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PURPOSE: Angioleiomyoma, predominantly arising from the extremities, is a benign soft tissue tumor. Reports on its intracranial location are rare. We assessed clinical, radiological, and pathological features of intracranial angioleiomyoma (iALM) treated at our neurosurgical institution. METHODS: We consecutively enrolled all patients with neuropathologically confirmed iALM treated at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were analyzed. A review of the literature on iALM was conducted. RESULTS: Seven patients with iALM (four female) with a median age of 45 years (range: 32-76 years) were identified. In three cases, the lesion was found incidentally. In magnetic resonance imaging (MRI), all tumors were hypo- to isointense on T1-weighted, hyperintense on T2-weighted sequences, and gadolinium-enhancing. A strong FLAIR signal was seen in six patients. Surgery consisted of gross total resection in all cases without perioperative complications. Neuropathological staining was positive for smooth muscle actin (SMA) in all lesions. Mature smooth muscle cells arranged around blood vessels were typically observed. The Ki-67 index was ≤ 3%. The patients were discharged after a median of 6 days (range: 4-9 days). During a median follow-up time of 14 months (range: 4-41 months), no tumor recurrence occurred. In the current literature, 42 additional cases of iALM were identified. CONCLUSION: Intracranial angioleiomyoma is a benign soft tissue tumor treated by gross total resection. Tumor morphology and positive staining for SMA lead to the neuropathological diagnosis.
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Angiomioma , Neoplasias Encefálicas , Humanos , Angiomioma/patologia , Angiomioma/cirurgia , Angiomioma/diagnóstico por imagem , Feminino , Pessoa de Meia-Idade , Adulto , Masculino , Idoso , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Imageamento por Ressonância MagnéticaRESUMO
PURPOSE: Mutations in the Isocitrate Dehydrogenase (IDH) genes, IDH1 or IDH2, define a group of adult diffuse gliomas associated with a younger age at diagnosis and better prognosis than IDH wild-type glioblastoma. Within IDH mutant gliomas, a small fraction of astrocytic tumors present with grade 4 histologic features and poor prognosis. In molecular studies, homozygous deletion of CDKN2A/B is independently predictive of poor prognosis and short survival. As a consequence, 2021 WHO classification now also recognizes this molecular feature, CDKN2A/B deletion, as sufficient for classifying an astrocytoma as IDH-mutant, WHO Grade 4, regardless of histological grading. Here, we investigate outcomes of patients with WHO Grade 4 IDH-mutant astrocytoma both with and without CDKN2A/B deletion, to compare these groups and evaluate clinical and radiographic factors that contribute to survival. METHODS: We retrospectively identified 79 patients with IDH-mutant astrocytoma with CDKN2A/B deletion detected at initial diagnosis across five international institutions as well as a comparison group of 51 patients with IDH-mutant, astrocytoma, histologically Grade 4 without detectable CDKN2A/B deletion. We assembled clinical and radiographic features for all patients. RESULTS: We find that CDKN2A/B deletion was associated with significantly worse overall survival (OS; p = 0.0004) and progression-free survival (PFS; p = 0.0026), with median OS of 5.0 years and PFS of 3.0 years, compared to 10.1 and 5.0 years for tumors with a grade 4 designation based only on histologic criteria. Multivariate analysis confirmed CDKN2A/B deletion as a strong negative prognosticator for both OS (HR = 3.51, p < 0.0001) and PFS (HR = 2.35, p = 0.00095). In addition, in tumors with CDKN2A/B deletion, preoperative contrast enhancement is a significant predictor of worse OS (HR 2.19, 95% CI 1.22-3.93, p = 0.0090) and PFS (HR = 1.74, 95% CI = 1.02-2.97, p = 0.0420). CONCLUSIONS: These findings underscore the severe prognostic impact of CDKN2A/B deletion in IDH-mutant astrocytomas and highlight the need for further refinement of tumor prognostic categorization. Our results provide a key benchmark of baseline patient outcomes for therapeutic trials, underscoring the importance of CDKN2A/B status assessment, in addition to histologic grading, in clinical trial design and therapeutic decision-making for IDH-mutant astrocytoma patients.
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PURPOSE: Surgery for recurrent glioma provides cytoreduction and tissue for molecularly informed treatment. With mostly heavily pretreated patients involved, it is unclear whether the benefits of repeat surgery outweigh its potential risks. METHODS: Patients receiving surgery for recurrent glioma WHO grade 2-4 with the goal of tissue sampling for targeted therapies were analyzed retrospectively. Complication rates (surgical, neurological) were compared to our institutional glioma surgery cohort. Tissue molecular diagnostic yield, targeted therapies and post-surgical survival rates were analyzed. RESULTS: Between 2017 and 2022, tumor board recommendation for targeted therapy through molecular diagnostics was made for 180 patients. Of these, 70 patients (38%) underwent repeat surgery. IDH-wildtype glioblastoma was diagnosed in 48 patients (69%), followed by IDH-mutant astrocytoma (n = 13; 19%) and oligodendroglioma (n = 9; 13%). Gross total resection (GTR) was achieved in 50 patients (71%). Tissue was processed for next-generation sequencing in 64 cases (91%), and for DNA methylation analysis in 58 cases (83%), while immunohistochemistry for mTOR phosphorylation was performed in 24 cases (34%). Targeted therapy was recommended in 35 (50%) and commenced in 21 (30%) cases. Postoperatively, 7 patients (11%) required revision surgery, compared to 7% (p = 0.519) and 6% (p = 0.359) of our reference cohorts of patients undergoing first and second craniotomy, respectively. Non-resolving neurological deterioration was documented in 6 cases (10% vs. 8%, p = 0.612, after first and 4%, p = 0.519, after second craniotomy). Median survival after repeat surgery was 399 days in all patients and 348 days in GBM patients after repeat GTR. CONCLUSION: Surgery for recurrent glioma provides relevant molecular diagnostic information with a direct consequence for targeted therapy under a reasonable risk of postoperative complications. With satisfactory postoperative survival it can therefore complement a multi-modal glioma therapy approach.
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Neoplasias Encefálicas , Glioma , Humanos , Reoperação , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Estudos Retrospectivos , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/cirurgia , Medicina de Precisão , Glioma/genética , Glioma/cirurgia , Glioma/patologiaRESUMO
PURPOSE: Quantitative pupillometry (QP) has been increasingly applied in neurocritical care as an easy-to-use and reliable technique for evaluating the pupillary light reflex (PLR). Here, we report our preliminary findings on using QP for clinical brain death (BD) determination. MATERIALS: This retrospective study included 17 patients ≥ 18 years (mean age, 57.3 years; standard deviation, 15.8 years) with confirmed BD, as defined by German Guidelines for the determination of BD. The PLR was tested using the NPi®-200 Pupillometer (Neuroptics, Laguna Hill, USA), a handheld infrared device automatically tracking and analyzing pupil dynamics over 3 s. In addition, pupil diameter and neurological pupil index (NPi) were also evaluated. RESULTS: Intracerebral bleeding, subarachnoid hemorrhage, and hypoxic encephalopathy were the most prevalent causes of BD. In all patients, the NPi was 0 for both eyes, indicating the cessation of mid-brain function. The mean diameter was 4.9 mm (± 1.3) for the right pupil and 5.2 mm (±1.2) for the left pupil. CONCLUSIONS: QP is a valuable tool for the BD certification process to assess the loss of PLR due to the cessation of brain stem function. Furthermore, implementing QP before the withdrawal of life-sustaining therapy in brain-injured patients may reduce the rate of missed organ donation opportunities. Further studies are warranted to substantiate the feasibility and potential of this technique in treating patients and identify suitable candidates for this technique during the BD certification process.
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Morte Encefálica , Reflexo Pupilar , Humanos , Pessoa de Meia-Idade , Reflexo Pupilar/fisiologia , Estudos Retrospectivos , Morte Encefálica/diagnóstico , Pupila/fisiologia , EncéfaloRESUMO
Visual field deficits (VFDs) are common in patients with temporal and occipital lobe lesions. Diffusion tensor fiber tractography (DTI-FT) is widely used for surgery planning to reduce VFDs. Q-ball high-resolution fiber tractography (QBI-HRFT) improves upon DTI. This study aims to evaluate the effectiveness of DTI-FT and QBI-HRFT for surgery planning near the optic radiation (OR) as well as the correlation between VFDs, the nearest distance from the lesion to the OR fiber bundle (nD-LOR), and the lesion volume (LV). This ongoing prospective clinical trial collects clinical and imaging data of patients with lesions in deterrent areas. The present subanalysis included eight patients with gliomas near the OR. Probabilistic HRFT based on QBI-FT and conventional DTI-FT were performed for OR reconstruction based on a standard diffusion-weighted magnetic resonance imaging sequence in clinical use. Quantitative analysis was used to evaluate the lesion volume (LV) and nD-LOR. VFDs were determined based on standardized automated perimetry. We included eight patients (mean age 51.7 years [standard deviation (SD) 9.5]) with lesions near the OR. Among them, five, two, and one patients had temporodorsal, occipital, and temporal lesions, respectively. Four patients had normal vision preoperatively, while four patients had preexisting VFD. QBI-FT analysis indicated that patients with VFD exhibited a significantly smaller median nD-LOR (mean, -4.5; range -7.0; -2.3) than patients without VFD (mean, 7.4; range -4.3; 27.2) (p = 0.050). There was a trend towards a correlation between tumor volume and nD-LOR when QBI-FT was used (rs = -0.6; p = 0.056). A meticulous classification of the spatial relationship between the lesions and OR according to DTI-FT and QBI-FT was performed. The results indicated that the most prevalent orientations were the FT bundles located laterally and intrinsically in relation to the tumor. Compared with conventional DTI-FT, QBI-FT suggests reliable and more accurate results when correlated to preoperative VFDs and might be preferred for preoperative planning and intraoperative use of nearby lesions, particularly for those with larger volumes. A detailed analysis of localization, surgical approach together with QBI-FT and DTI-FT could reduce postoperative morbidity regarding VFDs. The display of HRFT techniques intraoperatively within the navigation system should be pursued for this issue.
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Glioma , Campos Visuais , Humanos , Pessoa de Meia-Idade , Imagem de Difusão por Ressonância Magnética , Imagem de Tensor de Difusão/métodos , Glioma/cirurgia , Estudos ProspectivosRESUMO
INTRODUCTION: Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST. METHODS: We conducted a retrospective review of our institutional pediatric oncology and neurosurgery database, examining 14 patients under 18 years admitted with ST due to oncological diseases since 2005. We analyzed the clinical presentations, evaluations, molecular diagnostics and treatments for these patients. RESULTS: The study spanned 15 years and included 14 pediatric patients, each diagnosed with distinct spinal tumor entity. The mean patient age was approximately 19.6 ± 10.1 months. Severe axial pain along the vertebral column was observed in 13 patients, while acute neurological deterioration manifested in 7 patients. As a first-line intervention, 13 patients underwent decompressive surgery through laminectomy and tumor resection, and only one patient received chemotherapy solely. Before surgery, seven patients were unable to walk; post-surgery, six of them regained their ability to ambulate. The diagnosis encompassed a range of neoplasms: two instances of Ewing sarcoma, 3 instances of teratoma, one case presenting an atypical teratoid Rhabdoid tumor, two instances each of low-grade astrocytoma and neuroblastoma, and single instances of ependymoma, meningioma, rhabdomyosarcoma, and embryonal tumors with multilayered rosettes (ETMRs). Three patients succumbed two years after initiating therapy. CONCLUSION: Despite their rarity, intraspinal tumors in pediatric patients pose substantial therapeutic challenges. The intertwined complexities of the disease entity and the patient's neurological status demand swift initiation of an individualized therapeutic strategy. This crucial step helps optimize outcomes for this patient cohort, who frequently grapple with debilitating health conditions. Inclusion of these patients within a registry is mandatory to optimize treatment outcomes due to their rarity in pediatric population.
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Neoplasias da Coluna Vertebral , Humanos , Masculino , Feminino , Estudos Retrospectivos , Pré-Escolar , Criança , Lactente , Adolescente , Resultado do Tratamento , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/complicações , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/terapia , Sarcoma de Ewing/complicações , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Medula Espinal/complicações , Ependimoma/terapia , Ependimoma/cirurgia , Ependimoma/diagnóstico , Laminectomia , Descompressão Cirúrgica/métodos , Teratoma/complicações , Teratoma/cirurgia , Teratoma/diagnóstico , Teratoma/terapia , Procedimentos Neurocirúrgicos/métodos , Neuroblastoma/cirurgia , Neuroblastoma/complicações , Astrocitoma/complicações , Astrocitoma/cirurgia , Astrocitoma/terapia , Tumor Rabdoide/terapia , Tumor Rabdoide/complicações , Meningioma/cirurgia , Meningioma/terapia , Meningioma/complicações , Meningioma/diagnósticoRESUMO
OBJECTIVE: Otitis media and sinusitis are common childhood infections, typically mild with good outcomes. Recent studies show a rise in intracranial abscess cases in children, raising concerns about a link to COVID-19. This study compares a decade of data on these cases before and after the pandemic. METHODS: This retrospective comparative analysis includes pediatric patients diagnosed with otitis media and sinusitis, who later developed intracranial abscesses over the past decade. We collected comprehensive data on the number of cases, patient demographics, symptoms, treatment, and outcomes. RESULTS: Between January 2013 and July 2023, our center identified 10 pediatric patients (median age 11.1years, range 2.2-18.0 years, 60% male) with intracranial abscesses from otitis media and sinusitis. Of these, 7 cases (70%, median age 9.7 years, range 2.2-18.0 years) occurred since the onset of the COVID-19 pandemic, while the remaining 3 cases (30%, median age 13.3 years, range 9.9-16.7 years) were treated before the pandemic. No significant differences were found in otolaryngological associations, surgical interventions, preoperative symptoms, lab findings, or postoperative antibiotics between the two groups. All patients showed positive long-term recovery. CONCLUSION: This study reveals 5-fold increase of pediatric otogenic and sinogenic intracranial abscess cases in the last three-years since the onset of the COVID-19 pandemic. While further investigation is needed, these findings raise important questions about potential connections between the pandemic and the severity of otitis media and sinusitis complications in children. Understanding these associations can improve pediatric healthcare management during infectious disease outbreaks.
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Abscesso Encefálico , COVID-19 , Otite Média , Sinusite , Humanos , COVID-19/epidemiologia , COVID-19/complicações , Criança , Masculino , Feminino , Estudos Retrospectivos , Adolescente , Pré-Escolar , Otite Média/epidemiologia , Otite Média/complicações , Otite Média/cirurgia , Sinusite/epidemiologia , Sinusite/complicações , Abscesso Encefálico/epidemiologia , SARS-CoV-2 , PandemiasRESUMO
INTRODUCTION: Brain tumor surgery represents a critical and high-risk area within the field of neurosurgery. Our study aims to offer a comprehensive analysis of adverse events (AEs) from a prospectively maintained database at a leading neurosurgical tertiary center, with a specific focus on different types of tumor entities. METHODS: From January 2022 to September 2023, our study focused on adult patients, who underwent surgery for intracranial tumors. Each patient in this demographic was thoroughly assessed for adverse events (AEs) by their attending physicians at discharge. An AE was defined as any event occurring within the first 30 days post-surgery. RESULTS: A total of 1173 patients with an average age of 57.4 ± 15.3 years underwent surgical procedures. The majority of these surgeries were elective, accounting for 93.4% (1095 out of 1173), while emergency surgeries constituted 13.9% (163 out of 1173). The incidence of surgery-related AEs was relatively low at 12.7%. The most common surgical indications were meningioma and glioma pathologies, representing 31.1% and 28.2% of cases, respectively. Dural leaks occurred in 1.5% of the cases. Postoperative hemorrhage was a significant complication, especially among glioma patients, with ten experiencing postoperative hemorrhage and eight requiring revision surgery. The overall mortality rate stood at 0.8%, corresponding to five patient deaths. Causes of death included massive postoperative bleeding in one patient, pulmonary embolism in two patients, and tumor progression in two others. CONCLUSIONS: Surgical interventions for intracranial neoplasms are inherently associated with a significant risk of adverse events. However, our study's findings reveal a notably low mortality rate within our patient cohort. This suggests that thorough documentation of AEs, coupled with proactive intervention strategies in neurosurgical practices, can substantially enhance patient outcomes.
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Neoplasias Encefálicas , Glioma , Neurocirurgia , Adulto , Humanos , Pessoa de Meia-Idade , Idoso , Estudos Prospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/complicações , Hemorragia Pós-Operatória , Glioma/complicaçõesRESUMO
INTRODUCTION: The management of spinal sarcomas is complex, given their widespread involvement and high recurrence rates. Despite consensus on the need for a multidisciplinary approach with surgery at its core, there is a lack of definitive guidelines for clinical decision-making. This study examines a case series of primary spinal sarcomas, focusing on the surgical strategies, clinical results, and survival data to inform and guide therapeutic practices. METHODS: We conducted a retrospective analysis of patients who underwent surgical resection for primary spinal sarcomas between 2005 and 2022. The study focused on gathering data on patient demographics, surgical details, postoperative complications, overall hospital stay, and mortality within 90 days post-surgery. RESULTS: The study included 14 patients with a primary diagnosis of spinal sarcoma, with an average age of 48.6 ± 12.6 years. Chondrosarcoma emerged as the most common tumor type, representing 57.1% of cases, followed by Ewing sarcoma at 35.7%, and synovial sarcoma at 7.1%. Patients with chondrosarcoma were treated with en-bloc resection, while the patient with synovial sarcoma underwent intra-lesional excision and those with Ewing sarcoma received decompression and tumor debulking. Postoperative assessments revealed significant improvements in neurological conditions. Notably, functional status as measured by the Karnofski Performance Index (KPI), improved substantially post-surgery (from 61.4 to 80.0%) The mean follow-up was 34.9 ± 9.2 months. During this time period one patient experienced fatal bleeding after en-bloc resection complications involving the vena cava. None of the patient needed further surgery. CONCLUSIONS: Our 16-year study offers vital insights into managing primary spinal sarcomas, showcasing the effectiveness of surgical intervention, particularly en-bloc resection. Despite their rarity and complexity, our multidisciplinary treatment approach yields improved outcomes and highlights the potential for refined surgical strategies to become standardized care in this challenging domain.
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Sarcoma , Neoplasias da Coluna Vertebral , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Masculino , Feminino , Adulto , Sarcoma/cirurgia , Sarcoma/mortalidade , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/mortalidade , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos , Idoso , Sarcoma Sinovial/cirurgia , Sarcoma Sinovial/mortalidade , Condrossarcoma/cirurgia , Condrossarcoma/mortalidade , Condrossarcoma/patologia , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/mortalidade , Complicações Pós-Operatórias/etiologia , Equipe de Assistência ao PacienteRESUMO
BACKGROUND: Several individual predictors for outcomes in patients with cerebellar stroke (CS) have been previously identified. There is, however, no established clinical score for CS. Therefore, the aim of this study was to develop simple and accurate grading scales for patients with CS in an effort to better estimate mortality and outcomes. METHODS: This multicentric retrospective study included 531 patients with ischemic CS presenting to 5 different academic neurosurgical and neurological departments throughout Germany between 2008 and 2021. Logistic regression analysis was performed to determine independent predictors related to 30-day mortality and unfavorable outcome (modified Rankin Scale score of 4-6). By weighing each parameter via calculation of regression coefficients, an ischemic CS-score and CS-grading scale (CS-GS) were developed and internally validated. RESULTS: Independent predictors for 30-day mortality were aged ≥70 years (odds ratio, 5.2), Glasgow Coma Scale score 3 to 4 at admission (odds ratio, 2.6), stroke volume ≥25 cm3 (odds ratio, 2.7), and involvement of the brain stem (odds ratio, 3.9). When integrating each parameter into the CS-score, age≥70 years and brain stem stroke were assigned 2 points, Glasgow Coma Scale score 3 to 4, and stroke volume≥25 cm3 1 point resulting in a score ranging from 0 to 6. CS-score of 0, 1, 2, 3, 4, 5, and 6 points resulted in 30-day mortality of 1%, 6%, 6%, 17%, 21%, 55%, and 67%, respectively. Independent predictors for 30-day unfavorable outcomes consisted of all components of the CS-score with an additional variable focused on comorbidities (CS-GS). Except for Glasgow Coma Scale score 3 to 4 at admission, which was assigned 3 points, all other parameters were assigned 1 point resulting in an overall score ranging from 0 to 7. CS-GS of 0, 1, 2, 3, 4, 5, 6, and 7 points resulted in 30-day unfavorable outcome of 1%, 17%, 33%, 40%, 50%, 80%, 77%, and 100%, respectively. Both 30-day mortality and unfavorable outcomes increased with increasing CS-score and CS-GS (P<0.001). CONCLUSIONS: The CS-score and CS-GS are simple and accurate grading scales for the prediction of 30-day mortality and unfavorable outcome in patients with CS. While the score systems proposed here may not directly impact treatment decisions, it may help discuss mortality and outcome with patients and caregivers.
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Isquemia Encefálica , AVC Isquêmico , Acidente Vascular Cerebral , Humanos , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/terapia , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/terapia , Resultado do Tratamento , IdosoRESUMO
PURPOSE: The purpose of this study was to compare the potential of asymmetry-based (APTwasym ), Lorentzian-fit-based (PeakAreaAPT and MTconst ), and relaxation-compensated (MTRRex APT and MTRRex MT) CEST contrasts of the amide proton transfer (APT) and semi-solid magnetization transfer (ssMT) for early response assessment and prediction of progression-free survival (PFS) in patients with glioma. METHODS: Seventy-two study participants underwent CEST-MRI at 3T from July 2018 to December 2021 in a prospective clinical trial four to 6 wk after the completion of radiotherapy for diffuse glioma. Tumor segmentations were performed on T2w -FLAIR and contrast-enhanced T1w images. Therapy response assessment and determination of PFS were performed according to response assessment in neuro oncology (RANO) criteria using clinical follow-up data with a median observation time of 9.2 mo (range, 1.6-40.8) and compared to CEST MRI metrics. Statistical testing included receiver operating characteristic analyses, Mann-Whitney-U-test, Kaplan-Meier analyses, and logrank-test. RESULTS: MTconst (AUC = 0.79, p < 0.01) showed a stronger association with RANO response assessment compared to PeakAreaAPT (AUC = 0.71, p = 0.02) and MTRRex MT (AUC = 0.71, p = 0.02), and enabled differentiation of participants with pseudoprogression (n = 8) from those with true progression (AUC = 0.79, p = 0.02). Furthermore, MTconst (HR = 3.04, p = 0.01), PeakAreaAPT (HR = 0.39, p = 0.03), and APTwasym (HR = 2.63, p = 0.02) were associated with PFS. MTRRex APT was not associated with any outcome. CONCLUSION: MTconst , PeakAreaAPT, and APTwasym imaging predict clinical outcome by means of progression-free survival. Furthermore, MTconst enables differentiation of radiation-induced pseudoprogression from disease progression. Therefore, the assessed metrics may have synergistic potential for supporting clinical decision making during follow-up of patients with glioma.
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Neoplasias Encefálicas , Glioma , Humanos , Amidas , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/patologia , Glioma/diagnóstico por imagem , Glioma/radioterapia , Glioma/patologia , Imageamento por Ressonância Magnética/métodos , Estudos Prospectivos , Prótons , Curva ROCRESUMO
Glioneuronal tumors are a heterogenous group of CNS neoplasms that can be challenging to accurately diagnose. Molecular methods are highly useful in classifying these tumors-distinguishing precise classes from their histological mimics and identifying previously unrecognized types of tumors. Using an unsupervised visualization approach of DNA methylation data, we identified a novel group of tumors (n = 20) that formed a cluster separate from all established CNS tumor types. Molecular analyses revealed ATRX alterations (in 16/16 cases by DNA sequencing and/or immunohistochemistry) as well as potentially targetable gene fusions involving receptor tyrosine-kinases (RTK; mostly NTRK1-3) in all of these tumors (16/16; 100%). In addition, copy number profiling showed homozygous deletions of CDKN2A/B in 55% of cases. Histological and immunohistochemical investigations revealed glioneuronal tumors with isomorphic, round and often condensed nuclei, perinuclear clearing, high mitotic activity and microvascular proliferation. Tumors were mainly located supratentorially (84%) and occurred in patients with a median age of 19 years. Survival data were limited (n = 18) but point towards a more aggressive biology as compared to other glioneuronal tumors (median progression-free survival 12.5 months). Given their molecular characteristics in addition to anaplastic features, we suggest the term glioneuronal tumor with ATRX alteration, kinase fusion and anaplastic features (GTAKA) to describe these tumors. In summary, our findings highlight a novel type of glioneuronal tumor driven by different RTK fusions accompanied by recurrent alterations in ATRX and homozygous deletions of CDKN2A/B. Targeted approaches such as NTRK inhibition might represent a therapeutic option for patients suffering from these tumors.
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Neoplasias Encefálicas , Neoplasias do Sistema Nervoso Central , Neoplasias Neuroepiteliomatosas , Humanos , Adulto Jovem , Biomarcadores Tumorais/genética , Encéfalo/patologia , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Fusão Gênica , Neoplasias Neuroepiteliomatosas/genética , Neoplasias Neuroepiteliomatosas/patologia , Receptores Proteína Tirosina Quinases/genética , Proteína Nuclear Ligada ao X/genéticaRESUMO
Focal brain damage after aneurysmal subarachnoid haemorrhage predominantly results from intracerebral haemorrhage, and early and delayed cerebral ischaemia. The prospective, observational, multicentre, cohort, diagnostic phase III trial, DISCHARGE-1, primarily investigated whether the peak total spreading depolarization-induced depression duration of a recording day during delayed neuromonitoring (delayed depression duration) indicates delayed ipsilateral infarction. Consecutive patients (n = 205) who required neurosurgery were enrolled in six university hospitals from September 2009 to April 2018. Subdural electrodes for electrocorticography were implanted. Participants were excluded on the basis of exclusion criteria, technical problems in data quality, missing neuroimages or patient withdrawal (n = 25). Evaluators were blinded to other measures. Longitudinal MRI, and CT studies if clinically indicated, revealed that 162/180 patients developed focal brain damage during the first 2 weeks. During 4.5 years of cumulative recording, 6777 spreading depolarizations occurred in 161/180 patients and 238 electrographic seizures in 14/180. Ten patients died early; 90/170 developed delayed infarction ipsilateral to the electrodes. Primary objective was to investigate whether a 60-min delayed depression duration cut-off in a 24-h window predicts delayed infarction with >0.60 sensitivity and >0.80 specificity, and to estimate a new cut-off. The 60-min cut-off was too short. Sensitivity was sufficient [= 0.76 (95% confidence interval: 0.65-0.84), P = 0.0014] but specificity was 0.59 (0.47-0.70), i.e. <0.80 (P < 0.0001). Nevertheless, the area under the receiver operating characteristic (AUROC) curve of delayed depression duration was 0.76 (0.69-0.83, P < 0.0001) for delayed infarction and 0.88 (0.81-0.94, P < 0.0001) for delayed ischaemia (reversible delayed neurological deficit or infarction). In secondary analysis, a new 180-min cut-off indicated delayed infarction with a targeted 0.62 sensitivity and 0.83 specificity. In awake patients, the AUROC curve of delayed depression duration was 0.84 (0.70-0.97, P = 0.001) and the prespecified 60-min cut-off showed 0.71 sensitivity and 0.82 specificity for reversible neurological deficits. In multivariate analysis, delayed depression duration (ß = 0.474, P < 0.001), delayed median Glasgow Coma Score (ß = -0.201, P = 0.005) and peak transcranial Doppler (ß = 0.169, P = 0.016) explained 35% of variance in delayed infarction. Another key finding was that spreading depolarization-variables were included in every multiple regression model of early, delayed and total brain damage, patient outcome and death, strongly suggesting that they are an independent biomarker of progressive brain injury. While the 60-min cut-off of cumulative depression in a 24-h window indicated reversible delayed neurological deficit, only a 180-min cut-off indicated new infarction with >0.60 sensitivity and >0.80 specificity. Although spontaneous resolution of the neurological deficit is still possible, we recommend initiating rescue treatment at the 60-min rather than the 180-min cut-off if progression of injury to infarction is to be prevented.
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Lesões Encefálicas , Depressão Alastrante da Atividade Elétrica Cortical , Hemorragia Subaracnóidea , Lesões Encefálicas/complicações , Infarto Cerebral/complicações , Eletrocorticografia , Humanos , Estudos Prospectivos , Hemorragia Subaracnóidea/complicações , Hemorragia Subaracnóidea/diagnóstico por imagemRESUMO
PURPOSE: Inflammatory and infectious diseases of the pituitary gland (IIPD) are rare lesions often misdiagnosed preoperatively. Immediate surgery is indicated especially in cases of neurological impairment. However, (chronic) inflammatory processes can mimic other pituitary tumors, such as adenomas, and data on the preoperative diagnostic criteria for IIPD are sparse. METHODS: We retrospectively reviewed medical records of 1317 patients who underwent transsphenoidal surgery at our institution between March 2003 and January 2023. A total of 26 cases of histologically confirmed IIPD were identified. Patient records, laboratory parameters, and postoperative course were analyzed and compared with an age, sex, and tumor volume-matched control group of nonfunctioning pituitary adenomas. RESULTS: Pathology confirmed septic infection in ten cases, most commonly caused by bacteria (3/10) and fungi (2/10). In the aseptic group, lymphocytic hypophysitis (8/26) and granulomatous inflammation (3/26) were most frequently observed. Patients with IIPD commonly presented with endocrine and/or neurological dysfunction. No surgical mortality occurred. Preoperative radiographic findings (cystic/solid tumor mass, contrast enhancement) did not significantly differ between IIPD and adenomas. At follow-up, 13 patients required permanent hormone substitution. CONCLUSION: In conclusion, correct preoperative diagnosis of IIPD remains challenging, as neither radiographic findings nor preoperative laboratory workup unequivocally identify these lesions. Surgical treatment facilitates decompression of supra- and parasellar structures. Furthermore, this low-morbidity procedure enables the identification of pathogens or inflammatory diseases requiring targeted medical treatment, which is crucial for these patients. Establishing a correct diagnosis through surgery and histopathological confirmation thus remains of utmost importance.
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Adenoma , Doenças Transmissíveis , Hipopituitarismo , Neoplasias Hipofisárias , Humanos , Estudos Retrospectivos , Hipófise/cirurgia , Hipófise/patologia , Adenoma/patologia , Hipopituitarismo/diagnóstico , Neoplasias Hipofisárias/patologia , Resultado do TratamentoRESUMO
Rechargeable implantable pulse generators (r-IPGs) have been available for spinal cord stimulation (SCS) claiming to offer a longer service life but demanding continuous monitoring and regular recharging by the patients. The aim of the study (DRKS00021281; Apr 7th, 2020) was to assess the convenience, safety, and acceptance of r-IPGs and their effect on patient lives under long-term therapy. Standardized questionnaires were sent to all chronic pain patients with a r-IPG at the time of trial. Primary endpoint was the overall convenience of the charging process on an ordinal scale from "very hard" (1 point) to "very easy" (5 points). Secondary endpoints were charge burden (min/week), rates of user confidence and complications (failed recharges, interruptions of therapy). Endpoints were analyzed for several subgroups. Data sets n = 40 (42% return rate) were eligible for analysis. Patient age was 57.2 ± 12.6 (mean ± standard deviation) years with the r-IPG being implanted for 52.1 ± 32.6 months. The overall convenience of recharging was evaluated as "easy" (4 points). The charge burden was 112.7 ± 139 min/week. 92% of the patients felt confident recharging the neurostimulator. 37.5% of patients reported failed recharges. 28.9% of patients experienced unintended interruptions of stimulation. Subgroup analysis only showed a significant impact on overall convenience for different models of stimulators (p < 0.05). Overall, SCS patients feel confident handling a r-IPG at high rates of convenience and acceptable effort despite high rates of usage-related complications. Further technical improvements for r-IPGs are needed.
Assuntos
Dor Crônica , Estimulação Encefálica Profunda , Estimulação da Medula Espinal , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Eletrodos Implantados , Estudos Retrospectivos , Dor Crônica/terapia , Medula Espinal/cirurgiaRESUMO
Spinal epidural abscess (SEA) with pyogenic vertebral osteomyelitis (PVO) is a rare illness with a steadily increasing incidence. However, comparative analyses of young and older patients with SEA are lacking. We aimed to compare the clinical course of patients aged 18-64 years, 65-79 years, and ≥ 80 years undergoing surgery for SEA. Clinical and imaging data were retrospectively collected from the institutional database between September 2005 and December 2021. Ninety-nine patients aged 18-64 years, 45 patients aged 65-79 years, and 32 patients ≥ 80 years were enrolled. Patients ≥ 80 years presented with a poorer baseline history (9.2 ± 2.4), as indicated by the CCI, than their younger counterparts (18-74 years: 4.8 ± 1.6;6.5 ± 2.5; p < 0.001). Patients aged 65-79 years and 80 years had a significantly longer length of stay. In-hospital mortality was significantly higher in those aged ≥ 80 years compared to their younger counterparts (≥ 80 years, n = 3, 9.4% vs. 18-64 years, n = 0, 0.0%; 65-79 years, n = 0, 0.0%; p < 0.001), while no differences in 90-day mortality or 30-day readmission were observed. After surgery, a significant decrease in C-reactive protein levels and leukocytes and amelioration of motor scores were observed in all the groups. Of note, older age (> 65 years), presence of comorbidities, and poor preoperative neurological condition were significant predictors of mortality. Surgical management led to significant improvements in laboratory and clinical parameters in all age groups. However, older patients are prone to multiple risks, requiring meticulous evaluation before surgery. Nevertheless, the risk profile of younger patients should not be underestimated. The study has the limitations of a retrospective design and small sample size. Larger randomized studies are warranted to establish the guidelines for the optimal management of patients from every age group and to identify the patients who can benefit from solely conservative management.
Assuntos
Abscesso Epidural , Doenças do Sistema Nervoso , Osteomielite , Humanos , Abscesso Epidural/cirurgia , Abscesso Epidural/complicações , Abscesso Epidural/tratamento farmacológico , Estudos Retrospectivos , SeguimentosRESUMO
This study aimed to compare and assess clinical outcomes of spinal metastasis with epidural spinal cord compression (MESCC) in patients aged 65-79 years and ≥ 80 years with an acute onset of neurological illness who underwent laminectomy. A second goal was to determine morbidity rates and potential risk factors for mortality. This retrospective review of electronic medical records at a single institution was conducted between September 2005 and December 2020. Data on patient demographics, surgical characteristics, complications, hospital clinical course, and 90-day mortality were also collected. Comorbidities were assessed using the age-adjusted Charlson comorbidity index (CCI). A total of 99 patients with an overall mean age of 76.2 ± 3.4 years diagnosed with MESCC within a 16-year period, of which 65 patients aged 65-79 years and 34 patients aged 80 years and older were enrolled in the study. Patients aged 80 and over had higher age-adjusted CCI (9.2 ± 2.1) compared to those aged 65-79 (5.1 ± 1.6; p < 0.001). Prostate cancer was the primary cause of spinal metastasis. Significant neurological and functional decline was more pronounced in the older group, evidenced by Karnofsky Performance Index (KPI) scores (80+ years: 47.8% ± 19.5; 65-79 years: 69.0% ± 23.9; p < 0.001). Despite requiring shorter decompression duration (148.8 ± 62.5 min vs. 199.4 ± 78.9 min; p = 0.004), the older group had more spinal levels needing decompression. Median survival time was 14.1 ± 4.3 months. Mortality risk factors included deteriorating functional status and comorbidities, but not motor weakness, surgical duration, extension of surgery, hospital or ICU stay, or complications. Overcoming age barriers in elderly surgical treatment in MSCC patients can reduce procedural delays and has the potential to significantly improve patient functionality. It emphasizes that age should not be a deterrent for spine surgery when medically necessary, although older MESCC patients may have reduced survival.
Assuntos
Compressão da Medula Espinal , Neoplasias da Coluna Vertebral , Idoso , Masculino , Humanos , Idoso de 80 Anos ou mais , Seguimentos , Neoplasias da Coluna Vertebral/cirurgia , Procedimentos Neurocirúrgicos , Laminectomia , Avaliação de Estado de KarnofskyRESUMO
In aqueduct stenosis, pressure difference below and above level of obstruction leads to bulging of third ventricular floor (TVF) and lamina terminalis (LT). Endoscopic third ventriculocisternostomy (ETV) is the standard treatment in these patients. We tried to assess success of ETV depending on those two radiological changes in aqueduct stenosis. We implemented "Heidelberg ETV score" retrospectively to assess the state of TVF as well as LT in same manner in midsagittal MR image. Every patient had a preoperative, direct, 3-months and one-year postoperative score from -2 to + 2. We correlated the scores to clinical course to decide whether the score is reliable in defining success of ETV. Between 2017-2021, 67 (mean age 25.6 ± 23.9y) patients treated with ETV were included. Success rate of primary and Re-ETVs was 91% over 46.8 ± 19.0 months. A marked shift of score to the left after surgery in success group was noticed through the distribution of score immediate postoperative, 3-months later; 70.2% showed (+ 2) before surgery, 38.9% scored (0) after surgery and 50.9% showed further score drop to (-1) 3 months later, p < 0.001. In cases of failure, there was initial decrease after surgery followed by increase with ETV-failure (mean time to failure: 7.2 ± 5.7 months) in 100%. Significant difference was noticed in Heidelberg score at postoperative 1-year- and failure-MRI follow-up between two groups, p < 0.001. Heidelberg score describes anatomical changes in third ventricle after ETV and can serve in assessment of MR images to define success of the procedure in patients with aqueduct stenosis.
Assuntos
Hidrocefalia , Ventriculostomia , Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Constrição Patológica , Estudos Retrospectivos , Endoscopia , Hidrocefalia/cirurgiaRESUMO
OBJECTIVE: The prevalence of degenerative disorders of the spine, such as cervical spinal stenosis with cervical spine myelopathy (CSM) in the geriatric population, has rapidly increased worldwide. To date, there has been no systematic analysis comparing outcomes in older patients suffering from progressive CSM and undergoing surgery depending on their health insurance status. We sought to compare the clinical outcomes and complications after anterior cervical discectomy and fusion (ACDF) or posterior decompression with fusion in patients aged ≥ 65 years with multilevel cervical spinal canal stenosis and concomitant CSM with special focus on their insurance status. METHODS: Clinical and imaging data were retrieved from patients' electronic medical records at a single institution between September 2005 and December 2021. Patients were allocated into two groups with respect to their health insurance status: statutory health insurance (SHI) vs. private insurance (PI). RESULTS: A total of 236 patients were included in the SHI group and 100 patients in the privately insured group (PI) group. The overall mean age was 71.7 ± 5.2 years. Regarding comorbidities, as defined with the age-adjusted CCI, SHI patients presented with higher rates of comorbidities as defined by a CCI of 6.7 ± 2.3 and higher prevalence of previous malignancies (9.3%) when compared to the PI group (CCI 5.4 ± 2.5, p = 0.051; 7.0%, p = 0.048). Both groups underwent ACDF (SHI: 58.5% vs. PI: 61.4%; p = 0.618), and the surgical duration was similar between both groups. Concerning the intraoperative blood transfusion rates, no significant differences were observed. The hospital stay (12.5 ± 1.1 days vs. 8.6 ± 6.3 days; p = 0.042) and intenisve care unit stay (1.5 ± 0.2 days vs. 0.4 ± 0.1 days; p = 0.049) were significantly longer in the PI group than in the SHI group. Similar in-hospital and 90-day mortality rates were noted across the groups. The presence of comorbidities, as defined with the age-adjusted CCI, poor neurological status at baseline, and SHI status, was significant predictor for the presence of adverse events, while the type of surgical technique, operated levels, duration of surgery, or blood loss was not. CONCLUSIONS: Herein, we found that surgeons make decisions independent of health insurance status and aim to provide the most optimal therapeutic option for each individual; hence, outcomes were similar between the groups. However, longer hospitalization stays were present in privately insured patients, while SHI patients presented on admission with poorer baseline status.
Assuntos
Doenças da Medula Espinal , Fusão Vertebral , Estenose Espinal , Humanos , Idoso , Estudos de Coortes , Estenose Espinal/epidemiologia , Estenose Espinal/cirurgia , Resultado do Tratamento , Fusão Vertebral/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/cirurgia , Discotomia/métodos , Doenças da Medula Espinal/cirurgia , Vértebras Cervicais/cirurgia , Cobertura do Seguro , Alemanha/epidemiologia , Estudos RetrospectivosRESUMO
PURPOSE: Spontaneous spinal epidural hematoma (SSEH) is a rare but disabling disease. Although several cases have been reported in the literature, their treatment remains unclear, especially in patients with advanced age. We, therefore, aimed to describe the clinical outcomes of cervical SSEH in octogenarians with an acute onset of neurological illness undergoing laminectomy. METHODS: Electronic medical records from a single institution between September 2005 and December 2020 were retrieved. Data on patient demographics, neurological conditions, functional status, surgical characteristics, complications, hospital course, and 90-day mortality were also collected. RESULTS: Twenty-two patients aged ≥ 80 years with SSEH undergoing laminectomy were enrolled in this study. The mean Charlson comorbidity index was 9.1 ± 2.0, indicating a poor baseline reserve. Ten individuals (45.5%) were taking anticoagulant agents with a pathologic partial thromboplastin time (PTT) of 46.5 ± 3.4 s. Progressive neurological decline, as defined by the motor score (MS), was observed on admission (63.8 ± 14.0). The in-hospital and 90-day mortality were 4.5% and 9.1%, respectively. Notably, the MS (93.6 ± 8.3) improved significantly after surgery (p < 0.05). Revision surgery was necessary in 5 cases due to recurrent hematoma. Anticoagulant agents and pathological PTT are significant risk factors for its occurrence. Motor weakness and comorbidities were unique risk factors for loss of ambulation. CONCLUSIONS: Laminectomy and evacuation of the hematoma in octogenarians with progressive neurological decline induce clinical benefits. Emergent surgery seems to be the "state of the art" treatment for SSEH. However, potential complications associated with adverse prognostic factors, such as the use of anticoagulants, should be considered.