Scleral Invasion Research Articles

Dissémination locorégionale et systémique du mélanome choroïdien : corrélation anatomoclinique à partir de 2 cas

To describe didactically the local, regional and systemic spread of choroidal melanoma. Two patients who had undergone primary enucleation for the management of choroidal melanoma in 2018 at the University Hospital of Nice were included. Extrascleral extension and invasion of the vortex veins were evaluated, as well as synchronous and metachronous metastases, based on our database. Patient 1 was diagnosed with large choroidal melanoma with partial scleral invasion and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3, and a gain of chromosome 8q. Systemic work-up was unremarkable. Patient 2 was diagnosed with a large choroidal melanoma with extrascleral extension and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3 and a gain of chromosome 8q. Systemic work-up revealed several liver metastases. A total of 1762 patients were included in our database. Eighty-five patients (4.8%) and 46 patients (2.6%) experienced vortex vein invasion and extrascleral extension respectively. Patients with vortex vein invasion were diagnosed with synchronous and metachronous liver metastases in 1.2% and 18.8% respectively. Patients with extrascleral extension had synchronous and metachronous liver metastases in 6.5% and 30.4% respectively. The mean follow-up was 49.4 months (1-180). Extrascleral extension and vortex vein invasion illustrate the local, regional and systemic spread of choroidal melanoma. The latter are often associated with genetically aggressive tumours associated with high metastatic risk.

Conjunctival Leiomyosarcoma, a Rare Neoplasm Always Originating at the Limbus? Report of a New Case and Review of 11 Published Cases

Purpose of the Study: To describe the differential diagnosis and management of a rare conjunctival malignancy. Procedures: A 79-year-old man presented with a conjunctival mass at the limbus. Excisional biopsy revealed a malignant mesenchymal tumor with myogenic differentiation. Six months later, three suspect lesions developed at the border of the previous excision. Pathological diagnosis pointed to a leiomyosarcoma. Adjuvant radiotherapy with strontium-90 brachytherapy was applied. After 3 years, there was neither recurrence nor distant metastases. A literature review revealed 11 cases of conjunctival leiomyosarcoma. Results: All 12 cases seemed to originate at the limbal conjunctiva. Scleral invasion was found only in one patient with multiple previous resections. Corneal invasion was reported in two patients. Four patients had a globe-sparing resection. In two of them, margins were not tumor free and additional brachytherapy gave a tumor-free follow-up of 1 and 3 years. Four cases underwent an exenteration. Conclusion and Message: Primary conjunctival leiomyosarcoma is a rare tumor with a favorable prognosis due to early detection and consequently limited size. Diagnosis involves histopathological investigation including immunohistochemistry. If possible, complete resection has the best prognosis. Adjunctive radiotherapy can be effective when the margins are not free and should be considered.

Pathological Risk Factor Profile for Enucleated Retinoblastoma at Sanglah General Hospital

Retinoblastoma is a primary intraocular tumor on childhood. It has a good prognosis with early detection. Aim of this study is to know Pathological Risk Factors (PRF) profile of enucleated retinoblastoma at Sanglah General Hospital. This study is an observational retrospective study based on patient’s medical record and pathological examination. Twenty patients were included in this study, 50% were female and 50% male within age ranging from 7 month – 12 years old. Unilateral retinoblastoma was found in 18 patients (90.5%), bilateral retinoblastoma was found in 2 patients (9.5%). Majority PRF profile found that cancer cell invasions was 60% choroid infiltration and 45% of anterior segment invasion, 60% of optic nerve infiltration, and 50.1% scleral invasion. Tumor cell aggregation showed 61.9% poorly differentiated malignant cells. None of the patients had family history of retinoblastoma. This study revealed optic nerve and choroid invasion were the major PRF found in retinoblastoma, but there is a significant association between scleral infiltration and tumor cell differentiation (p = 0.025). There was no statistical difference of PRF based on age, gender, and laterality.

Upregulation of BCL2 19 kD Protein-Interacting Protein 3 (BNIP3) is Predictive of Unfavorable Prognosis in Uveal Melanoma.

BackgroundBCL2 19 kD protein-interacting protein 3 (BNIP3) is a BH3-containing protein of the BCL-2 family; it can regulate cell death, autophagy, and cytoprotection. The upregulation of BNIP3 has been reported to relate to progression and poor prognosis in different cancer types. However, the clinical significance of BNIP3 in uveal melanoma (UM) is still unknown.Material/MethodsIn our study, 47 patients with UM were enrolled; the expression of BNIP3 was detected with immunohistochemistry. According to BNIP3 immunohistochemical scores, the patients were divided into BNIP3 high- and low-expression subgroups. The correlation between the expression of BNIP3 and clinicopathological factors was evaluated with Fisher’s test; the associations with survival rates were analyzed with log-rank test. The independent prognostic factors were identified with the Cox-regression model.ResultsBNIP3 was mainly localized in the cytoplasm, and high expression of BNIP3 accounted for 31.9% (15/47) of the patients in our study. High expression of BNIP3 was demonstrated to be significantly associated with more pigment (P=0.018) and deeper scleral invasion (P=0.013). High expression of BNIP3 was also correlated with lower overall survival rate (P=0.006). Multivariate analysis confirmed positive ciliary body involvement and lymphatic infiltration as independent prognostic factors.ConclusionsHigh expression of BNIP3 was significantly associated with poor prognosis of patients with UM, indicating that BNIP3 detection could help stratify high-risk patients and identify new therapies targeting BNIP3 as a promising approach to treat UM.

Conjunctival Squamous Neoplasia: Staging and Initial Treatment.

To evaluate the clinical relevance of the American Joint Committee on Cancer (AJCC) classification in the initial management of squamous neoplasia of the conjunctiva. This retrospective study enrolled 95 histopathologically proven cases of treatment-naive conjunctival squamous neoplasia. Tumors were classified into 4 histological groups: conjunctival intraepithelial neoplasia (CIN) with mild dysplasia (grade 1/3), moderate dysplasia (grade 2/3), severe dysplasia (grade 3/3 or carcinoma in situ), and invasive squamous cell carcinoma (SCC). Clinical findings such as tumor location, largest basal diameter, growth pattern, and adjacent structures involved were recorded. CIN was observed in 74 cases (78%), and SCC was noted in 21 cases (22%). Based on the AJCC classification, all the 74 cases of CIN were classified as Tis (tumor in situ). Among the invasive SCC, there were 3 T1 tumors, 2 T2 tumors, and 16 T3 tumors. Complete excision with or without adjuvant therapy was selected as initial treatment in 80% of cases (76/95). Two cases of SCC with scleral invasion were treated using brachytherapy. The AJCC stage does not correlate with the initial treatment of CIN. The AJCC T3 category should be reviewed to differentiate diffuse SCCs with broad surface extension from tumors with deep scleral invasion.

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

Multi-institutional collaborative studies that include large patient populations for the management of retinoblastoma with histopathological risk factors could provide important information for patient management. To evaluate the implementation of a strategy for the management of nonmetastatic unilateral retinoblastoma in children based on standardized diagnostic and treatment criteria. This single-arm prospective study applied a strategy based on a single-center experience. The setting was a multicenter study in Latin America (Grupo de America Latina de Oncologia Pediatrica [GALOP]). Participants were children with nonmetastatic unilateral retinoblastoma (staged with the International Retinoblastoma Staging System). The study opened on July 1, 2008, and closed on December 31, 2014. Follow-up was updated until June 30, 2017. Stage 0 patients (without enucleation) were given conservative therapy without a protocol. Stage I patients (with enucleation and no residual tumor) were divided into a high-risk group (retrolaminar invasion and/or scleral invasion) and a low-risk group (all remaining patients). High-risk children received adjuvant chemotherapy with 4 alternating cycles of regimen 1 (cyclophosphamide [65 mg/kg/d] [plus sodium-2-mercaptoethane sulfonate], idarubicin hydrochloride [10 mg/m2/d], and vincristine sulfate [0.05 mg/kg/d]) and 4 cycles of regimen 2 (carboplatin [500 mg/m2/d, days 1 and 2] and etoposide [100 mg/m2/d, days 1-3]). Low-risk children did not receive adjuvant therapy. Children with buphthalmia received neoadjuvant and adjuvant chemotherapy for a total of 8 cycles. Probability of event-free survival (extraocular relapse and death from any cause were considered events). Among 187 children registered in the study, 175 were evaluable (92 [52.5%] female; median age, 22 months; age range, 3-100 months). Forty-two were stage 0 children, 84 were stage I low-risk children, and 42 were stage I high-risk children; there were 7 children in the buphthalmia group. With a median follow-up of 46 months, the 3-year probability of event-free survival was 0.97 (95% CI, 0.94-0.99), and the probability of overall survival was 0.98 (95% CI, 0.94-1.00). Stage 0 patients had no events, stage I low-risk patients had 1 event (orbital relapse treated with second-line therapy), stage I high-risk patients had 2 events (1 central nervous system relapse and 1 death from sepsis), and the buphthalmia group had 1 event (orbital relapse, followed by central nervous relapse and death). Adjuvant therapy may be effective for high-risk unilateral retinoblastoma but is toxic, and neoadjuvant chemotherapy for buphthalmus appears feasible.

Ocular surface squamous neoplasia in HIV-infected patients: current perspectives.

Ocular surface squamous neoplasia (OSSN) refers to a spectrum of conjunctival and corneal epithelial tumors including dysplasia, carcinoma in situ, and invasive carcinoma. In this article, we discuss the current perspectives of OSSN associated with HIV infection, focusing mainly on the epidemiology, pathophysiology, clinical manifestations, diagnosis, and treatment of these tumors in patients with HIV. Upsurge in the incidence of OSSN with the HIV pandemic most severely affected sub-Saharan Africa, due to associated risk factors, such as human papilloma virus and solar ultraviolet exposure. OSSN has been reported as the first presenting sign of HIV/AIDS in 26%–86% cases, and seropositivity is noted in 38%–92% OSSN patients. Mean age at presentation of OSSN has dropped to the third to fourth decade in HIV-positive patients in developing countries. HIV-infected patients reveal large aggressive tumors, higher-grade malignancy, higher incidence of corneal, scleral, and orbital invasion, advanced-stage T4 tumors, higher need for extended enucleation/exenteration, and increased risk of tumor recurrence. Current management of OSSN in HIV-positive individuals is based on standard treatment guidelines described for OSSN in the general population, as there is little information available about various treatment modalities or their outcomes in patients with HIV. OSSN can occur at any time in the disease course of HIV/AIDS, and no significant trend has been discovered between CD4 count and grade of OSSN. Furthermore, the effect of highly active antiretroviral therapy on OSSN is controversial. The current recommendation is to conduct HIV screening in all cases presenting with OSSN to rule out undiagnosed HIV infection. Patient counseling is crucial, with emphasis on regular follow-up to address high recurrence rates and early presentation to an ophthalmologist for of any symptoms in the unaffected eye. Effective evidence-based interventions are needed to allow early diagnosis and treatment, as well as prevention of the disease.

Outcomes of primary endoresection for choroidal melanoma

PurposeTo review long time treatment results in patients with small or medium sized choroidal melanoma who underwent surgical tumor endoresection as a primary treatment when plaque radiotherapy was unable and patients declined enucleation.Materials and methodsPatients were evaluated for best corrected visual acuity (BCVA), and underwent biomicroscopy, indirect ophthalmoscopy, retinography and ultrasound as well as the usual systemic workup. Study inclusion required the absence of scleral invasion or metastasis and an anterior margin not exceeding the pars plana or the ciliary body. Surgery consisted of a clear lens phacoemulsification with a PC-IOL, and a 23-gauge pars plana vitrectomy with anterior vitreous shave, lesional choroidal endodiathermy, followed by 23-gauge probe tumor endoresection and continuous endolaser. Patients were followed at post-operative 1 day, 1 week, 1, 3, and 6 months and then every 6 months with a complete ophthalmological exam including ultrasound biomicroscopy and systemic follow-up at 3, 6 and every 6 months thereafter.ResultsFourteen patients with choroidal melanoma were included the study. Pre-operative BCVA ranged from 20/20 to hand motion (HM): 20/20 (n = 2); 20/60 (n = 1); and HM (n = 10). Pathological analysis confirmed the diagnosis of uveal melanoma in all cases. Mean follow-up was 54.5 months (45–66 months) with a final BCVA ranging from 20/60 to HM: 20/60 (n = 1); 20/60 to 20/200 (n = 10); and HM (n = 2). The eye retention rate in our study was 100%. No intraocular recurrence was observed. One patient died 12 months after surgery from metastatic disease.ConclusionEndoresection appears to be an acceptable alternative to enucleation for the treatment of posteriorly-localized uveal melanoma, with excellent local control and eye salvage rates.

Retinoblastoma and retinocytoma (retinoma)

Retinoblastomas are extremely rare withabout half of the cases being hereditary eye neoplasms in young children. They show highly proliferative, CRX-positive undifferentiated tumour cells (occasionally forming rosettes). Staging is performed according to the pTNM classification and the International Retinoblastoma Staging System (IRSS). The diagnostic basis for systemic administration of chemotherapy and/or radiation is postlaminar optic nerve invasion, massive choroidal tumour infiltration, scleral invasion or even extraocular invasion. In cases that are difficult to diagnose, immunohistochemical stains (CRX and Ki67) may be very helpful. Retinocytomas (retinomas)completely lackundifferentiated tumour cell areas, showan exclusive photoreceptor differentiation and an exceptionally low Ki67 index.

Correlation between conventional MR imaging combined with diffusion-weighted imaging and histopathologic findings in eyes primarily enucleated for advanced retinoblastoma: a retrospective study.

To determine the diagnostic accuracy of conventional MRI in detecting tumour invasion of advanced intraocular retinoblastoma and to correlate ADC values with high-risk prognostic parameters. The sensitivities, specificities, positive predictive values (PPV), negative predictive values (NPV) and accuracies of MRI in detecting tumour-extent parameters of 63 retinoblastomas were determined. Furthermore, ADC values were correlated with high-risk prognostic parameters. MRI detected postlaminar optic nerve with a sensitivity of 73.3% (95% CI 44.9-92.2%) and a specificity of 89.6% (77.3-96.5%), while the specificity for choroidal invasion was only 31.8% (13.9-54.9%). Likewise, MRI failed to predicted early optic nerve invasion in terms of low sensitivity and PPV. In contrast, scleral and ciliary body invasion could be correctly excluded with high NPV. ADC values were significantly lower in patients with undifferentiated tumours, large tumour size, as with optic nerve and scleral invasion (all p < 0.05). However, no correlation was found between ADC values and the degree of choroidal or ciliary body infiltration. Additionally, ADC values were negatively correlated with Ki-67 index (r = -0.62, P = 0.002). Conventional MRI has some limitations in reliably predicting microscopic infiltration, with the diagnostic efficiency showing room for improvement, whereas ADC values correlated well with certain high-risk prognostic parameters for retinoblastoma. • Conventional MRI failed to predicted microscopic infiltration of the retinoblastoma. • Scleral and ciliary body invasion could be excluded with high NPV. • ADC values correlated well with some high-risk pathological prognostic parameters.

Evaluation of high-risk features of primary enucleation of patients with retinoblastoma in a tertiary center of a developing country in the era of intra-arterial chemotherapy.

To evaluate the frequency of high-risk histopathologic factors in Turkish children enucleated for retinoblastoma and to analyze the association between growth pattern, rosetta formation, tumor thickness, presence of necrosis, calcification, neovascularization, rate of mitosis, and high-risk histopathologic factors. Pathology reports of 59 eyes who had received enucleation for retinoblastoma were reviewed retrospectively. The histopathologic data included presence of choroidal invasion, optic nerve invasion, scleral extension, tumor thickness, presence of necrosis, calcification, neovascularization, rosetta formation and lymphocyte infiltration, rate of mitosis, and growth pattern. This study included 59 eyes from 30 (50.8%) male and 29 (49.2%) female patients. The mean age was 22.87±18.99months. There were 30 (50.8%) eyes with choroidal invasion, 30 (50.8%) eyes with optic nerve invasion, and 5 (8.5%) eyes with scleral invasion. Endophytic growth pattern was seen in 27 (45.8%) eyes, exophytic growth pattern was seen in 2 (3.4%) eyes, and combined growth pattern was seen in 30 (50.8%) eyes. Exophytic growth pattern was found statistically related to choroidal invasion (p=0.00). Although tumor with greater thickness tended to have more choroidal invasion (p=0.02), there was no relation between tumor thickness and optic nerve invasion (p=0.09). Incidences of choroidal and optic nerve invasion showed similarity with other developing countries. Because of higher incidence of high-risk factors, intra-arterial chemotherapy with its targeted effect should be preferred carefully.

Minimal Disseminated Disease in Nonmetastatic Retinoblastoma With High-Risk Pathologic Features and Association With Disease-Free Survival

Fatal metastatic relapse may occur in children with retinoblastoma and high-risk pathologic features (HRPFs). Minimal dissemination (MD) may be an additional tool for risk estimation. The use of cone-rod homeobox (CRX) transcription factor messenger RNA for MD evaluation in metastatic retinoblastoma was previously reported, but no data in nonmetastatic cases with HRPFs are available. To evaluate whether MD is detectable in patients with nonmetastatic retinoblastoma and to assess its prognostic effect on disease-free survival (DFS). This single-institution cohort study of patients with nonmetastatic retinoblastoma and HRPFs used prospectively defined inclusion criteria and a sampling strategy to procure bone marrow (BM) and cerebrospinal fluid (CSF) samples from May 1, 2007, through October 31, 2013. Median follow-up was 38 months (range, 8-89 months). Survival analysis was closed in December 2015, and no further updates were made after that point. The study evaluated CRX messenger RNA by quantitative polymerase chain reaction in BM and CSF at diagnosis and follow-up. In 14 patients, GD2 synthase was used instead of CRX for CSF evaluation. Patients were treated under uniform guidelines. Metastatic relapse. The study included 96 children (median age at study inclusion, 26 months; range, 1-168 months; 46 male [47.9%]; 50 female [52.1%]) with nonmetastatic retinoblastoma and HRPFs (isolated massive choroidal invasion in 14, postlaminar optic nerve invasion in 51 [26 with concomitant massive choroidal and 13 with scleral invasion], 12 with scleral invasion without postlaminar optic nerve invasion, and 7 with tumor at the resection margin of the optic nerve) were evaluated at the time of primary or secondary enucleation. Minimal dissemination was detected in 9 patients (7 BM samples and 2 CSF samples) and was associated with extension beyond the resection margin of the optic nerve and scleral involvement, but only the former was independently associated (adjusted odds ratio, 57.0; 95% CI, 4.8-678.2; P = .001). In addition, MD occurred in 8 of the 43 International Intraocular Retinoblastoma Classification group E eyes with glaucoma (18.6%) and in 8 of 80 (10%) and 1 of 16 children (6.3%) who underwent primary or secondary enucleation, respectively. Children with MD had a 3-year DFS of 0.78 compared with 0.98 in those without MD (95% CI for the difference in DFS, 0.17-0.23; P = .004). These findings identified a high-risk population of children with retinoblastoma and HRPFs with MD. Because the number of events was small, these results, which suggest that children with International Intraocular Retinoblastoma Classification group E retinoblastoma and glaucoma have a higher risk of MD at diagnosis, should not be considered definitive at this time.

Characteristics of retinal and scleral infiltration in uveal melanoma

Objective: To analyze features of retinal and scleral infiltration of uveal melanoma. Methods: It was a retrospective case series study. The pathological and clinical data of 102 cases of uveal melanoma were analyzed retrospectively, which were collected between Jun. 2001 and Apr. 2013 in Beijing Tongren Hospital, Capital Medical University. The tumor shape, size, cell types, retinal and scleral invasion were recorded and analyzed. The relationship between retinal invasion and scleral invasion was analyzed by trend Chi-square test. Results: There were 44 male patients and 58 female patients with an average age of 45.6±12.4 (range from 15 to 78 years old) in 102 cases. As for tumor size, there were 68 (66.7%) large size tumors, 27 (26.5%) medium size tumors and 7(6.8%) small size tumors; As for cell types, 76 (74.5%) were spindle cell, 6 (5.9%) were epithelioid cell, 16 (15.7%) were mixed cell, and 4 (4.0%) were others; As for tumor location, there were 3 (2.94%) ciliary body melanoma, 28 (27.5%) ciliary body and anterior choroid melanoma, and 71 (69.6%) choroid melanoma. Eighty-six cases (84.3%) showed retinal invasion, in which there were 20 cases of outer nuclear layer invasion (19.6%), 12 cases of inner nuclear layer invasion (11.8%), 30 cases of ganglion cell layer invasion (29.4%), and 24 cases of inner limiting membrane invasion (23.5%). Fifty-five cases (53.9%) showed scleral invasions, in which there were 11 cases (68.8%) of no retinal invasion, 13 cases of outer nuclear layer invasion (65.0%), 5 cases of inner nuclear layer invasion (41.7%), 15 cases of ganglion cell layer invasion (50.0%) and 11 cases of inner limiting membrane invasion (45.8%). In 76 spindle cell cases, the scleral invasion rate of different retinal invasion degree were 72.7% in 8 cases of none retinal invasion, 71.4% in 10 cases of outer nuclear layer invasion, 62.5% in 5 cases of inner nuclear layer invasion, 53.9% in 14 cases of ganglion cell layer invasion and 47.1% in 8 cases of inner limiting membrane invasion. No significant relationship was found between retinal and scleral invasion (γ=-0.21, P=0.127) . Conclusion: With the increase of scleral invasion, the retinal invasion decreased. However, the correlation show no statistical significance.(Chin J Ophthalmol, 2016, 52: 749-754).

Clinical and pathologic observation of uveal metastatic carcinoma

Objective: To observe the clinical and pathological features of uveal metastatic carcinoma. Methods: It was a retrospective case series study. The clinical manifestation, growth pattern, tumor types and relative pathological features of 13 patients visiting from January 1980 to December 2014 with uveal metastatic carcinoma in Tianjin Eye Hospital were analyzed retrospectively. Results: There were 13 cases, 6 cases of male and 7 of female. Age was from 37.0 to 66.0 years old. The mean age was 52.1 years old. all cases were monocular. There were 5 cases with right eye and 8 cases with left eye. Among 13 cases, 10 tumors were in posterior choroid, one tumor was in anterior choroid and ciliary body, 2 tumors were in the iris. There were 5 patients with lung cancer, 4 patients with breast cancer, 1 patient with prostate cancer, 1 patient with thyroid cancer and 1 patient with esophageal cancer. The primary tumor wasn't found in 1 patient. The rapid decrease of visual acuity showed in 10 patients with posterior choroidal metastatic carcinoma, 8 of them accompanied with extensive retinal detachment and 6 of them had secondary glaucoma. The multiple gray-white nodule or pink cauliflower mass on the papillary margin of iris were showed respectively in 2 patients with iris metastatic carcinoma. The pathological examination found that posterior choroidal metastatic carcinoma mainly located in temporal or nasal side choroids in 10 cases, among them, local or diffuse flat choroidal masses showed in 6cases, extensive mass involving choroid and ciliary body showed in 1 case, large nodular or globular choroidal mass showed in 2 cases, choroidal mass surrounded the optic disc in 1 case, optic nerve invasion showed in 3 cases and extraocular or orbital invasion showed in 3 cases. The scleral and subconjunctival invasion showed in 1 case of anterior choroid and ciliary body metastatic carcinoma. Conclusions: Uveal metastatic carcinoma manifested various growth pattern, the rapid decrease of visual acuity, flat or nodular choroidal solid mass, secondary retinal detachment and glaucoma were common clinical features. Some cases might invade extraocular or orbital tissue. (Chin J Ophthalmol, 2016, 52: 769-774).

Clinical and histopathological correlation: A study on 334 eyes with retinoblastoma from Vietnam

Purpose: To elucidate the clinical features which predict high-risk histopathological factors for subsequent metastatic disease as well as to report the incidence of these high-risk histopathological factors in a cohort of Asian patients with retinoblastoma.&#x0D; Design: A retrospective and non-randomized sequential cases series.&#x0D; Methods: A retrospective study was done on 334 eyes with retinoblastoma at Vietnam National Institute of Ophthalmology during a 10 year period (January 2004 – December 2013). All pathology specimens and medical records were reviewed and assessed for invasion and clinical signs.&#x0D; Results: Among 334 eyes, 225 (67.4%) had high-risk retinoblastoma and 109 (22.6%) had non-high-risk features on histopathology. The high-risk histopathological features included anterior chamber seeding (48.2%), iris infiltration (14.7%), ciliary body involvement (14.1%), massive choroidal invasion (29.9 %), post-laminar optic nerve invasion (21.2%), invasion of optic nerve transection (9.6 %), combined choroidal and optic nerve invasion (9.6 %), scleral invasion (3.3%), and extra-scleral infiltration (11.4%). The significant clinical features in high-risk group versus non-high-risk group included hyphema (19.6% vs 3.7%, p &lt; 0.001), pseudohypopyon (19.1% vs 6.4%, p = 0.001), iris neovascularization (25.3% vs 5.5%, p &lt; 0.001), vitreous seeding (72.4% vs 37.6%, p &lt; 0.001), staphyloma (24% vs 4.6%, p &lt; 0.001) and scleritis (20% vs 3.7%, p &lt; 0.001).&#x0D; Conclusions: Clinical signs including hyphema, pseudohypopyon, iris neovascularization, vitreous seeding, staphyloma, and scleritis were significantly associated with high-risk features on histopathology. Globe preserving methods should be used with caution in patients with these signs.&#x0D;

Globe Loss From Intraocular Invasion of MIRAgel Scleral Buckle Components.

To describe intraocular invasion of MIRAgel scleral buckles requiring evisceration. This is an Institutional Review Board-approved retrospective consecutive case series of eyes requiring evisceration secondary to intraocular intrusion of MIRAgel implants performed at the Cole Eye Institute from 2000 to 2014. Charts were reviewed for age at surgery, gender, laterality, time between MIRAgel placement and evisceration, preoperative examination and imaging results, intraoperative findings, postoperative complications, and duration of follow up. Five eyes of 5 patients underwent evisceration due to a blind, painful eye secondary to MIRAgel expansion. The mean time between MIRAgel placement and evisceration was 21 years (range: 17-30 years). Preoperative ultrasound identified intraocular MIRAgel in 3 of 5 cases; however, intraocular MIRAgel was identified during surgery in all 5 cases. A transocular-approach orbitotomy was performed at the time of evisceration in an effort to remove the MIRAgel. Postoperative complications included ptosis and inability to retain an ocular prosthesis. No cases of orbital implant extrusion occurred. Scleral invasion and intraocular penetration of MIRAgel may occur decades after placement. This may result in a blind, painful eye requiring evisceration and orbitotomy to remove residual material. Suspicion of intraocular penetration of implant should be high in blind, painful eyes. Surgical removal can be difficult due to MIRAgel fragmentation. Conjunctival insufficiency may result in the need for further surgery after evisceration.

Ocular Surface Squamous Neoplasia in 200 Patients: A Case-Control Study of Immunosuppression Resulting from Human Immunodeficiency Virus versus Immunocompetency

To describe and compare the clinical presentation, treatment outcomes, and histopathologic features of ocular surface squamous neoplasia (OSSN) based on human immunodeficiency virus (HIV) status. Case-control study. A total of 200 patients with OSSN, of whom 83 (41%) had positive results for HIV and were classified as cases and 117 (59%) had negative results for HIV and were classified as controls. Enzyme-linked immunosorbent assay for HIV, conjuntival excision biopsy, extended enucleation, orbital exenteration. Clinical features, treatment outcomes, and histopathologic characteristics. The mean age at presentation of OSSN in both cases and controls was 40 years (median, 40 years; range, 13-65 years) and in controls was 40 years (median, 38 years; range, 15-80 years). On comparison of cases versus controls with OSSN, HIV-positive individuals had larger (12 vs. 8 mm; P < 0.001) and thicker (3.2 vs. 2.3 mm; P = 0.041) tumors, with a higher incidence of corneal (60% vs. 40%; P = 0.007), scleral (19% vs. 9%; P = 0.044), and orbital (13% vs. 3%; P = 0.019) invasion and a higher need for extended enucleation or exenteration (27% vs. 11%; P < 0.001). The bilateral presentation (11% vs. 4%; P = 0.13), need for lamellar sclerectomy (13% vs. 8%; P = 0.29), and tumor recurrence after primary treatment (30% vs. 20%; P = 0.12) was higher in HIV-positive cases compared with HIV-negative controls. However, these features were not statistically significant. Based on American Joint Committee on Cancer classification, T1 tumor was more common in controls (13% in cases vs. 35% in controls; P = 0.0009), and T4 tumor was more common in cases (13% in cases vs. 4% in controls; P = 0.019). None of the patients demonstrated systemic metastases or died of disease during a mean follow-up period of 10 months (median, 4 months; range, <1-75 months) in cases and 9 months (median, 4 months; range, <1-99 months) in controls. Ocular surface squamous neoplasia in HIV-positive individuals is aggressive with larger and thicker tumors and with higher incidence of corneal, scleral, and orbital invasion. These patients are associated with poor ocular prognosis with higher need for extended enucleation, exenteration, or both.

High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma: Part 1: MRI vs. histopathology.

A reliable detection of metastatic risk factors is important for children with retinoblastoma to choose the right therapeutic regimen. First studies using high-resolution magnetic resonance imaging (MRI) with orbit surface coils were promising. The aim of this study was therefore to evaluate the ability of high-resolution MRI to detect metastatic and especially advanced metastatic risk factors in a large group of children with retinoblastoma. One hundred forty-three consecutive children with retinoblastoma (148 enucleated eyes, 64 girls, 79 boys, mean age 19.7 ± 15.3) who received pretherapeutical high-resolution MRI with orbit surface coils on 1.5 T MR scanner systems between 2007 and 2012 and subsequent primary enucleation within 14 days were included in this retrospective study. Image analysis was performed by two neuroradiologists experienced in ocular imaging in consensus. Histopathology served as gold standard. Sensitivity/specificity for the detection of metastatic risk factors using high-resolution MRI with orbit surface coils were 60 %/88.7 % for postlaminar optic nerve infiltration, 65.5 %/95.6 % for choroidal invasion, 100 %/99.3 % for scleral invasion, and 100 %/100 % for peribulbar fat invasion, respectively. The results increased for the detection of advanced metastatic risk factors, 81.8 %/89.1 % for deep postlaminar optic nerve infiltration, 70.6 %/97.6 % for massive choroidal invasion. High-resolution MRI is clinically valuable for the detection of metastatic, especially of advanced metastatic risk factors in children with retinoblastoma.

Neoadjuvant/adjuvant treatment of high-risk retinoblastoma: a report from the German Retinoblastoma Referral Centre

BackgroundRetinoblastoma can extend beyond the structures of the eye, where cells can enter the bloodstream and cause metastases. Various types of protocols for adjuvant treatment risk-adapted according to histopathological risk...

Anterior segment invasion in retinoblastoma: is it a risk factor for extraocular relapse?

We report a retrospective review of patients with retinoblastoma and anterior segment invasion (ASI) as risk factors for extraocular relapse. Only those with ASI combined with postlaminar optic nerve invasion and/or scleral invasion received adjuvant chemotherapy and those with tumor at the resection margin received orbital radiotherapy. Those with only uveal invasion did not receive adjuvant therapy. Of 479 evaluable patients, 67 patients had pathologically confirmed ASI, including 52 with anterior chamber invasion and 47 with iris or ciliary body invasion. ASI occurred with other pathology risk factors (25 had concomitant posterior uveal invasion, 36 had postlaminar optic nerve invasion, 11 with cut-end invasion, and 25 with scleral invasion). The 5-year disease-free survival (pDFS) was 0.9 (95% CI, 0.8-0.95) for children with ASI with no significant differences among children with other pathology risk factors with and without ASI. ASI was not significantly associated with extraocular relapse in multivariate analysis. There were no significant differences in pDFS for patients with anterior chamber invasion and those with iris-ciliary body invasion (pDFS 0.89 [95% CI, 0.65-0.96] vs. 0.93 [95% CI, 0.61-0.98]). To conclude, ASI was seen with other pathology risk factors and it did not add a significant risk for extraocular relapse.