningioma with cartilaginous areas is described. The tumour arose in the region of the right sphenoid wing in a 74-year-old woman. Histologically, it showed large areas of a typical meningothelial meningioma, among which numerous cartilaginous islands and some chondroid regions, obviously of intermediate (meningothelial/cartilaginous) differentiation, could be seen. Cartilaginous tumour areas showed lower MIB1-labelling indices than typical meningioma regions, where an increased proliferative activity was seen focally. The current WHO classification lists such tumours as metaplastic meningiomas, reflecting the potential of meningioma cells for mesenchymal differentiation. Metaplastic meningiomas may show different metaplasias (xanthomatous, osseous, lipomatous, cartilaginous, etc.). Extensive cartilaginous metaplasias are very uncommon. Identification of typical meningioma areas is the key for the diagnosis of this meningioma variant.     

脊索样脑膜瘤临床病理分析

目的：研究脊索样脑膜瘤的临床与病理特征,以提高其早期诊断率。方法：运用组织病理学及免疫组化标记（Ｓ－Ｐ法）对３例脊索样脑膜瘤进行临床病理学和组化（ＰＡＳ）、免疫组化（ＥＭＡ、Ｖｉｍ、Ｓ－１００、ＣＫ）及电镜观察。结果：组织学瘤细胞胞浆内外均见粘液,在粘液基质背景中有成簇或成行的类液滴样细胞,瘤组织中有典型的脑膜上皮漩涡结构,组化及免疫组化示ＰＡＳ（＋）、ＥＭＡ（＋）、Ｖｉｍ（＋）、Ｓ－１００（＋）     

Osteopontin predicts the behaviour of atypical meningioma

Lin C‐K, Tsai W‐C, Lin Y‐C & Hueng D‐Y
(2012) Histopathology  60, 320–325
Osteopontin predicts the behaviour of atypical meningioma Aim: Although the World Health Organization (WHO) histological criteria distinguishing benign from atypical and malignant meningioma are clear, discerning benign from atypical meningioma is still somewhat difficult, leading to interobserver diagnostic variability. Osteopontin (OPN) and cortactin play important roles in tumorigenesis, invasion and metastasis of several human cancers. The aim of this study was to evaluate the usefulness of OPN and cortactin immunohistochemistry for distinguishing between benign, atypical and malignant meningioma and predicting their recurrence. Methods and results: Seventy‐five specimens (48 benign, 17 atypical and 10 malignant meningiomas) were investigated immunohistochemically. The mean immunohistochemical scoreimmunohistochemical score ± SE of the mean of both OPN and cortactin were significantly higher in grade II or grade III meningiomas than in grade I meningioma. Discriminant analysis of immunohistochemical OPN expression showed correct classification of 97.7% of WHO grade I meningiomas and 88.2% of WHO grade II meningiomas (95.4% accuracy). However, the same analysis of cortactin expression showed correct classification of 95.8% of WHO grade I meningiomas and only 23.5% of WHO grade II meningiomas (76.9% accuracy). A cut‐off for predicting grades I and II meningioma recurrence was determined for OPN (3.0) but not for cortactin. Finally, logistic regression identified both this cut‐off (P < 0.05) and WHO grade (P < 0.05) as independent risk factors for recurrence. Conclusions: OPN expression is a valuable marker for diagnosis of atypical meningioma and prediction of grades I and II meningioma recurrence.     

Primary pancreatic leiomyosarcoma with metastasis to the liver diagnosed by endoscopic ultrasound‐guided fine needle aspiration and fine needle biopsy

Primary pancreatic leiomyosarcomas are rare tumors of the pancreas that are usually diagnosed after resection or by biopsy. One case in the literature has utilized endoscopic ultrasound‐guided fine needle aspiration (EUS‐FNA) cytology. We report a second case of a primary pancreatic leiomyosarcoma that yielded diagnostic material on EUS‐FNA cytology. A 72‐year‐old female presented with 3–4 months of abdominal pain. A CT scan showed a large heterogeneous, lobulated pancreatic head and uncinate mass and multiple hypoattenuating liver lesions. An EUS‐FNA was performed on one of the liver lesions with a 25‐gauge needle, yielding an adequate sample with lesional cells. The initial read was a spindle cell neoplasm. A subsequent endoscopic ultrasound‐guided fine needle biopsy with a 22‐gauge needle was performed on the pancreatic head mass to rule out two primaries and to provide tissue for a mitotic index in the case of gastrointestinal tumor. Both the cell block of the EUS‐FNA and the core biopsy were equally cellular and showed interlacing spindle cells that stained positive for SMA and negative for DOG‐1, CD 117, and CD34. In addition, the core biopsy of the pancreas stained positive for Desmin. A diagnosis of a primary pancreatic leiomyosarcoma was made and the patient was started on systemic chemotherapy. Primary pancreatic leiomyosarcomas are rare pancreatic tumors that may yield diagnostic material by EUS‐FNA with a 25‐gauge needle. Diagn. Cytopathol. 2016;44:1070–1073. © 2016 Wiley Periodicals, Inc.     

Loss of SMARCE1 expression is a specific diagnostic marker of clear cell meningioma: a comprehensive immunophenotypical and molecular analysis

Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers. We compared the performance of an anti‐SMARCE1 antibody and the molecular analysis of the SMARCE1 gene in a retrospective multicenter series of CCMs. All CCMs lossed SMARCE1 immunoexpression. Bi‐allelic inactivating events were found by NGS‐based sequencing in all of these cases, except for one, which was incompletely explored, but had a wild‐type sequence. We then validated the anti‐SMARCE1 antibody specificity by analyzing additional 305 pediatric and adult meningiomas of various subtypes and 15 non‐meningioma clear cell tumors by SMARCE1 immunohistochemistry. A nuclear immunostaining was preserved in all other meningioma variants, as well as non‐meningioma clear cell tumors. In conclusion, our series showed, for the first time, that SMARCE1 immunostaining is a highly sensitive biomarker for CCM, useful as a routine diagnostic biomarker.     

Meningioangiomatosis--a case report.

Meningioangiomatosis is a rare benign hamartomatous lesion. We describe a case of meningioangiomatosis in an 18-year-old boy with a 15 year history of seizures. Computed tomography reveals an irregular calcification density along the right temporal gyrus. Microscopically, irregularly branched blood vessels, surrounded by a concentric arrangement of proliferating spindle cells, are extending into the gray matter from the meningeal surface. Ultrastructural and immunohistochemical examination failed to demonstrate features of meningothelial cell origin in this case. This is the first case of meningioangiomatosis published in Korea along with immunohistochemical and electron microscopic studies. The pathogenesis and previous reports of this lesion will be discussed.     

Crush cytology of pituicytoma

The crush cytology of a pituicytoma is reported. The lesion was resected from a 54-yr-old man with a 7-mo history of headache. The intraoperative crush smears revealed plump spindle cells with elongated, nonwavy nuclei, and moderate, finely granular cytoplasm with distinct cytoplasmic borders. These cells were arranged in cohesive fascicles exhibiting a storiform pattern focally. Cytologically, this lesion can be differentiated from pituitary adenoma, astrocytoma, meningioma, and schwannoma.     

Cytopathologic features of orbital intraosseous chordoid meningioma: Report of a case and distinction from other myxoid/mucoid tumors

Chordoid meningioma (CM) is characterized by a striking histologic resemblance to chordoma and propensity for aggressive behavior or recurrence (WHO grade II designation). Orbital intraosseous CM is extremely uncommon and only one case report has been documented. A case is presented here in which squash smears of a left orbital tumor in a 53‐year‐old male revealed small clusters or cord‐like structures of bland tumor cells embedded in a myxoid or mucinous background. Whorl‐like structures were also identified. The tumor cells possessed uniformly round nuclei with a smooth nuclear outline, fine granular chromatin, and small nucleoli. Occasional intranuclear inclusions, coarse collageneous cytoplasmic filaments were observed. Many spindle‐shaped cells with similar nuclear findings were also seen. A cytologic diagnosis of a chordoid meningioma was suggested and histochemical and immunohistochemical studies were conducted on formalin‐fixed, paraffin‐embedded material. Immunohistochemically, the tumor cells showed diffuse and strong membranous and cytoplasmic staining for vimentin, epithelial membrane antigen (EMA) and faintly reactive with S‐100 protein but negative for pan‐neuroendocrine markers (i.e., NSE, chromogranin A, synaptophysin), cytokeratin AE1/AE3, smooth muscle actin, D2‐40, brachyury or class III beta‐tubulin. The proliferative index with MIB‐1 was less than 1%. The diagnosis of orbital intraosseous CM was confirmed based on cytopathologic, histopathological, immunohistochemical results, location of the tumor, and the lack connection to the duramater. We demonstrated here for the first time the cytopathological features of intraosseous CM with emphasis on differential diagnostic considerations. Diagn. Cytopathol. 2010; 38:818–821. © 2010 Wiley‐Liss, Inc.     

Miniaturized Handheld Confocal Microscopy Identifies Focal Brain Invasion in a Mouse Model of Aggressive Meningioma

Invasion of the brain parenchyma by a meningioma classified by histological criteria as World Health Organization (WHO) grade I meningioma, implies that the tumor has greater likelihood of recurrence and a biological behavior similar to the more aggressive WHO grade II meningiomas. It is therefore important to detect microscopic foci of brain invasion during surgery in order to maximize the resection and/or adapt imaging follow‐up. In this study, we tested the sensitivity of two handheld confocal imaging devices to detect foci of brain invasion in two types of meningioma mouse models: in a genetically engineered mouse model and in a syngeneic xenograft model. Confocal imaging offered precise images of meningothelial and fibroblastic mouse meningiomas as well as malignant meningiomas, which corresponded exactly to the pathological findings. Imaging showed a sharp definition of the brain‐tumor interface and enabled identification of embedded nerves and vessels. Importantly, in both mouse models used in this study, extension of tumor along Virchow–Robin spaces into adjacent brain was detected by imaging. In conclusion, this novel technique, following validation in clinical trials, may open new possibilities for use in operating rooms to influence both decision making during the surgery and planning for additional treatments.     

Rare growth of a psammomatous meningioma in a mature ovarian Teratoma: A case report

The presence of meningothelial elements along with abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma. Recognition of meningioma arising in the setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin.     

Kollisionstumor des Gehirns

A 51 year old caucasian male presented with headache, facial nerve paresis and continuing contraction of the visual field. CT scan revealed a singular intracerebral contrast enhancing lesion in the left frontal lobe. Intraoperatively the tumour was well demarcated. Frozen sections showed a high grade glioma. Paraffin sections revealed, in addition to the gliomatous component, some sharply demarcated nests of meningothelial cells. Immunohistochemistry with glial fibrillary acidic protein and epithelial membrane antigen confirmed a collision tumour consisting of a glioblastoma WHO-grade IV and a meningothelial meningioma WHO-grade I. The coincidence of these two different tumours at the same time and the same location leads us to the speculation, that the collision tumour might have been caused by malignant transformation of a reactive astrogliosis surrounding the meningioma.