RESUMO
PURPOSE: To present a descriptive analysis of pediatric craniopharyngiomas (PedCPG) treated in various Spanish hospitals, defining factors related to recurrence and performing a critical analysis of the results. METHODS: We undertook a multicenter retrospective review of PedCPG treated between 2000 and 2017. Data collected included epidemiological variables, clinical and radiological characteristics, goal of first surgery, rate of recurrence and its approach, adjuvant treatment, complications and permanent morbidity. Associations were studied between progression and number of progressions and independent variables. RESULTS: The study involved 69 children from 8 Spanish hospitals. Most of the tumors invaded several intracranial compartments at diagnosis, with the hypothalamus involved in 41.3% of cases. The first treatment strategy was usually gross total resection (GTR) (71%), with some patients treated with radiotherapy or intracystic chemotherapy. The progression rate after first surgery was 53% in a mean follow-up of 88.2 months (range 7-357). In the GTR group 38.8% of tumors recurred, 40% in the group of subtotal resection or biopsy and 93.3% in the cyst fenestration±Ommaya reservoir group. Mortality was 7.2%. Follow-up period, size of the tumor and goal of first surgery were significantly related with progression. CONCLUSIONS: Our results in terms of disease control, hormonal or visual impairment and mortality were acceptable, but there are several areas for improvement. Our short-term goals should be to create a national register of PedCPG, reach a consensus about a treatment algorithm, and improve diagnosis of hypothalamic dysfunction to avoid preventable morbidity.
Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Criança , Humanos , Craniofaringioma/diagnóstico , Craniofaringioma/patologia , Craniofaringioma/cirurgia , Resultado do Tratamento , Neoplasias Hipofisárias/cirurgia , Procedimentos Neurocirúrgicos/métodos , Estudos RetrospectivosRESUMO
BACKGROUND: An Ommaya reservoir can be used to treat posthemorrhagic hydrocephalus secondary to intraventricular hemorrhage of prematurity until an acceptable weight can be obtained to place a permanent shunt. Identifying newborns at higher risk of developing shunt conversion may improve the management of these patients. This study aimed to develop a predictive algorithm for conversion of an Ommaya reservoir to a permanent shunt using artificial intelligence techniques and classical statistics. METHODS: A database of 43 preterm patients weighing ≤1500 g with posthemorrhagic hydrocephalus (Papile grades III and IV with Levene ventricular index >4 mm above the 97th percentile) managed with an Ommaya reservoir at our institution between 2002 and 2017 was used to train a k-nearest neighbor algorithm. Validation of results was done with cross-validation technique. Three scenarios were calculated: 1) considering all features regardless whether or not they are correlated with the output variable; 2) considering the features as predictors if they have a correlation >30% with the output variable; 3) considering the output of the previous analysis. RESULTS: When considering the outputs of a previous multivariate analysis, the algorithm reached 86% of cross-validation accuracy. CONCLUSIONS: The use of machine learning-based algorithms can help in early identification of patients with permanent need of a shunt. We present a predictive algorithm for a permanent shunt with an accuracy of 86%; accuracy of the algorithm can be improved with larger volume of data and previous analysis.
Assuntos
Inteligência Artificial , Hidrocefalia , Hemorragia Cerebral/complicações , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/cirurgia , Derivações do Líquido Cefalorraquidiano , Humanos , Hidrocefalia/complicações , Hidrocefalia/cirurgia , Recém-Nascido , Aprendizado de Máquina , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Central nervous system (CNS) tumours comprise 25% of the paediatric cancer diagnoses and are the leading cause of cancer-related death in children. Current treatments for paediatric CNS tumours are far from optimal and fail for those that relapsed or are refractory to treatment. Besides, long-term sequelae in the developing brain make it mandatory to find new innovative approaches. Chimeric antigen receptor T cell (CAR T) therapy has increased survival in patients with B-cell malignancies, but the intrinsic biological characteristics of CNS tumours hamper their success. The location, heterogeneous antigen expression, limited infiltration of T cells into the tumour, the selective trafficking provided by the blood-brain barrier, and the immunosuppressive tumour microenvironment have emerged as the main hurdles that need to be overcome for the success of CAR T cell therapy. In this review, we will focus mainly on the characteristics of the deadliest high-grade CNS paediatric tumours (medulloblastoma, ependymoma, and high-grade gliomas) and the potential of CAR T cell therapy to increase survival and patients' quality of life.
Assuntos
Neoplasias Encefálicas/terapia , Imunoterapia Adotiva , Antígenos de Neoplasias/imunologia , Neoplasias Encefálicas/patologia , Criança , Ensaios Clínicos como Assunto , Humanos , Imunoterapia Adotiva/efeitos adversos , Gradação de Tumores , Microambiente TumoralRESUMO
PURPOSE: Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. To date, only eight spinal cases have been reported. This is the third reported case in pediatric population, the first one associated with genetic syndrome and the first benign to recur. We review the current literature on this topic. CASE DESCRIPTION: We present a 2-year-old boy affected by Beckwith-Wiedemann syndrome who developed a tumor at L4-L5 level. He underwent a gross total resection with MRI post-surgery demonstrating non-residual tumor. Histology disclosed an ectopic adrenal cortical adenoma with oncocytic features. Immunohistochemically was positive for inhibin-alpha, synaptophysin, and melan-A. It was negative for chromogranin A, GFAP, S-100, and other markers. One year later, he developed a recurrence at the same level being necessary a second surgery leaving a small sheet of residual tumor. CONCLUSION: Spinal adrenocortical adenomas are exceptional, and its behavior could be related to other conditions such as BWS. Gross total resection can be curative but a tight follow-up is needed. Immunohistochemical studies that include inhibin-alpha, synaptophysin, and melan-A can be useful in differential diagnosis as ultrastructural study. The decision on how to treat these patients is difficult given the low number of cases.
Assuntos
Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Adenoma Adrenocortical/diagnóstico por imagem , Síndrome de Beckwith-Wiedemann/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/complicações , Neoplasias do Córtex Suprarrenal/cirurgia , Adenoma Adrenocortical/complicações , Adenoma Adrenocortical/cirurgia , Síndrome de Beckwith-Wiedemann/complicações , Síndrome de Beckwith-Wiedemann/cirurgia , Pré-Escolar , Humanos , Masculino , Neoplasias da Coluna Vertebral/complicações , Neoplasias da Coluna Vertebral/cirurgiaRESUMO
BACKGROUND: Deep brain stimulation for obsessive-compulsive disorder (OCD) has targeted several subcortical nuclei, including the subthalamic nucleus (STN) and the nucleus accumbens. While the most appropriate target is still being looked for, little attention has been given to the side of the stimulated hemisphere in relationship to outcome. METHODS: We report 2 patients diagnosed with OCD, one having symmetry obsessions and the other one with sexual-religious obsessive thoughts. They were implanted bilaterally with deep electrodes located at both STN and nuclei accumbens. The effectiveness of the stimulation was tested for every possible paired combination of electrodes guided by the Yale-Brown Obsessive Compulsive Scale (Y-BOCS) score reduction. RESULTS: In both cases, the combination of electrodes which best relieved the OCD symptoms was both the left STN and left accumbens. In case 1, the preoperative Y-BOCS score was 33, and 1 month after stimulation it was 16. In case 2, the Y-BOCS scores were 33 and 3, respectively, with the patient being free of obsessions. CONCLUSION: Some reports suggest that lesion stimulation or stimulation of only the right side relieves OCD symptoms. However, anatomical and functional studies are not conclusive as to which side is most affected in OCD. Possibly, each OCD patient has an individualized optimal side to stimulate.
Assuntos
Estimulação Encefálica Profunda/métodos , Núcleo Accumbens/fisiopatologia , Transtorno Obsessivo-Compulsivo/terapia , Núcleo Subtalâmico/fisiopatologia , Adulto , Eletrodos Implantados , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Núcleo Accumbens/patologia , Transtorno Obsessivo-Compulsivo/fisiopatologia , Reprodutibilidade dos Testes , Núcleo Subtalâmico/patologia , Resultado do TratamentoRESUMO
Neurotrophic factors may be used to improve the growth and repair of injured peripheral nerves. In this study we determined the effectiveness of recombinant human growth hormone on peripheral nerve injury in the Wistar rat. The ulnar nerve of the rat was sectioned and its proximal and distal ends were sutured to either end of a silastic tube, with the aim of encouraging regeneration through the tube. 32 ulnar nerve specimens were randomized into two groups: 18 nerves regenerating under the influence of recombinant growth hormone, and 14 nerves regenerating in its absence. The study was performed over a period of 8 weeks and progression of regeneration was assessed with regular surface electroneurography every 1-2 weeks after surgery. In the group receiving recombinant growth hormone, it comprised a significant improvement in the recovery of conduction velocity, and a more gradual increase in the amplitude of motor potential from the fifth week onwards was observed. Histological analysis of study specimens in the recombinant hormone group revealed an improved architecture of the regenerating nerve, a greater density of nerve fibers, and increased myelination with a lesser degree of endoneural fibrosis. Our work demonstrates the positive effect of the administration of recombinant human growth hormone in obtaining significantly improved conduction velocities, and a greater improvement in nerve regeneration from the fifth week of monitoring when compared to the control group. Histological analysis in the group receiving hormone showed acceptable degree of myelination with little granulation tissue and fibrosis.