RESUMO
PURPOSE: To determine whether intraoperative adjunctive EVD placement in patients with a posterior fossa tumor (PFT) led to improved surgical, radiographic, and clinical outcomes compared to those who did not receive an EVD. METHODS: Patients were grouped as those who underwent routine intraoperative adjunctive EVD insertion and those who did not at time of PFT resection. Patients who pre-operatively required a clinically indicated EVD insertion were excluded. Comparative analyses between both groups were conducted to evaluate clinical, radiological, and pathological outcomes. Odds ratios (ORs) with corresponding 95% confidence intervals (CIs) were computed for post-operative outcomes. RESULTS: Fifty-five selected patients were included, 15 who had an EVD placed at the time of PFT resection surgery, and 40 who did not. Children without an EVD did not experience a higher rate of complications or poorer post-operative outcomes compared to those with an EVD placed during resection surgery. There was no significant difference in the degree of gross total resection (p = 0.129), post-operative CSF leak (p = 1.000), and post-operative hemorrhage (p = 0.554) between those with an EVD and those without. The frequency of new cranial nerve deficits post-operatively was higher in those with an EVD (40%) compared to those without (3%, p = 0.001). There was a trend towards more frequently observed post-operative hydrocephalus in the EVD group (p = 0.057). CONCLUSION: The routine use of EVD as an intraoperative adjunct in clinically stable pediatric patients with posterior fossa tumors and hydrocephalus may not be associated with improved radiological or clinical outcomes.
Assuntos
Neoplasias Encefálicas , Hidrocefalia , Neoplasias Infratentoriais , Humanos , Criança , Estudos Retrospectivos , Ventriculostomia/efeitos adversos , Complicações Pós-Operatórias/etiologia , Neoplasias Encefálicas/cirurgia , Neoplasias Infratentoriais/complicações , Neoplasias Infratentoriais/diagnóstico por imagem , Neoplasias Infratentoriais/cirurgia , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Drenagem/efeitos adversosRESUMO
BACKGROUND: Juvenile pilocytic astrocytoma (JPA) typically follows an indolent clinical course. The first-line treatment for most JPAs is surgical resection. However, a gross total resection may not be feasible for deep-seated lesions and/or infiltrative tumors, leading to multimodal treatment approaches that may be complicated by patient age and tumor location. Despite the prevalence of pediatric JPAs, there is no single approach to treating progressive disease. METHODS: We investigated the multifaceted management of progressive JPAs through a retrospective analysis of JPAs treated at a single center over an 18-year period (1998-2016). All cases were categorized according to location, whether supratentorial or infratentorial, and for each case we calculated the number of interventions and the time between interventions. RESULTS: We identified a total of 40 JPAs, (11 supratentorial, 29 infratentorial). Total number of interventions among all supratentorial JPA patients was 21 (average 2 interventions/patient). The total number of interventions among infratentorial JPAs was 40 (average 1.4 interventions/patient). CONCLUSIONS: Treatment of progressive JPA is variable and may require numerous surgeries and adjuvant therapies.
Assuntos
Astrocitoma , Neoplasias Encefálicas , Humanos , Criança , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Estudos Retrospectivos , Astrocitoma/cirurgia , Astrocitoma/patologiaRESUMO
BACKGROUND: Angiogenesis is a key process in the growth and maintenance of tumors. The Wnt signaling pathway is required for angiogenesis of the central nervous system though development of the blood-brain barrier and subsequent proliferation of endothelial cells during tumor growth. However, the specificity of the Wnt pathway in regulating endothelial cells of different central nervous systems remains to be investigated. MATERIALS & METHODS: Patient-derived tissue samples from 35 paraffin-embedded tumors were used to assess ß-catenin immunoexpression. Tumor samples consisted of the following pathologies: grade II diffuse astrocytoma, glioblastoma, hemangioblastoma, and metastatic adenocarcinoma (lung or breast primary). Average percent reactivity was recorded as a mean observed in ten high-power fields. The following scale was used to grade immunoreactivity: 0 = immunonegative, 1 = 1-25% reactive, 2 = 26-50% reactive, 3 = 51-75% reactive, 4 = 76-100% reactive. RESULTS: While we did not observe nuclear expression of ß-catenin in any samples, there was uniform cytoplasmic expression of ß-catenin within glial tumor cells. There was a clear distinction in tumor endothelial cells whereby diffuse staining was noted in areas of microvascular hyperplasia in GBM and a less immunoreactive profile in low-grade astrocytomas. By contrast, non-glial tumors, contained very minimal cytoplasmic ß-catenin expression in tumor and stromal cells and were devoid of immunoreactivity in endothelial cells. CONCLUSION: ß-catenin is unique marker of proliferating endothelial cells in GBM. Therapies targeting the spatial and structural heterogeneity inherent to GBM may prove to be efficacious and result in an improved survivorship.
Assuntos
Astrocitoma , Neoplasias Encefálicas , Glioblastoma , Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Células Endoteliais/metabolismo , Glioblastoma/patologia , Humanos , Neovascularização Patológica , Via de Sinalização Wnt , beta Catenina/metabolismoRESUMO
Intracranial Masson tumor (intravascular papillary endothelial hyperplasia, IPEH) is a benign lesion that is thought to originate from a reactionary process in response to compromised blood flow. IPEH may be classified into one of three subtypes based on etiology as it may result from the excessive proliferation of endothelial cells within a normal vessel (primary), vascular malformation (type II), or organized hematoma (type III). We report the case of a 79-year-old woman who presented with confusion, gait instability, and urinary incontinence. Neuroimaging revealed a hemorrhagic lesion within the right lateral ventricle, which was successfully resected. To our knowledge, this is the first reported case of an intraventricular IPEH and 33rd case of primary intracranial IPEH. We further performed a systematic review of the literature on all prior type I intracranial IPEH cases and discuss the importance of long-term follow-up in intracranial IPEH.
Assuntos
Neoplasias Encefálicas , Neoplasias do Ventrículo Cerebral , Malformações Vasculares , Idoso , Feminino , Humanos , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Diagnóstico Diferencial , Células Endoteliais , Hiperplasia/cirurgiaRESUMO
We present a young adult woman who developed a myxoid tumor of the pineal region having a SMARCB1 mutation, which was phenotypically similar to the recently described desmoplastic myxoid, SMARCB1-mutant tumor of the pineal region (DMT-SMARCB1). The 24-year-old woman presented with headaches, nausea, and emesis. Neuroimaging identified a hypodense lesion in CT scans that was T1-hypointense, hyperintense in both T2-weighted and FLAIR MRI scans, and displayed gadolinium enhancement. The resected tumor had an abundant, Alcian-blue positive myxoid matrix with interspersed, non-neoplastic neuropil-glial-vascular elements. It immunoreacted with CD34 and individual cells for EMA. Immunohistochemistry revealed loss of nuclear INI1 expression by the myxoid component but its retention in the vascular elements. Molecular analyses identified a SMARCB1 deletion and DNA methylation studies showed that this tumor grouped together with the recently described DMT-SMARCB1. A cerebrospinal fluid cytologic preparation had several cells morphologically similar to those in routine and electron microscopy. We briefly discuss the correlation of the pathology with the radiology and how this tumor compares with other SMARCB1-mutant tumors of the nervous system.
RESUMO
Medulloblastoma (MB) represents the most common malignant pediatric brain tumor and is defined by four molecular subgroups with WNT MB having the most favorable prognosis. Our work provides a rational therapeutic option in which the protective effects of WNT-driven MBs may be augmented in Group 3 and 4 MB.
RESUMO
Medulloblastoma (MB) is defined by four molecular subgroups (Wnt, Shh, Group 3, Group 4) with Wnt MB having the most favorable prognosis. Since prior reports have illustrated the antitumorigenic role of Wnt activation in Shh MB, we aimed to assess the effects of activated canonical Wnt signaling in Group 3 and 4 MBs. By using primary patient-derived MB brain tumor-initiating cell (BTIC) lines, we characterize differences in the tumor-initiating capacity of Wnt, Group 3, and Group 4 MB. With single cell RNA-seq technology, we demonstrate the presence of rare Wnt-active cells in non-Wnt MBs, which functionally retain the impaired tumorigenic potential of Wnt MB. In treating MB xenografts with a Wnt agonist, we provide a rational therapeutic option in which the protective effects of Wnt-driven MBs may be augmented in Group 3 and 4 MB and thereby support emerging data for a context-dependent tumor suppressive role for Wnt/ß-catenin signaling.
Assuntos
Neoplasias Cerebelares/terapia , Meduloblastoma/terapia , Proteínas Wnt/farmacologia , Proteínas Wnt/uso terapêutico , Animais , Carcinogênese , Linhagem Celular Tumoral , Proliferação de Células , Neoplasias Cerebelares/patologia , Modelos Animais de Doenças , Perfilação da Expressão Gênica , Regulação Neoplásica da Expressão Gênica , Xenoenxertos , Humanos , Meduloblastoma/genética , Meduloblastoma/patologia , Camundongos , Células-Tronco , Proteínas Wnt/genética , Via de Sinalização Wnt , beta Catenina/uso terapêuticoRESUMO
OBJECT: Lipomyelomeningocele (LMM) is a congenital spinal cord anomaly. While patients with LMM may initially be asymptomatic, neurological sequelae secondary to LMM may become apparent as the patient ages. Consequently, some pediatric neurosurgeons have advocated for upfront neurosurgical interventions irrespective of the presence of symptoms at diagnosis. By contrast, others pursue a conservative approach when overt neurological symptoms are not yet evident. In light of the various practice styles to the heterogeneous anatomical locations, symptoms, and ages associated with LMM, we have conducted a multi-center survey of Canadian pediatric neurosurgeons using clinical vignettes representative of LMM patients. METHODS: An online survey of the opinions of Canadian pediatric neurosurgeons was conducted using 5 separate cases with magnetic resonance imaging (MRI) scans of the lumbar spine. Each case was accompanied with the same three clinical vignettes, which varied in severity at time of presentation: asymptomatic, progressive somatic motor deficit, or longstanding overflow incontinence. Participants were asked the question, "Would you offer surgical management?" after each clinical vignette. After the five cases and their corresponding 3 clinical vignettes, participants were asked, "If you answered yes to any of the preceding questions, what type of surgery would you perform?". Options for surgical goals and techniques included complete removal, near-total removal, debulking, detethering, and expansile duroplasty. Surgical adjuncts included CUSA, LASER, and neurophysiologic monitoring. RESULTS: Twenty-three responses were received from the 38 questionnaires sent out to all staff pediatric neurosurgeons across academic medical centers in Canada. This represented a response rate of 61%. Canadian pediatric neurosurgeons generally maintain a conservative approach to the surgical management of LMM as only 13% (n = 3) of surgeons indicated that they would operate in all scenarios. By contrast, 43% (n = 10) indicated surgical management in only those cases presenting with symptoms, and another 43% (n = 10) displayed a variable surgical approach. Nine percent (n = 2) of participants would not perform surgery for incontinence. The greatest level of disagreement among participants pertained to the management of asymptomatic sacral LMM where 43% of participants favored prophylactic surgery, while 57% of participants preferred conservative management. CONCLUSIONS: The current study highlights the differences in management of LMM among Canadian pediatric neurosurgeons and provides further support for future prospective cohort studies to develop appropriate expert opinions and guidelines such that the care of LMM patients may be according to evidence-based best practice. This is especially true for the treatment of asymptomatic patients, a patient group that would benefit from a randomized controlled trial to assess the long-term outcomes of conservative and surgical management.
Assuntos
Caramujo Conus , Lipoma , Animais , Canadá , Criança , Humanos , Lipoma/diagnóstico por imagem , Lipoma/cirurgia , Estudos Prospectivos , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Postoperative length of stay (LOS) carries a high burden of healthcare costs. In resource-intense specialties such as neurosurgery, it is imperative to identify factors that influence LOS to improve care. The current study investigates the potential for variables that affect clinical presentation, tumor characteristics, treatment modalities, and postoperative complications to impact overall LOS in pediatric brain tumor patients. METHODS: A retrospective cohort study design was used with patients enrolled in the McMaster Pediatric Brain Tumor Study Group database. All patients up to 18 years of age, presenting with a newly diagnosed brain tumor admitted to and discharged from neurosurgery, were included. Patients were sorted into three cohorts: short LOS (≤3 days), extended LOS (≥20 days), and control LOS (4-19 days). RESULTS: Of the 124 patients included, 20 (65% male; median age: 9.1 years; range, 0.8-17.4 years) were considered short LOS, 28 (61% male; median age: 4.7 years; range, 0.4-14.7 years) were considered extended LOS, and 76 (57% male; median age: 8.5 years; range, 0.3-17.9 years) were considered control LOS. Variables that prolonged LOS were emesis at presentation (P < 0.001), developmental delay (P = 0.02), multiple surgeries (P = 0.004), tumor location (P < 0.05), subtotal resection (P = 0.02), feeding tube (P < 0.001), adjuvant chemoradiotherapy (P < 0.001), and posterior fossa syndrome (P = 0.004). CONCLUSIONS: This study identifies variables related to clinical presentation, tumor characteristics, treatment modalities, and postoperative complications associated with extended LOS. These findings uncover novel predictors of LOS that can be used to guide future research and improve health resource management.
Assuntos
Neoplasias Encefálicas/cirurgia , Tempo de Internação/estatística & dados numéricos , Procedimentos Neurocirúrgicos/normas , Complicações Pós-Operatórias , Adolescente , Neoplasias Encefálicas/patologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de RiscoRESUMO
Mechanistic insight into signaling pathways downstream of surface receptors has been revolutionized with integrated cancer genomics. This has fostered current treatment modalities, namely immunotherapy, to capitalize on targeting key oncogenic signaling nodes downstream of a limited number of surface markers. Unfortunately, rudimentary mechanistic understanding of most other cell surface proteins has reduced the clinical utility of these markers. CD133 has reproducibly been shown to correlate with disease progression, recurrence, and poor overall survivorship in the malignant adult brain tumor, glioblastoma (GBM). Using several patient-derived CD133high and CD133low GBMs we describe intrinsic differences in determinants of stemness, which we owe to a CD133-AKT-Wnt signaling axis in which CD133 functions as a putative cell surface receptor for AKT-dependent Wnt activation. These findings may have implications for personalized oncology trials targeting PI3K/AKT or Wnt as both pathways may be activated independent of their canonical drivers, leading to treatment resistance and disease relapse.
Assuntos
Antígeno AC133/metabolismo , Carcinogênese , Glioblastoma/patologia , Proteínas Proto-Oncogênicas c-akt/metabolismo , Via de Sinalização Wnt , Linhagem Celular Tumoral , Glioblastoma/metabolismo , HumanosRESUMO
PURPOSE: The present study aims to determine the tumor-related, clinical, and demographic factors associated with extent of resection (EOR) and post-operative outcomes in JPA patients. METHODS: All patients with JPA, identified from a single-center brain tumour data base, were included in this retrospective analysis. Pre-operative MRI scans were reviewed by a single neurosurgeon blinded to the EOR. JPA cases that exhibited no residual tumor post-operatively were assigned to the GTR group, all other tumors were assigned to the Assuntos
Astrocitoma/cirurgia
, Neoplasias Encefálicas/cirurgia
, Procedimentos Neurocirúrgicos/métodos
, Adolescente
, Astrocitoma/patologia
, Neoplasias Encefálicas/patologia
, Criança
, Pré-Escolar
, Estudos de Coortes
, Feminino
, Humanos
, Lactente
, Masculino
, Neoplasia Residual/patologia
, Procedimentos Neurocirúrgicos/efeitos adversos
, Estudos Retrospectivos
, Resultado do Tratamento
RESUMO
Medulloblastoma (MB) is the most frequent malignant pediatric brain tumor, representing 20% of newly diagnosed childhood central nervous system malignancies. Although advances in multimodal therapy yielded a 5-year survivorship of 80%, MB still accounts for the leading cause of childhood cancer mortality. In this work, we describe the epigenetic regulator BMI1 as a novel therapeutic target for the treatment of recurrent human Group 3 MB, a childhood brain tumor for which there is virtually no treatment option beyond palliation. Current clinical trials for recurrent MB patients based on genomic profiles of primary, treatment-naive tumors will provide limited clinical benefit since recurrent metastatic MBs are highly genetically divergent from their primary tumor. Using a small molecule inhibitor against BMI1, PTC-028, we were able to demonstrate complete ablation of self-renewal of MB stem cells in vitro. When administered to mice xenografted with patient tumors, we observed significant reduction in tumor burden in both local and metastatic compartments and subsequent increased survival, without neurotoxicity. Strikingly, serial in vivo re-transplantation assays demonstrated a marked reduction in tumor initiation ability of recurrent MB cells upon re-transplantation of PTC-028-treated cells into secondary recipient mouse brains. As Group 3 MB is often metastatic and uniformly fatal at recurrence, with no current or planned trials of targeted therapy, an efficacious targeted agent would be rapidly transitioned to clinical trials.
Assuntos
Neoplasias Cerebelares/tratamento farmacológico , Meduloblastoma/tratamento farmacológico , Células-Tronco Neoplásicas/efeitos dos fármacos , Complexo Repressor Polycomb 1/antagonistas & inibidores , Bibliotecas de Moléculas Pequenas/administração & dosagem , Animais , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/metabolismo , Criança , Epigênese Genética , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Meduloblastoma/genética , Meduloblastoma/metabolismo , Camundongos , Células-Tronco Neoplásicas/citologia , Células-Tronco Neoplásicas/metabolismo , Complexo Repressor Polycomb 1/genética , Bibliotecas de Moléculas Pequenas/farmacologia , Resultado do Tratamento , Regulação para Cima/efeitos dos fármacos , Ensaios Antitumorais Modelo de XenoenxertoRESUMO
BACKGROUND: The role of the extent of surgical resection (EOR) in clinical outcomes for patients with low-grade glioma requires further examination. The goal of the present study was to evaluate the association between variable degrees of EOR and clinical outcomes for patients with low-grade glioma. METHODS: We conducted a systematic review and meta-analysis and searched databases for reports of low-grade glioma EOR. Eligible studies compared patient outcomes, including ≥2 categories of EOR (biopsy, resection of any extent, subtotal resection [STR], or gross total resection [GTR]). Treatment effects were evaluated using pooled estimates, mean differences, or risk ratios (RRs) with corresponding 95% confidence intervals (CIs) using random effects modeling. RESULTS: Our literature search yielded 60 studies with 13,289 patients. Pooled estimates of overall survival (OS) showed an increase from 3.79 years in the biopsy group to 6.68 years in STR to 10.65 years in GTR. OS was favorable with resection of any extent compared with (mean difference, 3.24; 95% CI, 0.64-5.84; P = 0.015). Pooled estimates of seizure control showed an improvement from 47.8% with biopsy to 54.2% with STR and 81.0% with GTR. Compared with STR, GTR delayed malignant transformation (RR, 0.43; 95% CI, 0.20-0.93; P = 0.032), without increasing postoperative mortality (RR, 0.38; 95% CI, 0.07-1.97; P = 0.250) or morbidity (RR, 1.22; 95% CI, 0.65-2.28; P = 0.540). CONCLUSION: Among patients with low-grade gliomas, greater degrees of safe EOR were associated with longer OS and progression-free survival, better seizure control, and delayed malignant transformation, without increasing mortality or morbidity.
Assuntos
Biópsia , Neoplasias Encefálicas/cirurgia , Glioma/cirurgia , Procedimentos Neurocirúrgicos , Encéfalo/patologia , Encéfalo/cirurgia , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Glioma/mortalidade , Glioma/patologia , HumanosRESUMO
Central neurocytoma (CN) has long been regarded as a neuronal tumor based on the immunohistochemical expression of synaptophysin and the ultrastructural observation of neurosecretory granules, neurites, and synapses. Having diagnosed 11 CNs at our institution over the past thirty years, we set out to conduct an immunohistochemical study to assess the expression profile of neuronal markers across our cases. Markers of interest included synaptophysin, alpha-synuclein, chromogranin, neurofilament, and calretinin. Intriguingly, we observed a dichotomous expression profile between neurofilament and calretinin, suggesting the presence of histologic variants of CN based on the degree of neuronal maturation. We have further provided an overview of the clinico-pathologic heterogeneity within our series with respect to age of onset, overall outcome, and presence of anaplastic features. In highlighting the case of an infant with an incidental CN diagnosed at autopsy, we have discussed the role of a primitive neural cell of origin for driving tumor formation and accounting for our proposed differences in neuronal maturation within CN.
Assuntos
Biomarcadores Tumorais/análise , Neoplasias Encefálicas/patologia , Neurocitoma/patologia , Adulto , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND: Sinusitis is a common clinical condition, but sphenoid sinusitis is a less common form and even rarer is hypophysitis as a complication of the latter. Clinically, hypophysitis may mimic a pituitary neoplasm in presenting with mass effect and pituitary hormone dysfunction. CASE DESCRIPTION: We present 5 cases of sinusitis-related hypophysitis treated at the Royal Care International Hospital in Khartoum, Sudan. Clinical symptoms at presentation included headache, fever, ptosis, ophthalmoplegia, and history of sinusitis with running nose (nasal discharge). None of the patients were immunocompromised or showed signs of meningitis. Laboratory tests indicated neutrophilia and elevated inflammatory indices, namely C-reactive protein and erythrocyte sedimentation rate. There was also a disturbance of the hypothalamic-pituitary hormone axis, particularly impaired cortisol level. Magnetic resonance imaging scans on all patients revealed swollen masses in the pituitary fossa and enhancement of the sellar region and paranasal sinuses, especially the sphenoid sinus. All cases were empirically treated with hydrocortisone and amoxicillin-clavulanate, resulting in reversal of symptoms. CONCLUSIONS: Sinusitis is common in tropical regions where the climate is usually warm and often hot and dry. Here, the condition is considered a common incidental finding in magnetic resonance imaging examinations done for various indications. Hence it is not considered to be a serious health problem. Though our cohort of cases is small, we emphasize the importance of keeping a high index of suspicion for the diagnosis of hypophysitis in relevant case settings. This would help make an early diagnosis and ensure appropriate medical, perhaps nonsurgical, management.
Assuntos
Países em Desenvolvimento , Hipofisite/diagnóstico por imagem , Procedimentos Neurocirúrgicos , Seios Paranasais/diagnóstico por imagem , Sinusite/diagnóstico por imagem , Adulto , Idoso , Feminino , Humanos , Hipofisite/etiologia , Hipofisite/cirurgia , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Seios Paranasais/cirurgia , Sinusite/complicações , Sinusite/cirurgiaRESUMO
Brain metastases (BM) are the most common brain tumor in adults and are a leading cause of cancer mortality. Metastatic lesions contain subclones derived from their primary lesion, yet their functional characterization is limited by a paucity of preclinical models accurately recapitulating the metastatic cascade, emphasizing the need for a novel approach to BM and their treatment. We identified a unique subset of stem-like cells from primary human patient brain metastases, termed brain metastasis-initiating cells (BMICs). We now establish a BMIC patient-derived xenotransplantation (PDXT) model as an investigative tool to comprehensively interrogate human BM. Using both in vitro and in vivo RNA interference screens of these BMIC models, we identified SPOCK1 and TWIST2 as essential BMIC regulators. SPOCK1 in particular is a novel regulator of BMIC self-renewal, modulating tumor initiation and metastasis from the lung to the brain. A prospective cohort of primary lung cancer specimens showed that SPOCK1 was overexpressed only in patients who ultimately developed BM. Protein-protein interaction network mapping between SPOCK1 and TWIST2 identified novel pathway interactors with significant prognostic value in lung cancer patients. Of these genes, INHBA, a TGF-ß ligand found mutated in lung adenocarcinoma, showed reduced expression in BMICs with knockdown of SPOCK1. In conclusion, we have developed a useful preclinical model of BM, which has served to identify novel putative BMIC regulators, presenting potential therapeutic targets that block the metastatic process, and transform a uniformly fatal systemic disease into a locally controlled and eminently more treatable one.
Assuntos
Neoplasias Encefálicas/genética , Neoplasias Encefálicas/secundário , Regulação Neoplásica da Expressão Gênica , Metástase Neoplásica/genética , Metástase Neoplásica/fisiopatologia , Adenocarcinoma/genética , Adenocarcinoma/patologia , Adenocarcinoma/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Neoplasias Encefálicas/fisiopatologia , Carcinoma Pulmonar de Células não Pequenas/genética , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma Pulmonar de Células não Pequenas/fisiopatologia , Linhagem Celular Tumoral , Feminino , Humanos , Masculino , Camundongos Endogâmicos NOD , Camundongos SCID , Pessoa de Meia-Idade , Transplante de Neoplasias , Estudos Prospectivos , Proteoglicanas/genética , Proteoglicanas/metabolismo , Interferência de RNA , Proteínas Repressoras/genética , Proteínas Repressoras/metabolismo , Proteína 1 Relacionada a Twist/genética , Proteína 1 Relacionada a Twist/metabolismoRESUMO
Spindle cell oncocytoma (SCO) of the sella turcica is characterized as a WHO grade I non-endocrine neoplasm of the sella turcica. Histologically, these tumors contain spindled and variably oncocytic cellular processes. Although SCOs lack immunoreactivity for neuroendocrine markers and pituitary hormones, they are clinically indistinguishable from non-functioning pituitary adenomas. In contrast to the initially described benign clinical course, several reports have subsequently illustrated cases with multiple recurrences with or without histological features of anaplasia in the form of nuclear pleomorphism, frequent mitoses, high Ki-67 index, and/or necrosis. With a follow-up of 14years, we report a case of SCO with multiple recurrences along with an exhaustive clinico-pathological review of all 41 cases of SCO reported in the literature, of which recurrence has been described in 11 cases. Collectively, this report highlights the importance of long-term follow-up and the possible need for adjuvant radiotherapy in patients diagnosed with a sellar SCO and provides a comprehensive review of this rare nonadenomatous sellar tumor.
Assuntos
Adenoma Oxífilo/patologia , Sela Túrcica/patologia , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neoplasias Hipofisárias/patologia , Neoplasias da Base do Crânio/patologiaRESUMO
The surgical risk factors and neuro-imaging characteristics associated with cerebellar mutism (CM) remain unclear and require further investigation. Therefore, we aimed to examine surgical and MRI findings associated with CM in children following posterior fossa tumor resection. Using our data registry, we retrospectively collected data from pediatric patients who acquired CM and were matched based on age and pathology type with individuals who did not acquire CM after posterior fossa surgery. The strength of association between surgical and MRI variables and CM were examined using odds ratios (ORs) and corresponding 95% confidence intervals (CIs). A total of 22 patients (11 with and 11 without CM) were included. Medulloblastoma was the most common pathology among CM patients (91%); the remaining 9% were diagnosed with a pilocytic astrocytoma. Tumor attachment to the floor of the fourth ventricle (OR 6; 95% CI 0.7-276), calcification/hemosiderin deposition (OR 7; 95% CI 0.9-315.5), and post-operative peri-ventricular ischemia on MRI (OR 5; 95% CI 0.5-236.5) were found to have the highest measures of association with CM. Our results may suggest that tumor attachment to the floor of the fourth ventricle, pathological calcification, and post-operative ischemia have a relatively higher prevalence in patients with CM. Collectively, our work calls for a larger multi-institutional cohort study of CM patients to encourage further investigation of the determinants and management of CM in order to potentially minimize its development and predict onset.
Assuntos
Doenças Cerebelares/diagnóstico por imagem , Doenças Cerebelares/etiologia , Neoplasias Infratentoriais/diagnóstico por imagem , Neoplasias Infratentoriais/cirurgia , Mutismo/diagnóstico por imagem , Mutismo/etiologia , Astrocitoma/diagnóstico por imagem , Astrocitoma/cirurgia , Encéfalo/diagnóstico por imagem , Encéfalo/cirurgia , Criança , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/cirurgia , Procedimentos Neurocirúrgicos , Razão de Chances , Complicações Pós-Operatórias/diagnóstico por imagem , Estudos RetrospectivosRESUMO
We report a childhood case of thalamic atypical extraventricular neurocytoma that progressed to highly anaplastic ganglioglioma after 8 years of dormancy after subtotal resection and chemotherapy. The neurocytoma displayed immunoreactivity only for synaptophysin, ß-catenin, S100, and CD56. The ganglioglioma acquired strong immunoreactivity for chromogranin, glial fibrillary acidic protein, neuron-specific enolase, and p53 and showed a very high proliferation rate approaching 50% in some areas. Tumor transformation was associated with overexpression of components of the sonic hedgehog and Wnt developmental signaling pathways, which are known to regulate tumor-initiating cells in malignant brain neoplasms.