Primary Meningioma Research Articles

P2.04-038 Primary Pulmonary Meningioma: Rare Tumor with Malignant Potential: Topic: Esophageal Cancer and Other Malignancies

Primary Pulmonary Meningioma (PPM) is a very rare tumor with approximately 40 cases described in previous medical literature. Although majority of the cases documented previously are benign, there were 3 cases that showed malignant characteristics. We report a case of PPM with atypical features. A 65-year-old lady, non-smoker, had an incidental finding of 1.9 × 1.6cm nodule in the right middle lobe on routine chest x-ray. The computed tomography (CT) of the thorax demonstrated a well-defined, homogeneous soft tissue nodule measuring 1.5 × 1.5 cm in the lateral segment of the right middle lobe. CT-guided biopsy did not show features of malignancy. Hence, the patient was managed conservatively with surveillance CT scans. Four years later, repeat CT thorax revealed enlargement of the tumor size to 3.1 × 2.2 cm, with increased lobulations. The patient underwent completely portal robotic middle lobectomy and mediastinal lymph node dissection. The patient made an uneventful recovery and was discharged on post-operative day 3. Histological examination revealed a 3.8 cm solid, circumscribed, whitish, homogeneous nodule that abuts the pleural surface. Microscopically, the tumor comprised of bland spindle cells arranged in fascicles and occasional whorls. The cells had round to ovoid nuclei and moderate amounts of eosinophilic fibrillary cytoplasm. Up to 7 mitotic figures per 10 high power fields were identified. Stromal hyalinization, ectatic blood vessels and focal necrosis were seen in some area. The tumor cells were diffusely positive for vimentin and S-100, focally positive for cytokeratin AE1/3, synaptophysin and EMA, and negative for chromogranin. Overall, histology of the specimen demonstrated characteristic patterns of meningiomas with atypical features. The lymph nodes sampled were negative for malignancy. There were no intra-cranial or spinal lesions. There was no tumor recurrence at the 1-yr follow-up. PPM is a rare tumor which requires timely surgical resection for diagnosis and treatment. Untreated benign behaving lesions have the potential to become aggressive and demonstrate atypical features.

Primary Pulmonary Meningioma in an Elderly Patient: Case Report

Primary Pulmonary Meningioma in an Elderly Patient: Case Report

OS14 - 208 The prognostic role of pre-operative complete blood count (CBC) in progression-free survival in patients with meningioma

Factors which might influence outcome in patients with meningioma are not well-understood. Previous studies have examined associations of laboratory blood values including hemoglobin levels with patient outcomes in cancer. We hypothesized those changes in CBC before tumor resection can be used as one of the prognostic factors for tumor recurrence/progression in meningioma. To address this, we gathered the clinical and pre-operative CBC results for final analysis from 226 patients (64 males and 162 females) who underwent craniotomy for primary meningioma (grades: 157 WHO GI, 59 GII, 10 GIII) at our institution between 2001 and 2015.Individual parameters were analyzed for correlation with progression-free survival. The median recurrence free survival (RFS) was not reached and follow-up ranged 0.3-14 years. Fifty-six patients (25%) had anemia and 30% of the patients showed leukocytosis using standard cut-offs. On univariate analyses, low hemoglobin (Hb) level, as well as high leukocytes (Lkc), neutrophil (Neutro) and monocyte counts correlated with worse RFS. As expected, tumor grade was correlated with RFS. Low Hb level, high Lkc and Neutro counts were all significantly associated with RFS after adjusting for grade. Strikingly, 32% of patients with pre-operative anemia experienced a recurrence at 5 years, compared with only 11% of non-anemic patients. Conclusion: In this exploratory study, we find that pre-operative CBC data, which is readily available, may contain prognostic information relevant to subsequent risk of recurrence or progression in meningioma. While the biological mechanism for these associations is not clear, they represent hypotheses for further investigation.

Intraventricular Meningiomas: A Series of 42 Patients at a Single Institution and Literature Review

Primary intraventricular meningiomas (IVMs) make up 0.5%-5% of all intracranial meningiomas and represent one of the most challenging lesions in neurosurgery. Between 1990 and 2013, 42 patients (30 female, 12 male; mean age, 43.6 years) underwent resection of their IVM. The removal was performed by a posterior parietal approach in 19 of the 40 lateral ventricle tumors, and 1 third ventricle meningioma. The transcallosal approach was used for 3 meningiomas, and patients with other lesions underwent temporal (7 cases) and temporoparietal approaches (12patients), respectively. The most common presenting signs were increased intracranial pressure (83.3%), visual impairment (78.6%) and cognitive changes (50%). Forty lesions (95.2%) arose in the lateral ventricles, and 2 (4.8%) in the third ventricle, ranging in size from 3 to 10 cm. Total removal was achieved in 39 cases and the pathology report disclosed World Health Organization grade I lesions in 41 cases. Hydrocephalus, cerebrospinal fluid leakage, and cerebral edema were the postoperative complications (7.15%); 1 patient died of respiratory problems not directly related to surgery. Thirty-five patients (83.3%) showed a 6-month Glasgow Outcome Scale of 5. One patient, who underwent partial resection, presented a recurrence after 1year that remained stable until last follow-up. IVMs usually reach a large size before being diagnosed. Surgical treatment is the most suitable option and total removal should represent the main goal of the procedure. The posterior parietal transulcal approach and the temporoparietal approach are the most common surgical routes used in our series.

Primary intraosseous meningioma: an osteosclerotic bone tumour mimicking malignancy

BackgroundSclerotic tumours of the calvarial bones are rare and may be due to primary and secondary bone tumours as well as extradural tumours of meningeal origin.Case presentationWe report a case of primary intraosseous meningioma (PIM) which arose in the frontal bone of a 63 year old woman who complained of progressive pain and thickening of the right skull. Radiology showed a large osteosclerotic lesion in the right frontal bone. Histology showed an intraosseous lesion containing dense fibrous tissue in which there were scattered cells that expressed epithelial membrane antigen and progesterone receptor. The tumour was partially resected and 3 years after operation has not recurred.ConclusionsPIM is a rare tumour which needs to be distinguished from primary/secondary osteosclerotic calvarial bone tumours.

Post-Traumatic Cutaneous Meningioma.

Post-Traumatic Cutaneous Meningioma.

Microsurgical management of primary jugular foramen meningiomas: a series of 22 cases and review of the literature

This study summarized clinical manifestations, surgical management, histological grading, and long-term outcome of jugular foramen meningiomas (JFMs). Retrospective study was performed in 22 consecutive patients with primary JFMs from January 2004 to October 2010, enrolling 10 men and 12 women with average age of 39.4 (14-57years). The most common initial symptom is hearing disorder, followed by lower cranial nerve dysfunctions. The tumor was classified into type I (intracranial) in 1, type II (intracranial) in 15, type IV (intracranial-extracranial) in 6, and none type III (extracranial). Surgical approaches mainly included retrosigmoid in 7, far lateral in 10, and juxtacondylar in 5, with some variations. The gross total resection was achieved in 15 cases and subtotal resection in 7. Fourteen patients (63.6%) developed new or worse neurological deficits immediately after operation, of whom 11 (78.6%) got alleviation. Postoperatively, keeping airway patency and prevention from aspiration pneumonia is very important. Nasotracheal intubation is much more tolerated than orotracheal intubation for postoperative patient management. WHO grade 2 was found in four cases (18.2%) and grade 3 in one. During the average time of follow-up in 83.2months, only one (grade 3) died of tumor regrowth 20months after surgery and radiosurgery. Five of 17 patients of grade 1 developed tumor regrowth. Radiosurgery provides a good tumor control for tumor regrowth in grade 1, or postoperative grade 2 tumor. In conclusion, JFMs has a favorable long-term overall survival; however, neurological preservation is still challenging, especially low cranial nerves.

Brain Invasion in Meningiomas: Incidence and Correlations with Clinical Variables and Prognosis

In meningioma, correlation of brain invasion with prognosis and clinical variables remains controversial. Correlation of brain invasion with clinical and histopathologic variables was investigated in 467 patients with primary intracranial meningioma. Diffuse (n= 3; 10%), clusterlike (n= 11; 34%) or fingerlike (n= 18; 56%) invasion was detected in 32 patients (7%). Brain invasion was more common in males than in females (13% vs. 5%; odds ratio, 2.75; 95% confidence interval, 1.29-5.89; P= 0.009) and pattern of invasion differed between genders (P= 0.037). Brain invasion was absent in 401 benign meningiomas and present in 48% of 60 atypical (n= 29) and 50% of 6 anaplastic (n= 3) meningiomas (P < 0.001) but was independent of tumor location and extent of resection. Progression occurred in 11% and was more frequent (31% vs. 15%; P= 0.036) in invasive than in noninvasive tumors, but only after gross total resection and in univariate analyses, and independent of invasion pattern. In atypical meningiomas, frequency of adjuvant irradiation was similar comparing invasive and noninvasive tumors and grading solely based on brain invasion (n= 20; 33%), other World Health Organization (WHO) criteria (n= 31; 52%) or a combination of both (n=9; 15%). Risk of recurrence was lower (hazard ratio, 0.258, 95% confidence interval, 0.09-0.734; P= 0.011) when grading exclusively based on brain invasion than when further WHO criteria were in addition present and the progression-free interval among the first was similar to benign tumors. Brain invasion and its patterns are correlated to gender. In contrast to the current WHO classification, invasion was associated with recurrence only after gross total resection and not independent of further histopathologic criteria of atypia.

Primary intraspinal papillary meningioma: a rare aggressive variant of meningioma

Primary intraspinal papillary meningioma: a rare aggressive variant of meningioma

Giant intraosseous meningioma mimicking fibrous dysplasia

We illustrate a case of a giant primary intraosseous meningiomas (PIMs) with optic nerve compression treated by partial resection. A 48-year-old female presented with visual disturbances exophthalmus, diplopia and eye pain due to optic nerve compression. The patient had a past history of fibrous dysplasia treated surgically with partial resection by ENT in 2010. Histology confirmed the diagnosis. Two years later she presented with further decrease in visual acuity and diffuse hyperostosis. In this context she underwent a neurosurgical procedure consisting in craniotomy and further partial resection of the lesion including optic canal decompression. The second pathological examination demonstrated an extensive meningioma (WHO grade I) of the skull base. We recommend that in cases of diffuse hyperostosis the differential diagnosis include diffuse intraosseous meningioma. If complete surgical resection is not achievable, a biopsy to confirm the diagnosis is recommended. Treatment options include complete or partial resection followed by adjuvant radiotherapy.

Evidence for ancient meningiomas and a probable case from Medieval Tarbat, Scotland

We report the case of a probable meningioma in a cranium excavated from the Medieval site of Portmahomack on the Tarbat Peninsula in Scotland (Carver, 2008). Stratigraphic evidence enabled dating of the remains to a post-Pictish and pre-Reformation date. Meningiomas usually arise from the arachnoid membrane of the meninges (Yamazaki et al., 2001) and now represent approximately 20% of all primary intracranial tumours (Yener et al., 2009). They can result in hyperostosis of adjacent bone, osteolytic reactions, or both. We review the evidence for ancient meningiomas and describe a differential diagnosis of the pathology from the Tarbat cranium, including vascular and osteoid lesions, intracerebral malignancies, metastases and other benign lesions. The cranium that we present is from an adult male, and has a frontoparietal lesion approximately 3cm in diameter, which is characterised by bone growth and remodelling of the outer table, and endocranial bone destruction. Supporting photographic and radiological evidence is presented. We conclude that a diagnosis of meningioma is most likely in this case, and may represent the rarer primary extradural meningioma.

The role and regulatory mechanism of IL-1β on the methylation of the NF2 gene in benign meningiomas and leptomeninges.

Methylation of the neurofibromatosis type 2 (NF2) gene in low-grade meningioma (WHO grade I) has crucial roles in tumorigenesis and development. Meningioma formation might also occur in the setting of an inflammatory microenvironment. However, the association between inflammation and the methylation of NF2 remains unclear. The present study investigates the role and regulatory mechanism of IL-1β, one of the most important pro-inflammatory cytokines, in the methylation of NF2 in benign meningioma. Three primary low-grade meningioma cells and leptomeningeal cells were cultured. CCK-8 and BrdU assays demonstrated that proliferation of meningioma/leptomeningeal cells treated with IL-1β occurred in a dose- and time-dependent manner. Methylation-specific PCR verified that IL-1β induced methylation of the NF2 promoter and decreased NF2/merlin expression in meningioma/leptomeningeal cells. Real-time PCR, western blotting, and immunofluorescence showed that IL-1β up-regulated DNMT1 in meningioma cells and DNMT1/3b in leptomeningeal cells but did not up-regulate DNMT3a. After co-treatment with the DNMT inhibitor 5-Aza-2'-deoxycytidine and DNMT siRNA, methylation of NF2 induced by IL-1β was attenuated and merlin expression was restored. Furthermore, we showed that DNMT1 in meningiomas and DNMT1/3b in leptomeninges were regulated via activation of the MAPK (p38, ERK, JNK) and NF-κB pathways. These results suggest that IL-1β induces methylation of NF2 by up-regulating DNMT1 in benign meningioma cells and DNMT1/3b in leptomeningeal cells via MAPK and NF-κB pathways. Therefore, NF2 methylation is a linker between IL-1β and tumor development, and DNMTs might be potential therapeutic targets in meningioma for regulating NF2 and inhibiting tumor development. © 2016 Wiley Periodicals, Inc.

The Simpson grading revisited: aggressive surgery and its place in modern meningioma management.

OBJECTIVE Recent advances in radiotherapy and neuroimaging have called into question the traditional role of aggressive resections in patients with meningiomas. In the present study the authors reviewed their institutional experience with a policy based on maximal safe resections for meningiomas, and they analyzed the impact of the degree of resection on functional outcome and progression-free survival (PFS). METHODS The authors retrospectively analyzed 901 consecutive patients with primary meningiomas (716 WHO Grade I, 174 Grade II, and 11 Grade III) who underwent resections at the University Hospital of Bonn between 1996 and 2008. Clinical and treatment parameters as well as tumor characteristics were analyzed using standard statistical methods. RESULTS The median follow-up was 62 months. PFS rates at 5 and 10 years were 92.6% and 86.0%, respectively. Younger age, higher preoperative Karnofsky Performance Scale (KPS) score, and convexity tumor location, but not the degree of resection, were identified as independent predictors of a good functional outcome (defined as KPS Score 90-100). Independent predictors of PFS were degree of resection (Simpson Grade I vs II vs III vs IV), MIB-1 index (< 5% vs 5%-10% vs >10%), histological grade (WHO I vs II vs III), tumor size (≤ 6 vs > 6 cm), tumor multiplicity, and location. A Simpson Grade II rather than Grade I resection more than doubled the risk of recurrence at 10 years in the overall series (18.8% vs 8.5%). The impact of aggressive resections was much stronger in higher grade meningiomas. CONCLUSIONS A policy of maximal safe resections for meningiomas prolongs PFS and is not associated with increased morbidity.

Primary intraosseous atypical inflammatory meningioma presenting as a lytic skull lesion: Case report with review of literature

Primary extradural meningiomas of the skull comprise 1% of all meningiomas, and lytic skull meningiomas are still rarer and are said to be more aggressive. We present a case of 38-year-old male with an extradural tumor which on histopathological examination showed features of inflammatory atypical meningioma (WHO Grade II). The intense inflammatory nature of osteolytic primary intraosseous meningioma has not been reported before. This entity deserves special mention because of the need for adjuvant therapy and proper follow-up.

Case report of a rare case of primary sinonasal meningioma

The aim of this study was to report a case of sinonasal meningioma in Sudan. Meningiomas account for nearly 20% of all intracranial neoplasms and are the second most common tumor of the central nervous system. Primary meningiomas of the nose and paranasal sinuses are extremely rare. The clinical and radiological features of these tumors are nonspecific, and consequently an accurate diagnosis requires histologic evaluation. A 62-year-old woman from North Sudan presented to our hospital in May of 2016 complaining of right nasal obstruction associated with bloody nasal secretion and proptosis. Endoscopic endonasal examination revealed a soft mass in the right nasal cavity between the septum and the middle and superior turbinates posteriorly. MRI revealed the presence of a lobulated mass with soft-part density in the right nasal cavity (middle meatus) and the right ethmoid sinus invading the orbital contents and extending intracranially. The biopsy of the lesion revealed nodular neoplasm composed of regular cells arranged in whorled pattern. One psammoma body was noted and there was no evidence of pleomorphism or necrosis. The features are consistent with sinonasal meningioma. The patient was referred to the oncological department because it was an unresectable malignant meningioma and surgery was not feasible and would create more complications. Meningiomas involving the nasal cavity and paranasal sinuses are rare. The mortality is low, and its lethality is due to the complications of the surgery and injury of vital structures. Complete surgical extirpation of sinonasal tract meningiomas has an overall good prognosis. Hence, endoscopic nasal approach is an excellent surgical option in these cases, due to its low morbidity and satisfactory lesion resection.

Tumor after tumor in two years: A rare presentation of brain glioma, meningioma and supraclavicular meningiosarcoma

Introduction: Low-grade gliomas (LGG) and meningioma are common brain tumors but it is rare for both tumors to be diagnosed in a same patient. Furthermore, it is rare for the same patient to develop an extracranial primary or metastasis meningioma. Probably this is the first case report of a second primary with extracranial meningiosarcoma in literature. Case Report: We present a rare case of brain atypical meningioma which occurred shortly following craniotomy and radiotherapy after being diagnosed as brain glioma (diffused astrocytoma) in a 22-year-old Malay female. Tumors occurred at different sites of the brain namely the atypical meningioma at the temporal and the initial glioma at the frontal region. Subsequently, patient developed a right supraclavicular meningiosarcoma. We believe that the patient could have developed radiation-induced meningioma but the transformation period is very short. The patient's mother had history of brain tumor that added into the risk factors in this patient. However, we are unable to confirm whether the supraclavicular mass was a primary supraclavicular meningiosarcoma or metastatic meningiosarcoma progression from atypical meningioma. Conclusion: There are multiple variant revealed associations of glioma and meningioma. However, these variant could be coincidental. There is a possibility that one tumor acts locally as an irritating factor that induced another unrelated neoplasm. Relation of two or more types of cancers might lead to new therapies, provided target surveillance for cancer patients and risk factors or causal agent are identified.

Primary Pulmonary Meningioma Simulating a Pulmonary Metastasis.

Primary pulmonary meningiomas represent a rare tumor entity. Few cases have been reported in the English medical literature, and they have almost all been solitary and benign in nature, with the exception of several extremely rare cases. We report herein a case of PPM that raised suspicion of a pulmonary metastatic tumor initially, as it was depicted as a single, round, small, ground-glass opacity pulmonary nodule on a chest computed tomography scan, in a 55-year-old man with a history of buccal cancer. Increased awareness of the clinical and radiologic characteristics of this rare category can assist a multidisciplinary team to perform adequate management.

縦隔に生じた異所性髄膜腫の一手術例

縦隔に生じた異所性髄膜腫の一手術例

Calcified intraventricular meningioma in 82-years-old woman: An unusual finding

Introduction: Primary Intraventricular meningiomas are rare and occur in much younger age group than other the intracranial meningiomas. Calcified intraventricular meningioma in elderly has never been reported before. Case Report: Here we report the unique case of an 82 year-old female who presented for headache and vomiting following a traffic accident. The neurological examination disclosed no abnormalities. The CT scan revealed a calcified Intraventricular meningioma. Conclusion: Since the patient was aged over 70 years and was asymptomatic, the lesion was not removed. Follow-up imaging showed no changes in the tumor.

Pediatric intraventricular meningioma: A series of six cases.

Objective:Meningiomas in children is a rare occurrence. Primary intraventricular meningiomas (IVMs) are even rarer with reported incidence of 0.5%–5% of all meningiomas. The aim of the study was to describe the demographic profile, location, surgical approach, complications, and histopathology of six pediatric patients with IVM.Materials and Methods:We retrospectively analyzed all intraventricular tumors operated at our Institute from January 2010 till July 2015. Patients' clinical details and follow-up were obtained from hospital records and scans were obtained from picture archiving and communication system.Results:We found 6 pediatric patients (age ≤18 years) with histopathologically proven IVM. The mean age in this series was 14.6 years. Tumor was most commonly approached through the superior parietal lobule in this series. Gross total excision was achieved in all patients. The blood loss in the series was in the range of 600–2000 ml with a mean of 1100 ml. All were grade 1 meningioma on histopathological examination. Transitional meningioma was the most common histological subtype. None of the patients had a recurrence at last follow-up.Conclusions:Pediatric IVMs are rare tumors. They tend to have a male preponderance in contrast to adults which have a preference for females. Parietooccipital transcortical, transcallosal approach, middle/inferior temporal gyrus approach are the described techniques to tackle such tumors depending on the location of such tumors. Surgeons should watch out for massive blood loss during surgery, especially via the parietooccipital transcortical approach.