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Simple Summary: This study assesses for the first time the clinical outcomes in a group of adolescent and young adult (AYA: 15–39 years old) uveal melanoma (UM) patients (n = 270) treated with proton therapy, comparing them with matched elder adults (>40 years). Ocular outcomes, local tumor control, metastasis incidence, and overall survival were similar in both groups, in contrast with the better survival rates in previous, pediatric UM series. Moreover, when compared with the general population, the AYAs had reduced relative survival, while the elder adults had better relative survival. Objective: The aim of this study was to compare the clinical outcomes of adolescents and young adults (AYAs) with those of elder adult patients treated with proton therapy (PT) for uveal melanoma (UM). Material and Methods: A retrospective, comparative study was conducted in UM patients who underwent PT at the Ocular Oncology Unit of the Jules-Gonin Eye Hospital (University of Lausanne, Lausanne, Switzerland) and the Paul Scherrer Institute (PSI); (Villigen, Switzerland) between January 1997 and December 2007. Propensity score matching (PSM) was used to select for each AYA (between 15–39 years old) an elder adult patient (≥40 years) with similar characteristics. We assessed ocular follow-up, local tumor control, metastasis incidence, and overall and relative survival (OS and RS). Non-terminal outcomes were then compared between the two groups using competing risk survival analysis. Results: Out of a total of 2261 consecutive UM patients, after excluding 4 children (<15 years) and 6 patients who were metastatic at presentation, we identified 272 AYA patients and matched 270 of them with 270 elder adult patients. Before PSM, the AYA patients had a higher incidence of primary iris melanoma (4.0% vs. 1.4%; p = 0.005), while the elder patients were more likely to have other neoplastic diseases at presentation (9% vs. 3.7%; p = 0.004). Ocular outcomes and local tumor control were similar in both groups. Cumulative metastasis incidence for the AYA and elder adult groups was 13% and 7.9% at 5 years and 19.7% and 12.7% at 10 years, respectively, which was not significantly different between the groups (p = 0.214). The OS was similar in the two groups (p = 0.602), with estimates in the AYA and elder adult groups of 95.5% and 96.6% at 5 years and 94.6% and 91.4% at 10 years, respectively. However, the relative survival (RS) estimation was worse in the AYA group than the elder group (p = 0.036). Conclusion: While AYAs treated with PT for UM have similar ocular outcomes and present the same metastasis incidence and OS as elder adults, their RS is worse than that in elder adults, when compared with the population in general. [ABSTRACT FROM AUTHOR]

Uveal melanoma (UM) is the most common primary intraocular tumor in adults and usually has a very poor prognosis. Histologically, UMs have been classified in epithelioid cell type, spindle cell type, and mixed cell type. Balloon cells are large pale cells that contain small, hyperchromatic, central nuclei with vesiculated, clear, and lipid-rich cytoplasm. A balloon cell morphology is infrequently observed in naevi and even less frequently in malignant melanomas of the skin, conjunctiva, ciliary body and choroid. In this regard, UMs that exhibit balloon cell features are generally those previously treated with proton beam irradiation and then enucleated, rather than those that directly underwent primary surgery. To the best of our knowledge, very few cases of primary UM showing extensive balloon cell morphology have been reported in scientific literature to date. We herein present an unusual case of primary UM with diffuse balloon cell changes in a 69-year-old woman. [ABSTRACT FROM AUTHOR]

Background: Cumulative evidence suggests that the risk of eye tumors varies among different age groups and populations. The purpose of the present study was to assess the age distribution of eye tumors in China. Methods: In this retrospective study, the age distribution of various types of eye tumors was analyzed on surgically excised and histologically confirmed specimens obtained from 4492 patients (4526 eyes), collected between 2001 and 2017. Results: Of the 4526 specimens, 3156 eyes (69.7%) had benign eye tumors, while 1370 eyes (30.3%) had malignant tumors. The age-specific incidence of eye tumors was characterized by a bimodal distribution, one peak occurred at age 0–9 years (19.7%) and the other at 50–59 years (14.7%) of age. Malignant eyelid tumors were very rare under the age of 20 years, but increased to 78% of all eyelid tumors by the age of 70 years. Children aged 0–9 years old were 6.5 times as likely to have a malignant eye tumor (95% CI, 4.1–10.4) as those aged 10–19 years. The age-related variation of eye tumors was also observed in the top ten categories of both benign (p < 0.001) and malignant types (p = 0.001). Conclusions: These results showed that age is a major factor determining the type of eye tumor, confirmed by histopathological analysis.

Introduction: Depth-dose distribution curve of protons in the matter has a maximum is called Bragg peak. Bragg peak of a monoenergetic proton beam is too narrow. The spread out Bragg peak should be created for full coverage of the tumor. The spread out Bragg peak is obtained in the depth of the tumor with superposition of the several Bragg peaks. The aim of this study was coverage of an eye tumor in the proton therapy while healthy eye tissue absorbs less radiation. Methods: In this analytical study, the simulations were performed using MCNPX code. A tumor in the eye phantom was considered. The eye phantom has been irradiated with different proton beam energy. A Polystyrene modulator wheel was used for creating the spread out Bragg peak in the tumor region. Results: Bragg peaks were created in different depths of the tumor, by varying the proton beam energies from 20 MeV to 38 MeV. Bragg peak of the 32.85 MeV proton beam energy was precisely placed at the end of the tumor. Different pristine Bragg peaks were produced using a Polystyrene modulator wheel with different thicknesses and 32.85 MeV proton beam energy. The spread out Bragg peak was created in the tumor region by modulation of the pristine Bragg peaks. Neutrons and photons are produced by the inelastic nuclear interactions of protons with the nuclei of different tissues of eyes. The flux and absorbed dose of secondary neutrons and photons were considerably small compared to the depth-dose distribution of protons and the total absorbed dose in the tumor was more than other tissues of eyes. Conclusion: Using a modulator wheel the tumor can be treated, so that the minimal damage reaches the surrounding tissues. The results show that more than 92% of the total dose of secondary particles and protons is absorbed in the tumor.

Purpose Iris neoplasm is a non-symptom cancer that causes a gradual loss of sight. The first purpose of this study was to present a novel and automatic method for segmenting the iris tumors and detecting the corresponding areas changing along time. The second aim of this work was to investigate several recently published methods after being applied for the iris tumors segmentation. Methods Our approach consists firstly in segmenting the iris region by using the Vander Lugt correlator based active contour method. Secondly, by treating only the iris region, a K-means clustering model was used to assign the tumorous tissue to one pixel-cluster. This model is quite sensitive to the center initialization and to the choice of the distance measure. To solve these problems, a proportional probability based approach was introduced for the cluster center initialization, and the impact of several distance measure was investigated. The proposed method and the different comparative methods were evaluated on two databases: the Eye Cancer and the Miles Research. Results Results reported using several performance metrics reveal that the first step assures the detection of all iris tumors with an accuracy of 100%. Additionally, the proposed method yields better performance compared to the recently published methods. [ABSTRACT FROM AUTHOR]

Several ancillary tests could be used in the diagnosis, treatment, and management of complications of eye tumors. Among those, ultrasound biomicroscopy has a particular role in the evaluation of anterior segment tumors. Optic coherence tomography and fundus autofluorescence have values in determining the architecture of posterior segment tumors and the effects of tumors on the adjacent structures. However, it should be noted that those tests have a meaning when they are evaluated together with the clinical features of the lesions. (Turk J Ophthalmol 2014; 44: Supplement 66-70)

Neurofibromatosis type 2 (NF-2) is characterized by multifocal proliferation of neural crest-derived cells. The characteristics finding of NF-2 is bilateral vestibular schwannomas. Combined hamartoma of retina and retinal epithelium (CHRRPE) is another associated finding. A 9 year-old-male child presented with left eye decreased vision for 3 months. Visual acuity was 0.0 and 0.8 LogMAR in the right and left eye, respectively. Left fundus showed an elevated, pigmented lesion with surface wrinkling and vascular tortuosity suggestive of CHRRPE with multiple presumed retinal astrocytic proliferations in mid-periphery. He had multiple café-au-lait spots. Optical coherence tomography confirmed clinical findings. Magnetic resonance imaging brain showed bilateral acoustic neuroma. Recognition of this rare finding as presenting feature of NF-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention. It is recommended that children with CHRRPE be screened for NF-2.

We conducted a retrospective review of 11 eyes undergoing eye wall resection between October 1998 and October 2009. The median age of 11 patients was 29 years. Decreased vision (eight) was the most common presenting symptom. Ciliary body medulloepithelioma was the most common clinical diagnosis (six). Medulloepithelioma was the most common histopathological diagnosis (four). The duration of follow-up ranged from 0.5 to 67 months (median 11 months). Three eyes needed to be enucleated in the postoperative period (margin involvement two eyes, recurrence one eye). Postoperative complications among others included retinal detachment (three), vitreous hemorrhage (three), cataract (two), and suprachoroidal hemorrhage (two). To conclude, prognosis of this procedure continues to be guarded needing close postoperative follow-up.

Background and Objective: Distribution of dose depends on the treatment field and the presence of multiple collimators sheet at the direction of the photons. The aim of this study is evaluating and comparing the effects of the presence and absence of electrons contamination on the dose distribution with the use of computed tomography. Subjects and Methods: Varian linear accelerator (2100 C / D) was simulated with Primo and BEAMnrc codes for photon with 5.9 MV nominal energy. This study was done for 15 patients with eyes tumor undergoing single field photon therapy. Results: The maximum depth dose treatment volume aggregation in the presence and the absence of electrons contamination was occurred at 1.3cm3 and 2cm3, respectively. Differential dose volume histogram concluded 80% of total dose in the presence and absence of electrons, 90%±0.5 and 80%±2 of the eye volume covered, respectively. Conclusion: The main goal of radiation therapy is delivering the highest dose to the planning treatment volume and minimal radiation dose to organs at risk. In this case, the percent of the eye and treatment volume which taken 80% and 90% dose has 10% reduction and 5% increased by removing electrons contamination, respectively. [ABSTRACT FROM AUTHOR]

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ABSTRACT Objective: One of the primary factors in managing patients with retinoblastoma is early diagnosis. The main idea of this study was to recognize the consequences of delay in diagnosis on therapy of the disease. Methods: A retrospective review of all children with proven retinoblastoma, who had presented to MAHAK hospital in Tehran, from April 2007 to Dec 2011, was performed. Grouping of intraocular tumors was applied as A to E according to International Classification of Retinoblastoma. Findings: There were 157 (91 boys) children eligible for study. The mean age was 1.21±0.11 years with average delay in diagnosis of 3.4±0.53 months. Classification of D group in both unilateral (93 patients) and bilateral tumors was the largest category. A significant relation (P=0.05) between delayed diagnosis time and tumor grouping was evident. The most frequent symptoms were leukocoria and strabismus. Age was significantly lower in the subgroup of bilateral tumors than in unilateral retinoblastomas (0.6±0.12 year vs 1.6±0.15 years). The diagnosis was delayed in subgroup of extra ocular retinoblastoma more than in intraocular tumors (8.7±2.9 months vs 2.9±0.52 months). Conclusion: The authors recommend early referring of suspected cases to ophthalmologists and pediatric oncologists and to organize educational programs to publisize signs and symptoms of the disease such as leukocoria, strabismus and ocular inflammatory disorders through national media. In conclusion, early diagnosis of retinoblastoma can be the primary factor in managing the patients as the delay in diagnosis accounts for highly advanced disease and poor prognosis. [ABSTRACT FROM AUTHOR]

Retinoblastoma (RB) is a pediatric ocular malignancy that is initiated mostly by biallelic inactivation of the RB transcriptional corepressor 1 (RB1) tumor suppressor gene in the developing retina. Unlike the prevailing prediction based on multiple studies involving RB1 gene disruption in experimental models, human RB tumors have been demonstrated to possess a relatively stable genome, characterized by a low mutation rate and a few recurrent chromosomal alterations related to somatic copy number changes. This suggests that RB may harbor heightened genome maintenance mechanisms to counteract or compensate for the risk of massive genome instability, which can potentially be driven by the early RB1 loss as a tumor-initiating event. Although the genome maintenance mechanisms might have been evolved to promote RB cell survival by preventing lethal genomic defects, emerging evidence suggests that the dependency of RB cells on these mechanisms also exposes their unique vulnerability to chemotherapy, particularly when the genome maintenance machineries are tumor cell-specific. This review summarizes the genome maintenance mechanisms identified in RB, including findings on the roles of chromatin regulators in DNA damage response/repair and protein factors involved in maintaining chromosome stability and promoting survival in RB. In addition, advantages and challenges for exploiting these therapeutic vulnerabilities in RB are discussed. [ABSTRACT FROM AUTHOR]

We report a clinicopathological correlation of a 12-year-old female child who manifested as retinoblastoma clinically and radiologically but turned out to have acute panopthalmitis on histopathological examination. The authors feel that there is significant clinical implication in sharing the knowledge of this clinicopathological correlation. A 12-year-old female child born out of a consanguineous marriage reported to us with bilateral blindness. She was evaluated clinically. Ultrasound B mode and computerized tomogram (CT) examination were done. Based on all the inputs, right eye enucleation was performed, and the same was subjected to a histopathology examination. The patient had proptosis of the right eye with (LE) calcification of the lens. CT scan revealed irregular thickening of the right globe with heterogeneous uveoscleral enhancement. The vitreous showed irregular hyperdense thickening with multiple calcifications. There was mild thickening of the retrobulbar optic nerve and tendinous insertions of extraocular muscles. The right eye was enucleated, and its histopathology examination revealed panophthalmitis with intraocular ossification. The right globe revealed no evidence of malignancy. Painful blind eye is a major therapeutic dilemma for an ophthalmologist. Radiological findings of irregular thickening of sclera, vitreous with calcifications, and optic nerve thickening are highly suggestive of retinoblastoma. In our patient, we wanted to share the differential diagnosis of acute panopthalmitis which masqueraded as retinoblastoma. [ABSTRACT FROM AUTHOR]

Nodular fasciitis (NF) is a benign proliferation of fibroblasts and myofibroblasts that rarely occurs in the periorbital region. We report what we believe to be the first case of periorbital NF associated with pregnancy. A case of intravascular fasciitis, a NF variant, has been reported during pregnancy, but it was not located in the periorbital region. A weak presence of estrogen receptors has been reported in NF. This may make it more susceptible to the hormone-related changes during pregnancy and contribute to the development of the lesion by stimulating fibroblasts and smooth muscle cell types. Although rare, NF should be considered in the differential diagnosis of periorbital soft-tissue masses arising during pregnancy.

Purpose: To determine the local control and complication rates for children with papillary and/or macular retinoblastoma progressing after chemotherapy and undergoing stereotactic radiotherapy (SRT) with a micromultileaf collimator. Methods and Materials: Between 2004 and 2008, 11 children (15 eyes) with macular and/or papillary retinoblastoma were treated with SRT. The mean age was 19 months (range, 2–111). Of the 15 eyes, 7, 6, and 2 were classified as International Classification of Intraocular Retinoblastoma Group B, C, and E, respectively. The delivered dose of SRT was 50.4 Gy in 28 fractions using a dedicated micromultileaf collimator linear accelerator. Results: The median follow-up was 20 months (range, 13–39). Local control was achieved in 13 eyes (87%). The actuarial 1- and 2-year local control rates were both 82%. SRT was well tolerated. Late adverse events were reported in 4 patients. Of the 4 patients, 2 had developed focal microangiopathy 20 months after SRT; 1 had developed a transient recurrence of retinal detachment; and 1 had developed bilateral cataracts. No optic neuropathy was observed. Conclusions: Linear accelerator-based SRT for papillary and/or macular retinoblastoma in children resulted in excellent tumor control rates with acceptable toxicity. Additional research regarding SRT and its intrinsic organ-at-risk sparing capability is justified in the framework of prospective trials. [Copyright &y& Elsevier]

Neurofibromatosis type 2 (NF-2) is characterized by multifocal proliferation of neural crest-derived cells. The characteristics finding of NF-2 is bilateral vestibular schwannomas. Combined hamartoma of retina and retinal epithelium (CHRRPE) is another associated finding. A 9 year-old-male child presented with left eye decreased vision for 3 months. Visual acuity was 0.0 and 0.8 LogMAR in the right and left eye, respectively. Left fundus showed an elevated, pigmented lesion with surface wrinkling and vascular tortuosity suggestive of CHRRPE with multiple presumed retinal astrocytic proliferations in mid-periphery. He had multiple café-au-lait spots. Optical coherence tomography confirmed clinical findings. Magnetic resonance imaging brain showed bilateral acoustic neuroma. Recognition of this rare finding as presenting feature of NF-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention. It is recommended that children with CHRRPE be screened for NF-2. [ABSTRACT FROM AUTHOR]

Abstract: A three-dimensional boundary element model of the human eye is developed to investigate the thermal effects of eye tumor on the ocular temperature distribution. The human eye is modeled as comprising several regions which have different thermal properties. The tumor is one of these regions. The thermal effects of the tumor are simulated by taking it to have a very high metabolic heat generation and blood perfusion rate. Inside the tumor, the steady state temperature is governed by the Pennes bioheat equation. Elsewhere, in normal tissues of the eye, the temperature satisfies the Laplace''s equation. To compute the temperature on the corneal surface, the surface boundary of each region is divided into triangular elements. [Copyright &y& Elsevier]

Intraocular medulloepithelioma is a rare tumor usually arising from the ciliary body and presenting in childhood. We recently managed a case of an advanced medulloepithelioma in a 45-year-old man that went untreated for several years. The patient had an exenteration after the tumor destroyed the eye and extended into the central nervous system. The initial pathologic diagnosis was complicated by the undifferentiated nature of the tumor which mimicked a lymphoma on fine needle aspiration biopsy (FNAB) as well as on initial permanent sections. The diagnosis was established by immunohistochemical stains indicating a neuroepithelial origin; the relationship of the tumor to the ciliary body and pigmented ciliary epithelium indicated the site of origin. [ABSTRACT FROM AUTHOR]

Large granular lymphocyte leukemia (LGL) constitutes a heterogeneous entity with very different immunophenotypic, clonal and evolutionary characteristics. The most common LGL-T are CD3 +, CD8 +, CD16 +, CD57 +, CD56-. The majority of patients have a chronic disease, systemic signs are rare, and symptoms mainly result from neutropenia or associated autoimmune diseases. We report here a very special case of a 44-year-old woman patient diagnosed with aggressive LGL-T variant also expressing CD56.

The article describes the case of a 62-year-old male who was diagnosed with conjunctival squamous carcinoma in situ with incomplete excision at lateral margins and tumor-free deeper margins and was treated with subconjunctival and topical interferon alpha-2b.