ObjectiveTo investigate the clinical, ophthalmological and pathological features of primary uveal lymphoma.MethodsRetrospective clinical study. From 2012 to 2018 in Beijing Tongren Eye Cener, 4 cases and 4 eyes of patients with primary uveal lymphoma were included in the study. Among them, 3 cases were male and 1 case was female. The average age was (54 ± 13.58) years old. The average time from initial diagnosis to pathological diagnosis was (18.50 ± 9.29) months. 3 cases were enucleated and 1 case was biopsied. Extranodal marginal zone lymphoma (EMZL) of the mucosa associated lymphoid tissue (MALT) was confirmed by pathological examination. BCVA, fundus color photography, color Doppler ultrasound and orbital MRI were performed in all eyes. UBM, OCT, FFA and ICGA were performed in 2 eyes, 3 eyes, 3 eyes and 2 eyes respectively. The clinical, imaging and pathological changes were observed. Following up time was ≥ 6 months.ResultsAt the initial diagnosis, BCVA was 0.6, 0.02 and 0.01 in 1, 2 and 1 eye respectively. Choroid, ciliary body and iris were involved in 3 eyes, choroid in 1 eye. The fundus of the eyes showed infiltration of choroid in yellow and white color, and the lesions were beyond the vascular arch to the equator and peripheral areas. Color Doppler ultrasonography showed that choroidal diffuse thickening and extrascleral extension (ESE) which was the corresponding hypoechoic areas behind the sclera. Among them, ESE showed crescent thickening in 1 eye and nodular thickening in 3 eyes. UBM showed that the echo of ciliary body was thicken and the internal echo was decreased with the iris involved. OCT showed that RPE was wavy and local retinal neuroepithelial layer detached. FFA showed that the early lesions were mottled with strong and weak fluorescence, and the late fluorescence leakage. The posterior wall of the eyeball was thickened and enhanced in MRI.ConclusionThe clinical manifestations of uveal lymphoma are various, color Doppler ultrasound has characteristic manifestations and ESE of crescent or nodular thickening is valuable in diagnosis.
Objective To probe the relationship between the patients′prognosis and the gene of multidrugs and drug resistance in uveal melanoma.Methods The gene expression of cyclin D1, epithelial growth factor receptor (EGFR), non-metastasis gene 23 (nm 23), P glucose protein (P-gp ) , multidrug resistance relation protein (MRP) and lung resistance protein (LRP) expression in 96 cases of uveal melanoma were detected by depigment immunohistochemistry. The patients with complete anamnesis data were observed continuously, and the follow-up results were classified. Results Among the 96 cases of uveal melanoma, the epithelioid cell type was in 21, the mixed cell type in 56, and the spindle cell type in 19 ; including 76 at intraocular stage and 20 at extraocular stage. As the level of metastasis suppress gene nm 23 expression decreased and the level of cyclin D1 and EGFR expression increased, the expression level of drug resistance genes increased. The levels of LRP and MRP had negative correlation to the expressions of nm 23 and postive correlation to the expressions of nm 23, Cyclin D1 and EGFR. In 58 patients′who were observed continuously, 19 died in 5 years and 26 survived over 5 years.Conclusion There are significant as sociation between patients′prognosis and multidrug and drug resistance gene in uveal melanoma.(Chin J Ocul Fundus Dis，2003，19：1-4)
ObjectiveTo assess the use of 18-Fluorine-labelled 2-deoxy-2-fluoro-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) in the diagnosis of uveal melanoma. MethodsTwenty-three patients with uveal melanoma confirmed by histopathologic examination or imaging examination were enrolled. There were 16 male, 7 female, and the mean age was (49.8±12.3) years. All the lesions were unilateral, with 11 cases in OD, 12 cases in OS. Diagnosis was confirmed by histopathological examination of enucleated eyeballs in 15 cases, by ophthalmoscope, fundus fluorescein angiography, ocular B-mode ultrasound and magnetic resonance imaging and other imaging technology in 8 cases. 15 patients diagnosed by histopathologic examination of enucleated eyeballs were divided into three types including mixed (7 patients), spindle cell (6 patients) and epithelioid cell (2 patients) types. The mixed cell type and epithelioid cell type are considered as high-risk; spindle cell type is low-risk. All the patients were evaluated by whole body PET/CT. The location, size, shape, boundary of the lesions, and the relationship with adjacent structures were observed in CT images. 18F-FDG uptake was quantitative expression by standardized uptake value (SUV) in PET image; positive diagnosis should be made when the maximum standardized uptake value (SUVmax) was not less than 2.5. The correlation between SUVmax and maximum diameter of tumor base, tumor height was analyzed by Spearman rank correlation test. The detection rate of high-risk and low-risk patients between 18F-FDG PET and CT methods was comparative analyzed. ResultsAll the CT images showed abnormal high density ocular lesions. The shape of the lesions included 8 patients of semi sphere-like, 7 patients of flat-like, 4 patients of mushroom-like, 3 patients of round-like and 1 patient of diffuse lesions. The tumors were located in the posterior pole (9 patients), temporal equator (5 patients), nasal equator (4 patients), superior equator (1 patient), temporal ciliary body (1 patient), inferior ciliary body (1 patient), temporal iris (1 patient), and nasal iris and ciliary body (1 patient). SUVmax≥2.5 were found in 9 patients (39.13%), the largest basal diameter and height were (17.53±3.48), (11.37±3.85) mm respectively. SUVmax < 2.5 were found in 14 patients (60.87%), the largest basal diameter and height were (10.66±3.25), (5.33±2.23) mm respectively. The former's largest basal diameter and height were greater than the latter's and the difference was statistically significant (t=4.815, 4.786; P < 0.01). SUVmax was positively correlated with the largest basal diameter and height respectively (r=0.881, 0.809; P < 0.01). 15 patients (39.13%) were diagnosed by histopathological diagnosis after enucleation, of which SUVmax≥2.5 were found in 8 patients which included 6 patients of mixed type, 1 patient of epithelioid cell type, and 1 patient of spindle cell type. The detection rate of high-risk type (77.78%, 7/9) was higher than that of low-risk type (16.67%, 1/6), the difference was statistically significant (χ2=5.402, P < 0.05). Conclusions18F-FDG PET-CT examination can show large uveal melanoma tumor from cell metabolism, and may help to evaluate the prognosis of the preoperative patients. But, for small tumor, it has little value. We don't recommend 18F-FDG PET-CT is used as a routine examination for uveal melanoma.
Once uveal melanoma (UM) has distant metastasis, the median survival time of the patient is less than 12 months. There is currently a lack of standard treatment for metastatic UM. In recent years, immunotherapy is splendid in the field of oncology. Immune checkpoint therapy, cancer vaccine therapy and T cell adoptive therapy have been applied to UM therapy. However, most of the clinical effects are limited and the survival benefit is not high. The recent early research results of the new immunotherapeutic drug IMCgp100 are encouraging.
Radiotherapy is the prior treatment for uveal melanoma, but a major problem confronted most of the patients is radiation retinopathy, which accompanied with severe visual loss and secondary enucleation potential. There is no optium choice and normative strategy so far, the intraocular melanoma society has focused on application of anti-vascular endothelial growth factor drugs injection and glucocorticoids. This article reviews a series of potential managements for radiation retinopathy and its further stage .
Objective To observe the ocular features and analyze the cause of misdiagnosis of intraocular nonHodgkin's lymphoma (IONHL). Methods A retrospective study was performed in 6 patients and related literatures were reviewed. The age of those patients was 46 to 68 yrs with an average of 562 yrs. Four cases were female, two cases were male. Five patients were unilateral and one case was bilateral case. All patients were investigated by Goldmann tonometer, fundus fluorescein angiography (FFA) and ophthalmic Bscan. Pathological samples come from enucleation and vitrectomy, and were analyzed histologically and immunohistochemically. Results Ophthalmological signs of IONHL included decrease visual acuity, red eye, aqueous flare; keratic precipitates (Kp), vitreous opacity, retinal detachment and multiple whiteyellow retinal lesions of different sizes and fuzzy boundaries. IONHL patients were always misdiagnosed as diverse and nonspecific symptoms and signs, two cases were misdiagnosed as retinitis or chorioiditis, one case as intermediate uveitis, one case as secondary glaucoma in uveitis. The duration from onset of symptom and sign to a final diagnosis was from 6 months to 24 months with an average of 13.3 months. Conclusions The clinical manifestation of IONHL was diverse and non-specific，and this disease was unknown to lots of doctors and easily be misdiagnosed.
Uveal melanoma (UM) is one of the most common primary intraocular malignancy in adults. The incidence of UM is lower in Asia than in Europe and the United States, however, the age of onset of UM patients in Asia is earlier than in the European and American populations. With the improvement of economic living standards in recent years, UM as a rare intraocular tumor has been gradually recognized by the public. In the past 10 years, the research of UM in China has been characterized by a large number and rapid development. Among them, the direction of molecular genetics represented by non-coding RNA, the frontier development of potential anticancer drugs for UM and Chinese traditional medicines are the research hotspots for scholars in China. In the past 10 years, China has made a relatively complete understanding and research progress on the pathogenesis, diagnosis and treatment of UM. On the other hand, compared with European and American countries, China still lacks in frontier research such as immunotherapy. With the further efforts of Chinese ophthalmology researchers and research teams, and with the further development of scientific research in my country, it is believed that the mechanism affecting tumors can be further elucidated, providing more possibilities for treatment and improving the prognosis of UM patients in China.
Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B cell derived non-Hodgkin's lymphoma. It is the main type of uveal lymphoma and is extremely rare. The pathogenesis of ocular MALT lymphoma remains unclear. It is now considered to be associated with many causes. The manifestations of primary uveal MALT lymphoma differ. So sometimes it is necessary to diagnose depending on diversity of auxiliary tests. Ultrasound examination shows typical low and homogeneous internal reflectivity, with blood flow signal. Optical coherence tomography, fundus imaging, fundus angiography, magnetic resonance imaging and positron emission tomography computerized tomography can assist diagnosing. Primary uveal MALT lymphoma is sensitive to radiation therapy, chemotherapy and biotherapy have positive influence too. The prognosis of uveal MALT lymphoma is good, but its early diagnosis is rather challenging. The nonspecific clinical manifestations and the rarity of the disease can confound the initial diagnosis, resulting in delayed treatments which may cause irreversible vision loss.