Orbital Solitary Fibrous Tumors: A 20-Year Cases Series Study in a Single Center.

PURPOSE: To investigate the clinical manifestations, imaging, pathology, and prognosis of orbital solitary fibrous tumors (OSFTs). In addition, the surgical incisions and the treatment outcomes were also evaluated. METHODS: A total of 89 patients with pathologically confirmed primary OSFTs were enrolled onto this study. Clinical and histopathological characteristics, imaging data, treatment modalities, and follow-up time, including tumor recurrence and death, were documented. The outcome measures included overall survival and disease-free survival time. RESULTS: Among 89 eligible cases, the median age of presentation was 39 years (range: 5-80 years) at the initial diagnosis. The most common presenting symptom was painless proptosis (54, 60.67%), then palpable mass (31, 34.83%), swelling (29, 32.58%), and impaired ocular motility (27, 30.34%). Tumor-related severe impaired vision was found in 11 patients (12.36%), including no-light-perception blindness (6, 6.74%), light-perception (2, 2.25%), and hand-movement (1, 1.12%). The preoperative imaging (computed tomography and magnetic resonance imaging) accurate diagnostic rate of OSFTs was 17.98% (16, 95% CI: 0.098-0.261), and misdiagnosis rate was 25.84% (23, 95% CI: 0.166-0.351). Grossly intact masses were excised for 27 patients (30.34%). Among the 89 patients, 33 (37.08%) were recurrences, and the median of these recurrent patients' interval between the first and the last operation was 7.33 years (range: 0.12-29.69 years). In 81 patients with complete follow-up data, the median course of the disease was 9.64 years (range: 1.55-33.65 years) from the onset OSFT. The overall survival rate of the 81 patients was 93.83% with a median course of 8.48 years (range: 0.38-30.4 years) from diagnosis of OSFT, and the disease-free survival rate of 81 patients was 91.36% with a median follow-up of 4.76 years (range: 0.08-19.22 years) after the last surgery. Of all the 81 patients, 5 patients (6.17%) developed local recurrence, and 3 patients (3.70%) died from tumor-related diseases, including pulmonary metastasis (2, 2.47%) and complications from intracranial lesions (1, 1.23%). Ten patients (11.24%) received postoperational radiation therapy, including 125I seeds implantation (5, 6.17%) and external beam radiotherapy (5, 6.17%), and remained no recurrence. CONCLUSIONS: In this series, OSFTs showed long courses and easy recurrence. Although it was very important to choose a proper surgical incision for intact resection of OSFTs at the initial surgery to avoiding recurrence, preoperative imaging is of very limited use since it is not able to identify OSFTs effectively. Postoperative radiotherapy may be beneficial to reduce the recurrence of OSFTs with malignant pathologic features.

Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China.

BACKGROUND: In this study, we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma (RB) in a single center in China with a large sample collection spanning 17 years. METHODS: The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected, and a retrospective analysis was conducted. RESULTS: The median age of the participants was 28.3 months. There were 3624 affected eyes, 12.4% of which were in groups A-C, 67.1% in groups D-E and 16.2% were not specified. The primary symptom observed in most cases was a white pupil, accounting for 66.5%, followed by strabismus (12.8%). The median follow-up time was 59.7 months. The enucleation rate was 71.3% (703/986) in a single left eye and 72.5% (702/968) in a single right eye. The overall survival (OS) rate was 95.8% (2444/2552) because 237 patients dropped out, and 109 died. Kaplan‒Meier survival analysis showed that the median survival time (MST) was 125.92 months [95% confidence interval (CI) = 124.83-127.01]. Cox multivariate survival analysis showed that trilateral RB (P = 0.017), metastasis site (P = 0.001), and combined distant tissue metastasis (P = 0.001) were independent prognostic factors for RB. The OS of 44 cases of familial RB was 93.2% (41/44), with an MST of 80.62 months (95% CI = 67.70-93.54). CONCLUSIONS: The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay. More importantly, the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

Clinical Analysis of Primary Malignant Lacrimal Sac Tumors: A Case Series Study With a Comparison to the Previously Published Literature.

PURPOSE: To report management of a series of patients with primary malignant lacrimal sac tumors and to compare these results to the previously published literature. MATERIALS AND METHODS: A total of 27 patients with pathologically confirmed primary malignant lacrimal sac lesions were enrolled into this study. Pathological classifications, clinical characteristics, various treatment modalities and follow-up time, including tumor recurrence, were documented. The outcome measures included overall survival, progression-free survival, and median survival time. RESULTS: Among 27 eligible cases, 33.33% (9/27) of the tumor was non-Hodgkin B-cell lymphoma, and 33.33% (9/27) was squamous cell carcinoma; both were the most common tumor in this series, followed by adenocarcinoma 18.52% (5/27), then melanoma 7.41% (2/27). Treatment modalities included surgery, radiotherapy, and/or chemotherapy, the overall survival rate of 27 patients was 70.37%, with a median follow-up of 45 months (range: 7 mo-16 y), 8 patients had died from metastatic disease, but 13 patients remained without evidence of recurrent tumor. The 5-year overall survival and progression-free survival for all cases were 73.33% and 66.67%, respectively. The median survival time for 5 deceased patients with interstitial brachytherapy was 98 months, and 5-year survival rate was 60%. CONCLUSIONS: In this series, among primary malignant lacrimal sac tumors, the proportion of lymphoma had increased when compared with the previously published literature, and multidisciplinary therapy may lead to a good prognosis in the majority of patients with the tumors and patients may benefit more from interstitial brachytherapy than external beam radiotherapy.

Differential expression of microRNA related to irritable bowel syndrome in a rabbit model.

OBJECTIVE: This study aimed to evaluate the differential expressions of microRNAs (miRNAs) in white hair black eye (WHBE) rabbits of irritable bowel syndrome (IBS). METHODS: WHBE and Japanese white (JW) rabbits were divided into the control and IBS groups. The IBS groups were exposed to moist heat, stress and low-dose laxatives. Their intestinal movement rate was measured. Blood samples were taken to detect serum 5-hydroxytryptamine (5-HT) and dopamine levels and colonic tissues were obtained to detect c-Fos expression by immunohistochemistry. Deep sequencing technology was used to obtain miRNA sequences in the intestinal tissues of WHBE and JW control groups. Expressions of 14 miRNAs were measured by real-time polymerase chain reaction in both the control and the IBS model groups. RESULTS: Serum 5-HT and dopamine levels, intestinal movement rate and c-Fos expressions in the WHBE rabbits were significantly increased compared with the control group. MiR-29a-3p, miR-24-3p, miR-221-3p, let-7f-5p, let-7g-5p, let-7i-5p, miR-192-5p, miR-126-3p and miR-130b-3p expressions in WHBE IBS rabbits at day 14 were significantly higher than those in the control group while miR-324-3p and miR-132 were downregulated. MiR-29a-3p, let-7i-5p, miR-192-5p and miR-126-3p were significantly upregulated only in JW IBS rabbits at day 14 and miR-324-3p, miR-223-3p and miR-132 were significantly downregulated in JW IBS group. MiR-24-3p, miR-221-3p, let-7f-5p, miR-126-3p and miR-130b-3p expressions in WHBE IBS rabbits were higher than that in JW IBS rabbits. CONCLUSIONS: Twelve miRNAs were differentially expressed in IBS rabbits. Five are specific in WHBE IBS rabbits, suggesting that they play a role in increased sensitivity to IBS.

Analysis of post-operative endophthalmitis after pars plana vitrectomy: a 10-year experience at a single center.

BACKGROUND: Endophthalmitis can be a devastating complication after pars plana vitrectomy. The incidence of postvitrectomy endophthalmitis is significantly lower than that of endophthalmitis occurring after other intraocular operations. However, normal post-operative pain and inflammation may mask endophthalmitis and lead to delayed diagnosis and grave visual consequences. This study aimed to summarize the outcomes of cases that underwent pars plana vitrectomy over a 10-year period and to analyze the characteristics of post-vitrectomy endophthalmitis. METHODS: A retrospective observational case study was conducted on all the cases who underwent pars plana vitrectomy in the Beijing Tongren Hospital between January 1, 2002 and March 31, 2012. All cases of endophthalmitis that occurred during a period of 10 years and 3 months were reviewed, and the possible risk factors, clinical findings, causative organism(s), and the sources of infection were analyzed. RESULTS: Within the 10-year observational period, 14 patients developed endophthalmitis after pars plana vitrectomy. The incidence of post-vitrectomy endophthalmitis (0.05%) was lower than that reported previously from the same center (0.12%). Staphylococcus epidermidis (five patients, 35.71%) was the most common organism identified in aqueous or vitreous cultures. Eight patients (57.14%) had diabetes mellitus. There was no statistically significant difference (P > 0.05) in the incidence of endophthalmitis between period 1 (with antibiotic pretreatment) and period 2 (without antibiotic pretreatment). Surgical procedures for the treatment of endophthalmitis were performed in 10 patients (71.43%). CONCLUSIONS: This series of cases showed that the incidence of endophthalmitis after pars plana vitrectomy is lower than what was previously reported in our hospital. A variable degree of corneal edema with relatively normal or mildly increased intraocular pressure was one of the commonly observed characteristics of post-vitrectomy endophthalmitis. Staph. epidermidis was the most common causative organism, and antibiotic pretreatment did not lower the incidence of postvitrectomy endophthalmitis.

Effect of the regimen of Gaoshan Hongjingtian on the mechanism of poly (ADP-ribose) polymerase regulation of nuclear factor kappa B in the experimental diabetic retinopathy.

BACKGROUND: Poly(ADP-ribose) polymerase (PARP) plays an important role in the death of retinal capillary cells in diabetic retinopathy (DR) partly via its regulation of nuclear factor kappa B (NF-κB). The current study investigated the effect of the regimen of Gaoshan Hongjingtian (RG) on the mechanism of PARP regulation of NF-κB, and demonstrated the possible impact of the RG and Gaoshan Hongjingtian (Rhodiola sachalinensis, RS) on diabetic retinopathy. METHODS: Wistar rats were made diabetic by administering streptozotocin. They were then assigned to three groups at random. After 2 months, the three groups of these diabetic rats were treated with RS or RG, or untreated. Analyses of expression levels of PARP, NF-κB, and intercellular adhesion molecule-1 (ICAM-1) in the retinas of rats in different groups were performed by Western blotting and immunohistochemical assays, and mRNA levels of NF-κB and ICAM-1 were determined by real-time polymerase chain reaction (PCR). In addition, the basement membranes of capillaries in the rats' retinas were observed using electron microscopy, and diabetes-induced capillary degeneration (ghost pericytes and acellular capillaries) were quantitated. RESULTS: From the third month after the injection of streptozotocin, the diabetic rats were given daily RG, RS or tap water separately. The diabetic rats failed to gain weight compared with normal age-matched rats, whereas their glycated hemoglobin levels were significantly increased. After 5 months, the mRNA levels of NF-κB and ICAM-1 and the protein expression of PARP, NF-κB, and ICAM-1 were significantly increased in the retinas of diabetic rats in the untreated group compared with the nondiabetic controls. After 8 months, the number of degenerated retinal capillaries (ghost pericytes and acellular capillaries) was significantly increased in the diabetic rats in the untreated group compared with normal age-matched rats. RG and RS inhibited diabetes-induced over-expression of PARP, NF-κB, and ICAM-1 in the retinas of diabetic rats at the end of 5-month diabetic duration. Treatment using RG and RS significantly inhibited increases in the number of acellular capillaries and pericyte ghosts and suppressed the basement membrane thickening in the retinas of rats with diabetes for 8 months compared with the control diabetic rats. CONCLUSIONS: These results indicate that PARP plays an important role in the pathogenesis of diabetic retinopathy. RS and RG may have acted on the mechanism of PARP regulation of NF-κB, which suppressed the expression of NF-κB and ICAM-1, and led to the inhibition of retinal capillary degeneration.

[Analysis of the misdiagnosis of bilateral iridocorneal endothelial syndrome].

OBJECTIVE: To analyze the misdiagnosis of bilateral iridocorneal endothelial (ICE) syndrome and the possible reasons. METHODS: The patients seen from January 1998 to December 2011 at Beijing Tongren Hospital, misdiagnosed as bilateral ICE syndrome, were analyzed retrospectively. And a literature search was performed with a Wanfang computerized database website for the case studies of bilateral ICE syndrome. The history, clinical profiles and the findings of corneal, iris and anterior chamber examinations were analyzed. The clinical manifestations of patients with ICE syndrome were also compared with those of Axenfeld-Rieger syndrome, posterior polymorphous dystrophy (PPMD) and chronic iritis for differential diagnosis. RESULTS: Ten patients were diagnosed as bilateral ICE syndrome. However, among them, 5 patients were diagnosed as Axenfeld-Rieger syndrome, 2 patients as PPMD, 3 as chronic iritis and none as bilateral ICE syndrome. There were 9 Chinese reports of bilateral ICE syndrome in a total of 18 patients. Analysis showed that Axenfeld-Rieger syndrome needed to be ruled out in 10 patients, PPMD could not be ruled out in 2 patients while diagnosis could not be made in 6 patients for a lack of proof. CONCLUSION: Despite the reporting of bilateral cases, ICE syndrome is usually unilateral. With specific micrographic features for a definite diagnosis of ICE syndrome, ICE cells may become an important feature differentiating from Axenfeld-Rieger syndrome, PPMD and chronic iritis.

Dilemma in management of ocular medulloepithelioma in a child.

Medulloepithelioma, a rare tumor, arises from the epithelium of the medullary tube. In this article, we present a 3-year-old boy who suffered from secondary glaucoma, initially presumed the primary disease was endophthalmitis. Subconjunctival mass was later found, pathologically proved to be medulloepithelioma. We discuss the patient management with emphasis on the early signs of examination and the role of ultrabiomicroscopy (UBM) in evaluating pediatric secondary glaucoma and in influencing the management of patients with medulloepithelioma.