A case of choroidal melanocytoma treated by transscleral resection: A clinicopathological study.

Purpose: Choroidal melanocytoma is a rare benign melanocytic tumor. We report a case of choroidal melanocytoma that was definitively diagnosed by histopathological findings after local resection. Observation: A 71-year-old female complained of blurred vision in her left eye. Her best-corrected visual acuity (BCVA) was 1.0. A dark-brown elevated lesion, measuring 5 papilla-diameter was found in the periphery of the fundus in her left eye. The mass showed hyperfluorescence on fluorescein angiography, early hypofluorescence and late hyperfluorescence on indocyanine green angiography. B-mode echography indicated the mass was originated from the choroid. Orbital magnetic resonance imaging showed isointense signal intensity on T1-weighted images (WI) and hypointense signal intensity on T2-WI, and poor Gadolinium enhancement on T1WI. The tumor was suspected to be melanocytoma, but it was difficult to differentiate from malignant melanoma. Transscleral tumor resection combined with 25-gauge vitrectomy was performed. Histopathological examinations led to the diagnosis of choroidal melanocytoma. Two years after local resection, her BCVA was 1.0 with no tumor recurrence. Conclusions/importance: Local resection was useful as a diagnostic treatment for choroidal tumors confined to the periphery of the fundus that were difficult to clinically differentiate from malignant melanoma.

CLINICOPATHOLOGICAL ANALYSIS OF SECONDARY RETINAL VASOPROLIFERATIVE TUMOR/REACTIVE RETINAL ASTROCYTIC TUMOR SUCCESSFULLY TREATED BY ENDORESECTION.

PURPOSE: To report the clinicopathological findings of retinal vasoproliferative tumor/reactive retinal astrocytic tumor (VPT/RRAT) with retinal vasculitis, treated by tumor resection. METHODS: A retrospective single case report. PATIENT: A 29-year-old Japanese woman was referred with cystoid macular edema and retinal vasculitis in the right eye. Best-corrected visual acuity was 0.9. Results of fundus examination, optical coherence tomography, and fluorescein angiography demonstrated VPT/RRATs in the temporal retina surrounded by a subretinal exudate, serous retinal detachment and macular edema, and retinal vasculitis. Despite 3 months of oral prednisolone treatment, a full-thickness macular hole developed. Pars plana vitrectomy and endoresection of the VPT/RRATs were performed. Pathologic and immunohistochemical analyses with anti-CD34 antibody, antiglial fibrillary acidic protein antibody, anti-Ki67 antibody, and anti-vascular endothelial growth factor antibody were performed on the excised tissue. Inflammation was evaluated by immunohistological staining with leukocyte common antigen (LCA), anti-CD3 antibody, and anti-CD20 antibody. RESULTS: After surgery, the macular hole closed, best-corrected visual acuity improved to 1.2, retinal vasculitis was ameliorated, and retinal exudate disappeared. There was no recurrence of VPT/RRAT or retinal vasculitis. Pathologic examination showed that antiglial fibrillary acidic protein and anti-vascular endothelial growth factor were widely expressed, irrespective of the distribution of blood vessels. Ki67-positive proliferating cells were detected in the perivascular area. Leukocyte common antigen-positive leukocytes and CD3-positive T cells were detected throughout the samples, whereas CD20-positive B cells were rarely detected. CONCLUSION: Endoresection of VPT/RRAT could be a good treatment option for secondary VPT/RRAT accompanied by retinal vasculitis. Pathologic findings revealed for the first time that inflammatory cells infiltrate the tissue in secondary VPT/RRAT.

Clinicopathological findings in refractory diabetic macular edema: A case report.

The present study describes the case of a patient with refractory diabetic cystoid macular edema who underwent vitrectomy with en bloc removal of the cystoid lesion component. The current study also performed histopathological and immunohistochemical analyses of the cystoid lesion component to assess fibrin/fibrinogen and advanced glycation end-products (AGEs) immunoreactivity. A 69-year-old Japanese man presented with visual loss in the left eye due to residual cystoid macular edema (CME) refractory to anti-vascular endothelial growth factor therapy. Best-corrected visual acuity was 1.2 in the right eye (OD) and 0.5 in the left eye (OS). Fundus examination showed dot hemorrhages and hard exudates in the peri-macular region with pan-retinal photocoagulation scars in both eye. Swept-source optical coherence tomography revealed CME with slight hyperreflectivity in the cyst OS. A total of 3 months after the initial visit, pars plana vitrectomy was performed, and the translucent solidified component within the cystoid lesion was isolated. Histopathologically, the excised component was elliptical in shape, measuring 0.7x0.4 mm and exhibited homogeneous eosinophilic material without cellular components. No membranous structure was observed surrounding the component. Immunohistochemistry demonstrated that the tissue was positive for fibrin/fibrinogen and weakly positive for AGEs, but was negative for glial fibrillary acidic protein, type 1 collagen and receptor for AGEs. To the best of our knowledge, the present case report is the first to histopathologically examine the contents of refractory CME, and to immunohistochemically demonstrate that fibrin in diabetic CME may be post-translationally modified by AGEs. These results suggested that fibrin in CME may escape degradation by plasmin due to post-translational modifications.

Clinicopathologic features of conjunctival MALT lymphomas refractory to radiation therapy.

OBJECTIVE: Clinicopathologic features of patients with limited-stage mucosa-associated lymphoid tissue (MALT) lymphoma refractory to radiotherapy have not been fully elucidated. This study aimed to elucidate clinicopathologic features of localized conjunctival MALT lymphoma concerning radiosensitivity by analyzing cell proliferation and expression of mismatch repair proteins. METHODS: We enrolled 26 patients with localized conjunctival MALT lymphoma treated with radiotherapy from November 2007 to March 2020. Monoclonal immunoglobulin H gene rearrangement was tested in addition to histopathologic evaluation. Thirty-six specimens were immunostained with antibodies to Ki-67 and MutL protein homologue 1 (MLH1), MutS protein homologue 2 (MSH2), and MutS protein homologue 6 (MSH6). Positive rates under a high-power field at a hot spot were counted manually. RESULTS: After radiotherapy, 21 patients showed clinical disappearance of the tumour without recurrence (effective group). Three patients showed temporary disappearance of the tumour, which later recurred (relapse group). Two patients did not show disappearance of the tumour (ineffective group). The 2 ineffective patients were young, had bilateral lesions, and received x-ray beam therapy. The mean positive rates of Ki-67, MLH1, MSH2, and MSH6 were higher in tumours with complete remission (CR) than in those without CR (23.4% ± 4.0% and 18.7& ± 4.7%, 14.7% ± 2.3% and 7.1% ± 3.7%, 23.9% ± 4.7% and 14.4% ± 5.2%, and 11.5% ± 3.2% and 5.4% ± 2.2%; p > 0.05 for each, respectively). CONCLUSIONS: A few patients could not achieve CR following radiotherapy, whereas there were no significant differences in proliferation activity and mismatch repair proteins between tumours with and without CR.

Alterations of choroidal circulation and choroidal thickness before and after chemoradiotherapy in a case of metastatic choroidal tumor.

BACKGROUND: Metastatic choroidal tumors are hematogenous intraocular metastases of malignant tumors in systemic organs; however, the details of choroidal circulation and morphological changes in the choroid are unknown. The aim of this study is to present a case of metastatic choroidal tumor and examine laser speckle flowgraphy (LSFG)-based choroidal circulation and central choroidal thickness (CCT) before and after chemoradiotherapy. CASE PRESENTATION: A 66-year-old woman with a medical history of breast cancer 16 years ago was referred to our department struggling with blurred vision in her right eye. At the time of initial examination, her best-corrected visual acuity (BCVA) was 0.4 oculus dexter (OD) and 0.9 oculus sinister. Fundus revealed a yellowish-white choroidal elevated lesion measuring 8 papillary diameters with serous retinal detachment (SRD) in the posterior pole. Fluorescein angiography showed diffuse hyperfluorescence and fluorescent leakage due to SRD, and indocyanine green angiography demonstrated no abnormalities in the macula but hypofluorescence in the center of the tumor. Based on these clinical findings, she was diagnosed with metastatic choroidal tumor. After chemoradiotherapy, the metastatic choroidal tumor became scarred, and SRD disappeared. The rate of changes in macular blood flows assessed by mean blur rate on LSFG and CCT of her right eye were 33.8 and 32.8% decrease at 5 months after the initial visit, respectively. BCVA was 0.5 OD 27 months after the initial examination. CONCLUSION: Chemoradiotherapy resulted in regression of the metastatic choroidal tumor and disappearance of SRD, with a decrease in central choroidal blood flow and CCT. The choroidal blood flow on LSFG could reflect an increased oxygen demand by cancer cells invading the choroid and substantial blood supply.

Changes in choriocapillaris structure occurring in idiopathic macular hole before and after vitrectomy.

PURPOSE: The aim of this study was to analyze the anatomical choroidal vascular layers and the changes in idiopathic macular hole (IMH) eyes over time after vitrectomy. METHODS: This is a retrospective observational case-control study. Fifteen eyes from 15 patients who received vitrectomy for IMH and age-matched 15 eyes from 15 healthy controls were enrolled in this study. Retinal and choroidal structures were quantitatively analyzed before vitrectomy and 1 and 2 months after surgery using spectral domain-optical coherence tomography. Each choroidal vascular layer was divided into the choriocapillaris, Sattler's layer, and Haller's layer, and then, the choroidal area (CA), luminal area (LA), stromal area (SA), and central choroidal thickness (CCT) were calculated using binarization techniques. The ratio of LA to CA was defined as the L/C ratio. RESULTS: The CA, LA, and L/C ratios were 36.9 ± 6.2, 23.4 ± 5.0, and 63.1 ± 7.2 in the choriocapillaris of IMH and were 47.3 ± 6.6, 38.3 ± 5.6, and 80.9 ± 4.1 in that of control eyes, respectively. Those values were significantly lower in IMH eyes than in control eyes (each P < 0.01), whereas there was no significant difference in total choroid, Sattler's layer, and Haller's layer or CCT. The ellipsoid zone defect length showed a significant negative correlation with the L/C ratio in total choroid and with CA and LA in the choriocapillaris of IMH (R = - 0.61, P < 0.05, R = - 0.77, P < 0.01, and R = - 0.71, P < 0.01, respectively). In the choriocapillaris, the LA were 23.4 ± 5.0, 27.7 ± 3.8, and 30.9 ± 4.4, and the L/C ratios were 63.1 ± 7.2, 74.3 ± 6.4, and 76.6 ± 5.4 at baseline, 1 month, and 2 months after vitrectomy, respectively. Those values showed a significant increase over time after surgery (each P < 0.05), whereas the other choroidal layers did not alter consistently with respect to changes in choroidal structure. CONCLUSIONS: The current OCT-based study demonstrated that the choriocapillaris was exclusively disrupted between choroidal vascular structures in IMH, which may correlate with the ellipsoid zone defect. Furthermore, the L/C ratio of choriocapillaris recovered after IMH repair, suggesting an improved balance between supply and demand of oxygen that has collapsed due to temporary loss of central retinal function by IMH.

A Case of Intraocular Metastasis of Lung Cancer Diagnosed Using Cell Block Preparation of the Vitreous Humor.

BACKGROUND/AIM: Masquerade syndrome is characterized by uveitis-like manifestations caused by tumor cell infiltration into the ocular tissues. The aim of the study was to report a lung cancer patient with persistent unilateral vitreous opacity, who was eventually diagnosed with masquerade syndrome using cell block preparation. CASE REPORT: An 82-year-old female complained of blurred vision in her left eye (OS). Because of pulmonary adenocarcinoma, she had previously received anticancer drug treatment at another Hospital and achieved partial remission. Ophthalmic examinations revealed anterior chamber inflammation and vitreous opacity OS. Corticosteroid eye drops were administered, but the inflammation did not improve, and was referred to the Hokkaido University Hospital. The left best-corrected visual acuity was 0.1 with normal intraocular pressure. Anterior chamber inflammation was 2+ cells, and vitreous haze was 4+ OS. B-mode ultrasonography showed diffuse heterogeneous high echoic changes in the vitreous cavity. She underwent vitrectomy, and cell block preparation of the vitreous infusion fluids was performed. Cytopathology revealed adenocarcinoma cells with a high nuclear/cytoplasmic ratio and glandular formation. The immunocytochemical study showed that tumor cells were positive for thyroid transcription factor-1 (TTF-1), napsin A, and CK7, therefore diagnosis of masquerade syndrome due to intraocular metastasis of pulmonary adenocarcinoma was reached. Chemoradiotherapy was administered, and the eye got phthisis bulbi after irradiation 2 years after diagnosis. CONCLUSION: Cell block preparation using vitreous humor may be useful in the diagnosis and management of intraocular metastasis of pulmonary adenocarcinoma in patients with prolonged vitreous opacity.

Two-year clinical outcomes of triple therapy with photodynamic therapy, anti-vascular endothelial growth factor agent, and triamcinolone acetonide for neovascular age-related macular degeneration.

PURPOSE: To analyze the 2-year treatment outcomes of triple therapy with standard-fluence photodynamic therapy (PDT), intravitreal injection of ranibizumab (IVR)/aflibercept (IVA), and sub-tenon injection of triamcinolone acetonide (STTA) for neovascular age-related macular degeneration (nAMD) in Japanese patients. STUDY DESIGN: A retrospective, clinical case-series study. METHODS: Forty-four eyes of 44 patients with treatment-naïve nAMD followed for more than 24 months were evaluated. Initial treatment was given with triple therapy and retreatment with IVR/IVA as a pro re nata regimen. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), the number of treatments, and intraocular pressure elevation were analyzed. RESULTS: The mean age was 73.3 ± 10.0 years. The mean BCVA significantly improved from 0.61 ± 0.30 at baseline to 0.50 ± 0.46 at 24 months (p = 0.034). CRT significantly improved from 373 ± 162 µm at baseline to 200 ± 107 µm at 24 months (p < 0.001). The number of treatments given during the 2-year treatment period was 2.7 ± 1.8. No retreatments were necessary in 18 of 44 eyes (40.9%), with no significant difference between IVR (46.4%) or IVA (31.3%) used during the 2-year follow-up of triple therapy (p = 0.51). Four eyes (9.1%) temporarily required glaucoma eye drop treatments. CONCLUSION: In nAMD patients, induction treatment with triple therapy resulted in approximately 40% of the patients requiring no retreatment for 2 years. The type of anti-VEGF agents used made no difference in the results.

RETINAL HYPOPERFUSION DETECTED BY WIDE-FIELD OPTICAL COHERENCE TOMOGRAPHIC ANGIOGRAPHY IN A CASE OF RETINAL RACEMOSE HEMANGIOMA.

PURPOSE: Retinal racemose hemangioma is a rare, unilateral, nonhereditary, arteriovenous malformation characterized by the appearance of dilated and tortuous retinal vessels. Retinal racemose hemangioma can develop complications associated with retinal ischemia, such as vitreous hemorrhage, retinal vein occlusion, and neovascular glaucoma. Here, a case of retinal racemose hemangioma with retinal hypoperfusion detected by wide-field swept-source optical coherence tomographic angiography was reported, which was not unambiguously illustrated by fluorescein angiography. METHODS: Case report. RESULTS: A 57-year-old woman was referred to our hospital for the evaluation of severe retinal vascular tortuosity, dilation, and retinal hemorrhages in the left eye. Fundus examination revealed arteriovenous communications temporal to the fovea and multiple microaneurysms surrounded by retinal hemorrhages at the midperipheral temporal fundus. In fluorescein angiography, multiple hyperfluorescent lesions with leakage corresponding to microaneurysms were observed in the temporal and lower midperipheral areas; however, nonperfused areas were apparently absent. By contrast, wide-field optical coherence tomographic angiography clearly showed low-density retinal capillaries in the superotemporal quadrant in comparison with those in the inferotemporal quadrant. CONCLUSION: Wide-field optical coherence tomographic angiography detected sparse retinal capillaries, which were not well illustrated by fluorescein angiography, in a patient with retinal racemose hemangioma. This indicates the presence of low-grade retinal hypoperfusion caused by altered retinal hemodynamics, potentially leading to ischemia-related retinal disorders during a prolonged course, in patients with clinically quiescent retinal racemose hemangioma.

Clinical significance of serum and vitreous soluble interleukin-2 receptor in patients with intraocular lymphoma.

BACKGROUND: Intraocular lymphoma (IOL) is a masquerade syndrome that mimics uveitis, making diagnosis difficult. The serum soluble interleukin-2 receptor (sIL-2R), which is cleaved by matrix metalloproteinase (MMP) -2 and MMP-9, has been recognized as a tumor-related biomarker of malignant lymphomas. The aim of this study was to review the reliability of serum and vitreous sIL-2R for distinguishing IOL from uveitis. METHODS: Patients who underwent diagnostic vitrectomy for marked vitreous haze at Hokkaido University Hospital between April 2014 and June 2019 were enrolled. The patients were divided into an IOL group and a uveitis group, according to the pathology of their vitreous samples. The IOL group was further divided at the time of vitrectomy into patients who already had extraocular involvement (IOL with extraocular involvement group) and patients with no evidence of having extraocular involvement (IOL without extraocular involvement group). Serum sIL-2R, and intravitreal sIL-2R, MMP-2, and MMP-9 levels were assessed. RESULTS: Twenty-five eyes of 25 patients, and 15 eyes of 15 patients were included in the IOL group and uveitis group, respectively. The serum sIL-2R levels were significantly lower in the IOL group than in the uveitis group (P < 0.05), and 20.0% and 66.7% in the IOL and the uveitis group showed high sIL-2R value above the normal range. Vitreous sIL-2R tended to be higher in the IOL group than in the uveitis group (P = 0.80). Serum sIL-2R was significantly lower in the IOL without extraocular involvement group than in the IOL with extraocular involvement group (P < 0.05); 5.9% in the IOL without extraocular involvement group and 50.0% in the IOL with extraocular involvement group showed high sIL-2R value above the normal range. Vitreous sIL-2R, MMP-2, and MMP-9 tended to be higher in the IOL with extraocular involvement group than in the IOL without extraocular involvement group (P = 0.30, < 0.05, 0.16). CONCLUSIONS: Serum sIL-2R is often within the normal range in IOL patients. Even if it is within the normal range, the possibility of IOL should be considered. Serum sIL-2R is not a reliable biomarker for IOL, whereas vitreous sIL-2R may be useful for the diagnosis of IOL.

Diffuse large b-cell lymphoma of the ocular adnexa with flow cytometry analysis and review of the literature.

PURPOSE: The aim of this study was to evaluate the clinicopathological features and flow cytometry (FCM) of tumor tissues in ocular adnexal diffuse large B-cell lymphoma (DLBCL). METHODS: This retrospective, multicenter case study was designed to evaluate the clinical and immunohistochemical features of tumors. DLBCL was diagnosed based on histopathology, immunoglobulin (Ig) heavy chain gene rearrangement, and FCM in all surgically removed periocular tumor tissues. This study involved assessing percentages (%) of B-cell/T-cell markers, a natural killer cell marker, and cell-surface Ig kappa/lambda (κ/λ) expression measured by FCM analysis in tumor tissues. RESULTS: Eleven DLBCL patients (4 men and 7 women) with 11 tumors were enrolled in this study. The median age at the time of initial presentation was 73 years. The tumor cells were immunohistochemically positive for cluster of differentiation (CD) 20, while CD5 was negative in all 8 cases tested. At the time of ophthalmic diagnosis, two cases already showed systemic dissemination of DLBCL throughout the body. FCM of tumor tissues detected a high percentage of B-cell markers including CD19 and CD20 in all 11 tumors. One case with high CD10 levels in FCM was histologic transformation from follicular lymphoma. One case with a relatively low CD20 population involved a history of systemic treatments including intravenous rituximab. CONCLUSION: Although caution should be exercised when interpreting the data, FCM is useful for not only supportive diagnosis complementary to immunohistochemistry, but also facilitates a better understanding of immunopathology including histologic transformation of follicular lymphoma to DLBCL in the ocular adnexa.

Noninvasive Imaging of a Vasoproliferative Retinal Tumor Treated with Cryopexy.

Optical coherence tomographic angiography (OCTA) has emerged as a rapid, noninvasive imaging modality to visualize the vascular networks in the retina and choroid. Here, we report the clinical findings in a case of primary vasoproliferative retinal tumor (VPRT) observed by the wide-field swept-source OCTA. A 74-year-old male patient with central vision loss and metamorphopsia in his left eye was referred to our hospital. At the first visit, the best-corrected visual acuity was 20/20 OD and 20/40 OS. Fundus examination revealed the presence of the epiretinal membrane and inferotemporal reddish retinal tumor in the left eye. Fluorescein angiography and indocyanine green angiography (ICGA) showed leaky characteristics and sharply defined structure of vessels in the retinal tumor, respectively. The patient was diagnosed with the VPRT with secondary epiretinal membrane and underwent pars plana vitrectomy with internal limiting membrane peeling, retinal photocoagulation, and triple freeze and thaw procedure using cryopexy. Whereas wide-field swept-source OCTA preoperatively depicted the flow signals as distinctive vascular structures similar to ICGA, the tumor color turned out to be ischemic white, and the flow signals detected by wide-field OCTA disappeared after the surgery, indicating that the freezing effect of transscleral cryopexy sufficiently reached the surface of the tumor. In sum, wide-field swept-source OCTA is a useful imaging modality that can be noninvasively and repetitively performed to determine the treatment effect in cases of peripheral retinal tumors such as VPRT.

Placental growth factor stabilizes VEGF receptor-2 protein in retinal pigment epithelial cells by downregulating glycogen synthase kinase 3 activity.

Placental growth factor (PlGF) belongs to the vascular endothelial growth factor (VEGF) family of proteins that participate in angiogenesis and vasculogenesis. Anti-VEGF therapy has become the standard treatment for ocular angiogenic disorders in ophthalmological practice. However, there is emerging evidence that anti-VEGF treatment may increase the risk of atrophy of the retinal pigment epithelium (RPE), which is important for the homeostasis of retinal tissue. Whereas the cytoprotective role of VEGF family molecules, particularly that of VEGF A (VEGFA) through its receptor VEGF receptor-2 (VEGFR-2), has been recognized, the physiological role of PlGF in the retina is still unknown. In this study, we explored the role of PlGF in the RPE using PlGF-knockdown RPE cells generated by retrovirus-based PlGF-shRNA transduction. We show that VEGFA reduced apoptosis induced by serum starvation in RPE cells, whereas the antiapoptotic effect of VEGFA was abrogated by VEGFR-2 knockdown. Furthermore, PlGF knockdown increased serum starvation-induced cell apoptosis and unexpectedly reduced the protein level of VEGFR-2 in the RPE. The antiapoptotic effect of VEGFA was also diminished in PlGF-knockdown RPE cells. In addition, we found that glycogen synthase kinase 3 activity was involved in proteasomal degradation of VEGFR-2 in RPE cells and inactivated by PlGF via AKT phosphorylation. Overall, the present data demonstrate that PlGF is crucial for RPE cell viability and that PlGF supports VEGFA/VEGFR-2 signaling by stabilizing the VEGFR-2 protein levels through glycogen synthase kinase 3 inactivation.

Subretinal fluid accumulation in a patient with polycythemia vera after receiving a prostaglandin I2 analogue treatment.

Purpose: To report a case of polycythemia vera (PV) with subretinal fluid accumulation after the administration of prostaglandin I2 (PGI2) analogue. Observations: A 57-year-old woman diagnosed as having PV was referred to our department for the evaluation of severe metamorphopsia in the left eye, which gradually progressed after the initiation of oral administration of PGI2 mimetics. At the first visit, the patient's best-corrected visual acuities (BCVAs) were 20/20 OD and 20/30 OS. Fundus examination and optical coherence tomography revealed the presence of subretinal fluid (SRF) in the left eye and multiple serous pigment epithelial detachments (PEDs) in both eyes. Fluorescein angiography revealed central serous chorioretinopathy (CSC)-like lesions, consisting of dye pooling corresponding to the PEDs in both eyes and dye leakage in the left eye. Indocyanine green angiography and laser speckle flowgraphy revealed dilated choroidal veins and reduced choroidal blood flow, respectively. The central choroidal thickness (CCT) measured at the first visit showed a relatively thickened choroid in the left eye. Laboratory data showed mild pancytosis. The patient was diagnosed as having CSC associated with a background of PV, presumably triggered by the PGI2 analogue. One month after cessation of drug administration, the patient's BCVA improved, the CCT slightly decreased, and serous retinal detachment and PED disappeared in the left eye. Conclusions and importance: Our case of PV presenting with CSC-like lesions after PGI2 analogue administration indicates the possible risk of SRF accumulation by PGI2 analogues in patients with PV.

Alterations of choroidal circulation and vascular morphology in a patient with chronic myeloid leukemia before and after chemotherapy.

BACKGROUND: Chronic myeloid leukemia (CML) is known to cause leukemic retinopathy due to leukemia cell invasion into the choroid; however, details of the circulatory dynamics and morphological changes in the choroid are unknown. The aim of this study was to present a case of leukemic retinopathy and examine choroidal circulatory and structural analyses using laser speckle flowgraphy (LSFG) and optical coherence tomography with a binarization method, respectively. CASE PRESENTATION: A 15-year-old male diagnosed with CML complained of blurred vision in his right eye. He was ophthalmologically diagnosed with leukemic retinopathy due to retinal hemorrhage in both eyes. Tyrosine kinase inhibitors achieved complete cytogenetic remission and resolution of retinal hemorrhages at 6 months after treatment. After the treatment, the best-corrected visual acuity had recovered from 0.1 to 1.2 oculus dexter (OD) and remained at 1.5 oculus sinister (OS). The rate of change in macular blood flow assessed by the mean blur rate on LSFG was 18.3% increase OD and 25.2% decrease OS 19 months after treatment. The central choroidal thickness showed 0.4 and 3.1% reductions OD and OS, respectively. The binarization technique demonstrated that the rate of luminal areas in choroidal areas exhibited 3.2% increase OD but 4.8% decrease OS. CONCLUSION: Choroidal blood flow improved OD after treatment for CML, while it deteriorated OS, together with choroidal thinning due to reduction of luminal areas. The degrees of leukemia cell invasion into the choroidal tissue and tissue destruction might be different between the eyes in this case.

Undifferentiated Pleomorphic Sarcoma of the Conjunctiva: A Case Report and Review of the Literature.

Background/Aim: Undifferentiated pleomorphic sarcoma (UPS), previously called as malignant fibrous histiocytoma, is a mesenchymal neoplasm which shows no identifiable cellular differentiation when analyzed by presently available technology. UPS in the periocular region is extremely rare. This study describes a patient with UPS arising in the conjunctiva with literature review. Case Report: A 66-year-old man presented with a congested mass on the bulbar conjunctiva. The mass was totally excised. Histopathologically, it was a dome-shaped tumor comprising atypical short spindle cells growing as pattern-less pattern, with enlarged nuclei and eosinophilic cytoplasm. Based on immunohistochemistry, the direction of cellular differentiation was unidentifiable; therefore, it was diagnosed as UPS. The tumor showed high Ki-67 labeling index (70~80%). Conclusion: Twelve patients with conjunctival UPS have been reported with an average age of 49 years. Eight tumors of the 12 patients were observed in the limbus, and the rest in the bulbar conjunctiva. The appearance of the tumors was yellow, tan, pink, brown, or vascularized. Histopathologically, the tumors consisted of spindle-shaped cells with pleomorphism and many mitotic figures. In conclusion, conjunctival UPS is a rare malignancy with various colors, which can show aggressive nature. UPS should be differentiated from other conjunctival malignancies based on histopathological and immunohistochemical examinations including Ki-67.

Diagnostic Accuracy of Cell Block Preparations and Clinical Features Affecting It in Vitreoretinal Lymphoma.

Purpose: The purpose of this study was to examine the diagnostic accuracy of the cell block (CB) method and clinical features affecting it in patients with vitreoretinal lymphoma (VRL). Methods: This study enrolled 38 eyes in 33 VRL patients, and 7 eyes in 7 patients with idiopathic uveitis who underwent diagnostic vitrectomy. Medical records including the results of CB cytology, interleukin (IL)-10/-6 concentrations, and immunoglobulin heavy chain gene (IgH) rearrangement were retrospectively searched. Results: Patients with VRL comprised 16 women and 17 men, and the age of onset ranged from 44 to 85 years (mean: 70 years). CB preparations detected large malignant cells in 35 eyes (92%), whereas the other 3 VRL eyes were negative. Two of the latter three eyes showed subretinal infiltrates, which existed in 7 of 35 CB-positive eyes. Intravitreal IL-10 and -6 concentrations were 1866 ± 4088 pg/mL and 98 ± 139 pg/mL, respectively, and the rate of IL-10/-6 >1 was 86.9%. The presence of IgH monoclonality was 63.2%. In patients with uveitis, CB specimens revealed no atypical but small inflammatory cells. IL-6 concentration was 311.1 ± 240 pg/mL, whereas IL-10 was undetectable in six eyes, and the IL-negative rate was 85.7%. Six eyes (85.7%) with uveitis showed no IgH monoclonality. Conclusions: Diagnostic accuracy of CB preparations in VRL could achieve an equivalent outcome to IL ratio calculation and IgH monoclonality detection. The appearance of subretinal infiltrates may diminish the CB positivity.