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Purpose: The purpose of this study was to evaluate the morphologic magnetic resonance imaging (MRI) characteristics of the pineal gland in retinoblastoma (Rb) patients without and with pineoblastoma in comparison to age-matched controls to improve early identification of pineoblastomas (trilateral retinoblastoma, TRb)., Methods and Materials: 80 patients with retinoblastoma and 80 age-matched controls who had undergone brain MRI were included in this retrospective institutional review board approved cohort study. Two readers analyzed the following MR characteristics of the pineal gland: signal intensity on T1- and T2-weighted images, enhancement pattern, delineation of the gland, presence of cystic component, size of pineal gland and size of pineal cysts, respectively. A third reader assessed all images for the presence or absence of pineoblastoma., Results: 3 patients were positive (TRb cohort) and 77 negative for pineoblastoma (non-TRb cohort). The mean maximum diameter of the pineal gland was 6.4mm in Rb patients and 6.3mm in age-matched controls. The mean volume of the pineal gland in Rb patients was 93.1mm(3) and was 87.6mm(3) in age-matched controls. Considering all available MRI scans the mean maximum diameter of the pineal gland in TRb patients was 11.2mm and the mean volume in TRb patients was 453.3mm(3). The third reader identified pineoblastomas with a sensitivity of 100% (3 of 3) and a specificity of 94% (72 of 77)., Conclusion: Our non-TRb patients did not show significant differences in the size of the pineal gland and pineal gland cysts compared to age-matched controls. The presented data can serve as a reference for the volume of normal pineal glands and pineal cysts in the diagnostic work-up of Rb patients with suspected pineoblastoma., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Dynamic contrast-enhanced MRI is used for the assessment of microvasculature in several tumours. We aimed to assess the contrast signal enhancement characteristics of ocular melanoma. Forty patients with ocular melanoma were prospectively investigated with ocular MRI including dynamic contrast-enhanced sequences over a 13-month period. A region-of-interest analysis of the images was carried out to calculate signal enhancement characteristics after a contrast injection. Clinical follow-up data such as extraocular spread and development of liver metastasis were compared with the signal enhancement characteristics of the ocular melanoma. In 39 patients (98%), the ocular melanomas showed an early strong signal enhancement after contrast injection, resulting in a mean time of maximum enhancement of 49 s. Clinical follow-up was available in 28 patients (70%) and indicated that the peak signal intensity was significantly increased (P=0.039) in patients who developed extraocular spread or liver metastasis at a later stage. Ocular melanoma shows signal enhancement characteristics of hypervascular neoplasms. This study provides baseline curve pattern data that may be useful for assessing changes in vascularity, for example during therapy response. Furthermore, the study showed that a strong signal enhancement of the ocular melanoma might be linked to a less favourable prognosis., (Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To evaluate long-term outcomes of proton beam radiotherapy in the treatment of choroidal melanoma of the intermediate zone of the fundus., Design: Retrospective interventional single-center study., Methods: The study was a retrospective analysis with long-term follow-up of 62 patients with a minimum tumor-to-disc and tumor-to-fovea distance of 2 mm of choroidal melanoma in the intermediate zone of the fundus. Mean values of tumor prominence, largest basal diameter, and tumor distances to the optic disc and fovea were 7.6, 12.8, 5.2, and 4.6 mm, respectively. All patients were irradiated with a total proton dose of 60 cobalt gray equivalents., Results: After proton beam radiotherapy, 71.0% of the patients received subsequent endoresection of the tumor. Only 18 patients (29.0%) did not require additional tumor resection and were analyzed as a separate group. For the total of patients, the median follow-up time was 70.3 months. The 5-year Kaplan-Meier rates of local tumor relapse, enucleation, and distant metastasis were 3.9%, 3.7%, and 13.4%, respectively. Cataract surgery was the most frequent secondary treatment in our cohort., Conclusions: In this study we demonstrate the effectiveness of proton beam irradiation in tumor control and preservation of the globe in the analyzed patients. The rate of metastasis was not higher than previously described. Nevertheless, consecutive tumor surgery is frequently required to maintain the eye in patients with large-sized choroidal melanomas. In conclusion, proton beam radiotherapy allows preservation of the eye in mid-zone choroidal melanomas., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Choroidal melanoma is frequently accompanied by an exudative retinal detachment that can persist after proton beam therapy. This study investigates whether vitrectomy without tumor resection improves the clinical outcome., Methods: This is a retrospective interventional case series. Twenty patients with choroidal melanoma with exudative retinal detachment involving the macula were treated by vitrectomy, endodrainage, photocoagulation, and silicone oil tamponade after proton beam therapy., Results: The mean follow-up was 38.4 months (median 21.5, range 12.0-122.0). The mean time between proton beam therapy and surgery was 4.5 months (range 0.1-9.2). Reattachment was achieved in 95% of the patients after one vitrectomy. One patient was lost to follow-up because enucleation was performed after 45.9 months due to a secondary glaucoma. Mean visual acuity decreased from 1.1 to 1.8 logMAR before vitrectomy and after 4 years, respectively. No patient showed local tumor recurrence. Metastatic disease was present in 1 patient after 15.2 months, and this patient died after 19.2 months., Conclusion: Vitrectomy is indicated after therapeutic proton beam irradiation in patients who present with persisting exudative retinal detachment involving the macula and high local or systemic risk factors for hemorrhagic complications, thus excluding endoresection.

Background: Prognosis evaluation of patients with choroidal and ciliary melanoma has experienced recent progress through tumour sampling and cytogenetic analysis of metastatic risk. By allocating tumor extension, height and linear basal diameter to defined TNM stages, an estimation of prognosis can also be made without invasive tissue sampling., Methods: Therapeutic strategies of organ preserving irradiation using different sources have clearly come to the forefront., Results: Due to microscopic haematogenous spreading of tumour cells prior to treatment, the metastatic risk following radiation of any form is not influenced in comparison to primary enucleation., Conclusion: However, metastatic disease still remains a fatal condition which currently may only be influenced by early detection and treatment of uveal melanomas., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: Diffusion-weighted imaging (DWI) allows characterization of masses on the basis of their cellular density. We hypothesized that ocular melanoma has a marked diffusion restriction as seen in other malignant tumors. Furthermore, we aimed to assess whether DWI is useful to differentiate ocular melanoma from retinal detachment., Materials and Methods: The institutional review board approved the prospective study on 44 patients investigated with ocular magnetic resonance imaging including DWI during a 9-month period. A region-of-interest analysis of diffusion-weighted images with b values of 0 and 1000 s/mm was performed to calculate the apparent diffusion coefficient (ADC) of the ocular melanoma and the retinal detachment. Three patients were excluded because DWI was nondiagnostic owing to severe artifacts; in 1 patient, the melanoma was too small for ADC calculation. Therefore, 40 patients were included in the final analysis. Ocular melanomas and detachments were compared with respect to their ADC values. The image quality of DWI was qualitatively scored by 2 readers in consensus on a 3-point scale from 1 (minor artifacts) to 3 (major artifacts)., Results: Ocular melanomas showed a marked diffusion restriction, and the mean (SD) ADC was 891 (172) × 10 mm/s. Twenty-nine patients (66%) had retinal detachment. The mean ADC of the ocular melanoma differed significantly (P < 0.001) from the mean ADC of the retinal detachment (1986 [375] × 10 mm/s). The image quality of DWI was rated 1 in 38 patients, 2 in 3 patients, and 3 in 3 patients., Conclusions: Ocular melanoma shows a marked diffusion restriction with an ADC of less than 1000 mm/s, which is in concordance with other malignant tumor entities. Diffusion-weighted imaging helps differentiating ocular tumors from retinal detachment and should therefore be included in the ocular magnetic resonance imaging protocol if an ocular mass is suspected.

Purpose: Generally, high-resolution MRI of the eye is performed with small loop surface coils. The purpose of this phantom and patient study was to investigate the influence of magnetic field strength and receiver coils on image quality in ocular MRI., Materials and Methods: The eyeball and the complex geometry of the facial bone were simulated by a skull phantom with swine eyes. MR images were acquired with two small loop surface coils with diameters of 4 cm and 7 cm and with a multi-channel head coil at 1.5 and 3 Tesla, respectively. Furthermore, MRI of the eye was performed prospectively in 20 patients at 1.5 Tesla (7 cm loop surface coil) and 3 Tesla (head coil). These images were analysed qualitatively and quantitatively and statistical significance was tested using the Wilcoxon-signed-rank test (a p-value of less than 0.05 was considered to indicate statistical significance)., Results: The analysis of the phantom images yielded the highest mean signal-to-noise ratio (SNR) at 3 Tesla with the use of the 4 cm loop surface coil. In the phantom experiment as well as in the patient studies the SNR was higher at 1.5 Tesla by applying the 7 cm surface coil than at 3 Tesla by applying the head coil. Concerning the delineation of anatomic structures no statistically significant differences were found., Conclusion: Our results show that the influence of small loop surface coils on image quality (expressed in SNR) in ocular MRI is higher than the influence of the magnetic field strength. The similar visibility of detailed anatomy leads to the conclusion that the image quality of ocular MRI at 3 Tesla remains acceptable by applying the head coil as a receiver coil., (© Georg Thieme Verlag KG Stuttgart · New York.)

Hypoxia and its down-stream activated pathways are commonly involved in tumor progression. Genes involved in angiogenesis and glycolysis, i.e. vascular endothelial growth factor (VEGF) and lactase dehydrogenase A (LDHA), respectively, are transcriptionally controlled by the hypoxia inducible factors 1α and 2α (HIF1α and HIF2α). A series of 60 uveal melanomas were immunohistochemically assessed for the expression of VEGF and the phosphorylated/activated form of VEGF receptor 2 (pVEGFR2/KDR), after binding to VEGF. The expression of HIF1α, HIF2α and LDH5 was also investigated. Uveal melanomas overexpressing HIF2α (but not that of HIF1α) were significantly associated with high VEGF (P = 0.005), pVEGFR2/KDR (P < 0.0001) and LDH5 (P ≤ 0.0001). High LDH5 was linked with tumor necrosis (P = 0.01) and increased tumor size (P = 0.03). High VEGF was linked with phosphorylated pVEGFR2/KDR receptors. In univariate analysis high pVEGFR2/KDR receptor expression was significantly related with poor prognosis (P = 0.02). It is concluded that HIF2α plays an important role in the progression of uveal melanomas possibly by promoting the autocrine loop VEGF-pVEGFR2/KDR, and by enhancing the expression of LDHA gene, conferring thus a growth advantage. As pVEGFR2/KDR expression was significantly related with poor prognosis, inhibitors of this receptor may improve the clinical outcome of patients with pVEGFR2/KDR overexpressing uveal melanomas.

Purpose: The black iris-lens diaphragm (ILD) can be used in the treatment of traumatic aniridia and aphakia. The aim of our study was to show postoperative functional and anatomic results and complications in a small case series., Methods: We retrospectively analyzed the files of 16 patients managed with a black ILD or a sole iris diaphragm in the period 1994-2007. Four of them were female and 12 were male. The mean age of the group was 50±17 years. At the time of the implantation, all of the eyes had already undergone primary surgical repair., Results: The preoperative best-corrected visual acuity in half of the patients was =0.1 and remained stable after the first postoperative year. During the follow-up years, one eye developed a subluxation of the implant and one eye an anterior chamber hemorrhage. At the same time, out of 8 eyes carrying a silicone tamponade in the vitreous cavity, silicone oil entered the anterior chamber in 3 cases. In one case, enucleation was undertaken due to phthisis., Conclusions: In the case of severely traumatized eyes with aniridia and aphakia, the implantation of a black ILD can have a positive effect on functional and anatomic stabilization.

Aims: To describe the long-term tumour control and metastatic rate after transscleral resection (TSR) of large uveal melanomas in a single-centre study., Methods: The sample included 210 patients with large uveal melanomas. Univariate analysis of local tumour control and metastatic risk by Kaplan-Meier survival curves and log-rank testing. Cox proportional HR analysis with forward and backward selection was used to identify independent prognostic factors in patients submitted to TSR of a large uveal melanoma., Results: A residual tumour was diagnosed in 6% of the patients. The 5- and 10-year local tumour recurrence rates were 24% and 32%, respectively. Older age, a large basal tumour diameter, the lack of adjuvant ruthenium brachytherapy and retinal detachment led to a 2.6, 2.4, 4.4 and 7.8 times higher risk of melanoma recurrence, respectively. The 5- and 10-year metastatic rates were 28% and 44%, respectively, and were statistical significantly affected by extraocular spread, tumour thickness and local tumour recurrence., Conclusions: TSR is an alternative to enucleation for the treatment of large uveal melanomas. Results should improve with better patient selection and more effective methods of adjuvant radiotherapy.