This article presents a case study of a rare convexity meningioma located in the frontal lobe of the right cerebellar hemisphere. Meningiomas comprise a substantial part of central nervous system neoplasms and are classified into benign, atypical, or anaplastic categories, each encompassing a variety of histological subtypes, among which the secretory meningioma is notably rare. A 77-year-old male presented with a clinical history of headache, impaired memory functions, an initial form of apathetic-abulic syndrome, and a single seizure, which were considered to be indicative of epileptic symptoms that had been present for several weeks. The imaging studies conducted showed a convexity tumor characterized by a rounded morphology and homogeneous contrast enhancement, positioned adjacent to the frontal lobe\'s cortical surface. This clinical report details the pathology of a secretory type of meningioma, which is distinguished by the atypical epithelial differentiation of meningothelial cells, resulting in hyaline fiber production. The neoplasm\'s anatomical accessibility permitted successful surgical resection. The tumor\'s position was appropriate for surgical removal, and the histological variant, along with the patient\'s favorable clinical course, is of particular scientific interest.

Large, extremely intricate labyrinthine structures were observed during an electron microscopic examination of a secretory meningioma that originated in the sphenoid ridge of a 73-year-old man. In many neoplastic cells, these labyrinthine structures formed round-shaped regions within the cytoplasm, and profiles indicated their internal structure consisted of numerous thin fragments of cytoplasm and intervening flocculent material. These labyrinthine structures did not exhibit apparent topographic association with pseudopsammoma bodies, and direct communication with the extracellular space by very thin channels was rarely observed. The structures resembled the \"intracytoplasmic lumina\" commonly seen in epithelial neoplasms; but they did not appear to be true \"intracytoplasmic\" closed structures but rather deep and elaborate invaginations of the plasma membrane into the cytoplasm. These structures are distinct from pseudopsammoma bodies and might represent another expression of the epithelial properties of neoplastic cells in this subtype of meningioma. Although their pathogenesis or significance remain unknown, these structures may significantly increase the surface area of neoplastic cells and facilitate the uptake of extracellular material.

An isolated proliferation of pericytes is a unique vascular reaction seen almost exclusively in the stroma of secretory meningioma. We report the results of immunohistochemical and ultrastructural studies of a pericytic proliferation that was found in two cases of meningioma (a secretory meningioma of the sphenoid ridge and a parasagittal atypical meningioma showing predominantly fibroblastic features). Pericytes had hyperchromatic nuclei and scant cytoplasm, and showed stratification or formed small clusters within the walls of small blood vessels. They occasionally showed close contact with pseudopsammoma bodies in secretory meningioma. Pericytes showed immunoreactivity for α-smooth muscle actin but were not immunoreactive for desmin. They also exhibited characteristic ultrastructural features of pericytes, including the presence of microfilaments and abundant pinocytotic vesicles, and investment by the basal lamina. This isolated pericytic proliferation is likely a peculiar response of the vascular wall, probably induced by some cytokines secreted from neoplastic meningothelial cells. The close contact of proliferating pericytes with pseudopsammoma bodies suggests a close pathogenetic association between them. The occurrence of pericytic proliferation that was found in our second case (atypical meningioma with predominantly fibroblastic features) is exceptional and has not been documented to date.

Cranioplasty with titanium mesh provides a stable and cosmetically sound option for the correction of extensive skull bone defects following trauma or tumour surgery with osseous involvement. Meningiomas are for the most part benign lesions that are amenable to surgical cure, however lesions with extradural extension pose additional challenges not only due to increased technical difficulty in achieving gross total resection but also because of distinct biological behaviour. We describe the case of a 43-years-old woman that had been submitted to gross total resection of a WHO grade I falcine and superior sagittal sinus secretory meningioma with extradural and bone extension and cranioplasty with a titanium mesh who had a recurrence 4 years later as two tumour masses on top of the titanium mesh with no adjacent soft tissue invasion, and without dural involvement. To our knowledge, this is the first reported case of meningioma growth on top of titanium cranioplasty material. Seeded or incompletely removed tumoral cells might have exploited the biocompatibility of titanium to promote tumour regrowth.

Background: Although meningiomas are common intracranial tumors, multiple meningiomas (MMs) are rare entities in patients without neurofibromatosis type 2. Previous studies suggest most sporadic MMs are of monoclone in origin. Objective: To elucidate the clonal relationship between two sporadic meningiomas from the same patient by using the next-generation sequencing (NGS) platform. Methods: Two MMs, located frontally and parietally on the right side, were surgically removed from a 52-year-old male. Pathological examinations and whole exome sequencing were performed on tumor samples, followed by Sanger sequencing validation. Results: MMs were diagnosed as secretory and fibrous subtypes, respectively, on histology (WHO grade I) and tumor DNA exhibited distinctive somatic mutation patterns. Specifically, the secretory subtype carried more single nucleotide variant while the fibrous subtype had much higher copy number variation. Besides, the two tumors demonstrated different mutation profiles in predisposing genes and known driver mutations. For example, the secretory subtype had missense mutations in TRAF7 and KLF4, while the fibrous subtype had frameshift deletion of NF2 gene in addition to copy number loss of NF2 and SMARCB1, genetic events that have already been associated with the development of meningiomas. Significantly mutated gene analysis revealed novel mutations of LOC729159 in the secretory subtype and RPGRIP1L and DPP6 in the fibrous subtype. Sanger sequencing validated important point mutations in TRAF7 (c.1678G>A, p.G560S), KLF4 (c.1225A>C, p.K409Q) and CDH11 (c.169T>G, p.W57G). Conclusion: Our data suggest the two meningiomas might develop independently in this patient and molecular subtyping by NGS is a valuable supplement to conventional pathology. Further study is needed to ascertain whether these novel genetic events are tumorigenic or simply passenger mutations, as well as their clinical implications.

Secretory meningioma is a rare form of meningiomas which differentiates from the meningothelial subtype. It is characterized by significant peritumor edema and distinct immunohistochemical and molecular genetic profiles. We present a middle aged female patient with secretory meningioma infiltrating the orbital bone from the primary cranial base location and causing exophthalmos, features rarely described with this tumor. Surgical resection was challenging because of the associated brain swelling and rich vascularization of the tumor. Imaging and immunohistochemical studies revealed characteristic hallmarks of secretory meningioma. While histologically it was a benign tumor, due to the orbital bone and soft tissue infiltration, postoperative management of neurological sequelae was challenging. This case highlights distinctive clinical, imaging and histological features along with individual characteristics of a rare form of meningiomas.
A szekretoros meningeoma a meningeomák ritka formája, mely a meningothelialis altípusból differenciálódik. Jelentős peritumoralis oedema és sajátos immunhisztokémiai és molekuláris genetikai profil jellemzi. Tanulmányunkban bemutatunk egy középkorú nőbeteget, akinek a szekretoros meningeomája az elsődleges agyalapi lokalizációból terjedt a csontos orbitára és az orbitaűrbe, így exophthalmust okozott, amit ritkán írtak le ilyen tumorokban. A tumor gazdag érellátottsága és a társult agyi oedema sebésztechnikai kihívást jelentett az eltávolítás során. A képalkotó és immunhisztokémiai feldolgozás a szekretoros meningeo­mák jellegzetességeit tárta fel. Míg hisztológiailag jóindulatú volt a tumor, az orbitalis csont- és lágyszövetek infiltrációja miatti posztoperatív neurológiai maradványtünetek kezelése nem kis kihívást jelentett. Tanulmányunk bemutatja a meningeomák ritka formájának megkülönböztető klinikai, radiológiai és hisztológiai jellegzetességeit, melyek további ritka sajátosságokkal társultak esetünkben.

Secretory meningioma (SM) is a rare histological subtype of the meningioma family. Few reports investigating SM have been published due to its extremely low incidence; thus, the current understanding of this disease is poor. We analyzed the incidence and clinical, radiological, pathological, and prognostic features of SM. Approximately 12,380 intracranial meningiomas were surgically resected at Beijing Tiantan Hospital between April 2008 and January 2017. All pathologically confirmed SM cases were identified. SMs accounted for approximately 1.2% of the intracranial meningiomas (149 of 12,380). The patients with SM had a mean age of 51.0 years and were predominantly female (112 female and 37 male). Radiologically, peritumoral brain edema was observed in 49 (32.9%) patients. Gross total resection was achieved in 115 (77.2%) cases. At the 35-months median follow-up (range 4-109 months), six patients had tumor recurrence, and one patient died from the tumor recurrence. The 5-year progression-free survival rates were 95.9%, and the 5-year overall survival rate was 99.3%. A skull base location and a tumor size ≥ 3.5 cm were significantly associated with poor short-term outcomes, and a skull base location was significantly associated with an increased risk of poor long-term outcomes (P < 0.05). A skull base location (OR 3.797; 95% CI 1.071-13.468; P = 0.039) and tumor size ≥ 3.5 cm (OR 2.616; 95% CI 1.107-6.181; P = 0.028) were independent risk factors for non-gross total resection. A son-skull base location (OR 0.070; 95% CI 0.028-0.177; P = 0.001) was the only independent risk factor that correlated with more severe peritumoral brain edema. SM is a rare subtype of meningiomas with a female predominance and low recurrence. Our results highlight the risk factors for short- and long-term outcomes, which can be useful for selecting treatments and predicting prognosis. Microsurgical treatment of a skull base SM remains a formidable challenge due to a large tumor size and critical neurovascular structure encasement.

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We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspected to be a metastasis. Histological examination revealed an anaplastic meningioma containing epithelial nests arranged in a gland-like pattern suggestive of adenocarcinoma. This component strongly expressed cytokeratins whereas the meningothelial component was vimentin--epithelial membrane antigen--and progesterone receptor-immunoreactive, suggesting either anaplastic meningioma with adenocarcinoma-like metaplasia, or adenocarcinoma metastasis in a meningioma, but the search for a primitive neoplasia including thoracic-abdominal-pelvic computed tomography and mammography was negative. Anaplastic meningiomas with adenocarcinoma-like metaplasia are uncommon lesions, 4 cases having been reported in the literature so far. Their immunohistochemical and chromosomal characteristics are similar to those observed in secretory meningiomas. When available, fluorescence in situ hybridization detects the same chromosomal alterations in the two components, confirming a common clonal origin. This observation demonstrates the necessity to perform the correct diagnosis of malignant meningioma with adenocarcinomatous metaplasia, whose prognosis and treatment radically differ from those of metastatic adenocarcinoma located in a meningioma.