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The WHO Grade I Collagen-Forming Meningioma Produces Angiogenic Substances

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The WHO Grade I Collagen-Forming Meningioma Produces Angiogenic Substances ANTICANCER RESEARCH 36: 941-950 (2016) The WHO Grade I Collagen-forming Meningioma Produces Angiogenic Substances. A New Meningioma Entity JOHANNES HAYBAECK1*, ELISABETH SMOLLE1,2*, BERNADETTE SCHÖKLER3 and REINHOLD KLEINERT1 1Department of Neuropathology, Institute of Pathology, Medical University Graz, Graz, Austria; 2Department of Internal Medicine, Division of Pulmonology, Medical University Graz, Graz, Austria; 3Department of Neurosurgery, Medical University Graz, Graz, Austria Abstract. Background: Meningiomas arise from arachnoid seems to be most appropriate. Conclusion: The "WHO Grade cap cells, the so-called meningiothelial cells. They account I collagen-forming meningioma" reported herein produces for 20-36% of all primary intracranial tumours, and arise collagen and angiogenic substances. To the best of our with an annual incidence of 1.8-13 per 100,000 knowledge, no such entity has been reported on in previous individuals/year. According to their histopathological features literature. We propose this collagen-producing meningioma meningiomas are classified either as grade I (meningiothelial, as a novel WHO grade I meningioma sub-type. fibrous/fibroblastic, transitional/mixed, psammomatous, angiomatous, microcystic, secretory and the Meningiomas arise from arachnoid cap cells, the so-called lympholasmacyterich sub-type), grade II (atypical and clear- meningiothelial cells (1-5). They account for 20-36% of all cell sub-type) or grade III (malignant or anaplastic primary intracranial tumours, and arise with an annual phenotype). Case Report: A 62-year-old female patient incidence of 1.8-13 per 100,000 individuals/year (1-5). presented to the hospital because of progressive obliviousness Meningiomas are the second most common central nervous and concentration difficulties. In the magnetic resonance system neoplasms in adults (6-9). The incidence of imaging (MRI) of the brain, an occipital convexity- meningiomas is increasing, which may be due to more meningioma was found in the left hemisphere, which was sensitive diagnostic modalities (1, 10). The peak incidence subsequently resected. Within the tumour tissue there were of meningiomas is in the middle-aged population, with a multiple spheroid precipitates, i.e. secretion products that female-to-male ratio of approximately 2:1 (11-14). turned out to consist of collagen. Part of the tumour cells Benign or low-grade meningiomas tend to occur in a displayed positive reactions for vasogenic substances, namely higher frequency in females, compared to males. This may for vascular endothelial growth factor (VEGF) and epidermal be explained by a progesterone-dependent tumour growth growth factor receptor (EGFR). Correspondingly, the (14-18). The so-called Clemmesen's hook is a phenomenon diagnosis "WHO Grade I collagen-forming meningioma" describing two peaks in the age-grouped distribution among female tumour patients. It is also observed for meningiomas, meaning that cancers with early onset reflect a strong hereditary pathogenesis whilst the acquired phenotypes tend *These authors contributed equally to this work. to occur at a later age (13, 19, 20). Meningiomas mostly feature a benign behavioral course Abbreviations: CNS: Central nervous system; DAL-1: protein 4.1B; and grow slowly. The majority of meningiomas are localized ECM: extracellular matrix; EGFR: epidermal growth factor receptor; MRI: magnetic resonance imaging; PDGFRB: platelet and non-invasive. However, meningiomas may also feature a derived growth factor receptor beta; VEGF: vascular endothelial more aggressive behavior with tendencies towards invasion growth factor; WHO: World Health Organization; YAP: yes- of adjacent structures, a high recurrence rate and extracranial associated protein. metastases (6, 21). A high histological grade, sub-total resection, young age, aggressive tumour sub-type, brain Correspondence to: Assoc. Prof. Johannes Haybaeck, MD, Ph.D., infiltration and a high proliferation rate predispose for Department of Neuropathology, Insitute of Pathology, Medical recurrence (16, 22-25). University of Graz, Graz, Austria. E-mail: johannes.haybaeck@ medunigraz.at Meningiomas are a heterogeneous group of tumours, with variable clinical presentation, a broad spectrum of Key Words: Meningioma, collagen-forming, angiogenic substances, histological patterns and an inherent trend to recur (26, 27). entity. Meningiomas may be located anywhere in the central 0250-7005/2016 $2.00+.40 941 ANTICANCER RESEARCH 36: 941-950 (2016) nervous system (4). Notably, it has been observed that World Outcome. According to a study by Lim et al. on the outcome Health Organization (WHO) grade I meningiomas are more of patients with malignant meningiomas, the median time to frequently located at the skull-base than other meningioma recurrence was 35 months (range=12-61), and the 5-year subtypes (18, 28, 29). progression-free survival rate was 53.6% (37). Goldsmith et Vascular invasion is commonly observed in meningiomas, al. reported a 5-year progression-free survival of 48% (38) however, metastasis only occurs in 0.1% of cases and only and Hug et al. reported 52% for malignant meningiomas (37, in grade III tumours (30). The lungs and pleura are the most 39). Since surgical removal is the primary treatment for common sites of metastasis, followed by the musculoskeletal malignant meningiomas, the surgical status becomes an system, liver, reticuloendothelial system and kidneys (31). important variable for prediction of recurrence (40-43). The occurrence of several meningioma variants is probably Incomplete tumour mass removal has a negative effect on related to the progenitor cells’ various functions, for tumour recurrence (39, 44-46). However, malignant example, meningothelial cap cells may exhibit diverse meningiomas may even recur after complete surgical morphological features and functions that overlap with both removal and after radiation therapy (25, 28, 40, 47). Since it epithelial and mesenchymal cells. This is probably due to the is difficult to completely resect the tumour by surgery, there complex ontogenesis of the meninges that have their is sometimes need of adjuvant treatment after surgery, such ontogenetic roots in both mesodermal cells and cells of the as radiation therapy or radiosurgery (40, 48-51). Many neural crest (32-34). authors emphasize that adjuvant radiotherapy after surgery Histopathology. In clinical practice, light microscopy of should always be performed for the treatment of malignant hematoxylin-eosin stained sections, analyzed based on the WHO meningiomas, because mortality rates and morbidity is guidelines, is used for diagnosis of meningiomas (4). According generally high (25, 26, 28, 52, 53). Stereotactic radiosurgery to their histopathological features meningiomas are classified is also an important treatment option, with lower either as grade I (the meningiothelial, fibrous/fibroblastic, complication rates compared to fractionated radiation therapy transitional/mixed, psammomatous, angiomatous, microcystic, (46, 54-56). Stereotactic radiosurgery has been found to secretory and the lympholasmacyterich subtype), grade II (the control tumour recurrence and to improve patients' survival atypical and the clear-cell subtype) or grade III (malignant or rates (38, 46). anaplastic phenotype) (21). The grade I secretory subtype can be differentiated from other subtypes by the occurrence of Case Report eosinophilic, non-fibrillary cytoplasm with eosinophilic refractile hyaline globules, according to a recent study (35). The A 62-year-old female patient presented to hospital because prevalence is highest for the benign category (78-81%), followed of progressive obliviousness and concentration difficulties. by the atypical variant (15-20%), whilst only 1-4% of In the magnetic resonance imaging (MRI) of the brain, an meningiomas are of the anaplastic phenotype (6). The recurrence occipital convexity-meningioma was seen in the left rates of grade I, II and III meningiomas range from 7-25%, 29- hemisphere. 52% and 50-94%, respectively (4). In the WHO classification of 2007, a lack of anaplastic Histopathological features. The meningioma was resected in a features characterizes grade I meningiomas. Grade I piecemeal-fashion. Together, the pieces measured 3×3×2.8 cm. meningiomas comprise: the meningiothelial, fibrous/ The tumour tissue was highly vascularized. The mitotic fibroblastic, transitional/mixed, psammomatous, angiomatous, activity of the tumour cells was low. Tumour cells showed microcystic, secretory and the lympholasmacyterich subtype syncytial formations with a rosette-like appearance. There was (26). Grade II meningiomas (atypical meningiomas; including only mild anisokaryosis, and some parts of the tumour the atypical and the clear-cell subtype) feature at least one of featured angioma-like characteristics. the following criteria: 1) choroid or clear cell histology, 2) 4 to Within the tumour tissue there were multiple spheroid 19 mitotic figures per ten high-power fields, 3) infiltration of precipitates, i.e. secretion products that turned out to consist the brain, and 4) three or more of the following five of collagen. The immunohistochemical reaction with histological characteristics: small cell change, increased collagen 1 and 3 was positive. Part of the tumour cells cellularity, prominent nucleoli, sheet-like growth, or necrosis displayed positive reactions for vasogenic substances, (4). Atypical or grade II meningiomas grow faster than the namely for vascular endothelial growth
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