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1.
Acta Neuropathol ; 143(3): 403-414, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-35103816

RESUMEN

Tumors of the central nervous system (CNS) often display a wide morphologic spectrum that has, until recently, been the sole basis for tumor classification. The introduction of the integrated histomolecular diagnostic approach in CNS tumors has facilitated a classification system that is increasingly data-driven and with improved alignment to clinical outcome. Here, we report a previously uncharacterized glioma type (n = 31) using unsupervised clustering analysis of DNA methylation array data from approximately 14,000 CNS tumor samples. Histologic examination revealed circumscribed growth and morphologic similarities to pleomorphic xanthoastrocytoma (PXA), astroblastoma, ependymoma, polymorphous neuroepithelial tumor of the young (PLNTY), and IDH-wildtype glioblastoma (GBM). Median age (46.5 years) was significantly older than other circumscribed gliomas and younger than GBM. Dimensionality reduction with uniform manifold approximation and projection (UMAP) and hierarchical clustering confirmed a methylation signature distinct from known tumor types and methylation classes. DNA sequencing revealed recurrent mutations in TP53 (57%), RB1 (26%), NF1 (26%), and NF2 (14%). BRAF V600E mutations were detected in 3/27 sequenced cases (12%). Copy number analysis showed increased whole chromosome aneuploidy with recurrent loss of chromosome 13 (28/31 cases, 90%). CDKN2A/B deletion (2/31, 6%) and MGMT promoter methylation (1/31, 3%) were notably rare events. Most tumors showed features of a high-grade glioma, yet survival data showed significantly better overall survival compared to GBM (p < 0.0001). In summary, we describe a previously uncharacterized glioma of adults identified by a distinct DNA methylation signature and recurrent loss of chromosome 13.


Asunto(s)
Astrocitoma , Neoplasias Encefálicas , Glioma , Monosomía , Mutación , Proteína p53 Supresora de Tumor , Astrocitoma/genética , Astrocitoma/patología , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patología , Cromosomas Humanos Par 13 , Humanos , Persona de Mediana Edad , Mutación/genética , Proteína p53 Supresora de Tumor/genética
2.
Alzheimers Dement ; 15(2): 205-216, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30347188

RESUMEN

INTRODUCTION: We sought to establish the relationships between standard postmortem measures of AD neuropathology and antemortem [11C]PIB-positron emission tomography ([11C]PIB-PET) analyzed with the Centiloid (CL) method, a standardized scale for Aß-PET quantification. METHODS: Four centers contributed 179 participants encompassing a broad range of clinical diagnoses, PET data, and autopsy findings. RESULTS: CL values increased with each CERAD neuritic plaque score increment (median -3 CL for no plaques and 92 CL for frequent plaques) and nonlinearly with Thal Aß phases (increases were detected starting at phase 2) with overlap between scores/phases. PET-pathology associations were comparable across sites and unchanged when restricting the analyses to the 56 patients who died within 2 years of PET. A threshold of 12.2 CL detected CERAD moderate-to-frequent neuritic plaques (area under the curve = 0.910, sensitivity = 89.2%, specificity = 86.4%), whereas 24.4 CL identified intermediate-to-high AD neuropathological changes (area under the curve = 0.894, sensitivity = 84.1%, specificity = 87.9%). DISCUSSION: Our study demonstrated the robustness of a multisite Centiloid [11C]PIB-PET study and established a range of pathology-based CL thresholds.


Asunto(s)
Enfermedad de Alzheimer , Compuestos de Anilina , Autopsia , Neuropatología , Placa Amiloide , Tomografía de Emisión de Positrones , Tiazoles , Anciano , Enfermedad de Alzheimer/diagnóstico por imagen , Enfermedad de Alzheimer/patología , Femenino , Humanos , Masculino , Placa Amiloide/diagnóstico por imagen , Placa Amiloide/patología , Estudios Retrospectivos
3.
Brain ; 138(Pt 7): 2020-33, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25953778

RESUMEN

Amyloid-ß, a hallmark of Alzheimer's disease, begins accumulating up to two decades before the onset of dementia, and can be detected in vivo applying amyloid-ß positron emission tomography tracers such as carbon-11-labelled Pittsburgh compound-B. A variety of thresholds have been applied in the literature to define Pittsburgh compound-B positron emission tomography positivity, but the ability of these thresholds to detect early amyloid-ß deposition is unknown, and validation studies comparing Pittsburgh compound-B thresholds to post-mortem amyloid burden are lacking. In this study we first derived thresholds for amyloid positron emission tomography positivity using Pittsburgh compound-B positron emission tomography in 154 cognitively normal older adults with four complementary approaches: (i) reference values from a young control group aged between 20 and 30 years; (ii) a Gaussian mixture model that assigned each subject a probability of being amyloid-ß-positive or amyloid-ß-negative based on Pittsburgh compound-B index uptake; (iii) a k-means cluster approach that clustered subjects into amyloid-ß-positive or amyloid-ß-negative based on Pittsburgh compound-B uptake in different brain regions (features); and (iv) an iterative voxel-based analysis that further explored the spatial pattern of early amyloid-ß positron emission tomography signal. Next, we tested the sensitivity and specificity of the derived thresholds in 50 individuals who underwent Pittsburgh compound-B positron emission tomography during life and brain autopsy (mean time positron emission tomography to autopsy 3.1 ± 1.8 years). Amyloid at autopsy was classified using Consortium to Establish a Registry for Alzheimer's Disease (CERAD) criteria, unadjusted for age. The analytic approaches yielded low thresholds (standard uptake value ratiolow = 1.21, distribution volume ratiolow = 1.08) that represent the earliest detectable Pittsburgh compound-B signal, as well as high thresholds (standard uptake value ratiohigh = 1.40, distribution volume ratiohigh = 1.20) that are more conservative in defining Pittsburgh compound-B positron emission tomography positivity. In voxel-wise contrasts, elevated Pittsburgh compound-B retention was first noted in the medial frontal cortex, then the precuneus, lateral frontal and parietal lobes, and finally the lateral temporal lobe. When compared to post-mortem amyloid burden, low proposed thresholds were more sensitive than high thresholds (sensitivities: distribution volume ratiolow 81.0%, standard uptake value ratiolow 83.3%; distribution volume ratiohigh 61.9%, standard uptake value ratiohigh 62.5%) for CERAD moderate-to-frequent neuritic plaques, with similar specificity (distribution volume ratiolow 95.8%; standard uptake value ratiolow, distribution volume ratiohigh and standard uptake value ratiohigh 100.0%). A receiver operator characteristic analysis identified optimal distribution volume ratio (1.06) and standard uptake value ratio (1.20) thresholds that were nearly identical to the a priori distribution volume ratiolow and standard uptake value ratiolow. In summary, we found that frequently applied thresholds for Pittsburgh compound-B positivity (typically at or above distribution volume ratiohigh and standard uptake value ratiohigh) are overly stringent in defining amyloid positivity. Lower thresholds in this study resulted in higher sensitivity while not compromising specificity.


Asunto(s)
Enfermedad de Alzheimer/diagnóstico por imagen , Péptidos beta-Amiloides/análisis , Compuestos de Anilina , Tomografía de Emisión de Positrones/normas , Radiofármacos , Tiazoles , Anciano , Femenino , Humanos , Interpretación de Imagen Asistida por Computador , Masculino , Valores de Referencia , Adulto Joven
4.
Molecules ; 19(10): 17026-51, 2014 Oct 23.
Artículo en Inglés | MEDLINE | ID: mdl-25342555

RESUMEN

Six series of structurally different mono- and binuclear copper(II) complexes 5-10 were obtained by reacting N-(2-pyridyl)imidazolidin-2-ones (1a-l), N,N'-bis(2-pyridyl)imidazolidin-2-ones (2a,b), N-acyl-N'(2-pyridyl)imidazolodin-2-ones (3a-j) and N-(2-pyridyl)imidazolidine-2-thiones (4a-g) with copper(II) chloride at an ambient temperature. The coordination modes of the complexes obtained were established by elemental analysis, IR spectroscopic data and single crystal X-ray diffraction studies. The in vitro cytotoxic activities of both the free ligands and copper(II) complexes were evaluated using a crystal violet microtiter plate assay on five human tumor cell lines: LCLC-103H, A-427, SISO, RT-4 and DAN-G. The free ligands 1-4 at concentration attainable in cancer cells of 20 µM showed no meaningful cytotoxic effect with cell viability in the range of 88%-100%. The most potent copper(II) complex of 1-(6-ethoxy-2-pyridyl)imidazolidin-2-one (6b) exhibited selective cytotoxicity against A-427 lung cancer cell line, while the complexes of 1-(5-methyl-2-pyridyl)imidazolidine-2-thione (5h) and 1-(4-tert-butyl-2-pyridyl)imidazolidine-2-thione (5j) showed cytostatic effect against a whole panel of five human tumor cell lines. In conclusion, the only complexes that showed remarkably increased activity in comparison to the free ligands were those obtained from N-(2-pyridyl)imidazolidine-2-thiones 4c and 4e substituted with alkyl group at position 4 or 5 of pyridine ring.


Asunto(s)
Antineoplásicos/farmacología , Complejos de Coordinación/farmacología , Cobre/química , Neoplasias/tratamiento farmacológico , Compuestos Organometálicos/farmacología , Tionas/química , Antineoplásicos/química , Complejos de Coordinación/química , Cobre/metabolismo , Cristalografía por Rayos X , Humanos , Modelos Moleculares , Neoplasias/patología , Compuestos Organometálicos/química , Tionas/metabolismo , Células Tumorales Cultivadas , Difracción de Rayos X
5.
Cureus ; 15(5): e38452, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37273386

RESUMEN

Adenoviral infections in post-transplant patients have been described in multiple organ systems, most classically the lung, liver, and alimentary tract. In the genitourinary tract, hemorrhagic cystitis is most frequently observed. Clinically apparent renal involvement with adenovirus is rare, and adenovirus-associated interstitial nephritis (AAIN) is an uncommon cause of renal allograft failure. Here, we present three cases of AAIN in patients who, after prompt diagnosis and treatment adjustment, experienced a return of allograft function. All patients were on standard triple therapy with tacrolimus levels within the target range at the time of biopsy. None of the patients had respiratory symptoms, and despite diarrhea, colon biopsies were negative. Only case one had positive adenovirus serology (IgG only) and case three had positive urine; two patients had leukopenia without neutropenia. Renal biopsies showed a characteristic granulomatous tubulocentric mixed lymphocytic and neutrophilic infiltrate. Adenovirus immunohistochemistry (IHC) showed strong staining in the tubular epithelium (nuclear and cytoplasmic) while staining for polyomavirus was negative. A follow-up biopsy two months after the diagnosis of AAIN in one patient revealed persistent cytopathic effects with negative adenoviral IHC staining while a biopsy at one year in another patient showed glomerular and tubulointerstitial scarring. AAIN is an uncommon but important etiology to consider in cases of acute renal allograft dysfunction. Although the presenting symptoms for AAIN are nonspecific, hematuria is frequently noted. Adenovirus IHC should be considered in cases with granulomatous inflammation associated with necrosis and mixed inflammatory infiltrate. As demonstrated in this single-institution case series, prompt diagnosis can result in the preservation of the renal allograft. Lasting cytopathic effects after adenoviral infection should also be considered in patients with a history, or potential history, of AAIN.

6.
Cureus ; 15(4): e37361, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37182073

RESUMEN

Glioneural hamartomas are exceedingly rare lesions. When localized to the internal auditory canal (IAC), they can cause symptoms referrable to seventh and eighth cranial nerve compression. Here, the authors present a rare case of an IAC glioneural hamartoma. A 57-year-old male presented for evaluation of presumed intracanalicular vestibular schwannomas found on work-up of dizziness and progressive right-sided hearing loss. Surgical intervention pursued progressive symptoms and new onset headaches. The patient underwent uncomplicated retrosigmoid craniectomy for gross total resection. Histopathological evaluation revealed a glioneural hamartoma. A MEDLINE database search used the terms' cerebellopontine angle' OR 'internal auditory canal' AND 'hamartoma' OR 'heterotopia'. Clinicopathological characteristics and outcomes of the present case were compared to those in the literature. The literature review yielded nine articles describing 11 cases (eight females, three males; median age 40 years, range 11-71) of intracanalicular glioneural hamartomas. Patients most commonly presented with hearing loss and were presumed to have a diagnosis of vestibular schwannoma before histologic diagnosis. Glioneural hamartomas are rare lesions that may be found in the IAC. Although benign, they may be safely resected for cranial nerve function preservation goals with a low risk of recurrence.

7.
J Radiol Case Rep ; 17(12): 13-21, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-38828027

RESUMEN

The reported incidence of blastomycosis is increasing in certain regions of the United States. The diagnosis is primarily made via urine antigen testing, culture, or cytology smear. The differential diagnosis for blastomycosis includes pneumonia, tuberculosis, and non-infectious pulmonary disease. Clinical context and epidemiologic exposure play a crucial role in diagnosis. However, the differential can expand significantly if there is disseminated central nervous system involvement, especially if pulmonary manifestations are not seen. Imaging begins to play a vital role when differentiating disseminated blastomycosis from other etiologies such as malignancy. Herein we present a case of a 58-year-old male who presented with seizures and right sided gaze preference found to have disseminated central nervous system blastomycosis. In this article, we will discuss symptoms and imaging findings of disseminated blastomycosis to help guide diagnosis and management.


Asunto(s)
Blastomicosis , Imagen por Resonancia Magnética , Humanos , Masculino , Blastomicosis/diagnóstico por imagen , Blastomicosis/diagnóstico , Blastomicosis/tratamiento farmacológico , Persona de Mediana Edad , Diagnóstico Diferencial , Tomografía Computarizada por Rayos X , Antifúngicos/uso terapéutico , Infecciones Fúngicas del Sistema Nervioso Central/diagnóstico por imagen , Infecciones Fúngicas del Sistema Nervioso Central/diagnóstico
8.
Ann Hematol Oncol ; 10(2)2023.
Artículo en Inglés | MEDLINE | ID: mdl-37309512

RESUMEN

We present three cases of O6-Methylguanine-DNA Methyl-transferase (MGMT) methylated high grade gliomas with distant recurrence. All three patients had a radiographic stability of original tumor site at time of distant recurrence indicating impressive local control with Stupp protocol in patients with a MGMT methylated tumors. All patients had a poor outcome after distant recurrence. For one patient Next Generation Sequencing (NGS) was available for both original and recurrent tumor and did not reveal any difference other than high tumor mutational burden in the distant recurrent tumor. Understanding risk factors of distant recurrence in MGMT methylated tumors and investigating correlations between recurrences will help plan therapeutic strategies to prevent distant recurrence and improve survival of these patients.

9.
Acta Neuropathol Commun ; 10(1): 66, 2022 04 28.
Artículo en Inglés | MEDLINE | ID: mdl-35484610

RESUMEN

Pathologists can label pathologies differently, making it challenging to yield consistent assessments in the absence of one ground truth. To address this problem, we present a deep learning (DL) approach that draws on a cohort of experts, weighs each contribution, and is robust to noisy labels. We collected 100,495 annotations on 20,099 candidate amyloid beta neuropathologies (cerebral amyloid angiopathy (CAA), and cored and diffuse plaques) from three institutions, independently annotated by five experts. DL methods trained on a consensus-of-two strategy yielded 12.6-26% improvements by area under the precision recall curve (AUPRC) when compared to those that learned individualized annotations. This strategy surpassed individual-expert models, even when unfairly assessed on benchmarks favoring them. Moreover, ensembling over individual models was robust to hidden random annotators. In blind prospective tests of 52,555 subsequent expert-annotated images, the models labeled pathologies like their human counterparts (consensus model AUPRC = 0.74 cored; 0.69 CAA). This study demonstrates a means to combine multiple ground truths into a common-ground DL model that yields consistent diagnoses informed by multiple and potentially variable expert opinions.


Asunto(s)
Angiopatía Amiloide Cerebral , Aprendizaje Profundo , Péptidos beta-Amiloides , Angiopatía Amiloide Cerebral/diagnóstico , Humanos , Neuropatología , Estudios Prospectivos
10.
Front Neurol ; 13: 1030468, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36438967

RESUMEN

Background: Cases of isolated intramedullary spinal neurocysticercosis are extremely rare. Only 25 cases have been reported before 2022. Due to its rarity, the diagnosis of spinal neurocysticercosis may be missed. Case presentation: We describe a 37-year-old female patient who developed back pain and lower extremity weakness and was found to have an intramedullary thoracic spine cystic lesion. She was taken to the operating room for resection of the lesion. Pathology revealed a larval cyst wall consistent with neurocysticercosis. The patient was started on albendazole and dexamethasone. Her exam improved post-operatively, and she was able to ambulate with minimal difficulty at the time of follow up. Conclusion: The case provides insights on the diagnosis and treatment of isolated intramedullary spinal neurocysticercosis. Review of the literature suggests that combined surgical and medical intervention results in significant improvement in the patient's neurological exam, and decreases morbidity associated with the disease. We propose a treatment paradigm for this rare manifestation of neurocysticercosis.

11.
J Neurosurg Case Lessons ; 1(21): CASE20168, 2021 May 24.
Artículo en Inglés | MEDLINE | ID: mdl-35854865

RESUMEN

BACKGROUND: Hypertrophic cranial pachymeningitis is a rare inflammatory disorder characterized by thickening of the dura mater and multiple cranial neuropathies. Although an infectious etiology may be present, often no specific cause is discovered. OBSERVATIONS: The authors described a 71-year-old man with progressive right eye vision loss, ptosis, and complete ophthalmoplegia with imaging findings suggestive of hypertrophic cranial pachymeningitis. Extensive studies, including cerebrospinal fluid studies, showed negative results. Blood serum, cell-free evaluation, and paraffin-embedded dural tissue testing had positive results for Pseudomonas aeruginosa, which allowed treatment tailored to the organism and a salutary clinical outcome. LESSONS: The constellation of neurological and radiological findings may make a diagnosis difficult in an inflammatory setting. The most precise methodology for establishing a diagnosis involves sampling the dura and testing it for infectious pathology. However, if results are inconclusive, further cell-free serum sampling with next-generation sequencing is a viable option for identifying pathogens with infectious concerns. This case highlighted the importance of multimodality studies for identifying a targetable pathogen.

12.
J Neurol Surg B Skull Base ; 82(2): 182-188, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33777632

RESUMEN

Introduction For patients presenting with neurological changes from pituitary tumor apoplexy, urgent surgical intervention is commonly performed for diagnosis, tumor resection, and optic apparatus decompression. Although identification and preservation of the pituitary gland during the time of surgery can be challenging, it may lead to improve endocrine outcomes. Methods A retrospective case series of all patients with macroadenomas presenting with apoplexy at Loyola University Medical Center from 2016 to 2018 was studied. Demographic, radiographic, and intraoperative characteristics were collected including age, gender, comorbidities, presenting symptoms, preoperative size of pituitary adenoma, Knosp's grade, Hardy's grade, identification and/or preservation of the gland, pre- and postoperative hormonal levels, intraoperative and/or postoperative complications, and follow-up time. Results A total of 68 patients underwent endoscopic endonasal surgery for resection of a macroadenoma. Among them, seven (10.2%) presented with apoplexy; five patients were male and two were female and presenting symptoms and signs included headache (100%), endocrinopathies (57%), visual acuity deficit (71%), visual field deficit (71%), and oculomotor palsy (57%). A gross-total resection rate was achieved in 86% of patients. Among them, 71% of patients obtained complete symptomatic neurological improvement. A statistically significant difference between gender and endocrine function was found, as no females and all males required some form of postoperative hormonal supplementation ( p = 0.047) . Conclusion Endoscopic endonasal resection of macroadenomas with sparing of the pituitary gland in the setting of apoplexy is safe and effective. Preservation of the normal gland led to no posterior pituitary dysfunction, and a statistically significant difference between gender and postoperative endocrinopathy was identified. Further studies with larger samples sizes are warranted.

13.
Kidney Med ; 3(2): 282-285, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33851124

RESUMEN

Case reports of acute kidney injury in patients taking the glucagon-like peptide 1 (GLP-1) receptor agonists exenatide and liraglutide have been reported. We report 2 patients with chronic kidney disease due to diabetic kidney disease who experienced rapid worsening of kidney function and increased proteinuria after being prescribed the GLP-1 receptor agonist semaglutide. In 1 patient, kidney biopsy showed advanced diffuse and nodular glomerulosclerosis accompanied by interstitial lymphoplasmacytic and eosinophilic infiltrate and evidence of acute tubular injury. At this time, the long-term outcomes of patients who experience acute kidney injury associated with GLP-1 receptor agonists is not known. We recommend that caution be used with these agents in patients with moderate to severe chronic kidney disease due to limited kidney reserve in the event of an adverse kidney event. Because most adverse kidney events have occurred in patients who experience adverse gastrointestinal symptoms, such patients should have laboratory tests and discontinuation of the medication if there is acute worsening of kidney function.

14.
World Neurosurg ; 152: 84-94, 2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-34087464

RESUMEN

BACKGROUND: Hemangioblastomas are benign vascular neoplasms of the central nervous system that may occur sporadically or in association with Von Hippel-Lindau disease. A minority of these lesions can occur in the spine, mostly as intramedullary masses. The authors present a rare case of primary sporadic spinal hemangioblastoma occurring as an intradural extramedullary (IDEM) lesion. Diagnostic workup and surgical management of the patient are described. METHODS: A systematic MEDLINE search was conducted using the keywords "hemangioblastoma" and "intradural extramedullary," "extramedullary," or "cauda equina." Clinicopathological characteristics and outcomes of the present case were reviewed and compared with those in the literature. RESULTS: A 72-year-old man was found to have an IDEM lesion in his cervical spine after presenting with neck and shoulder pain. Gross total resection was successfully performed with sacrifice of an involved dorsal nerve rootlet. Screening for Von Hippel-Lindau was negative. Thirty-three additional patients with sporadic IDEM hemangioblastomas are reported in the literature. There was a slight male preponderance (54%) with a median age of 52 years. Patients presented with pain (54%), radiculopathy (33%), or myelopathy (32%). The majority of lesions were located in the lumbosacral spine (56%). All patients underwent maximal safe resection with stable or improved clinical status. CONCLUSION: Primary IDEM hemangioblastomas are a rare entity. Differential diagnosis includes other IDEM lesions, such as schwannomas, meningiomas, or some vascular malformations. Resection of these sporadic tumors can be safely performed and result in improvement of neurologic deficits associated with mass effect from the tumor with low likelihood of recurrence.


Asunto(s)
Hemangioblastoma/patología , Neoplasias de la Médula Espinal/patología , Anciano , Hemangioblastoma/cirugía , Humanos , Masculino , Neoplasias de la Médula Espinal/cirugía
15.
World Neurosurg ; 153: 11-20, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-34182175

RESUMEN

BACKGROUND: Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs. METHODS: A systematic MEDLINE search was conducted using key words "hemangiopericytoma" OR "solitary fibrous tumor" AND "jugular foramen" OR "extracranial" OR "skull base." Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature. RESULTS: A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n = 2) or JF schwannomas (n = 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy. CONCLUSION: The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.


Asunto(s)
Hemangiopericitoma/patología , Foramina Yugular/patología , Adulto , Errores Diagnósticos , Glomo Yugular/efectos de los fármacos , Glomo Yugular/patología , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/terapia , Humanos , Imagen por Resonancia Magnética , Masculino , Radiocirugia
16.
J Neurol Surg B Skull Base ; 81(6): 651-658, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33381369

RESUMEN

Objective Most pituitary adenomas are of soft consistency and can be resected during surgery with routine suction instruments. However, fibrous adenomas may require more aggressive techniques. The ability to predict consistency on magnetic resonance imaging (MRI) would improve preoperative preparation and may have implications on the extent of resection. Design A retrospective review of MRI and tumor histology of 50 consecutive patients who underwent endoscopic endonasal resection for nonfunctional adenomas was performed. Methods An intensity ratio was calculated based on quantitative MRI signal intensity of the adenoma and pons. Intraoperatively, a sequentially graded technique required for resection ranged from suction (R1) for softer tumors, curettes (R2) for tumors with intermediate consistency, and aspirators and/or other microinstruments (R3) for firmer tumors. Fibrotic content was determined from histologic collagen percentage, and rates of gross total resection (GTR) were calculated from postoperative imaging. Statistical analyses were performed to determine if resection classification could be predicted by intensity ratio or collagen percentage, calculate ratio of cut-off points for clinical use, and assess for correlation between intensity ratios and collagen percentage. Results Tumors with ratios < 1.6 on the T2-weighted coronal image and collagen content > 5.3% were likely to have required a more aggressive resection technique. Statistically significant lower rates of GTR and higher rates of perioperative complications were seen with such tumors. Conclusion Preoperative MRI analyses can be helpful but not definitive in predicting adenoma consistency. Fibrous adenomas, associated with higher collagen content, are more difficult to resect and have higher rates of subtotal resection.

17.
J Neurosurg ; 111(2): 301-5, 2009 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-19025354

RESUMEN

Balamuthia mandrillaris is a rare but increasingly recognized cause of amebic encephalitis, yet it remains poorly understood. The condition is almost universally fatal, and due to diagnostic difficulty, most cases are identified postmortem. The authors report a case of Balamuthia amebic encephalitis in a patient with combined variable immunodeficiency in which a rare antemortem diagnosis was made via brain biopsy. Despite broad-spectrum antimicrobial therapy, the outcome was fatal. Such presentations are challenging, and definitive diagnosis may require biopsy in consultation with a skilled neuropathologist.


Asunto(s)
Amebiasis/parasitología , Síndromes de Inmunodeficiencia/complicaciones , Meningoencefalitis/parasitología , Adulto , Amebiasis/diagnóstico , Amebiasis/patología , Resultado Fatal , Humanos , Masculino , Meningoencefalitis/diagnóstico , Meningoencefalitis/patología
18.
World Neurosurg ; 132: 239-244, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31520753

RESUMEN

BACKGROUND: Malignant transformation of a mature intracranial teratoma into an adenocarcinoma is an extremely rare event and portends a poor prognosis. The clinical progression, radiographic changes, histopathological findings, and immunohistochemistry findings from these unique cases might provide clues regarding this transformation. Caudal homeobox gene-2 (CDX-2) is a specific and robust marker for colonic adenocarcinomas and can also be used to identify differentiation of mature intracranial teratoma into colonic-type adenocarcinoma. To the best of our knowledge, this is the first case report of the use of this specific marker for an intracranial malignancy. CASE DESCRIPTION: We present the case of a 55-year-old right-handed Hispanic-American woman with progressive headaches who was found to have a left posterior parietotemporal tumor with intraventricular extension. Surgical debulking was performed, and the pathologic examination revealed a mature teratoma. Despite surgical resection and radiotherapy, the teratoma progressed to a malignant mature intracranial teratoma both radiographically and histologically. Histological analysis of the third specimen revealed a moderately differentiated adenocarcinoma. The tumor cells were positive for CDX-2 and cytokeratin 20 and negative for cytokeratin 7 and thyroid transcription factor-1, consistent with an enteric/colonic-type adenocarcinoma, demonstrating progressive atypia and malignancy. CONCLUSIONS: Malignant transformation of a mature intracranial teratoma portends a poor prognosis. The exact histopathological diagnosis can facilitate treatment of these patients. CDX-2 is a specific robust marker for identifying differentiation of a mature intracranial teratoma into a colonic adenocarcinoma. This positive staining can also be observed in primary colonic and other adenocarcinomas. To the best of our knowledge, this is the first report of the use of CDX-2 in the diagnosis of an intracranial malignancy. The triangulation of clinical progression, radiographic findings, and histopathological and immunohistochemistry findings provided clues regarding this unique transformation.


Asunto(s)
Adenocarcinoma/metabolismo , Neoplasias Encefálicas/metabolismo , Factor de Transcripción CDX2/metabolismo , Neoplasias del Colon/metabolismo , Teratoma/metabolismo , Adenocarcinoma/diagnóstico por imagen , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Diferenciación Celular , Procedimientos Quirúrgicos de Citorreducción , Progresión de la Enfermedad , Femenino , Humanos , Queratina-20/metabolismo , Queratina-7/metabolismo , Persona de Mediana Edad , Teratoma/diagnóstico por imagen , Teratoma/cirugía , Factor Nuclear Tiroideo 1/metabolismo
19.
J Neuroimaging ; 29(4): 423-430, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-30994961

RESUMEN

BACKGROUND AND PURPOSE: The use of ventricular assist devices (VADs) for the treatment of heart failure has become increasingly common. These patients have a considerable risk of cerebral embolism. We describe such a patient and his successful treatment by thrombectomy, compare his attributes with those previously published, and describe the construct of a clinical decision model, whose results bear practical implications for patient management. METHODS: The details of our patient and his treatment are presented, followed by a literature review of all previously reported similar cases. Using this information, as well as that available from published series, we constructed a probabilistic decision tree, completed all calculations (ie, "folding back"), and, in order to assess the strength of the results, subjected them to multiple independent sensitivity analyses of each of the variables. RESULTS: The therapeutic success of our case, the 14th reported to date, when combined with previous reports, shows: (1) recanalization times of 184 minutes, (2) "successful" recanalization (ie, TICI = 2b or 3) achieved in 71% of procedures, (3) ultimate functional outcome (ie, mRS = 0-2) achieved in 57% patients, and (4) ultimate successful heart transplantations in 66% of cases. The clinical decision model showed the predicted utility of thrombectomy to be superior to conservative management (3.33 QALY vs. 2.56 QALY, respectively). The sensitivity analyses support the validity of these results. CONCLUSIONS: In conclusion, thrombectomy appears to be a safe and effective method (and often the only viable one) for urgent treatment of patients with VAD-originated cerebral embolism.


Asunto(s)
Isquemia Encefálica/cirugía , Corazón Auxiliar/efectos adversos , Embolia Intracraneal/cirugía , Accidente Cerebrovascular/cirugía , Trombectomía/métodos , Isquemia Encefálica/etiología , Reglas de Decisión Clínica , Humanos , Embolia Intracraneal/etiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Accidente Cerebrovascular/etiología , Resultado del Tratamiento
20.
Case Rep Neurol Med ; 2019: 9769657, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30733881

RESUMEN

BACKGROUND: Pituitary metastasis of cervical adenocarcinoma is an exceedingly rare phenomenon. CASE DESCRIPTION: The authors present a case of a 66-year-old female with cervical adenocarcinoma who was discovered to have a rapidly growing intrasellar mass in the work-up of adrenal insufficiency and hypothyroidism. The patient underwent subsequent endoscopic endonasal subtotal resection of the mass. Histopathological analysis of the resected lesion demonstrated features consistent with metastatic mucinous adenocarcinoma of the cervix. While initially neurologically asymptomatic following surgery, the patient developed an oculomotor nerve palsy several weeks following surgical debulking, at which time neuroimaging revealed marked regrowth and suprasellar extension of the metastatic lesion. CONCLUSIONS: While metastatic cervical adenocarcinoma to the sella is rare, it should be considered in the differential based on the history of the patient.

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