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The evolution of classification systems of pituitary adenomas (now PitNETs) has culminated in the use of transcription factor (TF) immunohistochemistry (IHC), forming a cell lineage-based system. However, several issues remain to be addressed, including the additional financial and logistic burden of undertaking the complete array of anterior pituitary hormones and TF IHC. To that end, several groups have suggested algorithms to minimise the number of tests performed, with varying levels of diagnostic accuracy. Although the proportion of null cell tumours has decreased following the use of TFs, "multilineage" tumours have been reported and characterised using transcriptomic signatures, most prominently the PIT1-SF1 co-expressing PitNETs, which do not bear a position in the present system of classification. In this review, we examine the proposed practical approaches to the diagnosis of PitNETs. We review the literature on reported PitNET types that challenge the existing classification system, such as those that express multiple TFs, with their potential clinical implications. Finally, we assess limitations in the present system, such as the lack of a standardised system for IHC interpretation, that need to be addressed in the future.
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Trigonal meningiomas are rare intraventricular tumours that present a surgical challenge. There is no consensus on the optimal surgical approach to these lesions, though the transtemporal and transparietal approaches are most frequently employed. We aimed to examine the approach-related morbidity and surgical nuances in treating trigonal meningiomas. This retrospective review assimilated data from 64 trigonal meningiomas operated over 15 years. Details of clinicoradiological presentation, surgical approach and intraoperative impression, pathology and incidence of various postoperative deficits were recorded. In our study, Trigonal meningiomas most frequently presented with headache and visual deterioration. The median volume of tumours was 63.6cc. Thirty-one meningiomas each (48.4%) were WHO Grade 1 and WHO Grade 2, while 2 were WHO Grade 3. The most frequent approach employed was transtemporal (38 patients, 59.4%), followed by transparietal (22 patients, 34.4%). After surgery features of raised ICP and altered mental status resolved in all patients, while contralateral limb weakness resolved in 80%, aphasia in 60%, seizures in 70%, and vision loss in 46.2%. Eighteen patients (28.13%) developed transient postoperative neurological deficits, with one patient (1.5%) developing permanent morbidity. Surgery for IVMs results in rapid improvement of neurological status, though visual outcomes are poorer in patients with low vision prior to surgery, longer duration of complaints and optic atrophy. The new postoperative deficits in some patients tend to improve on follow up. Transtemporal and transparietal approaches may be employed, based on multiple factors like tumour extension, loculation of temporal horn, size of lesion with no significant difference in their safety profile.
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Neoplasias Meníngeas , Meningioma , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias , Humanos , Meningioma/cirurgia , Meningioma/complicações , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Idoso , Resultado do Tratamento , Estudos Retrospectivos , Neoplasias Meníngeas/cirurgia , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/epidemiologia , Neoplasias do Ventrículo Cerebral/cirurgia , Adulto JovemRESUMO
BACKGROUND: Meningiomas are the most frequent primary intracranial tumor. While histological grade and grade of excision are established predictors of recurrence, the predictive ability of other clinical features, such as the role of radical excision of dural attachment and postoperative radiation therapy in intermediate-risk groups, remains unknown. METHODS: Clinical and radiological features and surgical details were analyzed in 451 World Health Organization (WHO) grade 1 intracranial meningiomas and 248 WHO grade 2 meningiomas operated on between 2010 and 2015. Outcomes were assessed in 352 WHO grade 1 and 208 WHO grade 2 meningiomas, studying the effect of extent of resection and use of radiation therapy. Kaplan-Meier analysis was used to determine differences in survival by extent of resection and use of postoperative radiation therapy in the treatment of the meningiomas. RESULTS: The mean age of the cohort was 46.3 years, with a female predominance. On univariate analysis, sex, WHO grade, and Simpson grade were significant predictors of recurrence. On multivariate analysis, WHO grade and Simpson grade remained significant predictors of recurrence. Recurrence was significantly associated with poor performance status and mortality. Postoperative radiation significantly improved progression-free survival among patients with grade 2 meningiomas who underwent gross total resection, but not among patients with grade 1 and grade 2 meningiomas who underwent subtotal resection. CONCLUSIONS: WHO grade and Simpson grade are independent predictors of recurrence in meningiomas. Regardless of WHO grade, gross total resection must be performed when possible, and postoperative radiation therapy may be recommended in grade 2 meningiomas.
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Neoplasias Meníngeas , Meningioma , Gradação de Tumores , Recidiva Local de Neoplasia , Organização Mundial da Saúde , Humanos , Meningioma/cirurgia , Meningioma/patologia , Meningioma/mortalidade , Feminino , Masculino , Pessoa de Meia-Idade , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/mortalidade , Prognóstico , Adulto , Idoso , Resultado do Tratamento , Estudos Retrospectivos , Adulto Jovem , Adolescente , Procedimentos Neurocirúrgicos/métodos , Estimativa de Kaplan-Meier , Idoso de 80 Anos ou maisRESUMO
PURPOSE: Postoperative fever is a common problem following neurosurgery but data on the causes among paediatric patients is sparse. In this report, we determined the incidence, causes, and outcomes of postoperative fever in paediatric neurosurgical patients (< 18 years), and contrasted the findings with an adult cohort published recently from our unit. METHODS: We recruited 61 patients who underwent 73 surgeries for non-traumatic neurosurgical indications over 12 months. A standard protocol was followed for the evaluation and management of postoperative fever. We prospectively collected data pertaining to operative details, daily maximal temperature, clinical features, and use of surgical drains, urinary catheters, and other adjuncts. Elevated body temperature of > 99.9 °F or 37.7 °C for > 48 h or associated with clinical deterioration or localising features was considered as "fever"; elevated temperature not meeting these criteria was classified as transient elevation in temperature (TET). RESULTS: Twenty-six patients (35.6%) had postoperative fever, more frequent than in adult patients. TET occurred in 12 patients (16.4%). The most common causes of fever were aseptic meningitis (34.6%), followed by urinary tract infections (15.4%), pyogenic meningitis, COVID-19, and wound infections. Postoperative fever was associated with significantly longer duration of hospital admission and was the commonest cause of readmission. CONCLUSION: In contrast to adults, early temperature elevations in paediatric patients may portend infectious and serious non-infectious causes of fever, including delayed presentation with aseptic meningitis, a novel association among paediatric patients. Investigation guided by clinical assessment and conservative antibiotic policy in keeping with the institutional microbiological profile provides the most appropriate strategy in managing paediatric postoperative fever.
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Febre , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias , Humanos , Feminino , Febre/etiologia , Febre/epidemiologia , Masculino , Criança , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Adolescente , Pré-Escolar , Lactente , Estudos Prospectivos , IncidênciaRESUMO
INTRODUCTION: Meningiomas are the most common primary intracranial tumour. Gamma knife radiosurgery (GKRS) is a frequently employed non-invasive method of treatment, with good remission rates and low morbidity in literature. However, the role of GKRS in the management of "large" meningiomas is unclear, with reported outcomes that vary by centre. We aimed to assess the factors that influence long-term outcomes following GKRS in meningiomas >10 cc in volume. METHODS: A retrospectively analysed all patients with meningiomas exceeding 10 cc in volume who underwent GKRS between January 2006 and December 2021 at the National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru. Demographic, clinical, radiological, and follow-up data were acquired, and factors associated with progression following GKRS were assessed. RESULTS: The cohort comprised 76 patients 29 males (38.2%) and 47 females (61.8%) with a mean age of 46.3 ± 11.02 years. Thirty-nine patients had been previously operated (51.3%). Meningiomas were most frequently located in the parasagittal region (26 tumours, 34.2%) and sphenopetroclival region (23 tumours, 30.3%), with mean lesion volume of 12.55 ± 5.22 cc, ranging 10.3 cc-25 cc. The mean dose administered to the tumour margin was 12.5 Gy ± 1.2 Gy (range 6-15 Gy). The median duration of clinical follow-up was 48 months, over which period radiological progression occurred in 14 cases (20%), with unchanged tumour volume in 20 cases (28.6%) and reduction in size of the tumour in 36 cases (51.4%). Progression-free survival after GKRS was 72% at 5 years, was significantly poorer among meningiomas with tumour volume >14 cc (log-rank test p = 0.045), tumours presenting with limb motor deficits (log-rank test p = 0.012), and tumours that underwent prior Simpson grade 3 or 4 excision (log-rank test p = 0.032). CONCLUSIONS: Meningiomas >10 cc in volume appear to display a high rate of progression and subsequent need for surgery following GKRS. Primary surgical resection, when not contraindicated, may be considered with GKRS serving an adjuvant role, especially in tumours exceeding 14 cc in volume, and presenting with limb motor deficits. Long-term clinical and radiological follow-up is essential following GKRS as the response of large meningiomas may be unpredictable.
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Neoplasias Meníngeas , Meningioma , Radiocirurgia , Masculino , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Meningioma/radioterapia , Meningioma/cirurgia , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Resultado do Tratamento , Estudos Retrospectivos , SeguimentosRESUMO
Intracranial hypotension (IH) represents a syndrome secondary to low cerebrospinal fluid pressure. This case of IH following a lumbar drain inserted before the excision of a left intraconal lesion, leading to recurrent post-operative unilateral subdural and extradural collections, was treated successfully with the evacuation of the collection and simultaneous epidural blood patch (EBP) injection. Our report provides an important perspective on the management of IH with recurrent intracranial collections and reiterates that IH should be considered when dealing with recurrent unilateral intracranial collections in the post-operative period. Evacuation with a simultaneous EBP is an effective strategy for managing IH.
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BACKGROUND: The endoscopic approach has gained popularity in cerebrospinal fluid (CSF) rhinorrhea repair with high success rates, yet recurrence is frequent. We analyzed our cases to determine the outcomes of endoscopic repair of CSF rhinorrhea and the effect of several perioperative factors on the success of repair. METHODS: A retrospective review of 50 patients who underwent endoscopic repair of CSF rhinorrhea between January 2013 and July 2023 was performed, collecting details of presentation, surgery, and postoperative period. RESULTS: The most frequent etiology was nontraumatic CSF rhinorrhea (76%), in which the defect was most commonly located at the left cribriform plate, followed by traumatic CSF rhinorrhea (24%), in which sphenoid defects were most frequent. Traumatic CSF rhinorrhea was more common among male patients and was significantly associated with anosmia. Success rate at first repair attempt was 84%. Persistent CSF rhinorrhea was present in 3 patients (6%), and 5 patients (10%) developed recurrence of CSF rhinorrhea. Overall, 7 patients required reoperation, with 100% success rate after the second surgery. The use of 3-layered repair with fat, fascia lata, and mucosal flap was protective against repair failure, whereas bilateral defects and duration of symptoms >1 year were significantly associated with repair failure. The use of lumbar drain did not demonstrate a difference in repair success rate. CONCLUSIONS: Endoscopic repair of CSF rhinorrhea appears to be safe and effective when performed with accurate localization of the site of the lesion and multilayered repair. Potential predictors of recurrence include bilateral and long-standing defects.
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Rinorreia de Líquido Cefalorraquidiano , Recidiva , Humanos , Rinorreia de Líquido Cefalorraquidiano/cirurgia , Rinorreia de Líquido Cefalorraquidiano/etiologia , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem , Idoso , Adolescente , Neuroendoscopia/métodos , Resultado do Tratamento , Reoperação , Complicações Pós-Operatórias , CriançaRESUMO
BACKGROUND: The WHO 2021 introduced the term pituitary neuroendocrine tumours (PitNETs) for pituitary adenomas and incorporated transcription factors for subtyping, prompting the need for fresh diagnostic methods. Current biomarkers struggle to distinguish between high- and low-risk non-functioning PitNETs. We explored if radiomics can enhance preoperative decision-making. METHODS: Pre-treatment magnetic resonance (MR) images of patients who underwent surgery between 2015 and 2019 with available WHO 2021 classification were used. The tumours were manually segmented on the T1w, T1-contrast enhanced, and T2w images using 3D Slicer. One hundred Pyradiomic features were extracted from each MR sequence. Models were built to classify (1) somatotroph and gonadotroph PitNETs and (2) high- and low-risk subtypes of non-functioning PitNETs. Feature were selected independently from the MR sequences and multi-sequence (combining data from more than one MR sequence) using Boruta and Pearson correlation. Support vector machine (SVM), logistic regression (LR), random forest (RF), and multi-layer perceptron (MLP) were the classifiers used. Data imbalance was addressed using the Synthetic Minority Oversampling TEchnique (SMOTE). Performance of the models were evaluated using area under the receiver operating curve (AUC), accuracy, sensitivity, and specificity. RESULTS: A total of 222 PitNET patients (train, n = 149; test, n = 73) were enrolled in this retrospective study. Multi-sequence-based LR model discriminated best between somatotroph and gonadotroph PitNETs, with a test AUC of 0.84, accuracy of 0.74, specificity of 0.81, and sensitivity of 0.70. Multi-sequence-based MLP model perfomed best for the high- and low-risk non-functioning PitNETs, achieving a test AUC of 0.76, accuracy of 0.67, specificity of 0.72, and sensitivity of 0.66. CONCLUSIONS: Utilizing pre-treatment MRI and radiomics holds promise for distinguishing high-risk from low-risk non-functioning PitNETs based on the latest WHO classification. This could assist neurosurgeons in making critical decisions regarding surgery or alternative management strategies for PitNETs after further clinical validation.
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Tumores Neuroendócrinos , Doenças da Hipófise , Neoplasias Hipofisárias , Humanos , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Radiômica , Estudos Retrospectivos , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/cirurgia , Imageamento por Ressonância MagnéticaRESUMO
Purpose Due to the potential risk of COVID-19 transmission during endonasal surgery, we studied methods to reduce droplet and aerosol generation during these procedures. Methods Droplet spread was assessed using ultraviolet light and a camera that detected fluorescence in the operative field and surgeon's personal protective equipment. Density of aerosols sized <10 µm was measured using a photometric particle counter. We designed a face-mounted negative-pressure mask placed on the patients' face during endoscopic endonasal surgery. Sixteen patients were recruited between October 2020 and March 2021 and randomly assigned to the mask and no-mask groups. We compared droplet spread and aerosols generated in both groups, with copious irrigation and continuous suction during drilling forming the mainstay of surgical technique in all cases. Results Droplet contamination due to direct spillage of fluorescein from the syringe was noted in two patients. Aerosol density rose during sphenoid drilling in both groups, with no significant difference when continuous suction and irrigation were employed (1.27 times vs. 1.07 times the baseline, p = 0 .248 ). Aerosol density rose significantly when suction and irrigation were interrupted in the no-mask group (44.9 times vs. 1.2 times, p = 0 .028) , which was not seen when the mask was used. Conclusion Aerosol generation increases during drilling in endonasal procedures and is a concern during this pandemic. The use of a rigid suction close to the drill along with copious irrigation is effective in reducing aerosol spread. The use of a negative pressure mask provides additional safety when inadvertent blockage of suction and inadequate irrigation occur.
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BACKGROUND: Paragangliomas are tumours of extra-adrenal paraganglia. They may metastasize to the brain but primary paragangliomas are exceedingly rare in the supratentorial region and long-term outcomes after surgery is largely unknown. This description of an excellent outcome 13 years following surgery in a 40-year-old gentleman with a primary paraganglioma near the falx provides an important perspective on the value of gross total resection in these tumours. We also review the options for adjuvant therapy in tumours that cannot be excised completely. CASE DESCRIPTION: We describe a supratentorial paraganglioma in the parasagittal region in a 40-year-old gentleman who presented with clinical and radiological features suggestive of a right parafalcine meningioma. Histopathological examination following gross total excision of the tumour revealed histological and immunochemical features of a paraganglioma. A detailed search for a systemic primary was negative and the patient remains disease-free 13 years after the surgery. CONCLUSIONS: Differentiating between tumours arising primarily and those that are metastatic deposits in the central nervous system requires long-term follow-up and monitoring for the appearance of occult primary tumours. Gross total resection is likely to provide good long-term outcomes.
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Neoplasias Meníngeas , Meningioma , Paraganglioma , Masculino , Humanos , Adulto , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Paraganglioma/patologia , Encéfalo/patologia , Sistema Nervoso Central/patologiaRESUMO
BACKGROUND: Hybrid nerve sheath tumors (HNST) contain elements of more than one established sub-type of nerve sheath tumor and have been recently recognized in the 2016 WHO classification of central nervous system tumors. While common in the peripheral nerves and extracranial branches of cranial nerves, only one case has been previously documented of an intracranial HNST arising from a cranial nerve. CASE DESCRIPTION: We describe a large, multi-compartmental intracranial hybrid nerve sheath tumor arising from the trigeminal nerve in a 22-year-old lady who presented with clinical and radiological features suggestive of a right cerebellopontine angle mass. Histopathological examination following retrosigmoid excision of the tumor revealed histological and immunohistochemical features of a schwannoma and a perineurioma. CONCLUSIONS: HNSTs are likely to be underreported in the intracranial region. The clinical course of these tumors and the reason for their occurrence in this location are not known.
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Neoplasias Encefálicas , Neoplasias de Bainha Neural , Neurilemoma , Feminino , Humanos , Adulto Jovem , Adulto , Neoplasias de Bainha Neural/diagnóstico por imagem , Neoplasias de Bainha Neural/cirurgia , Neurilemoma/diagnóstico por imagem , Neurilemoma/cirurgia , Neurilemoma/complicações , Neoplasias Encefálicas/complicações , Nervos Cranianos , Nervo Trigêmeo/diagnóstico por imagem , Nervo Trigêmeo/cirurgiaRESUMO
Postoperative fever is mostly transient and inconsequential but may portend a serious postoperative infection requiring a thorough evaluation, especially during the recent COVID-19 pandemic. We aimed to determine the incidence, causes and outcomes of postoperative fever in neurosurgical patients, as well as to evaluate a protocol for management of postoperative fever. We conducted a prospective study over 12 months, recruiting 425 adult patients operated for non-traumatic neurosurgical indications. We followed a standard protocol for the evaluation and management of postoperative fever collecting data regarding operative details, daily maximal temperature, clinical features, as well as use of surgical drains, urinary catheters, and other invasive adjuncts. Elevated body temperature of > 99.9°F or 37.7 °C for over 48 h or associated with clinical deterioration or localising features was considered as "fever" and was evaluated according to our protocol. We classified elevated temperature not meeting this criterion as a transient elevation in temperature (TET). Sixty-five patients (13.5%) had postoperative fever. Transient elevation of temperature, occurring in 40 patients (8.8%) was most common in the first 48 h after surgery. The most common causes of fever were urinary tract infections (13.7%), followed by aseptic meningitis (10.8%), wound infections and pneumonia. Various aetiologies of fever followed distinct patterns, with COVID-19 and meningitis causing high-grade, prolonged fever. Multivariate analysis revealed cranial surgery, prolonged duration of surgery, urinary catheters and wound drains retained beyond POD 3 to predict fever. Postoperative fever was associated with significantly longer duration of hospital admission. COVID-19 had a high mortality rate in the early postoperative period.
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COVID-19 , Neurocirurgia , Adulto , Febre , Humanos , Procedimentos Neurocirúrgicos , Pandemias , Estudos ProspectivosRESUMO
BACKGROUND: The prevalence of BRAFV600E mutations in pleomorphic xanthoastrocytoma (PXA) World Health Organization (WHO) Grade 2 and PXA WHO Grade 3 reported varies from 60% to 80%, yet the prognostic implications remain unclear. METHODS: We reviewed the demographic and clinicoradiologic data of 20 PXAs WHO Grade 2 and 13 PXAs WHO Grade 3, operated between 2007 and 2020, to ascertain extent of excision, recurrence, progression-free survival (PFS), and overall survival (OS). PXAs WHO Grade 3 were defined by the presence of >5 mitoses/high-power field. PXAs WHO Grade 3 received adjuvant radiation therapy and chemotherapy whereas PXAs received radiation therapy if subtotally excised. All samples were analyzed for the presence of BRAFV600E mutation using DNA obtained from paraffin blocks using droplet-digital polymerase chain reaction. RESULTS: The median patient age at diagnosis was 22 years with a male preponderance. BRAFV600E mutations were noted in 30% of tumors; 8 PXAs WHO Grade 2 and 2 PXAs WHO Grade 3. Recurrence occurred in 6 of 13 PXA WHO Grade 3 (55%) and 1 of 20 PXAs WHO Grade 2 (5%). At median follow-up of 45 months, the OS was 54 months and 33 months in the PXA WHO Grade 2 and PXA WHO Grade 3 groups, respectively (P = 0.02). OS and PFS did not differ between BRAF-mutated and BRAF-negative tumors. CONCLUSIONS: BRAFV600E mutations are less frequent in our population than reported in the literature. The BRAF mutation does not significantly impact OS and PFS. PXAs WHO Grade 3 are a distinct clinical entity, associated with worse PFS and OS than PXAs WHO Grade 2.
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Astrocitoma , Neoplasias Encefálicas , Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Humanos , Masculino , Mutação/genética , Prevalência , Prognóstico , Proteínas Proto-Oncogênicas B-raf/genéticaRESUMO
Despite being the most common primary intracranial tumor, meningiomas are classified largely based on histological features. The current system of grading has been shown to be unsatisfactory due to its poor reproducibility as well as the considerable variability within grades. With the increasing availability of genomic and epigenomic profiling, several markers have been suggested to correlate with the location, histological subtype, and clinical behavior of meningiomas. These developments have enabled the development of targeted therapy, as well as individualized use of currently available adjuvant methods. These include copy number alterations (CNAs), specific genetic abnormalities (germline and sporadic), and genome-wide methylation profiles. In this review, we recapitulate the changes in the classification of meningiomas thus far, discuss the various histological subtypes recognized, and present the available literature on the genetic and epigenetic profiles of meningiomas. The recognition and further study of these markers have the potential to usher in an era of personalized therapy in the management of meningiomas, vastly improving outcomes as has been observed in the case of several other tumors.
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Neoplasias Meníngeas , Meningioma , Variações do Número de Cópias de DNA , Humanos , Neoplasias Meníngeas/patologia , Meningioma/genética , Meningioma/patologia , Gradação de Tumores , Reprodutibilidade dos Testes , Organização Mundial da SaúdeRESUMO
BACKGROUND: Supratentorial ependymomas (STEs) are an aggressive group of ependymomas, topographically distinct from their posterior fossa and spinal counterparts. Zinc finger translocation associated (ZFTA) fusion-positive cases have been reported to account for the majority of STEs, although data on its association with poorer outcomes are inconsistent. MATERIALS AND METHODS: We assessed the prevalence of the ZFTA fusion by reverse-transcription polymerase chain reaction and fluorescence in situ hybridization in a cohort of 61 patients (68 samples) with STE. Our primary outcome was to determine the role of the ZFTA fusion on progression-free and overall survival of patients with STE. Our secondary objectives were to assess the impact of ZFTA fusion on nuclear factor (NF)-kB pathway signaling via surrogate markers of this pathway, namely COX-2, CCND1, and L1 cell adhesion molecule. RESULTS: ZFTA fusion was noted in 21.3% of STEs in our cohort. The presence of this rearrangement did not significantly impact the progression-free or overall survival of patients with STEs and was not associated with upregulation of markers of the NF-kB pathway. Only gross total resection was significantly associated with better progression-free survival. CONCLUSIONS: In contradiction to previous reports from across the world, the ZFTA fusion is far less prevalent among our population. It does not appear to drive NF-kB signaling or significantly affect outcomes. Gross total resection must be attempted in all cases of STE and adjuvant radiation and/or chemotherapy employed when gross total resection is not achieved.
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Ependimoma , Neoplasias Supratentoriais , Ependimoma/genética , Ependimoma/metabolismo , Ependimoma/cirurgia , Humanos , Hibridização in Situ Fluorescente , NF-kappa B/metabolismo , Prevalência , Neoplasias Supratentoriais/genética , Neoplasias Supratentoriais/metabolismo , Neoplasias Supratentoriais/cirurgia , Fator de Transcrição RelA/genética , Fator de Transcrição RelA/metabolismo , Translocação Genética/genética , Dedos de ZincoRESUMO
Background: Cavernous sinus hemangiomas are benign vascular tumors of the cavernous sinus. Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's angioma is a rare, aberrant thrombus organization occurring independently or in pre-existent lesions, and information on its occurrence in the intracranial region, or its management is sparse. Objective: We aim to describe a case of IPEH occurring in a pre-existing cavernous sinus hemangioma, treated with surgical excision and radiation therapy, providing insight into the appropriate treatment of these tumors and their response to therapy. Materials and Methods: We describe a right cavernous sinus hemangioma, recurring after initial radiation therapy and requiring surgical excision, at which point the presence of IPEH was noted. A sellar recurrence following partial excision was treated with hypofractionated Stereotactic radiotherapy (SRT), and this provided an excellent long-term outcome. Conclusions: Cavernous sinus hemangiomas are treated with surgical excision or primary radiation therapy. IPEH must be looked for in all excised specimens, as there are no radiological features that indicate its presence, and it may resemble the original lesion histologically. When detected, adjuvant radiation must be administered as the IPEH responds well to the use of radiation therapy.
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Seio Cavernoso , Hemangioma Cavernoso , Hemangioma , Neoplasias Vasculares , Seio Cavernoso/diagnóstico por imagem , Seio Cavernoso/patologia , Hemangioma/patologia , Hemangioma Cavernoso/diagnóstico por imagem , Hemangioma Cavernoso/patologia , Hemangioma Cavernoso/cirurgia , Humanos , Hiperplasia , Neoplasias Vasculares/patologiaRESUMO
BACKGROUND: Thyrotroph pituitary adenomas have been reported to be a rare cause of 'thyroid storms', causing myriad metabolic and autonomic disturbances. In this case, we describe the second reported case in literature of a 'GH storm' in an infarcted somatotroph adenoma.Case description: We describe a residual invasive somatotroph macroadenoma that underwent infarction, producing a dramatic elevation in serum GH levels. While infarction of adenomas may in some cases lead to remission, the patient went on to require re-surgery and re-radiation due to growth of the residual viable tumour.Conclusions- 'GH storms' are rare but interesting events that may occur in somatotroph adenomas. Infarction or apoplexy must be considered when managing residual adenomas.
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PURPOSE: During the ongoing COVID-19 pandemic, endonasal surgeries for sellar-suprasellar lesions were discouraged due to the risk of transmission of the disease. We reviewed the changes in our management protocol for these lesions as our disease understanding and preparedness evolved. MATERIALS AND METHODS: This was a retrospective observational study including patients with sellar-suprasellar and clival lesions presenting to us between March and October 2020. Management protocols were divided into three phases based on the prevalence of the disease and the number of mandatory preoperative COVID-19 tests being conducted. The surgical approach used was analyzed in relation to the preferred approach during pre-COVID times, and surgical outcomes and complications were noted. RESULTS: A total of 31 cases were operated during this period. During Phase I (low prevalence; no preoperative COVID testing) endonasal surgeries were largely abandoned in favor of transcranial approaches. In Phase II (medium prevalence; one preoperative COVID test) we gradually resumed endonasal surgeries for 'emergent' and 'essential' cases, and subsequently in Phase III (high prevalence; two preoperative COVID tests), we had no hesitation in performing 'elective' endonasal surgeries with additional barriers for prevention of aerosol transmission. No patient developed COVID-19 infection postoperatively. Eight HCWs in our department acquired the disease during this period, none of whom were directly involved in the surgeries for the above cohort of patients. CONCLUSIONS: With a strict preoperative COVID testing protocol, adherence to proper drilling techniques and using additional barriers to prevent droplet and aerosol spread, endonasal surgeries for sellar-suprasellar lesions are safe during this COVID-19 pandemic.
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BACKGROUND: Silent corticotroph adenomas (SCAs) are a rare subtype of non-functional pituitary adenoma. While it has been suggested that they are more aggressive and recur more frequently following excision, there is limited literature on the optimum treatment strategy for these tumors, especially regarding the role of radiation therapy in incompletely resected tumors. METHOD: We assimilated data from 62 SCAs and 238 other non-functional adenomas (ONAs), defined according to the WHO 2017 criteria that incorporates transcription factor analysis. We compared their clinicoradiological characteristics, such as hormonal levels, tumor configuration, size, and invasiveness. For 52 SCAs and 205 ONAs with serial follow-up imaging, we studied outcomes for progression after subtotal resection with or without radiation therapy or recurrence after gross total resection. Kaplan Meier analysis for recurrence or progression was used to determine the need for a differential treatment strategy for SCAs compared with other non-functional adenomas specifically concerning the role of radiotherapy. RESULTS: Patients with SCAs present at a younger age than ONAs (43.9 years vs. 48.2 years, p = 0.014), with larger (14.9 cc vs. 9.7 cc, p = 0.006) and more invasive adenomas (61.2% vs. 45.8%, p = 0.021). Overall, SCAs are more likely to recur or progress (48.7 vs. 15.7%, p < 0.001) following excision than ONAs, with significantly poorer event-free survival (Log rank test p < 0.001). Early adjuvant radiotherapy provides favorable outcomes among SCAs with postoperative residual tumor, on par with ONAs. Multivariate analysis identified male gender (HR: 2.217; p = 0.017), MIB index ≥ 3% (HR: 2.116; p = 0.012), and SCA tumor pathology (HR: 3.787; p < 0.001) as factors predicting recurrence. CONCLUSIONS: Based on the results of this retrospective, single-center review of 300 non-functional adenomas, we conclude that silent corticotroph adenomas are an aggressive subtype of non-functional pituitary adenomas that are larger, more likely to be invasive, and tend to recur more frequently after a subtotal excision compared with other non-functional adenomas. A gross total resection must be attempted whenever possible and earlier adjuvant radiation is recommended when re-surgery for residual tumor is difficult.
Assuntos
Adenoma Hipofisário Secretor de ACT , Neoplasias Hipofisárias , Fatores de Transcrição , Adenoma Hipofisário Secretor de ACT/diagnóstico por imagem , Adenoma Hipofisário Secretor de ACT/terapia , Hormônio Adrenocorticotrópico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/terapia , Estudos RetrospectivosRESUMO
OBJECTIVE: Acromegaly is a syndrome of metabolic alterations secondary to increased growth hormone levels from a somatotroph pituitary adenoma. A multidisciplinary approach beginning with surgery, followed by adjuvant radiation or medical therapy for residual disease, is considered standard of care. Several factors affect the likelihood of remission after surgery, but the impact of surgical experience on remission rates has not been adequately assessed. METHODS: Retrospective review of 203 patients, divided into 2 eras (era 1, 102 patients; era 2, 101 patients) of patients who underwent transsphenoidal surgery for acromegaly by a single surgeon over 11 years, was performed, determining the effect of surgical experience on rates of remission and various complications. Remission was defined according to the 2014 Endocrine Society Clinical Practice Guideline. RESULTS: The rate of surgical remission was 40.6% (62.9% among noninvasive adenomas). Rates of surgical remission significantly improved in the latter half of this cohort (31.2% in the first half vs. 50% in the second half), despite other factors being comparable. On multivariate analysis, surgeon experience, cavernous sinus invasion, and preoperative growth hormone levels affected the rates of surgical remission. Rates of cerebrospinal fluid leak and hypopituitarism were lower in the second half, whereas resolution of acromegaly-associated comorbidities was increased. CONCLUSIONS: We report, in this large single-surgeon review of endoscopically operated acromegaly cases, increased rates of surgical remission and reduced complications with increasing surgeon experience. The overall experience of the treating team in dealing with perioperative and intraoperative factors also contributes to improved outcomes.