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PURPOSE: Belzutifan is a Hypoxia Inducible Factor 2-alpha inhibitor approved in 2021 by the FDA for the treatment of renal cell carcinoma (RCC) in patients with Von-Hippel Landau (VHL) disease. These patients can also present with central nervous system (CNS) hemangioblastomas (HBs). We aim to study the effectiveness and adverse effects of belzutifan for CNS HBs, by reporting our preliminary institutional experience. METHODS: We present a series of VHL patients with CNS HBs undergoing treatment with belzutifan for RCC. All the included patients met the RECIST inclusion criteria. The clinical and radiological outcome measures included: Objective response rate (ORR), time-to-response (TTR), adverse events (AE), and patient response. Patient response was classified as partial response (PR), complete response (CR), progressive disease (PD), or stable disease (SD). RESULTS: Seven patients with 25 HBs were included in our study. A belzutifan dose of 120 mg/day PO was administered for a median of 13 months (range 10-17). Median follow up time was 15 months (range 10-24). An ORR of 71% was observed. The median TTR was 5 months (range: 1-10). None of the patients showed CR, while 5 patients (71.4%) showed PR and 2 (28.5%) showed SD. Among patients with SD the maximum tumor response was 20% [increase/decrease] of the lesion diameter. All the patients experienced decreased hemoglobin concentration, fatigue, and dizziness. None of the patients experienced severe anemia (grade 3-4 CTCAE). CONCLUSION: Belzutifan appears to be an effective and safe treatment for CNS hemangioblastoma in VHL patients. Further clinical trials to assess the long-term effectiveness of the medication are required.
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Carcinoma de Células Renais , Neoplasias do Sistema Nervoso Central , Hemangioblastoma , Neoplasias Renais , Doença de von Hippel-Lindau , Humanos , Hemangioblastoma/tratamento farmacológico , Hemangioblastoma/patologia , Estudos Retrospectivos , Doença de von Hippel-Lindau/complicações , Doença de von Hippel-Lindau/tratamento farmacológico , Doença de von Hippel-Lindau/patologia , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Sistema Nervoso Central/patologia , Proteína Supressora de Tumor Von Hippel-LindauRESUMO
INTRODUCTION: Patchy alopecia is a common adverse effect of stereotactic radiosurgery (SRS) on the calvarium and/or scalp, yet no guidelines exist for its prevention. This study aims to investigate the incidence and outcomes of patchy alopecia following SRS for patients with calvarial or scalp lesions and establish preventive guidelines. METHODS: The study included 20 patients who underwent CyberKnife SRS for calvarial or scalp lesions, resulting in a total of 30 treated lesions. SRS was administered as a single fraction for 8 lesions and hypofractionated for 22 lesions. The median SRS target volume was 9.85 cc (range: 0.81-110.7 cc), and the median prescription dose was 27 Gy (range: 16-40 Gy), delivered in 1-5 fractions (median: 3). The median follow-up was 15 months. RESULTS: Among the 30 treated lesions, 11 led to patchy alopecia, while 19 did not. All cases of alopecia resolved within 12 months, and no patients experienced other adverse radiation effects. Lesions resulting in alopecia exhibited significantly higher biologically effective dose (BED) and single-fraction equivalent dose (SFED) on the overlying scalp compared to those without alopecia. Patients with BED and SFED exceeding 60 Gy and 20 Gy, respectively, were 9.3 times more likely to experience patchy alopecia than those with lower doses. The 1-year local tumor control rate for the treated lesions was 93.3%. Chemotherapy was administered for 26 lesions, with 11 lesions receiving radiosensitizing agents. However, no statistically significant difference was found. CONCLUSION: In summary, SRS is a safe and effective treatment for patients with calvarial/scalp masses regarding patchy alopecia near the treated area. Limiting the BED under 60 Gy and SFED under 20 Gy for the overlying scalp can help prevent patchy alopecia during SRS treatment of the calvarial/scalp mass. Clinicians can use this information to inform patients about the risk of alopecia and the contributing factors.
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OBJECTIVE: Brain metastases (BMs) secondary to sarcoma are rare, and their incidence ranges from 1% to 8% of all bone and soft tissue sarcomas. Although stereotactic radiosurgery (SRS) is widely used for BMs, only a few papers have reported on SRS for sarcoma metastasizing to the brain. The purpose of this study was to evaluate the safety and effectiveness of SRS for sarcoma BM. METHODS: The authors retrospectively reviewed the clinical and radiological outcomes of patients with BM secondary to histopathologically confirmed sarcoma treated with SRS, either as primary treatment or as adjuvant therapy after surgery, at their institution between January 2005 and September 2022. They also compared the outcomes of patients with hemorrhagic lesions and of those without. RESULTS: Twenty-three patients (9 females) with 150 BMs secondary to sarcoma were treated with CyberKnife SRS. Median age at the time of treatment was 48.22 years (range 4-76 years). The most common primary tumor sites were the heart, lungs, uterus, upper extremities, chest wall, and head and neck. The median Karnofsky Performance Status on presentation was 73.28 (range 40-100). Eight patients underwent SRS as a primary treatment and 15 as adjuvant therapy to the resection cavity. The median tumor volume was 24.1 cm3 (range 0.1-150.3 cm3), the median marginal dose was 24 Gy (range 18-30 Gy) delivered in a median of 1 fraction (range 1-5) to a median isodose line of 76%. The median follow-up was 8 months (range 2-40 months). Median progression-free survival and overall survival were 5.3 months (range 0.4-32 months) and 8.2 months (range 0.1-40), respectively. The 3-, 6-, and 12-month local tumor control (LTC) rates for all lesions were respectively 78%, 52%, and 30%. There were no radiation-induced adverse effects. LTC at the 3-, 6-, and 12-month follow-ups was better in patients without hemorrhagic lesions (100%, 70%, and 40%, respectively) than in those with hemorrhagic lesions (68%, 38%, and 23%, respectively). CONCLUSIONS: SRS, both as a primary treatment and as adjuvant therapy to the resection cavity after surgery, is a safe and relatively effective treatment modality for sarcoma BMs. Nonhemorrhagic lesions show better LTC than hemorrhagic lesions. Larger studies aiming to validate these results are encouraged.
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Neoplasias Encefálicas , Radiocirurgia , Sarcoma , Feminino , Humanos , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Radiocirurgia/métodos , Estudos Retrospectivos , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Resultado do Tratamento , Sarcoma/radioterapia , Sarcoma/cirurgia , Encéfalo/patologiaRESUMO
OBJECTIVE: Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare, malignant tumor of neuroectodermal origin that arises from the olfactory neuroepithelium. In this study the authors present the first series in the literature on distant brain metastases (BMs) secondary to ENB that were treated with stereotactic radiosurgery (SRS), to evaluate the safety and effectiveness of SRS for this indication. METHODS: A retrospective analysis of clinical and radiological outcomes of patients with ENB who underwent CyberKnife (CK) SRS at a single center was conducted. The clinical and radiological outcomes of patients, including progression-free survival, overall survival, and local tumor control (LTC) were reported. RESULTS: Between 2003 and 2022, 32 distant BMs in 8 patients were treated with CK SRS at Stanford University. The median patient age at BM diagnosis was 62 years (range 47-75 years). Among 32 lesions, 2 (6%) had previously been treated with surgery, whereas for all other lesions (30 [94%]), CK SRS was used as their primary treatment modality. The median target volume was 1.5 cm3 (range 0.09-21.54 cm3). CK SRS was delivered by a median marginal dose of 23 Gy (range 15-30 Gy) and a median of 3 fractions (range 1-5 fractions) to a median isodose line of 77% (range 70%-88%). The median biologically effective dose was 48 Gy (range 21-99.9 Gy) and the median follow-up was 30 months (range 3-95 months). The LTC at 1-, 2-, and 3-year follow-up was 86%, 65%, and 50%, respectively. The median progression-free survival and overall survival were 29 months (range 11-79 months) and 51 months (range 15-79 months), respectively. None of the patients presented adverse radiation effects. CONCLUSIONS: In the authors' experience, SRS provided excellent LTC without any adverse radiation effects for BMs secondary to ENB.
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Neoplasias Encefálicas , Estesioneuroblastoma Olfatório , Neoplasias Nasais , Radiocirurgia , Humanos , Pessoa de Meia-Idade , Idoso , Radiocirurgia/efeitos adversos , Estesioneuroblastoma Olfatório/radioterapia , Estesioneuroblastoma Olfatório/cirurgia , Estesioneuroblastoma Olfatório/etiologia , Estudos Retrospectivos , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Cavidade Nasal/cirurgia , Neoplasias Nasais/radioterapia , Neoplasias Nasais/cirurgia , Neoplasias Nasais/etiologia , Resultado do TratamentoRESUMO
We describe a COVID-19 patient who presented with persistent headache and anosmia that was related to viral encephalomyelitis with acute lesions on MRI in both the brain and upper cervical cord.
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COVID-19 , Encefalomielite , Humanos , COVID-19/complicações , COVID-19/patologia , Encefalomielite/diagnóstico por imagem , Encefalomielite/patologia , Encéfalo/patologia , Medula Espinal/patologia , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: People with spinal cord injury (SCI) frequently develop neuropathic pain (NP) that worsens disability and diminishes rehabilitation efficacy. Chronic NP is presently incurable due to poor understanding of underlying mechanisms. We hypothesized that multilocus neuroinflammation (NIF) might be a driver of SCI NP, and tested it by investigating whether NP coexisted with central NIF, neurotransmission (NTM), neuromodulation (NML) and neuroplasticity (NPL) changes post-SCI. METHODS: Female Sprague-Dawley rats (230-250 g) with T10 compression or laminectomy were evaluated for physical conditions, coordinated hindlimb functions, neurological reflexes, and mechanical/thermal sensitivity thresholds at 1 day post-injury (p.i.) and weekly thereafter. Eight weeks p.i., central nervous system tissues were histochemically and immunohistochemically characterized for parameters/markers of histopathology and NIF/NTM/NML/NPL. Also analyzed was the correlative relationship between levels of selected biomarkers and thermosensitivity thresholds via statistical linear regression. RESULTS: SCI impaired sensorimotor functions, altered reflexes, and produced spontaneous pain signs and hypersensitivity to evoked nociceptive, mechanical, and thermal inputs. Only injured spinal cords exhibited neural lesion, microglia/astrocyte activation, and abnormal expression of proinflammatory cytokines, as well as NIF/NTM/NML/NPL markers. Brains of SCI animals displayed similar pathophysiological signs in the gracile and parabrachial nuclei (GrN and PBN: sensory relay), raphe magnus nucleus and periaqueduct gray (RMN and PAG: pain modulation), basolateral amygdala (BLA: emotional-affective dimension of pain), and hippocampus (HPC: memory/mood/neurogenesis). SCI augmented sensory NTM/NPL (GrN and PBN); increased GAD67 (PAG) level; reduced serotonin (RMN) and fear-off neuronal NTR2 (BLA) expressions; and perturbed neurogenesis (HPC). CONCLUSION: T10 compression caused chronic hyperalgesia that coexisted with NIF/NTM/NML/NPL responses at multilevel neuroaxis centers. The data have provided multidimensional biomarkers as new mechanistic leads to profile SCI NP for therapeutic/therapy development.
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Neuralgia , Traumatismos da Medula Espinal , Ratos , Animais , Feminino , Hiperalgesia/metabolismo , Ratos Sprague-Dawley , Neuralgia/metabolismo , Traumatismos da Medula Espinal/patologia , Inflamação/complicações , BiomarcadoresRESUMO
BACKGROUND: On October 23, 2011, a devastating earthquake, magnitude 7.2 on the Richter Scale, jolted the province of Van in the east of Turkey and led to hundreds of casualties. OBJECTIVES: In this study, we aimed to present our clinical experience in the management of patients with cranial and spinal injuries who were admitted to the Van Regional Training and Research Hospital. METHODS: The retrospective study included 44 (77.2%) patients who were referred to the neurosurgery department after being diagnosed with spinal and cranial injuries due to earthquake at the emergency department between October 23 and 27, 2011. RESULTS: The patients comprised 32 male (72.7%) and 12 (27.3%) female patients with a mean age of 23.5 years. The injuries included scalp injury (n = 16), burst fracture (n=7), compression fracture (n=3), epidural hematoma (n=9), subdural hematoma (n=3), contusion (n=1), traumatic subarachnoid hemorrhage (n=2), depressed skull fracture (n=3), linear fracture (n=9), cervical fracture (n=2), and pneumocephalus (n=1). Most of the patients (90.9%) had isolated injuries and the others (9.1%) presented with combined cranial and spinal injuries. At discharge, the 3 patients with spinal fractures were paraplegic, and of the 2 patients who were operatively treated due to subdural hematoma, 1 was hemiparesic and the other was hemiplegic. No mortality occurred in our patients. CONCLUSIONS: The results of this study demonstrated that, in the aftermath of a natural disaster, conducting correct triage procedures and performing a prompt intervention with appropriate and qualified equipment play key roles in reducing morbidity and mortality.
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Traumatismos Craniocerebrais/epidemiologia , Terremotos , Serviço Hospitalar de Emergência/estatística & dados numéricos , Traumatismos da Coluna Vertebral/epidemiologia , Adolescente , Adulto , Idoso , Criança , Traumatismos Craniocerebrais/etiologia , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/estatística & dados numéricos , Estudos Retrospectivos , Fraturas da Coluna Vertebral/epidemiologia , Turquia/epidemiologia , Adulto JovemRESUMO
OBJECTIVE: The aim of this study was to examine the distribution of olfactory filaments (OFs) in the nasal mucosa to facilitate preservation of olfactory function in endonasal approaches and preparation of a nasoseptal flap. METHODS: One formalin-fixed and 9 fresh cadaveric silicone-injected specimens with 20 total sides were studied to measure the distance of the OFs to the anatomical landmarks and compare the OF presence in the nasal septum mucosa (NSM) and ethmoidal mucosa (EM). RESULTS: The mean distance from the first to the last OF was 19.37 ± 2.16 mm in the NSM and 23.44 ± 5.42 mm in the EM. The NSM had a mean of 7.55 ± 1.31 OFs and the EM had 14.3 ± 1.78. Average OF lengths were measured at 6.44 ± 1.48 (range 3.75-12.40) mm in the NSM and 8.05 ± 1.76 (range 4.14-13.20) mm in the EM. The mean values of the EM measurements were compared with those of the NSM; the number of OFs, the distance between the first and last OF, the average OF length, and the number of OFs between anterior and posterior ethmoidal arteries in the NSM were significantly less (p < 0.05) than those in the EM. The distance between the first OF to the nasal bone on the NSM was greater than on the EM. CONCLUSIONS: Compared with the EM, the OFs are significantly fewer in number and smaller in size in the NSM. The uppermost edge of the nasoseptal flap incision in the NSM might be safer to start below 12 mm from the cribriform plate for OF protection.
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Cadáver , Microcirurgia , Mucosa Nasal , Septo Nasal , Humanos , Mucosa Nasal/anatomia & histologia , Septo Nasal/cirurgia , Septo Nasal/anatomia & histologia , Microcirurgia/métodos , Retalhos CirúrgicosRESUMO
BACKGROUND: Vestibular schwannomas (VSs) are benign tumors often monitored over time, with measurement techniques for assessing growth rates subject to significant interobserver variability. Automatic segmentation of these tumors could provide a more reliable and efficient for tracking their progression, especially given the irregular shape and growth patterns of VS. METHODS: Various studies and segmentation techniques employing different Convolutional Neural Network architectures and models, such as U-Net and convolutional-attention transformer segmentation, were analyzed. Models were evaluated based on their performance across diverse datasets, and challenges, including domain shift and data sharing, were scrutinized. RESULTS: Automatic segmentation methods offer a promising alternative to conventional measurement techniques, offering potential benefits in precision and efficiency. However, these methods are not without challenges, notably the "domain shift" that occurs when models trained on specific datasets underperform when applied to different datasets. Techniques such as domain adaptation, domain generalization, and data diversity were discussed as potential solutions. CONCLUSIONS: Accurate measurement of VS growth is a complex process, with volumetric analysis currently appearing more reliable than linear measurements. Automatic segmentation, despite its challenges, offers a promising avenue for future investigation. Robust well-generalized models could potentially improve the efficiency of tracking tumor growth, thereby augmenting clinical decision-making. Further work needs to be done to develop more robust models, address the domain shift, and enable secure data sharing for wider applicability.
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Redes Neurais de Computação , Neuroma Acústico , Humanos , Processamento de Imagem Assistida por Computador/métodos , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/patologiaRESUMO
OBJECTIVE: Leiomyosarcoma (LMS) is a rare, aggressive soft-tissue sarcoma that seldom spreads to the bone. The spine can be either the site of LMS osseous metastases or the primary tumor site. The optimal treatment option for spinal LMS is still unclear. The authors present a cohort of patients with spinal LMS treated with either upfront surgery or upfront CyberKnife stereotactic radiosurgery (SRS). METHODS: The authors retrospectively studied the clinical and radiological outcomes of 17 patients with spinal LMS treated at their institution between 2004 and 2020. Either surgery or SRS was used as the upfront treatment. The clinical and radiological outcomes were assessed. A systematic review of the literature was also conducted. RESULTS: Of the 17 patients (20 spinal lesions), 12 (70.6%) were female. The median patient age was 61 years (range 41-80 years). Ten patients had upfront surgery for their spinal lesions, and 7 had upfront CyberKnife radiosurgery. The median follow-up was 11 months (range 0.3-130 months). The median overall survival (OS) for the entire cohort was 13 months (range 0.3-97 months). In subgroup analysis, the median OS was lower for the surgical group (13 months, range 0.3-50 months), while the median OS for the SRS group was 15 months (range 5-97 months) (p = 0.5). Forty percent (n = 4) of those treated with surgery presented with local recurrence at a median of 6.7 months (range 0.3-36 months), while only 14% (n = 1) of those treated with CyberKnife radiosurgery had local recurrence after 5 months. Local tumor control (LTC) rates at the 6-, 12-, and 18-month follow-ups were 72%, 58%, and 43%, respectively, for the SRS group and 40%, 30%, and 20%, respectively, for the surgery group (p < 0.05). The literature review included 35 papers with 70 patients harboring spinal LMS; only 2 patients were treated with SRS. The literature review confirms the clinical and radiological outcomes of the surgical group, while data on SRS are anecdotal. CONCLUSIONS: The authors present the largest series in the literature of spinal LMS and the first on SRS for spinal LMS. This study shows that LTC is statistically significantly better in patients receiving upfront SRS instead of surgery. The OS does not appear different between the two groups.
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Leiomiossarcoma , Radiocirurgia , Neoplasias da Coluna Vertebral , Humanos , Leiomiossarcoma/cirurgia , Leiomiossarcoma/patologia , Radiocirurgia/métodos , Pessoa de Meia-Idade , Idoso , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/radioterapia , Feminino , Adulto , Estudos Retrospectivos , Masculino , Idoso de 80 Anos ou mais , Resultado do TratamentoRESUMO
Background: Glioblastoma (GBM) is the most common primary malignant brain tumor. The aim of this study was to elucidate the role of microenvironment and intrinsic T-type calcium channels (Cav3) in regulating tumor growth and progression. Methods: We grafted syngeneic GBM cells into Cav3.2 knockout mice to assess the role of microenvironment T-Type calcium channels on GBM tumor growth. We performed single-cell RNA-seq (scRNA-seq) of tumors from WT and Cav3.2 KO mice to elucidate the regulation of tumors by the microenvironment. We used neurons from WT and Cav3.2 KO mice in co-culture with GBM stem cells (GSC) to assess the effects of Cav3.2 on neuron/GSC synaptic connections and tumor cell growth. Results: Cav3.2 KO in the microenvironment led to significant reduction of GBM growth and prolongation of animal survival. scRNA-seq showed that microenvironment Cav3.2 regulates neuronal and glial biological processes. Microenvironment Cav3.2 downregulated numerous genes associated with regulating the OPC cell state in GBM tumors such as SOX10 and Olig2. Neuronal Cav3.2 promoted neuron/GSC synaptic connections and GSC growth. Treatment of GSCs with the Cav3 blocker mibefradil downregulated genes associated with neuronal processes. The Cav3 blocker drug mibefradil synergized with temozolomide (TMZ) and radiation to reduce in vivo tumor growth and prolong animal survival. Conclusions: Together these data reveal a role for microenvironment Cav3 in promoting GBM tumor progression through regulating neuronal and glial processes particularly associated with the OPC-cell state. Targeting both intrinsic and microenvironment Cav3 with the inhibitor mibefradil significantly enhanced the anti-GBM effects of TMZ and radiation.
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Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant tumor of neuroectodermal origin that arises from the olfactory epithelium. We present a case of ENB metastasizing through the leptomeningeal route to the spinal dura, which was treated with CyberKnife (CK) stereotactic radiosurgery (SRS), and aim to assess the safety and effectiveness of SRS in such cases. To the best of our knowledge, this is the first case report in the literature that discusses ENB spinal leptomeningeal metastases treated with CK radiosurgery. We retrospectively review the clinical and radiological outcomes in a 70-year-old female with ENB metastasis to the spine. Progression-free survival (PFS), overall survival (OS), and local tumor control (LTC) are investigated. In our patient, ENB had been diagnosed at the age of 58 years and spinal metastases had been first noted at the age of 65 years. A total of six spinal lesions received CK SRS. Lesions were present at the level of C1, C2, C3, C6-C7, T5, and T10-11. The median target volume was 0.72 cc (range: 0.32-2.54). A median marginal dose of 24 Gy was delivered to the tumors with a median of three fractions to a median isodose line of 80% (range: 78-81). LTC at the 24-month follow-up was 100%. PFS and OS were 27 months and 40 months, respectively. No adverse radiation effects were reported. Even though the treated spinal lesions remained stable, the number of new metastatic lesions had increased with progressive osseous and dural metastatic lesions within the cervical, thoracic, and lumbar spine at the last follow-up. SRS provides relatively good LTC for patients with ENB metastasizing to the spine, with no radiation-induced adverse events.
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BACKGROUND: Choroid plexus papillomas (CPPs) are rare intraventricular brain tumors derived from the epithelium of the choroid plexus. Gross total resection has traditionally been considered curative, but residual tumor or recurrence remains possible. Stereotactic radiosurgery (SRS) has become a more important strategy for subtotally resected and recurrent tumors. The evidence-based rationale of SRS treatment for residual or recurrent CPP in adult patients is still lacking because of the rarity of the disease. METHODS: We retrospectively reviewed the cases of histopathologically confirmed patients with residual or recurrent CPP treated with SRS at our institute in the adult population between 2005 and 2022. Three patients, with 5 lesions, were identified, with a median age of 63 years. Patients presented initially with hydrocephalus-related symptoms, although ventriculomegaly was noted in only 1 patient radiographically. The tumor location was most commonly in the fourth ventricle or along the foramen of Luschka. Treatment was delivered in a single fraction in 4 lesions and in 3 fractions in 1 patient. The median follow-up was 26 months. RESULTS: The local tumor control rate for the lesions was 80%. One patient developed a new lesion outside the SRS field, and 1 lesion developed progression without the need for subsequent treatment. There was no significant shrinkage of the lesions radiographically. None of the patients showed radiation-related adverse events. No patients required surgical management after SRS treatment at our institution. Based on the literature review, our case series was the second largest retrospective series from a single institution on SRS for recurrent or residual CPP. CONCLUSIONS: SRS for patients with recurrent or residual CPP was a safe and effective treatment modality in this case series. Larger studies are encouraged to validate the role of SRS in the treatment of recurrent or residual CPP.
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BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans histiocytes in various parts of the body and capable of leading to organ damage and tumor formation. Reports of cranial LCH in the adult population are extremely rare. Although surgery remains the preferred option for localized LCH lesions, the role of stereotactic radiosurgery (SRS) is emerging. OBJECTIVE: To retrospectively review a rare case series to determine the safety and effectiveness of SRS for patients with localized cranial LCH. METHODS: We retrospectively reviewed histopathologically confirmed cases of localized cranial LCH treated with SRS at our institute in the adult population between January 2005 and September 2022. Five patients were identified with a median age of 34 years (19-54 years). The tumor location was in the pituitary stalk in 3 patients, the orbit in one patient, and the parietal skull in one patient. The median target volume was 2.8 cc (range: 0.37-6.11). Treatment was delivered in a single fraction in 4 patients (median margin dose of 8 Gy, range: 7-10 Gy) and in 3 fractions (22.5 Gy) in 1 patient. The median follow-up was 12 years (range: 4-17). None of the patients required craniotomy for tumor debulking before or after SRS. RESULTS: The local tumor control rate for the lesions was 100%. All 3 patients with LCH in the pituitary stalk had diabetes insipidus at the initial presentation and developed panhypopituitarism after SRS. Diabetes insipidus was not improved after SRS. The other 2 patients presented no adverse radiation effects. Based on the literature review, our case series was the largest retrospective series on SRS for localized cranial LCH, with the longest median follow-up. CONCLUSIONS: SRS for patients with localized cranial LCH was a safe and effective treatment modality in this case series. Larger studies are encouraged to validate the role of SRS in the treatment of localized cranial LCH.
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Diabetes Insípido , Diabetes Mellitus , Histiocitose de Células de Langerhans , Radiocirurgia , Adulto , Humanos , Estudos Retrospectivos , Radiocirurgia/efeitos adversos , Resultado do Tratamento , Diabetes Insípido/epidemiologia , Diabetes Insípido/etiologia , Crânio/patologia , Histiocitose de Células de Langerhans/radioterapia , Histiocitose de Células de Langerhans/cirurgia , Histiocitose de Células de Langerhans/tratamento farmacológico , SeguimentosRESUMO
Introduction Glioblastoma (GBM) is the most common malignant adult brain tumor and is invariably fatal. The standard treatment for GBM involves resection where possible, followed by chemoradiation per Stupp's protocol. We frequently use stereotactic radiosurgery (SRS) as a single-fraction treatment for small (volume ≤ 1cc) nodular recurrent GBM to the contrast-enhancing target on T1 MRI scan. In this paper, we aimed to evaluate the safety and efficacy of SRS for patients with contrast-enhancing satellite nodules in recurrent GBM. Methods This retrospective study analyzed the clinical and radiological outcomes of five patients who underwent CyberKnife (Accuray Inc., Sunnyvale, California) SRS at the institute between 2013 and 2022. Results From 96 patients receiving SRS for GBM, five (four males, one female; median age 53) had nine distinct new satellite lesions on MRI, separate from their primary tumor beds. Those nine lesions were treated with a median margin dose of 20 Gy in a single fraction. The three-, six, and 12-month local tumor control rates were 77.8%, 66.7%, and 26.7%, respectively. Median progression-free survival (PFS) was seven months, median overall survival following SRS was 10 months, and median overall survival (OS) was 35 months. Interestingly, the only lesion that did not show radiological progression was separate from the T2-fluid attenuated inversion recovery (FLAIR) signal of the main tumor. Conclusion Our SRS treatment outcomes for recurrent GBM satellite lesions are consistent with existing findings. However, in a unique case, a satellite nodule distinct from the primary tumor's T2-FLAIR signal and treated with an enlarged target volume showed promising control until the patient's demise. This observation suggests potential research avenues, given the limited strategies for 'multicentric' GBM lesions.
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Pigmented villonodular synovitis is a benign proliferative disease of the synovial structures. This disease is most commonly seen in the extremities, and spinal involvement is extremely rare. In the relevant literature, 53 cases have been reported. We present a 66-year-old female patient with a long history of back pain, which progressed and radiated to the left leg over a period of 2 months. The patient also had a mass in the lumbar region on the grounds of spinal degenerative changes. Following gross total excision, the diagnosis was established by histopathology. In this report, we present our experience and discuss our neuroradiological and clinical findings.
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Histiocitoma Fibroso Benigno/patologia , Histiocitoma Fibroso Benigno/cirurgia , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/cirurgia , Sinovite Pigmentada Vilonodular/patologia , Sinovite Pigmentada Vilonodular/cirurgia , Idoso , Dor nas Costas/patologia , Dor nas Costas/cirurgia , Biópsia , Descompressão Cirúrgica , Feminino , Humanos , Vértebras Lombares/patologia , Vértebras Lombares/cirurgia , Imageamento por Ressonância Magnética , Estenose Espinal/patologia , Estenose Espinal/cirurgiaRESUMO
Adolescent idiopathic scoliosis patients treated with spinal fusion may develop adjacent segment disease and curve progression into adulthood. Revision operations can be challenging, especially for adult patients treated with outdated instrumentation such as sublaminar hooks and/or wires. The authors demonstrate revision lumbar spine surgery in a 38-year-old female with scoliosis progression from junctional degeneration below a prior T5-L3 posterior instrumented arthrodesis with a hook-and-rod wire system. They also demonstrate safe application of an ultrasonic bone scalpel for completion of a Smith-Petersen osteotomy. The patient provided written, informed consent for all material presented in this case demonstration. The video can be found here: https://youtu.be/3PmaFtNcqKc.
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OBJECTIVE: Sacral insufficiency fracture after lumbosacral (LS) arthrodesis is an uncommon complication. The objective of this study was to report the authors' operative experience managing this complication, review pertinent literature, and propose a treatment algorithm. METHODS: The authors analyzed consecutive adult patients treated at their institution from 2009 to 2018. Patients who underwent surgery for sacral insufficiency fractures after posterior instrumented LS arthrodesis were included. PubMed was queried to identify relevant articles detailing management of this complication. RESULTS: Nine patients with a minimum 6-month follow-up were included (mean age 73 ± 6 years, BMI 30 ± 6 kg/m2, 56% women, mean follow-up 35 months, range 8-96 months). Six patients had osteopenia/osteoporosis (mean dual energy x-ray absorptiometry hip T-score -1.6 ± 0.5) and 3 received treatment. Index LS arthrodesis was performed for spinal stenosis (n = 6), proximal junctional kyphosis (n = 2), degenerative scoliosis (n = 1), and high-grade spondylolisthesis (n = 1). Presenting symptoms of back/leg pain (n = 9) or lower extremity weakness (n = 3) most commonly occurred within 4 weeks of index LS arthrodesis, which prompted CT for fracture diagnosis at a mean of 6 weeks postoperatively. All sacral fractures were adjacent or involved S1 screws and traversed the spinal canal (Denis zone III). H-, U-, or T-type sacral fracture morphology was identified in 7 patients. Most fractures (n = 8) were Roy-Camille type II (anterior displacement with kyphosis). All patients underwent lumbopelvic fixation via a posterior-only approach; mean operative duration and blood loss were 3.3 hours and 850 ml, respectively. Bilateral dual iliac screws were utilized in 8 patients. Back/leg pain and weakness improved postoperatively. Mean sacral fracture anterolisthesis and kyphotic angulation improved (from 8 mm/11° to 4 mm/5°, respectively) and all fractures were healed on radiographic follow-up (mean duration 29 months, range 8-90 months). Two patients underwent revision for rod fractures at 1 and 2 years postoperatively. A literature review found 17 studies describing 87 cases; potential risk factors were osteoporosis, longer fusions, high pelvic incidence (PI), and postoperative PI-to-lumbar lordosis (LL) mismatch. CONCLUSIONS: A high index of suspicion is needed to diagnose sacral insufficiency fracture after LS arthrodesis. A trial of conservative management is reasonable for select patients; potential surgical indications include refractory pain, neurological deficit, fracture nonunion with anterolisthesis or kyphotic angulation, L5-S1 pseudarthrosis, and spinopelvic malalignment. Lumbopelvic fixation with iliac screws may be effective salvage treatment to allow fracture healing and symptom improvement. High-risk patients may benefit from prophylactic lumbopelvic fixation at the time of index LS arthrodesis.
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Meningiomas are the most common benign intracranial neoplasms. Nearly 20% of all primary intracranial tumors are meningiomas. Primary intraosseous meningiomas are a subtype of the meningiomas that represents the most uncommon manifestation of meningiomas. Although rare, these tumors can be found to occur in unexpected areas of the head and neck. The patient was a 78- year-old male who was operated two times for urinary bladder cancer. During his routine oncology follow-ups, the PET scan demonstrated a hyperactive area in the right parietal bone. Preoperative diagnosis was a metastasis, but histological examination revealed an osteolytic interosseous meningioma. The possibility of an intraosseous meningioma mimicking a metastatic tumor should be kept in mind.