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BACKGROUND AND IMPORTANCE: Calcifying pseudoneoplasms of the neuraxis (CAPNON) is an extremely rare tumor, with nearly 150 cases have been reported in the literature. We present a case of CAPNON at foramen magnum (cervicomedullary junction). We also discuss the histological and radiological features of this rare pathology. CLINICAL PRESENTATION: A 35-year-old male patient presented to our center complaining of neck pain for the last 6 months and for the last 3 months has had headaches associated with nausea, blurred vision and papilledema. The patient's magnetic resonance imaging showed the presence of right craniocervical junction intra-dural extramedullary tumor. Neuro examination showed no neurological deficits. He underwent midline suboccipital craniotomy with C1 laminectomy. The mass was completely resected, and histopathology confirmed it to be a CAPNON. CONCLUSION: We presented a new case of CAPNON at cervicomedullary junction. The tumor was radically resected, without any complications. Resection of this kind of tumor is curative, and the need for post-operative chemo or radiotherapy is unnecessary, which depends on the outcome of future research and the long-term follow ups.
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Calcifying pseudoneoplasm of the neuraxis (CAPNON) is thought to be a rare tumefactive lesion with unknown pathogenesis. Its prevalence is questionable with few previously reported cases of incidental CAPNON, and likely underdiagnosis. We report a unique case of incidental multifocal CAPNON. A 64-year-old female was admitted with loss of consciousness due to a ruptured right middle cerebral artery aneurysm with subarachnoid and intraventricular hemorrhage. She has a craniotomy and clipping. At time of operation, numerous small dural-based nodules were found, and one was excised for biopsy and was diagnosed as CAPNON. Retrospective review of her CT images identified nodules that were all ipsilateral to the ruptured aneurysm. A literature review revealed that incidental and/or multifocal CAPNONs are rare but likely underreported. Our case suggests a reactive process in the pathogenesis of CAPNON.
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Calcinosis , Humanos , Femenino , Persona de Mediana Edad , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Calcinosis/cirugía , Sistema Nervioso Central/patología , Craneotomía , Hemorragia Cerebral/cirugíaRESUMEN
Neuropathic pain is a typical patient disorder resulting from damage and dysfunction of the peripheral neuraxis. Injury to peripheral nerves in the upper extremities can result in a lifelong reduction in quality of life and a devastating loss of sensory and motor function. Since some standard pharmaceutical therapies can cause dependence or intolerance, nonpharmacological treatments have gained great interest in recent years. In this context, the beneficial effects of a new combination of palmitoylethanolamide and Equisetum arvense L. are evaluated in the present study. The bioavailability of the combination was initially analyzed in a 3D intestinal barrier simulating oral intake to analyze its absorption/biodistribution and exclude cytotoxicity. In a further step, a 3D nerve tissue model was performed to study the biological effects of the combination during the key mechanisms leading to peripheral neuropathy. Our results demonstrate that the combination successfully crossed the intestinal barrier and reached the target site, modulating the nerve recovery mechanism after Schwann cell injury and offering the initial response of relieving pain. This work supported the efficacy of palmitoylethanolamide and Equisetum arvense L. in reducing neuropathy and modifying the major pain mechanisms, outlining a possible alternative nutraceutical approach.
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Equisetum , Neuralgia , Humanos , Calidad de Vida , Distribución Tisular , Neuralgia/tratamiento farmacológicoRESUMEN
A 53-year-old man with a history of an untreated brain mass was taken to Toyama Prefectural Central Hospital by emergency transport. Computed tomography revealed an intracranial hypo-attenuated lesion exhibiting mass effect. Several calcified foci were observed around the lesion. His radiographical diagnosis was meningioma with calcification and edema. He suddenly showed tonic seizure after admission; therefore an emergency craniotomy was performed. However, he unfortunately died due to advanced cerebral edema. Microscopic findings of the surgically obtained materials were consistent with neurenteric cyst (NC). Intracranial hard masses were found adjacent to NCs, and the masses were composed of fibrous cartilage-like matrix with extensive linear calcification and the presence of surrounding round-to-oval epithelioid cells. Thus, calcifying pseudoneoplasm of the neuraxis (CAPNON) associated with NC was considered the most appropriate diagnosis of the present case. To the best of our knowledge, this is the first report of such a case. The present case suggests that delay of treatment might cause a poor outcome, at least in CAPNON associated with NC. Careful investigations, including the underlying pathology, may be essential when considering the etiology of CAPNON and its treatment strategies.
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Calcinosis , Neoplasias Meníngeas , Meningioma , Defectos del Tubo Neural , Masculino , Humanos , Persona de Mediana Edad , Calcinosis/complicaciones , Calcinosis/patología , Meningioma/complicaciones , Sistema Nervioso Central/patología , Defectos del Tubo Neural/complicaciones , Neoplasias Meníngeas/complicacionesRESUMEN
To validate a simultaneous analysis tool for the brain and cervical cord embedded in the statistical parametric mapping (SPM) framework, we compared trauma-induced macro- and microstructural changes in spinal cord injury (SCI) patients to controls. The findings were compared with results obtained from existing processing tools that assess the brain and spinal cord separately. A probabilistic brain-spinal cord template (BSC) was generated using a generative semi-supervised modelling approach. The template was incorporated into the pre-processing pipeline of voxel-based morphometry and voxel-based quantification analyses in SPM. This approach was validated on T1-weighted scans and multiparameter maps, by assessing trauma-induced changes in SCI patients relative to controls and comparing the findings with the outcome from existing analytical tools. Consistency of the MRI measures was assessed using intraclass correlation coefficients (ICC). The SPM approach using the BSC template revealed trauma-induced changes across the sensorimotor system in the cord and brain in SCI patients. These changes were confirmed with established approaches covering brain or cord, separately. The ICC in the brain was high within regions of interest, such as the sensorimotor cortices, corticospinal tracts and thalamus. The simultaneous voxel-wise analysis of brain and cervical spinal cord was performed in a unique SPM-based framework incorporating pre-processing and statistical analysis in the same environment. Validation based on a SCI cohort demonstrated that the new processing approach based on the brain and cord is comparable to available processing tools, while offering the advantage of performing the analysis simultaneously across the neuraxis.
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Encéfalo/diagnóstico por imagen , Médula Cervical/diagnóstico por imagen , Neuroimagen/métodos , Traumatismos de la Médula Espinal/diagnóstico por imagen , Adulto , Encéfalo/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neuroimagen/normas , Tractos Piramidales/diagnóstico por imagen , Tractos Piramidales/patología , Corteza Sensoriomotora/diagnóstico por imagen , Corteza Sensoriomotora/patología , Traumatismos de la Médula Espinal/patología , Tálamo/diagnóstico por imagen , Tálamo/patologíaRESUMEN
BACKGROUND: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like lesion with unknown pathogenesis. It is likely under-reported due to diagnostic challenges including the nonspecific radiographic features, lack of diagnostic markers, and often asymptomatic nature of the lesions. METHODS: We performed detailed examination of 11 CAPNON specimens diagnosed by histopathology, with the help of electron microscopy and immunohistochemistry. RESULTS: Electron microscopy revealed the presence of fibrillary materials consistent with neurofilaments. In addition to some entrapped axons at the periphery of CAPNONs, we discovered that all specimens stained positive for neurofilament-light (NF-L) within the granular amorphous cores, but not neurofilament-phosphorylated (NF-p). CAPNONs also showed variable infiltration of CD8+ T-cells and a decreased ratio of CD4/CD8+ T-cells, suggesting an immune-mediated process in the pathogenesis of CAPNON. CONCLUSION: NF-L and CD4/CD8 immunostains may serve as diagnostic markers for CAPNON and shed light on its pathogenesis.
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Calcinosis , Axones , Linfocitos T CD8-positivos , Calcinosis/diagnóstico por imagen , Sistema Nervioso Central , Humanos , InmunohistoquímicaRESUMEN
Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare, slow-growing, benign lesions occurring throughout the neuroaxis that are frequently misdiagnosed and overlooked by clinicians. Here, we report a case of a 56-year-old woman who presented with a history of recurrent headache for the previous six years. Magnetic resonance imaging (MRI) revealed a 2.3-cm-sized solid mass in the right frontal lobe that was surrounded by marked edematous areas. The lesion demonstrated dense calcification and avid enhancement. The lesion was initially diagnosed as oligodendroglioma, and then found to be CAPNON based on histopathology of a surgically resected tissue. Genetic analysis revealed a nonsense mutation in the CUL4B gene. The patient's condition appeared to reflect a reactive, rather than neoplastic, process. Clinicians should be prepared to detect such pseudotumors histopathologically in order to avoid unnecessary differential tests of neoplastic or infectious diseases, as well as potentially harmful therapies.
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Calcinosis , Oligodendroglioma , Sistema Nervioso Central , Proteínas Cullin , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
BACKGROUND: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base. METHODS: Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas. RESULTS: Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient's cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies. CONCLUSION: While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.
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Encefalopatías/diagnóstico por imagen , Encefalopatías/cirugía , Calcinosis/diagnóstico por imagen , Calcinosis/cirugía , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Neoplasias de la Base del Cráneo/diagnóstico , Anciano , Encefalopatías/complicaciones , Encefalopatías/patología , Calcinosis/complicaciones , Calcinosis/patología , Ataxia Cerebelosa/etiología , Ataxia Cerebelosa/fisiopatología , Enfermedades de los Nervios Craneales/etiología , Enfermedades de los Nervios Craneales/fisiopatología , Diagnóstico Diferencial , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Base del CráneoRESUMEN
We report a case of multiple calcifying pseudoneoplasms of the neuraxis (MCAPNON) with associated multifocal perivascular microcalcifications and vascular calcinosis. Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a very rare condition that may arise in extra-axial and occasionally, in intra-axial locations. Moreover, it is nearly always a solitary mass with only one case with two lesions reported. While the etiology and pathogenesis of CAPNON remains unclear, the histopathology findings of this entity have been well described. We report a case of a 62-year-old woman with 18 calcifying radiologic lesions involving bilateral cerebral hemispheres. Histologically, these lesions have features similar to that reported for CAPNON, including nodular calcification with fibro-osseous components and peripheral histiocytic reaction. The patient had a poorly documented diagnosis of neurocyticercosis 32 years prior, although without tissue confirmation. The lack of detectable cysticercus serum antibody titers, and absence of residual larval or cyst wall tissue render multifocal calcific involution of that parasite unprovable although still plausible. We also raise the possibility of a blood-brain barrier derangement and/or a metabolic disorder as an alternative etiology. Whether this case of MCAPNON shares the same pathogenesis as the usual solitary CAPNON is unclear.
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Encefalopatías/patología , Encéfalo/patología , Calcinosis/patología , Neurocisticercosis/patología , Encéfalo/diagnóstico por imagen , Encefalopatías/diagnóstico por imagen , Calcinosis/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Neurocisticercosis/diagnóstico por imagenRESUMEN
We present a case of a 29-year-old male with a calcifying pseudoneoplasm of the neuraxis (CAPNON) located in the region of the foramen magnum, treated successfully by complete resection. After a 2-year follow-up the patient remains recurrence free. Clinical and histopathological characterization of CAPNON is provided with special emphasis on the intraoperative and neuroradiological features of the lesion.
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Calcinosis/cirugía , Foramen Magno/cirugía , Adulto , Calcinosis/patología , Foramen Magno/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Procedimientos Neuroquirúrgicos/métodos , Resultado del TratamientoRESUMEN
Background: The popularity of tortoises kept in captivity is increasing and has caused concern regarding the necessity to establish safe and straightforward anaesthesia for those reptiles. Aim: This study aimed to compare four protocols using levobupivacaine in spinal anaesthesia for the blockade of the caudal neuraxis of red-footed tortoises (Chelonoidis carbonarius). Methods: Twenty-four tortoises were randomly assigned into four groups: G1, levobupivacaine 0.75% (1.15 mg kg-1); G2, levobupivacaine 0.37% (1.15 mg kg-1); G3, levobupivacaine 0.75% (2.3 mg kg-1); and G4, levobupivacaine 0.75% (0.1 ml 5 cm-1 of straight carapace length). Tortoises were evaluated for respiratory rate, muscle relaxation, response to hindlimb or tail pinch, and cloacal reflex. Results: A 1.15 mg kg-1 dose of levobupivacaine 0.37% appears adequate for shorter procedures, whereas a 1.15 mg kg-1 dose of levobupivacaine 0.75% should be appropriate for longer procedures in red-footed tortoises. Conclusion: Our results are the first to show the effects of levobupivacaine on spinal anaesthesia in reptiles. Weight-based doses presented more intense and more homogeneous effects than carapace length-based doses in red-footed tortoises. Spinal anaesthesia of red-footed tortoises was safe and effective with any of the weight-based protocols.
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Anestesia Raquidea , Anestésicos Locales , Levobupivacaína , Tortugas , Animales , Levobupivacaína/administración & dosificación , Levobupivacaína/farmacología , Anestesia Raquidea/veterinaria , Anestesia Raquidea/métodos , Anestésicos Locales/farmacología , Anestésicos Locales/administración & dosificación , Masculino , FemeninoRESUMEN
Calcifying pseudoneoplasm of the neuraxis (CAPNON) is an uncommon entity and a rare cause of third cranial nerve palsy. We review the case of a 17-year-old male with a 9-month history of progressive left third cranial nerve palsy. Cerebral magnetic resonance image showed a left clinoidal lesion with low signal intensity in T2 and T1 sequences with signs of calcification in the computed tomography and without vascular lesion in AngioMRI. A left pterional approach was performed with posterior clinoidectomy and total resection of the lesion. Calcifying pseudoneoplasm of the neuraxis is an infrequent pathology that presents in the skull base and spine that requires surgical treatment in the presence of compressive phenomena and differential diagnosis as meningioma, chordoma, and metastasis should be considered.
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Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare, non-neoplastic, slow-growing tumors that can present anywhere throughout the central nervous system. While the etiology of these lesions remains unknown, the mainstay of treatment is surgical excision. We describe a case of CAPNON at our institution in a 66 year-old female patient who presented with 5 months of pain and burning sensation in her thigh. On MRI, an intradural extramedullary lesion was identified at the level of T11-T12. The mass was surgically excised and the patient reported resolution of her symptoms by her six week follow-up appointment. We reviewed 79 spinal CAPNON cases, covering all cases reported in the literature thus far. In summary, we find that spinal CAPNON are most commonly lumbar and extradural in location, with pain as the most common presenting symptom. Lesions are well-defined and hypointense on T1 and T2 MRI sequence. The majority of cases had favorable surgical outcomes with near complete resolution of pain and associated symptoms.
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Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like fibro-osseous lesion that can develop anywhere in the neuraxis. Approximately a half of reported CAPNONs developed in the spinal region, mostly close to the facet joint (FJ). The diagnosis of spinal CAPNONs is challenging given the existence of mimics and associated pathologies including calcific degeneration of the FJ ligaments (DFJL) and synovial cysts (SCs). The pathogenesis of CAPNON remains elusive, although there have been a few hypotheses including degenerative, reactive, proliferative and immune-mediated processes. Our present study examined clinical, radiological and pathological features of 12 spinal CAPNONs in comparison to 9 DFJL foci, and diagnostic and pathogenic relationship between CAPNONs and FJ pathologies. On imaging, CAPNONs were all tumor-like and typically bigger than DFJL foci. All CAPNONs showed pathologically diagnostic features including characteristic cores, consistently identifiable core-surrounding/peripheral palisading of macrophages and other cells including multinucleated giant cells, variable infiltration of CD8+ T-cells, and multifocal immunopositivity of neurofilament light chain (NF-L). These features were absent or limited in the DFJL foci with statistically significant differences from CAPNONs, except calcifications. Spinal CAPNONs co-existed with DFJL foci in all cases; some had transitional foci with overlapping focal CAPNON and DFJL-like features. These findings, along with our previously reported relationship between CAPNONs and SCs, suggest that spinal CAPNONs may occur in association with or in transition from calcifying/calcified degenerative lesions of FJ ligaments and/or SCs when a reactive proliferative process is complemented by other pathogenic changes such as immune-mediated pathology and NF-L deposition/expression.
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Neoplasias , Articulación Cigapofisaria , Humanos , Linfocitos T CD8-positivos , Columna Vertebral , Sistema Nervioso CentralRESUMEN
Pilocytic astrocytoma (PA), recognized as the most prevalent central nervous system (CNS) tumor, has long been associated with calcifications, a characteristic often attributed to benign or indolent growth patterns. In this study, we explored the calcified attributes in these tumors that beckon a deeper understanding. This is a retrospective study, on a set of seven cases, with a histopathological diagnosis of pilocytic astrocytoma with calcifications and psammoma bodies (PB). Despite an encouraging overall survival outcome, the recurrence in four cases cast some doubt on the conventional classification. The histological study of these cases revealed a spectrum of calcifications, varying in size and morphology, all of which exhibited positive reactivity to glial fibrillary acidic protein (GFAP), osteoconduction, and osteopontin. Notably, the immunohistochemistry showed hyaline bodies displaying an atypical immune profile, strikingly negative for vimentin and GFAP, and a robust positivity for epidermal growth factor receptors (EGFR), tumor necrosis factor-alpha (TNF-α), and interleukin 1 beta (IL-1ß). These results stimulated speculation that the identity of these calcified tumors may have extended and potentially embraced the realm of calcifying pseudoneoplasms of the neuraxis (CAPNON), underscored by intense pilot gliosis. This study transcends mere anatomical exploration; it delves into the intricacies of calcified tumors, casting a spotlight on the dynamic interplay between PA and CAPNON. As we traverse the frontiers of neuro-oncology, these findings pave the way for innovative avenues in the diagnostics and therapeutics of these tumors.
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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.
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INTRODUCTION AND IMPORTANCE: Calcifying pseudoneoplasms of the neuraxis (CAPNON) are infrequent benign brain tumors. They demonstrate slow growth and are characterized by calcium deposits within the tumor tissue. CAPNONs are observed in the supratentorial compartment of the brain. Due to their rarity, increasing knowledge of CAPNONs is essential for accurate diagnosis and effective management of affected patients. This report describes a case of CAPNON diagnosed in a 56-year-old female and discusses the clinical, imaging, and histopathological findings of this rare entity. CASE PRESENTATION: A 56-year-old female presented with a history of recurrent holocranial headache and dizziness, progressively worsening over the last month. Physical and neurological examinations revealed no evident abnormalities. Brain magnetic resonance imaging revealed a calcified and cystic mass, measuring 40 × 32 mm in the right frontal lobe. Complete excision of the mass was done. Histologically, the lesion was composed of glial tissue with abundant amorphous lamellar calcification and a myxoid matrix in the background. Concentric circular calcifications were observed with osseous metaplasia present in some areas. Palisading spindle to epithelioid cells was noted around the lesion. The final diagnosis was CAPNON. The postoperative course was uneventful, and one year of follow-up revealed no signs of recurrence. DISCUSSION: CAPNON typically occurs in middle-aged adults and can present with variable symptoms depending on its location within the brain, including seizures, headaches, or neurological deficits. Surgical resection is considered the optimal treatment for CAPNON. Raising awareness and understanding of this rare entity is necessary for accurate diagnosis and management of patients affected by this condition.
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BACKGROUND: There are numerous atypical lesions of the sellar and suprasellar region that are often mistaken for pituitary adenomas. It is important to consider rare mimics of more common pathologies in this region. OBSERVATIONS: The authors detail the case of a 37-year-old woman with hypopituitarism who was found to have an atypical sellar mass with slow growth on interval imaging. The lesion was debulked via a microscopic endonasal transsphenoidal approach and found to be a calcifying pseudoneoplasm of the neuraxis (CAPNON). LESSONS: CAPNON is a rare disease entity that may affect the sellar region. CAPNON should be on the differential diagnosis for sellar masses that are associated with T1 and T2 hypointensity on magnetic resonance imaging with minimal enhancement. Although CAPNON is not at risk for malignant progression, these benign lesions can continue to grow after a subtotal resection and require follow-up.
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Calcifying pseudoneoplasms of the neuraxis (CAPNONs) are rare and can occur along the neural axis. The pathogenesis of these masses is still unknown, and they are diagnosed by histopathological analysis. We report the largest CAPNON in the temporal lobe reported to date and a review of the literature on all previously reported CAPNON cases located in the temporal lobe. According to the literature review, prior to 2020, the largest CAPNON in the temporal lobe that had ever been reported measured 30 × 30 × 20 mm (Mohapatra et al.). However, we report a larger temporal lobe CAPNON (45 × 35 × 35 mm) in a female patient admitted to our hospital. In addition, among 22 patients with CAPNONs aged from 6 to 62 years, 45.5% were female and 54.5% were male. A total of 72.8% of patients presented with seizures, 9.1% had pituitary dysfunction and 9.1% did not have symptoms. Of the cases in the patients with seizures, 83.2% were completely surgically resected, 5.6% were partially surgically resected, and one was treated medically; one patient refused treatment. Except for one patient who had multiple lesions, all patients who underwent surgery exhibited improved or the disappearance of symptoms of epilepsy. The patient in whom epilepsy resolved had undergone total resection. CAPNON is a rare benign lesion that occurs throughout the nervous system, and the pathogenesis remains unclear. Although the hardness of these lesions vary, surgery is still the preferred treatment and yields good results, and total resection is recommended for patients with epilepsy in the temporal lobe.
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Calcinosis , Epilepsia del Lóbulo Temporal , Humanos , Masculino , Femenino , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Calcinosis/cirugía , Lóbulo Temporal/diagnóstico por imagen , Lóbulo Temporal/cirugía , Lóbulo Temporal/patología , Sistema Nervioso Central/patología , ConvulsionesRESUMEN
BACKGROUND: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is an extremely rare entity with fewer than 150 cases reported in the literature and mostly with a supratentorial or spinal location. Posterior fossa CAPNON has been reported scarcely, and association with perilesional edema is a topic not yet approached which might play a significant role in treatment decision and clinical progression. Our objective is to report, to our knowledge, the first series of 3 posterior fossa CAPNON surgically treated in a single institution and assess features that help provide a systematic approach to diagnosis and timely treatment. METHODS: This was a monocentric, retrospective study of surgical patients diagnosed with a posterior fossa CAPNON in the last 5 years. A thorough bibliographic research was conducted. RESULTS: Three patients were included. Locations involved IV ventricle, right cerebellopontine angle with extension to foramen magnum, and cerebellar vermis. Two of them presented with symptoms linked to acute hydrocephalus, and the other one presented with progressive cranial nerve palsy and brainstem compression signs. The 3 of them showed radiological signs of perilesional edema on their preoperative magnetic resonance imaging. Gross total resection was accomplished in one case, with near and subtotal resections in the others. There were no complications. The outcome was favorable in all cases. CONCLUSIONS: It is essential to contemplate this infrequent diagnosis in cases of calcified lesions involving the posterior fossa. When symptoms manifest, surgery should be considered. Perilesional edema could be associated with symptomatic progression and hence a sign suggesting the need for surgical treatment.