Radiation therapy for optic nerve sheath meningiomas: Local control and treatment related visual changes.

Objective: Our primary objective is to evaluate the local control of optic nerve sheath meningiomas (ONSMs) treated with ionizing radiation and related visual changes after treatment. Our secondary objective is to describe the clinical characteristics and perform an analysis of the treatment impact on the functional status of this group of patients. Methods: We present our series of 19 patients treated with ionizing radiation therapy at our radio-neurosurgery unit between 2016 and 2022. The setting, ophthalmological follow-up, morbidity, and survival are analyzed and discussed. Results: Patients were followed up, and the impact of treatment on local disease control, visual alterations of the affected eye, and functional status of the patient were analyzed. The progression-free survival (PFS) median was 60 months (95% CI 50.3-69.6 months). The estimated PFS rates at 48 and 66 months were 100% and 66%, respectively. At diagnosis, nine (47.3%) eyes were in amaurosis and ten (52.6%) with vision. Of the ten patients without amaurosis at the time of diagnosis, three (30%) maintained unchanged visual acuity, and seven (70%) had decreased visual acuity; three of them developed amaurosis during the first year after treatment (p = 0.018). Conclusions: Using ionizing radiation therapy is a successful treatment for the local control of ONSMs. This therapeutic modality can compromise the visual acuity of the affected eye and improve dyschromatopsia and campimetry defects. The life prognosis is good for these patients, with a zero mortality rate, but their vision prognosis is poor.

Spinal Ependymomas: An Updated WHO Classification and a Narrative Review.

Ependymomas are neuroepithelial tumors that develop from ependymal cells found in the brain parenchyma and can spread to any part of the spinal cord. Three to six percent of all malignancies affecting the central nervous system (CNS) are ependymomas. Even the most talented surgeons are challenged by spinal cord ependymomas; as a result, research into this clinical phenomenon should continue. Since 1979, the World Health Organization (WHO) has published a classification and grading system for CNS malignancies to ensure consistent diagnostic standards worldwide. The WHO prepared an update on these tumors, paying particular attention to molecular techniques to categorize the therapeutic management of each patient with greater accuracy and clarity. We thoroughly reviewed the literature on the epidemiology, etiology, diagnosis, and treatment of spinal ependymomas since there has not been a recent review of these tumors. This included modifications to the 2021 WHO Classification of Tumors of the Central Nervous System.

Lhermitte-Duclos Disease: A Case Series.

Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma, is a rare benign tumor characterized by unilateral hemispheric cerebellar expansion. It is linked to mutations in the phosphatase and tensin homolog (PTEN) gene, which inhibit the phosphatidylinositol-3'-kinase pathway, leading to increased cell division and defective neuronal migration. This study aims to compare the clinical, radiological, histopathological, surgical resolution, and follow-up characteristics of reported cases of this rare condition. An in-depth search of LDD patients' clinical records at our institute between 2003 and 2023 was conducted, in addition to a systematic literature review on PubMed. Three patients with a diagnosis of LDD were found. Cerebellar abnormalities, varying headaches, and visual impairment were all present clinically. On T2 in the posterior fossa, all three MRI scans displayed the typical hyperintense parallel streak appearance. The histopathological report showed that large ganglion cells had replaced the granular layer, Purkinje cells had degenerated, the molecular layer had become hyper-myelinated, and synaptophysin and chromogranin were positive. Partial tumor resection and avoiding intracranial hypertension were the main goals of treatment. Genetic follow-up was conducted for all three patients. Neurosurgeons must be aware of LDD to provide close genetic monitoring despite the benign nature of the tumor because of its link to Cowden syndrome and elevated risk of cancer in other organs.

Effects and Assessment of the Optic Pathway After Management with Stereotactic Radiosurgery for Intracranial Tumors: A Comprehensive Literature Review.

Intracranial tumors are treated through a minimally invasive procedure called stereotactic radiosurgery (SRS), which uses precisely targeted radiation beams. When SRS is used to treat tumors in or near the optic pathway, which is responsible for transmitting visual information from the eyes to the brain, it is essential to assess the effects of treatment on visual function. The optic pathway is considered relatively radiation-sensitive, and high doses of radiation can lead to visual impairment or loss. Various methods can be used to assess the effects of SRS on the optic pathway, including visual acuity testing, visual field testing, and imaging studies. These assessments can be performed before and after treatment to track changes in visual function and detect potential complications or side effects. Assessing the optic pathway after management with SRS for intracranial tumors is essential to the treatment process to ensure that patients receive the best possible outcomes while minimizing the risk of complications. Close collaboration between the multidisciplinary team is often necessary to optimize treatment planning and monitoring of treatment response. In this review, we conducted an extensive analysis of the effects of radiation in patients with intracranial tumors after receiving radiotherapy.

Nelson's Syndrome: A Narrative Review.

Nelson's syndrome (NS) is an uncommon disease occurring as a complication of bilateral adrenalectomy (BLA) in patients with persistent Cushing's disease (CD) due to an adrenocorticotropin-producing pituitary tumor. The first reports of this syndrome were done in the 50s, although its pathophysiology is still not understood. Every year, between 1.8 and 2.6 cases are thought to occur per million people. It is characterized by hyperpigmentation, elevated adrenocorticotropic hormone (ACTH) plasma levels, and typical signs and symptoms related to pituitary adenomas, such as visual deficits due to optic pathway compression or decreased hormone production from the adenohypophysis. NS represents a challenge due to the lack of accepted diagnostic criteria and the complexity of its treatment. Moreover, the development of stereotactic radiosurgery (SRS) in the last few years has become an essential but controversial strategy for this syndrome. This review presents a comprehensive overview of NS.

Crusted Hyperkeratotic Scabies: A Case Report.

Crusted scabies is a rare form of classic scabies characterized by severe symptoms, mainly observed in immunosuppressed patients. This disease has been associated with a variety of health problems, such as delayed diagnosis, infection risk, and high mortality, mainly from sepsis. We report the case of a patient with hyperkeratotic scabies in the context of immunosuppression associated with malnutrition and the use of topical corticosteroids. Ivermectin is critical for successfully treating crusted scabies. However, a higher cure rate has been reported with the combination of oral ivermectin and topical permethrin. In our study, we chose to use a plan suitable for grade two scabies, resulting in a subtotal regression of the lesions. Crusted scabies is a highly contagious parasitic cutaneous disease, and there are few reports in the national and international literature. It is necessary to suspect this presentation form in order to establish a timely diagnosis and detect and treat associated comorbidities.