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Surgical Neurology International 2021Low-grade gliomas (LGG) are described by the World Health Organization as Grades I and II. Among LGGs, the most common primary brain tumor is pilocytic astrocytoma (PA)...
BACKGROUND
Low-grade gliomas (LGG) are described by the World Health Organization as Grades I and II. Among LGGs, the most common primary brain tumor is pilocytic astrocytoma (PA) and carries an excellent prognosis when treated with complete surgical resection. Cases, in which this is not possible, are associated with less favorable outcomes and worse progression-free survival.
CASE DESCRIPTION
This report describes a case of a 22-year-old male, who presented with progression of a primary brainstem tumor previously treated with stereotactic radiosurgery and chemotherapy. Patient underwent surgical exploration and was diagnosed with juvenile PA, but debulking was limited by the very dense and fibrous tumor. Complete surgical resection was not possible at this time. Despite efforts to treat with chemotherapy, the patient presented a year later with clinical deterioration and severe neurologic deficits, prompting surgical re-exploration. During the second operation, the tumor was found to have undergone very significant softening in consistency, allowing for gross total resection (GTR).
CONCLUSION
Aggressive treatment of brainstem LGG should be pursued whenever possible, given its generally favorable prognosis. Repeat microsurgical resection, even with a different approach, might be reasonable and safe. Finally, chemotherapy may be associated with changes in the tumor consistency that can render previously unresectable lesions amenable to successful aggressive resection.
PubMed: 33500827
DOI: 10.25259/SNI_594_2020 -
Cureus Aug 2020We present a very rare case of chronic encapsulated intracerebral hematoma involving the septum pellucidum and the foramen of Monro that by location radiological...
We present a very rare case of chronic encapsulated intracerebral hematoma involving the septum pellucidum and the foramen of Monro that by location radiological appearance, and clinical history was mimicking a recurrent astrocytoma or a shunt-related foreign body granuloma. A young adult underwent the resection of a juvenile pilocytic astrocytoma as a child, and with a mass encasing the tip of an old non-functioning ventricular catheter, the differential diagnosis of shunt-related foreign body granuloma versus recurrent low-grade glioma was raised. Although chronic encapsulated intracerebral hematomas have been reported in the literature, the anatomical location of the lesion in the presented case was unique, with radiological and history findings also posing a peculiar diagnostic challenge. Chronic encapsulated intracerebral hematomas are benign entities that may also be found to involve deep and midline supra-tentorial structures usually not prone to spontaneous intraparenchymal hemorrhages. When symptomatic, surgical resection of the hematoma can be both diagnostic and curative.
PubMed: 32953346
DOI: 10.7759/cureus.9839 -
Orphanet Journal of Rare Diseases Jun 2020Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease with many manifestations, and it involves any organ. In this study, we report a TSC patient...
OBJECTIVE
Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease with many manifestations, and it involves any organ. In this study, we report a TSC patient with new type skin lesions.
METHODS
A 7-month-old TSC boy with multiple cutaneous nodules was admitted in our hospital. We collected the clinical data of the patient. We performed biopsy of cutaneous nodules and whole-exome sequencing in both paraffin block tissue and blood samples.
RESULTS
The patient presented with a 2 month history of gradual growth multiple cutaneous nodules. He had cardiac rhabdomyoma, subependymal giant cell astrocytoma (SEGA) and hypomelanotic macules. The pathological finding of cutaneous nodules was consistent with juvenile xanthogranuloma (JXG). After 3 months of sirolimus treatment, the multiple nodules disappeared. The whole-exome sequencing identified TSC1 (c.2356C > T, p.R786*) mutation in both paraffin block tissue and blood samples. We overturned the original pathological diagnosis and finally identified JXG as a new type of skin lesions in TSC.
CONCLUSION
This is the first report on the occurrence of JXG skin lesions in TSC patient. Genetic testing is necessary in JXG. These findings expand the phenotype of skin in patients with TSC and contribute to the elucidation of JXG pathogenesis and treatment.
Topics: Genetic Testing; Humans; Hypopigmentation; Infant; Male; Sirolimus; Tuberous Sclerosis; Xanthogranuloma, Juvenile
PubMed: 32532290
DOI: 10.1186/s13023-020-01396-7 -
BMJ Case Reports Feb 2019
Topics: Astrocytoma; Brain Neoplasms; Female; High-Throughput Nucleotide Sequencing; Humans; Mutation, Missense; Proto-Oncogene Proteins p21(ras); Sequence Analysis, DNA; Young Adult
PubMed: 30798277
DOI: 10.1136/bcr-2018-228128 -
Cureus Oct 2018Hyponatremia post-neurosurgical intervention can be dangerous and potentially life-threatening. Two of its most common causes are cerebral salt wasting (CSW) and...
Hyponatremia post-neurosurgical intervention can be dangerous and potentially life-threatening. Two of its most common causes are cerebral salt wasting (CSW) and syndrome of inappropriate anti-diuretic hormone release (SIADH). CSW is proposed to be secondary not only to the elevated levels of circulating atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) but inhibition of steroidogenesis in the zona glomerulosa of the adrenal cortex, thus resulting in mineralocorticoid deficiency. We present a two-year-old male who had developed acute hyponatremia secondary to CSW on post-operative day two after a sub-total resection of a low-grade juvenile pilocytic astrocytoma (WHO grade I). Fludrocortisone was successfully used to manage the refractory hyponatremia and alleviated the need to use very large amounts of oral sodium supplementation.
PubMed: 30648045
DOI: 10.7759/cureus.3505 -
Cureus Oct 2017The resection of deep-seated brain tumors has been associated with morbidity due to injury to critical neural structures during the approach. Recent technological...
The resection of deep-seated brain tumors has been associated with morbidity due to injury to critical neural structures during the approach. Recent technological advancements in navigation and stereotaxy, surgical planning, brain tractography and minimal-access brain ports present the opportunity to overcome such limitations. Here, we present the case of a pediatric patient with a left thalamic/midbrain juvenile pilocytic astrocytoma (JPA). The tumor displaced the corticospinal fibers posteriorly and resulted in hemiparesis. Using whole brain tractography to plan a corridor for the approach, neuronavigation, a tubular retractor and an exoscope for visualization, we obtained gross total resection of the tumor, while minimizing injury to white matter bundles, including the corticospinal fibers. We propose that surgical planning with whole brain tractography is essential for reducing morbidity while accessing deep-lying brain lesions via retractor tubes, by means of sparing critical fiber tracts.
PubMed: 29234572
DOI: 10.7759/cureus.1768 -
Journal of Neuro-oncology Jun 2017Medulloblastoma patients are treated with surgery, radiation and chemotherapy. Radiation dose to the temporal lobe may be associated with neurocognitive sequelae....
Medulloblastoma patients are treated with surgery, radiation and chemotherapy. Radiation dose to the temporal lobe may be associated with neurocognitive sequelae. Longitudinal changes of temporal lobe cortical thickness may result from neurodevelopmental processes such as synaptic pruning. This study applies longitudinal image analysis to compare developmental change in cortical thickness in medulloblastoma (MB) patients who were treated by combined modality therapy to that of cerebellar juvenile pilocytic astrocytoma (JPA) patients who were treated by surgery alone. We hypothesized that the rates of developmental change in cortical thickness would differ between these two groups. This retrospective cohort study assessed changes in cortical thickness over time between MB and JPA patients. High-resolution magnetic resonance (MR) images of 14 MB and 7 JPA subjects were processed to measure cortical thickness of bilateral temporal lobe substructures. A linear mixed effects model was used to identify differences in substructure longitudinal changes in cortical thickness. The left temporal lobe exhibited overall increased cortical thickness in MB patients relative to JPA patients who showed overall cortical thinning (mean annual cortical thickness change: MB 0.14 mm/year versus JPA -0.018 mm/year across all substructures), particularly in the inferior temporal lobe substructures (p < 0.0001). The cortical thickness change of the right temporal lobe substructures exhibited similar, though attenuated trends (p = 0.002). MB patients exhibit overall increased cortical thickness rather than cortical thinning as seen in JPA patients and as expected in normal cortical development. These observations are possibly due to chemoradiation induced-disruption of normal neuronal mechanisms. Longitudinal image analysis may identify early biomarkers for neurocognitive function with routine imaging.
Topics: Adolescent; Brain Neoplasms; Cerebral Cortex; Chemoradiotherapy; Child; Child, Preschool; Female; Functional Laterality; Humans; Infant; Longitudinal Studies; Magnetic Resonance Imaging; Male; Medulloblastoma; Radiation Injuries; Retrospective Studies; Young Adult
PubMed: 28534154
DOI: 10.1007/s11060-017-2453-5 -
PloS One 2017Growth monitoring of apparently healthy children aims at early detection of serious conditions through the use of both clinical expertise and algorithms that define...
BACKGROUND
Growth monitoring of apparently healthy children aims at early detection of serious conditions through the use of both clinical expertise and algorithms that define abnormal growth. Optimization of growth monitoring requires standardization of the definition of abnormal growth, and the selection of the priority target conditions is a prerequisite of such standardization.
OBJECTIVE
To obtain a consensus about the priority target conditions for algorithms monitoring children's growth.
METHODS
We applied a formal consensus method with a modified version of the RAND/UCLA method, based on three phases (preparatory, literature review, and rating), with the participation of expert advisory groups from the relevant professional medical societies (ranging from primary care providers to hospital subspecialists) as well as parent associations. We asked experts in the pilot (n = 11), reading (n = 8) and rating (n = 60) groups to complete the list of diagnostic classification of the European Society for Paediatric Endocrinology and then to select the conditions meeting the four predefined criteria of an ideal type of priority target condition.
RESULTS
Strong agreement was obtained for the 8 conditions selected by the experts among the 133 possible: celiac disease, Crohn disease, craniopharyngioma, juvenile nephronophthisis, Turner syndrome, growth hormone deficiency with pituitary stalk interruption syndrome, infantile cystinosis, and hypothalamic-optochiasmatic astrocytoma (in decreasing order of agreement).
CONCLUSION
This national consensus can be used to evaluate the algorithms currently suggested for growth monitoring. The method used for this national consensus could be re-used to obtain an international consensus.
Topics: Algorithms; Child; Consensus; Growth and Development; Humans; Interdisciplinary Studies; Pilot Projects
PubMed: 28448550
DOI: 10.1371/journal.pone.0176464 -
International Journal of Particle... 2017Proton therapy is increasingly used to treat pediatric brain tumors. However, the response of both tumors and healthy tissues to proton therapy is currently under...
PURPOSE
Proton therapy is increasingly used to treat pediatric brain tumors. However, the response of both tumors and healthy tissues to proton therapy is currently under investigation. One way of assessing this response is magnetic resonance (MR) diffusion tensor imaging (DTI), which can measure molecular mobility at the cellular level, quantified by the apparent diffusion coefficient (ADC). In addition, DTI may reveal axonal fiber directional information in white matter, quantified by fractional anisotropy (FA). Here we report use of DTI to assess tumor and unexposed healthy brain tissue responses in a child who received proton therapy for juvenile pilocytic astrocytoma.
MATERIALS AND METHODS
A 10-year-old boy with recurrent juvenile pilocytic astrocytoma of the left thalamus received proton therapy to a dose of 50.4Gy (RBE) in 28 fractions. Functional magnetic resonance imaging was used to select beam angles for treatment planning. Over the course of the 7-year follow-up period, magnetic resonance imaging including DTI was done to assess response. The MR images were registered to the treatment-planning computed tomography scan, and the gross tumor volume (GTV) was mapped onto the MR images at each follow-up. The GTV contour was then mirrored to the right side of brain through the midline to represent unexposed healthy brain tissue.
RESULTS
Proton therapy delivered the full prescribed dose to the target while completely sparing the contralateral brain. The MR ADC images obtained before and after proton therapy showed that enhancement corresponding to the GTV had nearly disappeared by 25 months. The ADC and FA measurements confirmed that contralateral healthy brain tissue was not affected, and the GTV reverted to clinically normal ADC and FA values.
CONCLUSION
Use of DTI allowed quantitative evaluation of tumor and healthy brain tissue responses to proton therapy.
PubMed: 31772991
DOI: 10.14338/IJPT-16-00024.1 -
BMJ Case Reports Dec 2016
Topics: Astrocytoma; Cerebellar Neoplasms; Cerebellum; Child; Computed Tomography Angiography; Cranial Fossa, Posterior; Fatal Outcome; Humans; Intracranial Aneurysm; Magnetic Resonance Imaging; Male; Subarachnoid Hemorrhage
PubMed: 27927709
DOI: 10.1136/bcr-2016-217583