Meningioangiomatosis Combined with Calcifying Pseudoneoplasms of Neuraxis
1
Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430062, China
2
Department of Pathology, Zhongnan Hospital of Wuhan University, Wuhan 430062, China
*
Author to whom correspondence should be addressed.
Brain Sci. 2023, 13(5), 786; https://doi.org/10.3390/brainsci13050786
Submission received: 3 April 2023
/
Revised: 20 April 2023
/
Accepted: 9 May 2023
/
Published: 11 May 2023
(This article belongs to the Special Issue Advances in Diagnosis and Treatment of Brain Tumors and Nervous System Tumors)
Abstract
:Meningioangiomatosis (MA) is a rare hamartomatous or meningovascular lesion involving the central nervous system, and is sometimes associated with intracranial meningiomas. Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare, slow-growing benign tumor-like lesions that can occur anywhere along the neuraxis. Here, we report a rare case of MA combined with CAPNON. A 31-year-old woman was admitted to our hospital because of a high-density mass in the left frontal lobe, detected by computed tomography (CT) during a physical examination. She had a 3-year history of obsessive–compulsive disorder. We describe the imaging, histopathological, and molecular characteristics of the patient. To our knowledge, this is the first report describing MA combined with CAPNON. We reviewed the literature on MA and CAPNON over the last decade and summarized the points for differential diagnosis and treatment. It is difficult to preoperatively distinguish between MA and CAPNON. However, this coexisting condition should be considered when intra-axial calcification lesions are observed on radiological imaging. Accurate diagnosis and appropriate treatment are likely to benefit this patient group.
1. Introduction
Meningioangiomatosis (MA) is a rare hamartomatous or meningovascular lesion involving the central nervous system. It occurs sporadically, or may be associated with neurofibromatosis type 2 (NF-2). The sporadic symptoms are headaches, seizures, and a variety of other neurological symptoms. MA associated with NF2 is usually asymptomatic and found incidentally [1]. MA was first described by Bassoe and Nuzum in the autopsy of a patient with NF2 in 1915 [2]. Later, the brain lesion was named ”meningioangiomatosis” by Worster-Drought et al. in 1937 [3]. Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare, slow-growing, benign tumor-like lesions that can occur anywhere along the neuraxis, including the brain and spine [4,5]. Originally described by Rhodes and Davis in 1978, CAPNON is also called fibro-osseous lesions [6]. In this article, we describe a rare co-existence of MA and CAPNON that has never been previously reported.
The authors report a case involving a 30-year-old woman with a high-density mass in the left frontal lobe, suspected to be a meningioma on computed tomography (CT). However, after surgical resection (Operative video see Supplementary Material: Video S1), the histopathological diagnosis of the lesion was MA combined with CAPNON. This study aims to provide a detailed analysis of this rare entity, including its clinical presentation and histopathological and imaging features, by collecting reports from other authors and our practical experience with a patient who underwent surgical resection at our institution. It is crucial to distinguish benign lesions from the more common vascular malformations and calcified vascular, neoplastic, or non-neoplastic differential diagnoses, because complete resection is curative [7]. In addition, we aim to analyze the possible mechanisms of its occurrence and development to provide references for treatment decisions.
2. Materials and Methods
We present a case of a patient with a histopathologically confirmed diagnosis of MA combined with CAPNON. Additionally, we conducted a comprehensive review of the literature on MA and CAPNON published in PubMed over the past 10 years. The search keywords included: meningioangiomatosis, calcifying pseudoneoplasm, and calcifying pseudotumor. The following parameters were collected from the qualifying articles: study type, number of patients, anatomical area, clinical presentation, radiological presentation, therapy, complications, and outcomes. In this review, we analyzed MA and CAPNON separately and discussed the mechanisms underlying their occurrence and development. This study was approved by the Ethics Committee of the Zhongnan Hospital of Wuhan University (no. 2019048).
3. Case Presentation
A 31-year-old woman was admitted for physical examination due to a high-density mass found by computed tomography (CT) in the left frontal lobe. The patient had a 3-year history of obsessive–compulsive disorder and experienced progressive improvement after taking sertraline tablets for 6 months. The CT scans (Figure 1A–C) revealed an irregular hyperdense mass in the left frontal lobe, measuring approximately 19 × 15 × 14 mm. Subsequent magnetic resonance imaging (MRI) revealed a hypo-intense mass with an unclear boundary on the T1-weighted image (Figure 1D) and an irregular mixed hypo-intense mass on the T2-weighted image (Figure 1E) and T2 FLAIR (Figure 1F). Irregular linear enhancement was observed on gadolinium-enhanced T1-weighted MRI (Figure 1G–I). Based on these initial images, meningioma was suggested.
The patient underwent a left frontotemporal craniotomy and complete resection. The gray-white and gray-brown lesions were relatively compact, and the boundary between the lesions and the surrounding brain tissue was unclear (Figure 2A). Immediate postoperative MRI confirmed the complete removal of the tumor. (Figure 1J–L). The patient recovered well, and no recurrence was observed on the MRI (Figure 1M–O) 3 months after surgery. However, the symptoms of the obsessive–compulsive disorder did not show considerable improvement.
4. Histopathology
Microscopic observation using hematoxylin and eosin (H&E) staining revealed that the lesion was primarily located between the pia mater and the cerebral cortex. A clear border was observed between the MA and CAPNON (Figure 2B,E). In the superficial cortical areas of the brain parenchyma, proliferating vessels surrounded by spindle cells were observed. Psammoma bodies, partially surrounded by small proliferating blood vessels, were visible, which are a classic pathological feature of MA (Figure 2B–D). Large calcifications were observed in the pia mater, and the calcified components were irregular or nested with proliferating spindle cells around them, which are typical signs of CAPNON (Figure 2E–G).
Immunohistochemically, the meningothelial cells positively expressed CD34 (Figure 2H), the somatostatin receptor (SSTR-2), the epithelial membrane antigen (EMA), vimentin (Figure 2I), and the progesterone receptor (PR) to varying extents. The stained meningothelial cells were mainly located on the surface of the pia mater and around the blood vessels of the cerebral cortex. Neurons invading the lesion were immunopositive for neuronal nuclei (NeuN), whereas the glial cells were immunopositive for glial fibrillary acid protein (GFAP) and oligodendrocyte line transcription factor 2 (Olig-2). The Ki-67 proliferation index was low (1%).
5. Results
Based on the results of H&E and immunohistochemical staining for specific biomarkers, the histopathological diagnosis of this lesion was MA combined with CAPNON, accompanied by the proliferation of meningothelial cells.
6. Discussion
MA and CAPNON are two rare lesions of the nervous system. We conducted a comprehensive review of the literature on MA and CAPNON published on PubMed in the last 10 years. Our review included 62 cases of MA (Table 1) and 50 cases of CAPNON (Table 2).
Among the 62 patients with MA, 43 were male (69.35%) and 19 were female (30.65%) patients, suggesting that MA has a male predominance. The ages of the 62 patients ranged from 8 months to 73 years, with an average of 19.85 years. However, 35 of the 62 patients were under 18 years of age (56.45%), indicating that MA has predominance in young patients. Most MA lesions are sporadic. From 2013 to 2022, NF-2 signs were only reported in 1 of the 62 MA cases in PubMed [8]. NF2 is a neurocutaneous disorder that may potentially develop into schwannomas, meningiomas, and ependymomas. A 2-year-old boy presented with multiple cystic meningioangiomatosis and a grade II ependymoma in the right cerebellum, which was found incidentally after trauma. In sporadic MA cases, the most common symptoms were refractory seizures (46/61; 75.41%) and/or headaches (9/61; 14.75%). Among the 62 MA cases, the lesions were mostly located in the temporal (22/62, 35.48%), frontal (21/62, 33.87%), and parietal lobes (16/62, 25.81%), and could be associated with epilepsy and mental symptoms. Only six cases occurred in the occipital lobe (9.68%). Intracranial lesions coexisting with MA have been reported in 17 patients [8,9,10,11,12,13,14,15], and meningioma was the most common combined tumor (11/17, 64.71%) [9,10,13,14,15]. Notably, the perivascular spread of meningioma-associated-type MA should not be interpreted as evidence of a grade II meningioma.
Among the 50 patients with CAPNON, 23 were male and 27 were female, with a mean age of 47.63 years and ranging from 8 to 73 years old. Although CAPNON can occur anywhere along the neuraxis, the intracranial location was the most common (39/50, 78%), with the remaining cases occurring in the spine (9 cases) and the craniocervical junction (2 cases). Furthermore, among the 39 intracranial CAPNON cases, 26 (66.67%) were supratentorial and 13 (33.33%) were infratentorial. Ho et al. reported that nearly one-third of CAPNONs were “collision” lesions, where the CAPNON tissue coexisted with a separate, distinct entity [16]. In seven cases, coexistent primary tumors were described, including meningioma [17], lipoma [18,19,20], and low-grade glioma [21]. The most common symptoms of intracranial CAPNON were headaches (17/39, 43.59%) and/or seizures (13/39, 33.33%), with a few patients presenting with cranial nerve defects, such as hoarseness, decreased hearing, or gait disturbance. In a 2013 study by Stienen et al. [7]., the authors reviewed all reported cases of CAPNON between 1977 and December 2011. Of the 22 patients with intracranial CAPNON, who had a mean age of 45 years, 19 had supratentorial CAPNON. The modes of presentation included epileptic seizures in eight patients, headache in five, cranial nerve affection in four, and dizziness and limb paresis in three. This finding is consistent with that of our literature review.
Diagnosing MA and CAPNON can be challenging due to the lack of characteristic signs to differentiate between them on preoperative imaging. MA often presents with a certain extent of calcification and contrast enhancement on CT and MRI [22]. One study reported that calcification was prevalent in 89.6% of the patients with MA. The lesion is generally confined to the cortex [23,24], with mostly low signals on MRI-T1WI and dark, wavy “cyclotron” signals on T2WI. In contrast, CAPNON shows little or no contrast enhancement with severe or peripheral calcification on CT and MRI. Due to the lack of specific clinical presentations and typical imaging features [25], postoperative pathological diagnosis is crucial for both MA and CAPNON. MA is generally characterized by proliferative meningothelial and fibroblast-like cells that infiltrate the leptomeninges with hypercellular areas and sclerosis [10], which majorly involves the outer layers of the cortex. In the adjacent cortex, large ganglion cells are isolated by thickened blood vessels, and the neuronal fibers in the trapped ganglion cells show tangling changes. The histological features of MA include meningovascular and leptomeningeal hyperplasia and calcification. Immunohistochemical staining is not very effective in diagnosing MA due to the lack of consistent markers [13]. The common histological features of CAPNON include chondromyxoid regions, palisading spindle cells, fibrous stroma, calcifications, and psammoma bodies [26]. However, the presence of these components is highly variable among the reported cases. The major immunohistochemical findings were the presence of epithelial membrane antigen (EMA) and vimentin, as well as the absence of glial fibrillary acid protein (GFAP) and S-100 protein [7]. As shown in Table 2, EMA was positive in 17 cases (70.83%; 17/24), and vimentin was detected in all 10 cases (100%; 10/10). However, the S-100 and GFAP were negative in 14 (82.35%; 14/17) and 11 cases (61.11%; 11/18), respectively.
The differential diagnoses of MA mainly include meningioma, vascular malformation, and cavernous hemangioma. Meningiomas are usually associated with dural diseases, whereas MAs often involve the cerebral cortical surface. Vascular malformations and cavernous hemangiomas generally lack perivascular meningothelial cell proliferation or psammoma bodies. In contrast, the most prominent feature of CAPNON is its highly dense calcification; therefore, oligodendrocytoma, astrocytoma, meningioma, osteosarcoma, chondrosarcoma, and neoplastic calcinosis are all important differential diagnoses.
Surgical resection is the most effective treatment for MA and CAPNON. In this study, 58 MA cases (93.55%;58/62) and 46 CAPNON cases (92%;46/50) were selected for surgery, particularly in high-risk areas. For instance, Lucila Domecq Laplace et al. [27] reported three cases of CAPNON located in the posterior fossa with perilesional edema, and the vast majority were cured after total resection. However patients may experience recurrence because of incomplete evacuation [28], neoplastic malignancy [12], or coexisting with other malignant tumors [20,21]. In addition to surgery, medical treatment and follow-up observations have been reported [1,29,30,31]. Shlomit et al. [29] reported a 67-year-old man with diffuse meningioma involving the occipital and right temporal lobes. After being diagnosed with MA by biopsy, the patient benefited from antiangiogenic therapy with bevacizumab, which led to clinical stability and marked radiological improvement. Similarly, indomethacin, an anti-inflammatory drug, was administered as a treatment option in a 44-year-old woman with chest CAPNON. After completing the treatment, the patient’s symptoms were completely relieved, and the lesion disappeared on the CT and MR imaging [31]. These successful non-surgical treatments shed light on the mechanisms underlying the development and progression of MA and CAPNON.
7. Conclusions
Herein, we report a rare case of MA coexisting with CAPNON, which was treated with tumor resection and had a good prognosis. Histological H&E staining revealed proliferating vessels and psammoma bodies encased by blood vessels, suggesting that the pathogenesis of MA is associated with cortical vascular malformations and the secondary proliferation of meningothelial cells. The formation of psammoma bodies may be stimulated by vascular proliferation. Most current studies suggest that CAPNON may represent a series of reactive processes that can arise in association with diverse underlying pathologies, including inflammatory, degenerative, vascular, and neoplastic lesions. In the present case, meningeal vascular proliferation and fine leptomeningeal epithelial proliferation may have led to the benign reactive lesions of CAPNON. Although nonsurgical treatment has been successful in some cases, complete resection is still attempted, when feasible, to relieve the patient’s symptoms and perform histopathological analysis. Definitive diagnosis is based on histopathological analyses to prevent the patient from having to undergo aggressive adjuvant therapy.
Supplementary Materials
The following supporting information can be downloaded at: https://www.mdpi.com/article/10.3390/brainsci13050786/s1. Video S1: Operative video.
Author Contributions
Z.L. contributed to the conception and design of the manuscript and reviewed and modified the manuscript. X.S. collected the data and drafted the manuscript. C.X. performed the operation, provided samples, and gave clinical guidance. Y.C. offered immunohistochemistry. Z.P. performed the formal analysis. All authors have read and agreed to the published version of the manuscript.
Funding
This research was supported by the National Health Commission of China (no. 2018ZX-07S-011).
Institutional Review Board Statement
This study was approved by the Ethics Committee of the Zhongnan Hospital of Wuhan University (no. 2019048).
Informed Consent Statement
Informed consent was obtained from all subjects involved in the study. Written informed consent was obtained from the patient(s) to publish this paper.
Data Availability Statement
Data are available upon request.
Conflicts of Interest
The authors declare no conflict of interest.
References
- Free, S.; Berg, A.; Asfora, W.; Freeman, J. Sporadic Meningioangiomatosis: Bystander or Curious Culprit. South Dakota Med. 2018, 71, 120–124. [Google Scholar]
- Bassoe, P.; Nuzum, F. Report of a Case of Central and Peripheral Neurofibromatosis. J. Nerv. Ment. Dis. 1915, 42, 785–796. [Google Scholar] [CrossRef]
- Worster-Drought, C.; Dickson, W.E.C.; Mcmenemey, W.H. Multiple Meningeal and Perineural Tumours with Analogous Changes in the Glia and Ependyma (Neurofibroblastomatosis): With Report of Two Cases. Brain 1937, 60, 85–117. [Google Scholar] [CrossRef]
- Lu, J.-Q.; Popovic, S.; Provias, J.; Cenic, A. Collision Lesions of Calcifying Pseudoneoplasm of the Neuraxis and Rheumatoid Nodules: A Case Report with New Pathogenic Insights. Int. J. Surg. Pathol. 2021, 29, 314–320. [Google Scholar] [CrossRef] [PubMed]
- Li, W.-Q.; Wang, S.-H.; Zhang, Z.-W.; Chen, J.; Li, Y.-M.; Lv, Z.-C.; Cao, H.-T.; Ma, X.-M.; Liu, H.-M.; Zhu, Z. Calcifying pseudoneoplasms of the neuraxis (CAPNON). A case report. Neuropathology 2021, 41, 371–375. [Google Scholar] [CrossRef] [PubMed]
- Rhodes, R.H.; Davis, R.L. An unusual fibro-osseous component in intracranial lesions. Hum. Pathol. 1978, 9, 309–319. [Google Scholar] [CrossRef]
- Stienen, M.N.; Abdulazim, A.; Gautschi, O.P.; Schneiderhan, T.M.; Hildebrandt, G.; Lücke, S. Calcifying pseudoneoplasms of the neuraxis (CAPNON): Clinical features and therapeutic options. Acta Neurochir. 2013, 155, 9–17. [Google Scholar] [CrossRef] [PubMed]
- Wheeler, A.; Metrock, K.; Li, R.; Singh, S. Cystic meningioangiomatosis and cerebellar ependymoma in a child with neurofibromatosis type 2. Radiol. Case Rep. 2022, 17, 1082–1087. [Google Scholar] [CrossRef] [PubMed]
- Hassan, O.; Ghanchi, H.; Reyes, K.D.; Raghavan, R.; Minasian, T. Pediatric Temporal Lobe Meningioma with Meningioangiomatosis Mimicking Invasive Meningioma. Cureus 2021, 13, e18819. [Google Scholar] [CrossRef]
- Galloway, L.; Zilani, G.; Lammie, A.; Leach, P. Meningioma with rhabdoid features combined with meningioangiomatosis in infancy: A novel combination. Child’s Nerv. Syst. 2020, 36, 1311–1314. [Google Scholar] [CrossRef]
- Iorgulescu, J.B.; Ferris, S.; Agarwal, A.; Zambrano, S.C.; Hill, D.A.; Schmidt, R.; Perry, A. Non-meningothelial meningeal tumours with meningioangiomatosis-like pattern of spread. Neuropathol. Appl. Neurobiol. 2018, 44, 743–746. [Google Scholar] [CrossRef] [PubMed]
- Rossi, S.; Brenca, M.; Zanatta, L.; Trincia, E.; Guerriero, A.; Pizzato, C.; Fiorindi, A.; Viscardi, E.; Giangaspero, F.; Maestro, R.; et al. A Pediatric Intra-Axial Malignant SMARCB1-Deficient Desmoplastic Tumor Arising in Meningioangiomatosis. J. Neuropathol. Exp. Neurol. 2018, 77, 883–889. [Google Scholar] [CrossRef] [PubMed]
- Zhang, C.; Wang, Y.; Wang, X.; Zhang, J.-G.; Li, J.-J.; Hu, W.-H.; Zhang, K. Sporadic meningioangiomatosis with and without meningioma: Analysis of clinical differences and risk factors for poor seizure outcomes. Acta Neurochir. 2015, 157, 841–853. [Google Scholar] [CrossRef] [PubMed]
- Jamil, O.; Ramkissoon, S.; Folkerth, R.; Smith, E. Multifocal meningioangiomatosis in a 3-year-old patient. J. Neurosurgery Pediatr. 2012, 10, 486–489. [Google Scholar] [CrossRef]
- Cui, H.; Shi, H.; Chen, X.; Wang, W.; Lai, R.; Han, A. Clinicopathological Features of Meningioangiomatosis Associated with Meningioma: A Case Report with Literature Review. Case Rep. Oncol. Med. 2012, 2012, 296286. [Google Scholar]
- Ho, M.; Eschbacher, K.L.; Paolini, M.A.; Raghunathan, A. New insights into calcifying pseudoneoplasm of the neuraxis (CAPNON): A 20-year radiological–pathological study of 37 cases. Histopathology 2020, 76, 1055–1069. [Google Scholar] [CrossRef]
- Paolini, M.A.; Ho, M.-L.; Monahan, H.R.; Raghunathan, A. Supratentorial CAPNON associated with WHO grade II meningioma: A case report. Neuropathology 2018, 38, 535–538. [Google Scholar] [CrossRef]
- Soukup, J.; Kohout, A.; Vosmikova, H.; Hacova, M.; Kaiser, M.; Klener, J.; Krejci, T.; Syrucek, M.; Wozniakova, M.; Gabalec, F.; et al. Calcifying pseudoneoplasm of neuroaxis (CAPNON): A comprehensive immunohistochemical and morphological characterization of five cases. Virchows Arch. 2022, 480, 415–423. [Google Scholar] [CrossRef]
- Inukai, M.; Shibahara, I.; Hotta, M.; Miyasaka, K.; Sato, S.; Hide, T.; Saegusa, M.; Kumabe, T. Case of Calcifying Pseudoneoplasms of the Neuraxis Coexisting with Interhemispheric Lipoma and Agenesis of the Corpus Callosum: Involvement of Infiltrating Macrophages. World Neurosurg. 2020, 134, 635–640.e1. [Google Scholar] [CrossRef]
- Watanabe, A.; Nakanishi, K.; Kataoka, K.; Wakasa, T.; Ohta, Y. Regrowth and progression of multiple calcifying pseudoneoplasms of the neuraxis: Case report. Surg. Neurol. Int. 2018, 9, 243. [Google Scholar] [CrossRef]
- Higa, N.; Yokoo, H.; Hirano, H.; Yonezawa, H.; Oyoshi, T.; Goto, Y.; Arita, K. Calcifying pseudoneoplasm of the neuraxis in direct continuity with a low-grade glioma: A case report and review of the literature. Neuropathology 2017, 37, 446–451. [Google Scholar] [CrossRef] [PubMed]
- Makary, M.S.; Kobalka, P.; Giglio, P.; Slone, H.W. Meningioangiomatosis: Clinical, Imaging, and Histopathologic Characteristics. J. Clin. Imaging Sci. 2020, 10, 36. [Google Scholar] [CrossRef] [PubMed]
- Kashlan, O.N.; LaBorde, D.V.; Davison, L.; Saindane, A.M.; Brat, D.; Hudgins, P.A.; Gross, R.E. Meningioangiomatosis: A Case Report and Literature Review Emphasizing Diverse Appearance on Different Imaging Modalities. Case Rep. Neurol. Med. 2011, 2011, 361203. [Google Scholar] [CrossRef] [PubMed]
- Bulut, E.; Mut, M.; Soylemezoglu, F.; Oguz, K.K. Meningioangiomatosis of the cerebellum: Radiopathologic characteristics of a case. Acta Neurochir. 2015, 157, 1371–1372. [Google Scholar] [CrossRef]
- Yao, Z.; Wang, Y.; Zee, C.; Feng, X.; Sun, H. Computed Tomography and Magnetic Resonance Appearance of Sporadic Meningioangiomatosis Correlated with Pathological Findings. J. Comput. Assist. Tomogr. 2009, 33, 799–804. [Google Scholar] [CrossRef]
- Dallimore, C.A.; Quelle, M.; Désir, L.L.; Sham, S.; Harshan, M.; Wahl, S.J.; Zlochower, A.; Goodman, R.R.; Langer, D.J.; D’Amico, R.S. Calcifying Pseudoneoplasm of the Neuraxis in the Posterior Fossa: A Case Report and Literature Review. Cureus 2022, 14, e21562. [Google Scholar] [CrossRef]
- Domecq-Laplace, L.; Ruella, M.; Caffaratti, G.; Villamil, F.; Monsalve, M.; Alcorta, S.C.; Cervio, A. Posterior Fossa Calcifying Pseudoneoplasm of the Neuraxis (CAPNON). Presentation of three surgical cases. World Neurosurg. 2022, 167, e423–e431. [Google Scholar] [CrossRef]
- Anand, R.; Garling, R.J.; Poulik, J.; Sabolich, M.; Goodrich, D.J.; Sood, S.; Harris, C.A.; Haridas, A. Sporadic Meningioangiomatosis: A Series of Three Pediatric Cases. Cureus 2017, 9, e1640. [Google Scholar] [CrossRef]
- Yust-Katz, S.; Fuller, G.; Fichman-Horn, S.; Michaeli, N.; Inbar, E.; Lukman, J.; Limon, D.; Steiner, I.; Siegal, T. Progressive diffuse meningioangiomatosis: Response to bevacizumab treatment. Neurology 2016, 86, 1643–1644. [Google Scholar]
- Pisano, A.; Boschi, A.; Romoli, S. Calcifying pseudoneoplasms of the neuraxis: Not only surgical treatment. Asian J. Neurosurg. 2020, 15, 796–797. [Google Scholar]
- Kwan, M.K.; Abdelhai, A.M.; Saw, L.B.; Chan, C.Y.W. Symptomatic calcifying pseudotumor of the thoracic spine that resolved with the indomethacin treatment: A case report. Spine 2012, 37, E1676–E1679. [Google Scholar] [CrossRef] [PubMed]
Figure 1.
(A–C) Computed tomography (CT) shows an irregular hyper-dense mass in the left frontal lobe, measuring approximately 19 × 15 × 14 mm. Subsequent magnetic resonance imaging (MRI) demonstrates (D) a hypo-intense mass with an unclear boundary on the T1-weighted image and (E,F) an irregular mixed hypo-intense mass on the T2-weighted image and T2 FLAIR. (G–I) Irregular linear enhancement is visible on gadolinium-enhanced T1-weighted MRI. (J–L) Postoperative MRI confirms complete resection of the lesion. (M–O) Three months of postoperative follow-up; MRI shows no recurrence.
Figure 1.
(A–C) Computed tomography (CT) shows an irregular hyper-dense mass in the left frontal lobe, measuring approximately 19 × 15 × 14 mm. Subsequent magnetic resonance imaging (MRI) demonstrates (D) a hypo-intense mass with an unclear boundary on the T1-weighted image and (E,F) an irregular mixed hypo-intense mass on the T2-weighted image and T2 FLAIR. (G–I) Irregular linear enhancement is visible on gadolinium-enhanced T1-weighted MRI. (J–L) Postoperative MRI confirms complete resection of the lesion. (M–O) Three months of postoperative follow-up; MRI shows no recurrence.
Figure 2.
(A) Intraoperative microscopic photograph. (B) MA area within the superficial cortex of the brain parenchyma with proliferating small blood vessels (green arrows) and perivascular spindle cells (green asterisks) (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (C) Vascular proliferation, spindle cells, and psammoma bodies are visible (H&E stain, original magnification ×100, scale bar: 1 cm = 100 μm). (D) Proliferating blood vessels (green arrows) and psammoma bodies encased by blood vessels (red asterisks), possibly stimulated to form due to vascular proliferation (H&E stain, original magnification ×200 scale bar: 1 cm = 50 μm). (E) Large calcifications in the pia mater (blue arrows) (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (F) Large irregular calcifications and surrounding proliferating spindle cells (green asterisks) can be seen (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (G) Obvious calcification and some lymphocytes are visible (in red circle) (H&E stain, original magnification ×100, scale bar: 1 cm = 100 μm). (H) Meningioangiomatosis area: Spindle cells from perivascular hyperplasia were positive for CD34 (immunohistochemical stain, ×200, scale bar: 1 cm = 50 μm). (I) Calcifying pseudoneoplasms of the neuraxis area: Spindle cells around calcification are vimentin-positive (immunohistochemical stain, ×200, scale bar: 1 cm = 50 μm).
Figure 2.
(A) Intraoperative microscopic photograph. (B) MA area within the superficial cortex of the brain parenchyma with proliferating small blood vessels (green arrows) and perivascular spindle cells (green asterisks) (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (C) Vascular proliferation, spindle cells, and psammoma bodies are visible (H&E stain, original magnification ×100, scale bar: 1 cm = 100 μm). (D) Proliferating blood vessels (green arrows) and psammoma bodies encased by blood vessels (red asterisks), possibly stimulated to form due to vascular proliferation (H&E stain, original magnification ×200 scale bar: 1 cm = 50 μm). (E) Large calcifications in the pia mater (blue arrows) (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (F) Large irregular calcifications and surrounding proliferating spindle cells (green asterisks) can be seen (H&E stain, original magnification ×40, scale bar: 1 cm = 250 μm). (G) Obvious calcification and some lymphocytes are visible (in red circle) (H&E stain, original magnification ×100, scale bar: 1 cm = 100 μm). (H) Meningioangiomatosis area: Spindle cells from perivascular hyperplasia were positive for CD34 (immunohistochemical stain, ×200, scale bar: 1 cm = 50 μm). (I) Calcifying pseudoneoplasms of the neuraxis area: Spindle cells around calcification are vimentin-positive (immunohistochemical stain, ×200, scale bar: 1 cm = 50 μm).
Table 1.
Summary of clinical features and surgical outcomes of 62 MA cases in the literature of PubMed in the last ten years.
Table 1.
Summary of clinical features and surgical outcomes of 62 MA cases in the literature of PubMed in the last ten years.
| Authors | Case | Age (yr)/Sex | Site | Clinical Presentation | Combine with | Treatment | Outcome |
|---|---|---|---|---|---|---|---|
| Austin Wheeler | 1 | 2/male | Multiple intracranial lesions; light posterior fossa; bilateral basal ganglia; left temporal lobe; right frontal lobe, etc. | Unsteady gait | Cerebellar ependymoma | Surgery | No recurrence/alive |
| Omron Hassan | 1 | 11/male | Left temporal lobe | Diplopia; headache | Meningioma(WHO II) | Surgery | No recurrence/alive |
| Mina S. Makary | 1 | 17/female | Left temporal lobe | seizures; headache; dizziness | - | Surgery | No recurrence/alive; seizure-free |
| Brian Y.L. Chan | 1 | 2/male | Right temporal lobe; right frontal lobe | Squint; ptosis | Arachnoid cyst | Surgery | No recurrence/alive |
| Kunle Oyedokun | 1 | 3/male | Left parietal region | Seizures | - | Surgery | No recurrence/alive; seizure-free |
| Luke Galloway | 1 | 18-month old/male | Right temporal lobe | Seizures | Meningioma (WHO II) | Surgery | No recurrence/alive; seizure-free |
| Salvatore Stilo | 1 | 55/male | Left parietal region; head of the caudate nucleus, putamen, and thalamus | Seizures; memory and verbal impairment | B-cell central nervous system lymphoma | Surgery | NA |
| Alexandre Roux | 1 | 11/male | Left frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free |
| Laura Lavalle | 1 | 13/male | Right frontal | Stumble upon | - | Surgery | No recurrence/alive |
| Sara Free | 2 | 45/male | Left occipital region | Visual disturbances; Headache | - | Topiramate | Improvement/alive |
| 19/male | Left occipital lobe | Epilepsy history; headache; blurring of vision | - | Observation | Improvement/alive | ||
| J. Bryan Iorgulescu | 3 | 31/male | Frontal lobe | headache; nausea; vomiting; personality changes | Solitary fibrous tumour/hemangiopericytoma | Surgery | NA |
| 4/female | Parietal lobe | Seizures | Atypical teratoid/rhabdoid tumour | Surgery | No recurrence/alive; seizure-free | ||
| 2/male | Parietal lobe | Seizures | Rhabdomyosarcoma | Surgery and chemoradiotherapy | No recurrence/alive; seizure-free | ||
| Sabrina Rossi | 1 | 6/female | Right occipital lobe; right temporo-parietal region | Seizures; intellectual disability; dysfunctional behavior | Atypical teratoid/rhabdoid tumor | Surgery | Recurrence 2 months after operation; died 14 months from first diagnosis |
| Raja Anand | 3 | 6/female | Left frontal lobe | Blank stares; whole-body stiffening and rolling of the eyes | - | Surgery | One revision seven months after initial resection |
| 16/female | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| 2/male | Right parietal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| Fábio A Nascimento | 1 | 25/male | Left parietal lobe | Seizures | - | Surgery | NA |
| Shlomit Yust-Katz | 1 | 67/male | Bilateral occipital lobes; right temporal lobe | Visual impairment | - | bevacizumab | Blind and clinically stable |
| Dorna Motevalli | 1 | 13/male | Frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free |
| Daniel Joseph Donovan | 1 | 16/female | Right temporal lobe | Seizures | - | Limited resection | No further growth/alive; seizure-free |
| Elif Bulut | 1 | 55/female | Left cerebellum | Vertigo | - | Surgery | No recurrence/alive |
| Zhihua Sun | 3 | 73/female | Left temporal lobe | Binocular diplopia; limited abduction of the left eye | - | Surgery | NA |
| 23/male | Light temporal lobe | Left hemianesthesia | - | Surgery | NA | ||
| 9/female | Left parietal lobe | Seizures | - | Surgery | NA | ||
| Y. Fu | 1 | 23/male | Right insular lobe | Seizures | - | Surgery | No recurrence/alive |
| Chao Zhang | 14 | 30/male | Right frontal lobe | Headache | Meningioma | Surgery | No recurrence/alive; seizure-free |
| 32/male | Right temporal lobe | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free | ||
| 3/male | Corpus callosum | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free | ||
| 12/male | Left parietal lobe | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free | ||
| 23/male | Right parietal lobe | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free | ||
| 13/male | Third ventricle | Diabetes insipidus | Meningioma | Surgery | Dead | ||
| 23/male | Right temporal lobe | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free | ||
| 10/female | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| 25/female | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; seizure recurrence | ||
| 26/female | Right parietal lobe | Seizures | - | Surgery | No recurrence/alive; seizure improved | ||
| 5/female | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| 10/male | Right parietal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| 3.5/male | Anterior cranial fossa | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| 27/male | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; seizure improved | ||
| Peifeng Li | 1 | 21/female | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free |
| Nobutaka Mukae | 2 | 17/male | Left frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free |
| 16/male | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free | ||
| A. Abdulazim | 1 | 41/male | Right frontoparietal lobe | Monoparesis of the left leg | - | Surgery | No recurrence/alive |
| Ayush Batra | 1 | 23/male | Right frontal lobe | Seizures; migraine headaches | - | Surgery | No recurrence/alive; seizure-free |
| Rui Feng | 10 | 18/male | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free |
| 18/male | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| 13/female | Left parietal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| 39/female | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; Engel II seizure improved | ||
| 8/male | Right frontal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| 21/male | Left parietal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| 14/male | Left frontal lobe | Seizures | - | Surgery | No recurrence/alive; Engel III seizure improved | ||
| 17/female | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; Engel II seizure improved | ||
| 34/female | Left occipital lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| 13/male | Right parietal lobe | Seizures | - | Surgery | No recurrence/alive; Engel I seizure-free | ||
| Sara Marzi | 1 | 37/male | Right frontal lobe | Headache | - | Surgery | No recurrence/alive |
| Osama Jamil | 1 | 3/female | Left frontotemporal; left gyrus rectus | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free |
| Everton Barbosa-Silva | 1 | 32/male | Right frontal lobe; Right parietal lobe; Right occipital lobe | Seizures | - | Sedation; anti-epileptic drugs | Recurrent seizures/dead |
| T. C. Yasha | 1 | 19/male | Left temporal lobe | Headache; Seizures | - | Surgery | NA |
| Huajuan Cui | 1 | 33/male | Left temporal lobe | Seizures | Meningioma | Surgery | No recurrence/alive; seizure-free |
| Katrien Jansen | 1 | 8-month-old/male | Right temporal lobe | Seizures | - | Surgery | No recurrence/alive; seizure-free |
Table 2.
Summary of clinical features and surgical outcomes of 49 CAPNON cases in the literature of PubMed in the last ten years.
Table 2.
Summary of clinical features and surgical outcomes of 49 CAPNON cases in the literature of PubMed in the last ten years.
| Authors | Case | Age/Sex | Site | Presentation | Treatment | Outcome | EMA | Vimentin | S-100 | GFAP |
|---|---|---|---|---|---|---|---|---|---|---|
| Jiri Soukup | 5 | 38/F | Intracranial; supratentorial; central sulcus | Seizures | Surgery | No recurrence/alive | + | + | - | - |
| 72/F | Intracranial; supratentorial; falx cerebri | Right-sided hemiparesis; Organic psychosyndrome | Surgery | Died | + | + | - | - | ||
| 68/F | Intracranial; supratentorial; right lateral ventricle | Headaches; fainting; hydrocephalus with organic psychosyndrom | Surgery | No recurrence/alive | + | + | - | - | ||
| 50/F | Intracranial; subtentorial; right cerebellar hemisphere; and vermis | - | Surgery | No recurrence/alive | + | + | - | - | ||
| 53/F | Intracranial; subtentorial; partially intraaxial; pons and pontocerebellar angle | Headache; facial nerve palsy; tinnitus; fainting | Surgery | No recurrence/alive | + | + | - | - | ||
| Colin A. Dallimore | 1 | 53/F | Intracranial; subtentorial; posterior fossa | Headache | Surgery | No recurrence/alive | - | NA | - | - |
| Wei-Qing Li | 1 | 56/F | Intracranial; supratentorial; right frontal lobe | Headache | Surgery | No recurrence/alive | + | + | - | - |
| Jian-Qiang Lu | 1 | 51/F | Spinal; paravertebral fascia in the midline at the levels of L3–4 vertebral bodies | Lower back pain; lower back mass | Surgery | NA | NA | NA | NA | NA |
| Lei Yan | 1 | 44/F | Intracranial; subtentorial; skull base | Headache | Surgery | NA | - | NA | - | NA |
| John C. Benson | 1 | 58/M | Intracranial; subtentorial; posterior fossa | Headache | Surgery | No recurrence/alive; Hydrocephalus | NA | NA | NA | NA |
| Yujian Li | 1 | 19/F | Intracranial; supratentorial; right temporal | Seizures | Surgery | No recurrence/alive; Seizure-free | - | + | NA | + |
| Andrea Boschi | 1 | 44/F | Spinal; right preforaminal extradural lesion | Back pain | Indomethacin | No recurrence/alive | NA | NA | NA | NA |
| Marian Preetham Suresh | 1 | 63/M | Intracranial; supratentorial; posterior third ventricle | Cognitive impairment; gait disturbance | V-P shunt | No progress/alive | + | NA | NA | NA |
| Kaiyun Yang | 2 | 57/M | Intracranial; subtentorial; extraaxial, right cerebellopontine angle (CPA) | Hoarseness; dysphagia; gait imbalance | Surgery | No recurrence/alive | + | NA | NA | NA |
| 70/M | Intracranial; supratentorial; right frontal lobe | Headache; gait difficulty, with falls; confusion and mood changes | Surgery | Headache improved | + | NA | NA | NA | ||
| Madoka Inukai | 1 | 64/F | Intracranial; supratentorial; corpus callosum | Weakness of the left leg persisting | Surgery | No recurrence/alive; weakness improvement | NA | NA | - | - |
| Jiahua Huang | 1 | 39/M | Intracranial; subtentorial; skull base | Visual disturbance; headache | Surgery | No recurrence/alive | + | NA | - | NA |
| Prashanth Raghu | 1 | NA/M | Intracranial; supratentorial; right medial temporal lobe | Seizures | Anti-epileptic drugs | EEG found normal; symptomatically better | NA | NA | NA | NA |
| Frederic A Vallejo | 1 | 35/M | Intracranial; supratentorial; left-posterior temporal lobe | Seizures; headaches; vertigo | Surgery | No recurrence/alive; seizure-free | NA | + | NA | + |
| Pithon RFA | 1 | 17/M | Intracranial; supratentorial; left frontal lobe | Seizures | Surgery | No recurrence/alive; seizure-free | NA | NA | NA | NA |
| Yuta Tanoue | 1 | 52/M | Intracranial; supratentorial; left medial temporal lobe | Seizures | Surgery | No recurrence/alive; seizure-free | - | + | + | + |
| Zaman SKU | 1 | 10/M | Intracranial; supratentorial; right thalamic | Left-sided hemiparesis with mixed movement disorder with hemiballism, choreoathetosis, and dystonia | Medication; physiotherapy | No progress/alive | NA | NA | NA | NA |
| A J Gauden | 1 | 69/M | cranio-cervical junction | Neck pain | Surgery | No recurrence/alive | + | NA | NA | NA |
| Thakur B | 1 | 67/F | Intracranial; subtentorial; cerebellum | Difficulty walking | Surgery | No recurrence/alive | - | NA | NA | + |
| Eric S Nussbaum | 1 | 39/F | Intracranial–extradural; subtentorial | Right-sided deafness and tinnitus | Surgery | No recurrence/alive | NA | NA | NA | NA |
| Akira Watanabe | 1 | 40/F | Intracranial; supratentorial; right frontal lobe | Somnolence | Surgery | Recurrence (after 14 months) | NA | NA | NA | NA |
| Atin Saha | 1 | 67/M | Spinal; vertebral canal | Left lower extremity pain; weakness and gait instability | Surgery | No recurrence/alive; symptom improvement | NA | NA | NA | NA |
| Zerehpoosh FB | 1 | 25/M | Intracranial; supratentorial; left temporal lobe | Incidental finding | Surgery | No recurrence/alive | NA | NA | NA | NA |
| Sean M Barber | 1 | 31/F | Intracranial; supratentorial; right temporal lobe | Seizures | Surgery | No recurrence/alive; symptom improvement | + | + | - | - |
| Michael M Safaee | 1 | 8/M | Intracranial; supratentorial; right frontal lobe | Seizures | Surgery | No recurrence/alive; seizure-free | NA | NA | NA | NA |
| Timothy C Blood | 1 | 65/F | Intracranial; supratentorial; anterior cranial fossa | Deafness | Surgery | No recurrence/alive | + | NA | NA | NA |
| Michael A Paolini | 1 | 17/M | Intracranial; supratentorial; left occipitoparietal lobe | Seizures | Surgery | No recurrence/alive | + | NA | NA | NA |
| Brasiliense LB | 1 | 67/F | Intracranial; subtentorial; ventral midbrain and supratentorial; left frontal lobe | Seizures | Surgery | No recurrence/alive; seizure-free | NA | NA | - | - |
| Abdaljaleel M | 1 | 62/F | Intracranial; supratentorial; temporal, parietal, and occipital lobes | Seizures; headaches | Surgery | NA | NA | NA | - | NA |
| Nayuta Higa | 1 | 62/M | Intracranial; supratentorial; left cingulate gyrus | Headache | Surgery | Recurrence | NA | NA | + | + |
| Hong Gang Wu | 1 | 39/F | Spine; sacral canal | Sacrococcygeal pain | Surgery | No recurrence/alive | NA | NA | NA | NA |
| Sara García Duque | 4 | 48/F | Intracranial; supratentorial; left occipital lobe | Headache | Surgery | No recurrence/alive | NA | NA | NA | NA |
| 51/F | Spinal cord; L2 | Lower back pain | Surgery | No recurrence/alive | NA | NA | NA | NA | ||
| 46/F | Spinal cord; C3 | Posterior neck pain | Surgery | No recurrence/alive | NA | NA | NA | NA | ||
| 73/M | Spinal cord; T2 | Progressive paraparesis | Surgery | No recurrence/alive | NA | NA | NA | NA | ||
| Joseph Ghaemi | 1 | 18/M | Intracranial; subtentorial; skull base | Headache; diplopia | Surgery | NA | + | NA | NA | NA |
| Arthur J M Lopes | 1 | 72/F | spinal cord; L2 | Low back pain | Surgery | No recurrence/alive | + | NA | + | + |
| Mohammed Alshareef | 1 | 59/F | cranio-cervical junction | Gait instability; balance difficulty | Surgery | No recurrence/alive | NA | NA | NA | NA |
| Molly Hubbard | 1 | 38/F | Intracranial; supratentorial; bilateral frontal lobe | Headache; left facial numbness | Surgery | No recurrence/alive; symptom improvement | NA | NA | NA | NA |
| Karol Wiśniewski | 1 | 29/M | Intracranial; subtentorial; foramen magnum | Headache | Surgery | No recurrence/alive | + | NA | - | NA |
| Kirill Lyapichev | 1 | 24/M | Intracranial; supratentorial; right temporo-occipital lobe | Headache; seizures; loss of vision | Surgery | No recurrence/alive | - | NA | NA | + |
| M N Stienen | 2 | 46/M | Intracranial; supratentorial; right parietal lobe | Seizures | Surgery | No recurrence/alive | NA | NA | NA | NA |
| 55/F | Intracranial; supratentorial; left frontoparietal lobe | Progressive hallucinosis; behavioral disorders | Surgery | No recurrence/alive; symptom improvement | NA | NA | NA | NA | ||
| Edward E Kerr | 1 | 56/M | Intracranial; subtentorial; posterior fossa | Headache | Surgery | No recurrence/alive; symptom improvement | + | NA | NA | - |
| Mun Keong Kwan | 1 | 48/M | Spinal, extradural mass located dorsal to the T9–T10 disc | Radicular pain | Indomethacin | No recurrence/alive | NA | NA | NA | NA |
Disclaimer/Publisher’s Note: The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content. |
© 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Share and Cite
MDPI and ACS Style
Sun, X.; Xu, C.; Cai, Y.; Pan, Z.; Li, Z. Meningioangiomatosis Combined with Calcifying Pseudoneoplasms of Neuraxis. Brain Sci. 2023, 13, 786. https://doi.org/10.3390/brainsci13050786
AMA Style
Sun X, Xu C, Cai Y, Pan Z, Li Z. Meningioangiomatosis Combined with Calcifying Pseudoneoplasms of Neuraxis. Brain Sciences. 2023; 13(5):786. https://doi.org/10.3390/brainsci13050786
Chicago/Turabian StyleSun, Xiangyu, Chengshi Xu, Yuxiang Cai, Zhiyong Pan, and Zhiqiang Li. 2023. "Meningioangiomatosis Combined with Calcifying Pseudoneoplasms of Neuraxis" Brain Sciences 13, no. 5: 786. https://doi.org/10.3390/brainsci13050786
Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.
