Search Results (43)

Background: Manually contouring the organs at risk (OARs) is a time-consuming process with significant variability. Multiple commercial options are available to streamline this process by utilizing artificial intelligence. This study compares the performance of two commercially available systems, MIM Contour ProtégéAI v4.0.0 (MIM) and Radformation/Limbus AI v1.7.0 (Limbus), in auto-contouring head and neck (H&N) OARs. Methods: We conducted a retrospective single-institution study with 20 patients who received curative-intent H&N radiation therapy. Seventeen OARs, bilateral where applicable, were manually contoured for each patient, which established a reference for comparison. Dice coefficient (DC), mean Hausdorff distance (HD_mean), and maximum Hausdorff distance (HD_max) were compared relative to the manual reference. Subjective contour quality was graded on a Likert scale. Wilcoxon signed-rank test with Benjamini–Hochberg false discovery rate procedures were used to compare outcomes. Results: Twenty patients were selected with a variety of H&N primary sites. The total number of OARs used for comparison was 480. Three quarters of patients were male, the mean age was 60.5 years (SD = 12.8), and the mean BMI was 23.53 kg/m² (range 16.9–42.4). MIM performed more consistently with the manual reference (adjusted p < 0.05) compared to Limbus when contouring the bilateral brachial plexus and mandible. Limbus performed more consistently than MIM when contouring the left lacrimal gland, larynx, optic chiasm, bilateral optic nerves, right parotid, and esophagus. The DC, HD_mean, and HD_max were not statistically different between MIM and Limbus for the remaining structures. Both AI algorithms had high performance on the mandible, eyes, thyroid, and salivary glands, and poor performance on the optic chiasm, lacrimal glands, and brachial plexuses. The subjective quality of the auto-contours from both systems were good, with most structures requiring no or minor changes only. The structures most likely to require revisions were the pharyngeal constrictors, larynx, and submandibular glands. Limbus achieved better subjective scores than MIM for four of seventeen structures. Conclusions: This comparison of two AI auto-contouring systems demonstrated performance consistent with the manual reference for most H&N OARs. Each system had strengths and weaknesses in auto-contouring specific OARs. Full article

Pituitary macroadenomas often cause visual pathway impairment due to optic chiasm compression. The association between systemic neurotrophic factors and visual recovery remains insufficiently explored. This prospective observational cohort study included 53 patients (106 eyes); 36 patients (72 eyes) completed a 12-month follow-up. Patients were assigned to a treatment group (surgical and/or pharmacological; n = 23) or an observation group (n = 13). Serum brain-derived neurotrophic factor (BDNF) and tumor necrosis factor-α (TNF-α) were measured at baseline and 12 months. Structural parameters (retinal nerve fiber layer [RNFL], ganglion cell–inner plexiform layer [GCIPL]) and visual field indices (mean sensitivity [MS], mean deviation [MD], square root of loss variance [sLV]) were assessed using optical coherence tomography and automated perimetry. Serum BDNF levels differed significantly between groups at baseline (p = 0.0022) and at 12 months (p < 0.0001), while TNF-α levels showed no significant changes. The treatment group demonstrated significant improvement in visual field parameters and modest RNFL thickening in the right eye (p = 0.0087). Baseline BDNF levels correlated inversely with OCT and visual field measures, particularly in non-functioning adenomas (R = −0.70 to −0.80, p < 0.01). Baseline BDNF predicted treatment qualification (AUC = 0.815). Pituitary macroadenomas are associated with visual dysfunction and systemic neurotrophic alterations. Elevated BDNF may reflect a compensatory neuroprotective response, supporting combined molecular and ophthalmic monitoring. Full article

Background: Differentiating hypophysitis from non-functioning pituitary macroadenomas (NFPMA) remains a clinical and radiological challenge. Both entities present as sellar masses with overlapping features but require distinct therapeutic approaches. Accurate preoperative identification is necessary to avoid unnecessary surgery in inflammatory forms. This review aims to compare the clinical, endocrine, and imaging characteristics of hypophysitis and NFPMA, incorporating recent findings and evaluating the performance of three diagnostic scoring systems currently in use. Methods: A comprehensive narrative literature review was conducted using original articles, clinical series, radiological studies, and systematic reviews retrieved from international databases. The analysis focused on demographic characteristics, clinical presentation, hormonal profiles, magnetic resonance imaging (MRI) features, and the comparative evaluation of the three published diagnostic scoring systems designed to differentiate hypophysitis from NFPMA. Results: Hypophysitis predominantly affects women, particularly during late pregnancy or the postpartum period, and is frequently associated with autoimmune diseases. Corticotropic deficiency and central diabetes insipidus (CDI) are disproportionately frequent in hypophysitis, whereas somatotropic deficiency is more characteristic of NFPMA. Radiologically, hypophysitis typically appears as a smaller, symmetric, and homogeneous mass with intense, uniform contrast enhancement, associated with pituitary stalk thickening and loss of the posterior pituitary bright spot. In contrast, NFPMA generally present as larger, asymmetric, and heterogeneous lesions, frequently invading the cavernous sinus and compressing the optic chiasm. Analysis of the three diagnostic scores indicates that combining clinical, hormonal, and imaging data improves accuracy compared to relying on single features. The most recent score includes hormonal markers, which significantly enhance sensitivity and specificity, emphasizing the importance of integrated assessment. Conclusions: No single clinical, hormonal, or imaging feature is pathognomonic. However, integrating clinical context, endocrine profile, imaging characteristics, and validated diagnostic scores significantly enhances preoperative diagnostic accuracy. The systematic use of composite scores may help optimize therapeutic decision-making and reduce unnecessary surgical interventions in patients with hypophysitis. Full article

Background: This study was designed as a dosimetric feasibility analysis to compare hippocampal-avoidance whole-brain radiotherapy (HA-WBRT) using 3D-CRT, IMRT, and VMAT techniques, with particular attention to clinical applicability in resource-limited settings. While 3D-CRT was used as a reference for conventional WBRT, the primary aim was to determine whether IMRT can serve as an effective and accessible alternative to VMAT for HA-WBRT in centers without advanced technology infrastructure. Methods: Fifteen patients undergoing WBRT for symptom relief were planned using 3D-CRT, IMRT, and VMAT on the Elekta Monaco 6.1.4.0 system. Key organs at risk (OARs) such as the optic nerves, chiasm, eyes, and lenses were considered in the treatment planning. Plans were evaluated based on PTV dose distribution, Conformity Index (CI), Homogeneity Index (HI), and OAR dose constraints (RTOG 0933, NRG-CC001). Gamma pass rate analysis (3%/3 mm) was performed for the IMRT and VMAT plans. Results: IMRT and VMAT significantly reduced the hippocampal dose compared to 3D-CRT, with similar PTV coverage and OAR sparing. The mean D_max for the hippocampus was 15.4 Gy for IMRT and 15.5 Gy for VMAT compared to 31.2 Gy for 3D-CRT. The D100% for the hippocampus was 7.5 Gy for IMRT and 7.6 Gy for VMAT, both well below the RTOG 0933 threshold of 9 Gy, while 3D-CRT delivered 30.3 Gy. Additionally, IMRT and VMAT delivered lower doses to the optic nerves and chiasm. QA results showed gamma pass rates above 96% for all plans. This study focused solely on treatment-planning and dosimetric feasibility without evaluating patient outcomes or clinical follow-up. Conclusions: HA-WBRT with IMRT and VMAT significantly reduced the hippocampal dose while maintaining optimal PTV coverage. VMAT is preferred for its balance of efficacy, protection, and treatment time, while IMRT represents a feasible approach for facilities without VMAT, though it requires stricter dose control and longer treatment times. Full article

We present a rare case of delayed retrobulbar and adrenal metastases from renal cell carcinoma (RCC), diagnosed 5.5 years after radical nephrectomy. The patient exhibited symptomatic orbital involvement, with imaging revealing a hypervascular retrobulbar mass and an incidental right adrenal lesion, indicative of an oligometastatic state. Owing to the patient’s refusal of surgical resection, stereotactic ablative radiotherapy (SABR) was delivered to the retrobulbar lesion at a total dose of 40 Gy in five fractions, concurrently with immune checkpoint inhibitor therapy. Treatment planning prioritized sparing adjacent critical structures, including the optic chiasm and brainstem. Follow-up over 4 years demonstrated sustained radiologic stability and volume reduction in both metastatic lesions without evidence of progression. This case underscores the potential efficacy of SABR in achieving durable local control of RCC metastases, particularly in anatomically constrained regions where surgery is unfeasible. Moreover, it highlights the value of a multidisciplinary, multimodal treatment approach incorporating advanced radiotherapy techniques and systemic immunotherapy. Lastly, it reinforces the importance of prolonged surveillance in RCC survivors due to the potential for late metastatic recurrence at uncommon sites. Full article

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of coronavirus disease 2019 (COVID-19), is known to affect multiple organ systems, including the respiratory tract and nervous and ocular systems. This retrospective study aimed to characterize the spatiotemporal distribution of viral antigen and associated pathological changes in the nose, lungs, brain, and eyes of K18-hACE2 mice intranasally infected with SARS-CoV-2. Using histology and immunohistochemistry, tissues were examined at 3, 6, and 7/8 days post-infection (dpi). In addition, lung and brain tissues were analyzed by means of RT-qPCR to determine viral RNA titers. Viral antigen was most pronounced in the nose, brain, and lung at 3, 6, and 7/8 dpi, respectively, whereas viral antigen was detected at 6 and 7/8 dpi in the retina. Quantitative PCR confirmed increasing viral RNA levels in both lung and brain, peaking at 7/8 dpi. Nasal and lung inflammation mirrored viral antigen distribution and localization. In the brain, the predominantly basal viral spread correlated with lymphohistiocytic meningoencephalitis, neuronal vacuolation, and altered neurofilament immunoreactivity. Retinal ganglion cells showed viral antigen expression without associated lesions. Microglial activation was evident in both the optic chiasm and the brain. These findings highlight the K18-hACE2 model’s utility for studying extrapulmonary SARS-CoV-2 pathogenesis. Understanding the temporal and spatial dynamics of viral spread enhances insights into SARS-CoV-2 neurotropism and its clinical manifestations. Full article

Background: The main mechanism of optic nerve damage in patients with pituitary adenoma (PA) is the pressure of optic chiasm. The retinal nerve fiber layer (RNFL), the ganglion cell layer (GCL)+, and GCL++ thickness measurement by optical coherence tomography (OCT), visual function evaluation, and magnetic resonance imaging (MRI) can be used to predict visual function recovery. In our study, we investigated the associations between visual acuity (VA), visual field (VF), RNFL, GCL changes, and the findings of MRI in patients with PA. Methods: This study was conducted in the Departments of Ophthalmology and Neurosurgery of the Lithuanian University of Health Sciences Hospital. A total of 25 patients diagnosed with PA were included in the study group, and 27 healthy subjects were included in the control group. The thickness of the RNFL and ganglion cell layer (GCL+, GCL++) and optic nerve disc diameter was analysed with OCT. Moreover, an MRI was performed for patients with PA. Results: The RNFL thickness around the optic disk measured preoperatively was reduced significantly in the temporal quadrant in PA patients compared with the control group (median (min; max); mean rank: 73.5 (52; 109); 58.39 vs. 69.5 (16; 168); 46.14; p = 0.038). We found that it was reduced significantly only in the inferior quadrant of the macro-PA group compared to the micro-PA group (median (min; max); mean rank: 99.5 (61; 115); 21.07 vs. 106.5 (90; 121); 32.15), p = 0.008, respectively). The RNFL thickness was reduced significantly only in the inferior quadrant of the non-active PA group compared to the active PA group (median (min; max); mean rank: 118.5 (49; 144); 17.42 vs. 130.5 (77; 156); 28.05), p = 0.028, respectively). RNFL thickness was reduced significantly only in the temporal quadrant in the PA with suprasellar extension group compared to the PA without suprasellar extension group (median (min; max); mean rank: 67.5 (16; 99); 21.66 vs. 72 (58; 168); 30.39), p = 0.036, respectively). Furthermore, GCL++ thickness was reduced significantly in total and in superior and inferior sectors of the PA with suprasellar extension group compared to the PA without suprasellar extension group (median (min; max); mean rank: 98.5 (57; 113); 21.8; 101 (61; 121); 21.48 and 102.5 (59; 116); 21.71 vs. 103.5 (95; 115); 30.2; 106.5 (90; 115); 30.61 and 104.5 (95; 113); 30.32), p = 0.043; p = 0.028 and p = 0.038, respectively). In the control group, significant positive correlations were found between optic disc area and the total RNFL thickness (r = 0.440, p < 0.001). In the PA group, significant correlations were observed between optic rim area and total RNFL thickness (r = 0.493, p < 0.001) and all quadrants, with the strongest in the nasal quadrant (r = 0.503, p < 0.001). A moderate to strong negative correlation was found between visual field (VF) defects and RNFL thickness, with the strongest correlation observed in the superior quadrant. Conclusions: OCT offers a detailed insight into the microscopic structural and functional changes throughout the entire visual pathway in patients with PA. Our findings demonstrate a significant negative correlation between RNFL thickness and visual field defects, highlighting the clinical relevance of OCT measurements in visual function assessment. Moreover, the results suggest that optic rim area may be a more reliable indicator of RNFL thickness variations than optic disc area in patients with PA. Full article

The study aimed to analyze hsa-miR-16-5p, hsa-miR-143-3p, hsa-miR-423-5p, hsa-miR-137-3p, hsa-miR-489-5p, hsa-miR-520-3p, hsa-miR-486-5p, and hsa-miR-200a-3p expression in the serum of patients with invasive non-functioning pituitary adenomas (NFPAs) and prolactinomas, as candidates for non-invasive biomarkers. The study included 62 patients with NFPAs and 18 with macroprolactinoma who qualified for transsphenoidal surgical resection. MicroRNAs were isolated from serum samples. The expression levels of hsa-miR-16-5p, hsa-miR-143-3p, hsa-miR-423-5p, hsa-miR-137-3p, hsa-miR-489-5p, hsa-miR-520-3p, hsa-miR-486-5p, and hsa-miR-200a-3p were determined using TaqMan MicroRNA assays. The statistical analyses were performed with MedCalc. The total concentration of microRNA was significantly lower in NFPAs than in the CG (p = 0.0419). ROC curve analysis showed that the cutoff point of miRNA lower than 10.73 predicted the PA (sensitivity = 70.0%; specificity = 57.7%; AUC = 0.629; p = 0.052). No correlation between selected miRNAs and tumor type was found: hsa-miR-143-3p (p = 0.4610), hsa-miR-16-5p (p = 0.8767), and hsa-miR-423-5p (p = 0.1459). miRNA expression also did not correlate with invasiveness (cavernous or sphenoid sinus invasion, optic chiasm compression). Although the total expression of microRNA was significantly lower in NFPAs, hsa-miR-16-5p, hsa-miR-143-3p, and hsa-miR-423-5p are not useful as non-invasive biomarkers in patients with invasive non-functioning pituitary adenomas and prolactinomas. Full article

Chronic fungal sinusitis (FS) can cause bone erosion and extend to the sellar region, often misdiagnosed as pituitary tumors or malignancies. We report a 56-year-old immunocompetent female with sphenoid FS presenting as a giant sellar mass compressing the optic chiasm, with normal pituitary function. The surgery successfully resolved her symptoms, and a histological examination confirmed the presence of a fungal hyphal mass. We conducted a literature review of 52 publications on FS cases with bone erosion and inflammatory extension to the sellar region, which included analyses of 67 patients (35 females, mean age 49.6 years, half immunocompetent). The most common symptom was headache (73.1%), followed by visual complaints (71.7%), visual deterioration (40.3%), ophthalmoplegia (38.8%), and visual field defects (13.4%). Symptom duration averaged 4.5 months in 65.7% of cases. Aspergillus was the most frequent (71.6%). Hormonal abnormalities included hypopituitarism (25.4%) and hyperprolactinemia (13.4%). Surgery was performed in 92.5% of patients. Common diagnoses included pituitary abscess (41.8%), fungal granuloma (16.4%), aspergillosis (16.4%) and allergic FS (14.9%). Antifungal therapy was administered in 53.7% of cases. Cure was achieved in 67.2%, while the mortality rate was 10.4%. Early recognition of fungal involvement, supported by a multidisciplinary approach, is essential for the accurate diagnosis and effective treatment. This highlights the need for vigilance to improve the outcomes in similar cases. Full article

Background: The current standard therapy for pediatric optic pathway/hypothalamic glioma (OPHG) is systemic anticancer therapy (SAT) over surgery and radiotherapy. Nevertheless, recurrent radiological or clinical tumor progression after SAT forms a considerable challenge. Sporadic OPHGs are considered to have a higher tendency toward progression after first-line systemic anticancer therapy (SAT) compared to neurofibromatosis type-1-associated (NF1) OPHGs. Methods: The objective of this study was to conduct a national retrospective cohort analysis of children who received various treatments for a progressive OPHG, involving the hypothalamus and/or chiasm and/or optic radiations. The study aimed to examine the differences in clinical course and the range of treatment modalities applied to both sporadic and NF1-associated OPHGs between 1995 and 2020. Additionally, we sought to identify risk factors for 3- and 5-year progression following first- and second-order SAT. Results: In total, 136 children received treatment, of whom 49 of 136 (36.0%) had NF1. Within a median of 7.5 years (range: 0.1–23.8 years) of follow-up, sporadic OPHGs received more treatments compared to NF1-associated OPHGs (median of 2 (range: 1–8) vs. median of 1 (range: 1–7) (p < 0.01)). Nine children with sporadic OPHGs (6.6%) died. Of 112 children (82.4%) receiving SAT, 92% received combined first-line vincristine and carboplatin. These children had a 3- and 5-year progression-free survival of 61.8% (95% CI: 51.0–72.6%) and 48.4% (95% CI: 38.0–58.8%), respectively. Sporadic OPHGs had a higher rate of second progression (p < 0.01). Starting first-line vincristine and carboplatin at an age below one year was the only independent risk factor for progression. Conclusions: In this national historic cohort of pediatric OPHGs, four out of five children received SAT. Sporadic OPHGs received a higher number of various SATs compared to NF1-associated OPHGs, but the sporadic appearance of OPHGs was not an independent risk factor for progression after combined vincristine and carboplatin, as ‘age below one year at the start’ was the only factor. Full article

Background/Objectives: Spindle cell oncocytomas (SCOs) of the pituitary gland are rare tumors often misdiagnosed for nonfunctioning pituitary macroadenomas. Although classified as grade 1, they are often challenging in terms of diagnosis and treatment. Pituitary SCOs harbor peculiar features such as hypervascularity and stronger adherence to surrounding structures, with increased risk of hemorrhage, partial resection, and significantly higher recurrence rate. Almost 100 cases have been reported so far. The role of surgery is still crucial for the decompression of the optic chiasm as well as for achieving diagnosis. However, given the higher tendency of recurrence, the role of postoperative radiotherapy has been investigated over the last few years. Case presentation: Here, we reported a case of a 48-year-old female with a pituitary SCO treated at our institution, in which we focused on diagnosis, treatment, and follow-up. Conclusions: This type of tumor presents a challenge related to its higher vascularity and strong adherence to the surrounding structures. Adjuvant radiotherapy is something that should be considered, especially when gross total resection is not achieved, and finally, SCOs require diligent follow-up to monitor for any signs of disease recurrence or progression. Full article

Background: optic nerve glioblastoma is an uncommon pathology. The optic chiasm, optic tract, or optic nerves are possible places from which the tumor can originate. Most of the neuroimaging findings are nonspecific. To confirm the diagnosis, a biopsy is required. A delay to the treatment plan for optic nerve glioblastoma results in poor patient survival rates. Case report: a 68-year-old woman with an uncomplicated medical history presented with exophthalmos, deteriorating eyesight, and partial loss of vision. Using radiological data together with postoperative histopathological and histochemical analysis, optic nerve glioblastoma, IDH-wildtype, with optic chiasm involvement was diagnosed. Conclusion: optic nerve glioblastoma is a rare and aggressive form of cancer that affects the optic nerve, leading to significant vision impairment and potentially life-threatening complications. Treatment options are restricted and difficult because of the location and nature of the condition; surgery, radiation therapy, and chemotherapy are frequently needed as part of a multidisciplinary approach. Full article

Background: Proton therapy requires caution when treating patients with targets near neural structures. Intuitive and quantitative guidelines are needed to support decision-making concerning the treatment modality. This study compared dosimetric profiles of intensity-modulated proton therapy (IMPT) and intensity-modulated radiation therapy (IMRT) using helical tomotherapy (HT) for adaptive re-planning in cT3-4 nasopharyngeal cancer (NPCa) patients, aiming to establish criteria for selecting appropriate treatment modalities. Methods: HT and IMPT plans were generated for 28 cT3-4 NPCa patients undergoing definitive radiotherapy. Dosimetric comparisons were performed for target coverage and high-priority organs at risk (OARs). The correlation between dosimetric parameters and RT modality selection was analyzed with the target OAR distances. Results: Target coverages were similar, while IMPT achieved better dose spillage. HT was more favorable for brainstem D₁, optic chiasm D_max, optic nerves D_max, and p-cord D₁. IMPT showed advantages for oral cavity D_mean. Actually, 14 IMPT and 14 HT plans were selected as adaptive plans, with IMPT allocated to most cT3 patients (92.9% vs. 42.9%, p = 0.013). The shortest distances from the target to neural structures were negatively correlated with OAR doses. Receiver operating characteristic curve analyses were carried out to discover the optimal cut-off values of the shortest distances between the target and the OARs (temporal lobes and brainstem), which were 0.75 cm (AUC = 0.908, specificity = 1.00) and 0.85 cm (AUC = 0.857, specificity = 0.929), respectively. Conclusions: NPCa patients with cT4 tumor or with the shortest distance between the target and critical neural structures < 0.8 cm were suboptimal candidates for IMPT adaptive re-planning. These criteria may improve resource utilization and clinical outcomes. Full article

Background: Although visual field (VF) defects are common in compressive pituitary adenoma (CPA), their pathophysiology has not been fully elucidated. The mechanical theory (i.e., direct compression of the optic chiasm by the CPA) and the vascular theory (i.e., compression of the vessels supplying the visual path by the CPA) or their association could explain the visual impairment. The aim of this study was to determine whether the vascular density (VD) improved after surgical decompression of the optic chiasm in CPA patients and whether OCT-A could help to identify predictive factors for postoperative visual recovery. Methods: A prospective controlled study was conducted in patients who underwent transsphenoidal pituitary adenoma surgery. Patients were divided into two groups: with CPA and without CPA (NCPA). All patients underwent a neuro-ophthalmological examination, VF testing, macular and optic disc structural OCT [retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thicknesses] and OCT-A before and then 1 and 6 months after surgery. Results: Twenty-four eyes and fourteen eyes were included, respectively, in the CPA and NCPA groups. None of the VD parameters assessed by OCT-A were significantly improved after surgery in the CPA group. In the CPA group, the mean macular superficial VD was significantly decreased at 6 months. The multivariate analysis failed to identify any preoperative parameters predictive of postoperative VF improvement. Conclusions: Our preliminary findings suggest that the visual impairment observed in CPA patients could not be explained by the vascular theory. None of the preoperative OCT-A parameters allowed a postoperative VF recovery assessment. Trial registration number NCT04074642, ID-RCB 2019-A01186-51 date of registration 30 July 2019. Full article

Neurofibromatosis type 1 (NF1) is an inherited autosomal dominant disorder primarily affecting children and adolescents characterized by multisystemic clinical manifestations. Mutations in neurofibromin, the protein encoded by the Nf1 tumor suppressor gene, result in dysregulation of the RAS/MAPK pathway leading to uncontrolled cell growth and migration. Neurofibromin is highly expressed in several cell lineages including melanocytes, glial cells, neurons, and Schwann cells. Individuals with NF1 possess a genetic predisposition to central nervous system neoplasms, particularly gliomas affecting the visual pathway, known as optic pathway gliomas (OPGs). While OPGs are typically asymptomatic and benign, they can induce visual impairment in some patients. This review provides insight into the spectrum and visual outcomes of NF1, current diagnostic techniques and therapeutic interventions, and explores the influence of NF1-OPGS on visual abnormalities. We focus on recent advancements in preclinical animal models to elucidate the underlying mechanisms of NF1 pathology and therapies targeting NF1-OPGs. Overall, our review highlights the involvement of retinal ganglion cell dysfunction and degeneration in NF1 disease, and the need for further research to transform scientific laboratory discoveries to improved patient outcomes. Full article