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 and Pathological Features of Primary Orbital Liposarcoma in Chinese Patients.

PURPOSE: Liposarcomas are rare in the orbit. We analyzed a series of primary liposarcomas to determine the features unique to the orbit. METHODS: Records from 10 Chinese patients treated for primary orbital liposarcoma at Beijing Tongren Hospital, Capital Medical University, between September 2009 and September 2020 were reviewed. RESULTS: This cohort included four men and six women with age of onset ranging from 18 to 80 years. The pathology was myxoid liposarcoma in five patients, dedifferentiated liposarcoma in two patients, well-differentiated liposarcoma and pleomorphic liposarcoma in one patient each, and dedifferentiated liposarcoma and well-differentiated liposarcoma co-existing in one case. Magnetic resonance imaging (MRI) revealed a well-defined, irregular, or lobulated mass in the orbit, which contained components that were suppressible in the fat-suppression sequence, as well as components that were enhanced by gadolinium enhancement. Nine patients relapsed after surgery, with a mean recurrence of 2.44, and one patient was lost to follow-up. The interval between treatment and first recurrence ranged from 4 months to 16 years; 55.6% of patients with orbital liposarcoma relapsed within one year. Three patients underwent local excision alone, four patients underwent excision combined with radiotherapy, and three patients underwent exenteration. Half of the patients were misdiagnosed in the pathologic diagnosis after their first or multiple surgeries. No distant metastasis, death from tumors, or invasion of adjacent organs was observed after 21-150 months of follow-up. CONCLUSION: Orbital liposarcoma is easily misdiagnosed and prone to recurrence; however, MRI findings may help identify orbital liposarcoma prior to surgery. The optimal treatment choice remains to be discussed.

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.

Automatic retinoblastoma screening and surveillance using deep learning.

BACKGROUND: Retinoblastoma is the most common intraocular malignancy in childhood. With the advanced management strategy, the globe salvage and overall survival have significantly improved, which proposes subsequent challenges regarding long-term surveillance and offspring screening. This study aimed to apply a deep learning algorithm to reduce the burden of follow-up and offspring screening. METHODS: This cohort study includes retinoblastoma patients who visited Beijing Tongren Hospital from March 2018 to January 2022 for deep learning algorism development. Clinical-suspected and treated retinoblastoma patients from February 2022 to June 2022 were prospectively collected for prospective validation. Images from the posterior pole and peripheral retina were collected, and reference standards were made according to the consensus of the multidisciplinary management team. A deep learning algorithm was trained to identify "normal fundus", "stable retinoblastoma" in which specific treatment is not required, and "active retinoblastoma" in which specific treatment is required. The performance of each classifier included sensitivity, specificity, accuracy, and cost-utility. RESULTS: A total of 36,623 images were included for developing the Deep Learning Assistant for Retinoblastoma Monitoring (DLA-RB) algorithm. In internal fivefold cross-validation, DLA-RB achieved an area under curve (AUC) of 0.998 (95% confidence interval [CI] 0.986-1.000) in distinguishing normal fundus and active retinoblastoma, and 0.940 (95% CI 0.851-0.996) in distinguishing stable and active retinoblastoma. From February 2022 to June 2022, 139 eyes of 103 patients were prospectively collected. In identifying active retinoblastoma tumours from all clinical-suspected patients and active retinoblastoma from all treated retinoblastoma patients, the AUC of DLA-RB reached 0.991 (95% CI 0.970-1.000), and 0.962 (95% CI 0.915-1.000), respectively. The combination between ophthalmologists and DLA-RB significantly improved the accuracy of competent ophthalmologists and residents regarding both binary tasks. Cost-utility analysis revealed DLA-RB-based diagnosis mode is cost-effective in both retinoblastoma diagnosis and active retinoblastoma identification. CONCLUSIONS: DLA-RB achieved high accuracy and sensitivity in identifying active retinoblastoma from the normal and stable retinoblastoma fundus. It can be used to surveil the activity of retinoblastoma during follow-up and screen high-risk offspring. Compared with referral procedures to ophthalmologic centres, DLA-RB-based screening and surveillance is cost-effective and can be incorporated within telemedicine programs. CLINICAL TRIAL REGISTRATION: This study was registered on ClinicalTrials.gov (NCT05308043).

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.

Similar therapeutic effects of 125I seed radiotherapy and γ-ray radiotherapy on lacrimal gland adenoid cystic carcinoma.

AIM: To evaluate the survival outcomes of patients with lacrimal gland adenoid cystic carcinoma who underwent eye-sparing surgery combined with 125I seed implantation radiotherapy or local external γ-ray radiotherapy. METHODS: In this retrospective comparative case series, the clinical records of 27 primary and 8 recurrent patients were reviewed. Univariate and multivariate analyses were used to identify risk factors associated with distant metastasis (DM), and the overall survival (OS) after the initial surgery was analyzed. RESULTS: The median follow-up after radiotherapy was 36mo (range 6-120mo). At the last follow-up after radiotherapy, 26 (74.3%) patients had no evidence of disease, 7 (20%) patients had DM, 2 (5.9%) patients died of DM, and 1 patient with DM was lost to follow-up. Univariate analyses showed that duration of symptoms, bone destruction, T stage classification, and wide excision surgery were risk factors influencing DM (P<0.05). The 5-year and 10-year OS rates after the initial surgery were 95.8% and 79.9%, respectively. The 5-year DM-free survival and disease-free survival rates after radiotherapy were 66.4% and 52.7%, respectively. CONCLUSION: 125I seed radiotherapy and local external γ-ray radiotherapy may have similar therapeutic effects in preventing DM. Patients with T1/T2 stage disease have a better prognosis than those with T3/T4 stage disease.

Clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma and intracranial segment of Retrobulbar optic nerve invasion.

BACKGROUND: To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion. METHODS: A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method. RESULTS: Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05). CONCLUSIONS: The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis.

Characterization, treatment and prognosis of retinoblastoma with central nervous system metastasis.

BACKGROUND: Retinoblastoma is the most common primary intraocular tumor and more and more attention has been paid to the developing countries. This study was aimed to evaluate the clinical features, treatment, and prognosis of retinoblastoma patients with central nervous system (CNS) metastasis in Beijing Tongren Hospital, one of the largest tertiary eye centers in China. METHODS: Clinical data of 31 consecutive retinoblastoma patients with CNS metastases, who were diagnosed at the Department of Pediatrics in Beijing Tongren Hospital between September 2005 and December 2015, were retrospective analyzed. RESULTS: The median age at presentation was 29 months (range from 5 to 108 months). Magnetic resonance imaging (MRI) results indicated that 16 patients (56.6%, 16/31) presented with meningeal involvement, 12 (38.7%, 12/31) presented with intracranial mass, 11 (35.5%, 11/31) presented with thickened optic nerve, and 5 (16.1%, 5/31) presented with concurrent meningeal and spinal cord membrane involvement. Retinoblastoma cells were detected in the cerebrospinal fluid (CSF) of 12 patients (44.4%, 12/27). Laboratory examinations on the blood and CSF were performed for 11 patients who had received six cycles of systemic chemotherapy, indicated that the serum level of neurone-specific enolase (NSE) after chemotherapy was significantly lower than that before chemotherapy (P < 0.05). At the end of the follow-up, 25 patients were dead with a median survival time of 6 months (1 d - 21 months), and 6 cases were alive and continued to receive treatment. CONCLUSION: Our results were basically consistent with previous reports in the developing countries, and it could be guidance for clinical treatment, prognosis and prevention of CNS metastases in retinoblastoma.

Retinoblastoma in Chinese Children Aged Five to Fourteen Years.

OBJECTIVE: To assess the characteristics of retinoblastomas enucleated from Chinese children aged 5-14 years. METHODS: This retrospective hospital-based study included all eyes with retinoblastomas consecutively enucleated in the Beijing Tongren Hospital between August 2003 and July 2013. RESULTS: Out of 1,205 patients, 47 (3.9%) were 5 years or older. All tumors in this age group occurred unilaterally, the patients had a negative family history, and the tumors were detected at an age of 6.9 ± 1.8 years (range: 5-14). The main clinical features at the time of examining the as yet untreated children aged 5-7 years (n = 30) or >7-14 years (n = 10) were leukocoria, strabismus, pseudohypopyon, hypertension, vitreous seeds ('snowballs'), and calcifications. In 12 patients (26%), the retinoblastoma had not initially been diagnosed as a tumor. Histopathology revealed tumor invasion into nonretinal tissue in 19 eyes (40%). Therapy included enucleation only (n = 22; 47%), adjuvant systemic chemotherapy (n = 24; 51%), and additional orbital exenteration (n = 1). After a mean follow-up of 3.0 ± 2.1 years (range: 0.2-9.8), which was done for 40 children, none of these children showed a tumor recurrence. CONCLUSIONS: Of the children undergoing enucleation for retinoblastoma in Beijing, 3.9% were aged 5 years or more. As in Western countries, the tumor occurrence was unilateral, their family history was negative, and the survival rate was relatively high in these children. In 1 out of 4 children, the tumor had initially been misdiagnosed due to a masquerade syndrome. Retinoblastoma should be considered in the differential diagnosis of any unclear intraocular situation in children.

[Prognostic observation of 465 patients with retinoblastoma with optic nerve invasion].

OBJECTIVE: To observe the treatment and prognosis of optic nerve invasion in retinoblastoma (RB). METHOD: The children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited. Tumor extension into the optic nerve were disclosed. Optic nerve involvement was classified into four grades according to the degree of invasion. Grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. Grade I and II are called invasion of the optic nerve before the sieve plate. Grade III and IV are called invasion of the optic nerve after the sieve plate. Other high-risk factors included extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. They were divided into two groups according to whether the merger of other high histopathologic risk factors. Treatment was delivered accordingly, and the prognosis of different degrees of optic nerve invasion was observed. The subjects were followed up for 6 months to 7 years (average: 43 months). RESULT: There were 465 subjects in this study, including 279 boys and 186 girls. The right eye was affected in 260 patients and the left eye in 205 patients. The average time from onset of symptoms to visit was 2.7 months (range 1 day-24 months). Twenty-five patients died, resulting in an overall survival rate of 94.6%. The mortality rate of patients with optic nerve involvement with grade I was 0.4%, grade II was 1.0%, grade III was 8.7% was and grade IV was 60.9%. Of the 338 with invasion of the optic nerve before the sieve plate, two died of recurrence, with a survival rates of 99.4% (336/338). Of the 127 patients who had invasion of the optic nerve after the sieve plate, twenty-three died of recurrence, with a survival rate of 81.9% (104/127) , the difference was statistically significant (χ² = 52.299, P = 0.000). A total of 379 patients did not have any other merged pathology high-risk factors, 8 died, the mortality rate was 2.1%. Of the 86 patients who had complicated with other high-risk factors, 17 died, the mortality rate was 19.8%, the difference was statistically significant (χ² = 42.955, P = 0.000). Of the 338 patients, 304 had invasion of the optic nerve before the sieve plate had not merged other pathology high-risk factors, none died, of the 34 patients who had complicated with other pathology high-risk factors, 2 had died, the mortality rate was 5.9%, the difference was statistically significant (P = 0.010). Of the 127 patients with invasion of the optic nerve after the sieve plate, 76 had not complicated with other pathological high-risk factors, 9 of whom had died, the mortality rate was 11.8%, 51 had complicated with other pathological high-risk factors, 14 of whom had died, the mortality rate was 27.5%, outcomes did significantly differ between the two subgroups (χ² = 5.014, P = 0.025). Cox multivariate analysis showed that invasion of the retrolaminar optic nerve, surgical margin of the optic nerve and sclera were influential factors of colorectal cancer. CONCLUSION: Patients with optic nerve invasion have an excellent outcome with current therapy. But for those whose resection margin was invaded, which has a high incidence of recurrence, chemotherapy is recommended for patients with postlaminar optic nerve involvement.

Clinical treatment and prognostic observation for different pathological infiltrations in 537 patients with unilateral retinoblastoma.

BACKGROUND: The use of post-enucleation adjuvant therapy to decrease the extraocular relapse rate is frequently considered, but there is much controversy about the indications for adjuvant therapy. The aim of this retrospective study was to observe the treatment and prognosis for different degrees of invasion of eye tissue in retinoblastoma (RB) and identify the indications for post-enucleation adjuvant therapy. METHODS: We recruited 537 children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2012 in our hospital, and divided them into three groups according to their number of histopathologic risk factors: 0 factor, 1 factor, or ≥2 factors. Histopathologic high-risk factors included invasion of the optic nerve posterior to the ethmoid plate (including optic nerve stumps) and extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. Treatment was delivered accordingly, and the prognosis of different degrees of histopathologic invasion was observed. The subjects were followed up for 6 months to 7 years (average follow-up time: 35 months). Statistical analysis was analyzed using χ(2) test. P < 0.05 was considered significant. RESULTS: Of the 537 RB patients who received enucleation, 25 died (overall survival: 95.3%). Of the 369 (68.7%) with no histopathologic risk factors, 1 died of recurrence, with a mortality rate of 0.3%, whereas of the 168 (31.3%) with histopathologic risk factors, 26 had recurrences and 24 died (mortality rate: 14.3%; P = 0.000). Of the 93 patients (17.3%), each of whom had a single risk factor, nine had recurrences, 16 died (8.6%). Of the 75 patients (14%) with two or more high-risk factors, 16 died (21.3%). These differences were statistically significant between the three (P = 0.000). CONCLUSION: Chemotherapy is recommended for patients with histopathologic risk factors, especially those with two or more histopathologic risk factors.

Clinical research on the treatment effects of radioactive (125)I seeds interstitial brachytherapy on children with primary orbital rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is one of the most common primary orbital malignancies. However, orbital RMS is a very rare disease, especially in childhood, and the tumor has a high degree of malignancy and rapid development. The objective of the present study was to investigate the clinical treatment effects of radioactive (125)I seeds interstitial brachytherapy on children with primary orbital RMS, which may provide a new method for treating RMS in clinical applications. Radioactive (125)I seeds were used in the present study. Primary lesions from ten children with orbital RMS, including three male and seven female patients, were selected as the targeted areas. The activity, number and spatial location of the seeds were optimized and simulated by applying computer three-dimensional treatment planning system (TPS) software. The interstitial implantation of the radioactive (125)I seeds was conducted on children under general anesthesia according to the TPS simulation results. Quality verifications of the operation were conducted by orbital computed tomography and X-ray plain film at the early stage after operation, and the children were followed up. The patients were followed up by October 2012 with an average follow-up time of 57 ± 17.43 months and a median follow-up time of 55 months. Nine cases achieved complete remission, and one case achieved partial remission, resulting in a total efficiency and survival rate of 100.0 % (10/10). Most patients recovered after treatment or had no radiotherapy side effect after the operations, though 20.0 % of the patients (2/10) experienced corneal opacity, eyeball movement disorder, or loss of sight. Radioactive (125)I seeds interstitial brachytherapy was an effective treatment for children with primary orbital RMS. Results from this study may provide a new clinical approach for the treatment of child patients with primary orbital RMS.

[Clinical feature of chronic compressive optic neuropathy without optic atrophy].

OBJECTIVE: To investigate the clinical feature of the chronic compressive optic neuropathy without optic atrophy. METHODS: Retrospective cases series study. The clinical data of 25 patients (37 eyes) with chronic compressive optic neuropathy without optic atrophy, treated in Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, from October, 2005 to March, 2014, were collected. Those patients had been showing visual symptoms for 6 months or longer, but missed diagnosed or misdiagnosed as other eye diseases due to their normal or slightly changed fundi. The collected data including visual acuities, visual fields, neuroimaging and/or pathologic diagnosis were analyzed. RESULTS: Among the 25 patients, there were 5 males and 20 females, and their ages range from 9 to 74 years [average (47.5 ± 13.4) years]. All patients suffered progressive impaired vision in single eye or both eyes, without exophthalmos or abnormal eye movements. Except one patient had a headache, other patients did not show systemic symptoms. The corrected visual acuities were between HM to 1.0, and their appearances of optic discs and colors of fundi were normal. After neuroimaging and/or pathological examination, it was proven that 14 patients suffered tuberculum sellae meningiomas, 5 patients with hypophysoma, 3 patient with optic nerve sheath meningioma in orbital apex, 1 patient with cavernous hemangioma, 1 patient with vascular malformation in orbital apex and 1 patient with optic nerve glioma. Among the 19 patients whose suffered occupied lesions of saddle area, 14 patients underwent visual field examinations, and only 4 patients showed classic visual field defects caused by optic chiasmal lesions. CONCLUSION: Occult progressive visual loss was the most important clinical feature of the disease.

[The clinical characteristics and prognosis of 92 cases of delayed occurrence of retinoblastoma].

OBJECTIVE: To retrospectively summarize the clinical features, diagnosis and treatment of retinoblastoma (RB) patients in the children aged 5 years or above. METHODS: This was a case-series study. Total of 92 patients (107 eyes) with RB aged 5 years or above in Department of Pediatric,Beijing Tongren Hospital from September 2005 to May 2010 were included in the study. The diagnosis of RB was established using clinical examination and histopathology analysis.Statistical analysis in the characteristics of disease and initial symptoms was performed in 92 cases. Kaplan-Meier curve was employed to analyze the survival function. RESULTS: (1) The medium age of the patients was 6.25-year. Male accounted for 66.30% (61/92) and female was 33.69% (31/92) . According to the results of statistical scatter diagram, the age of the disease onset was ranged from 5.00 to 8.33 years. Percentile for age 5.77, 5.83 and 7.85 years were the 25th, 50th, 75th respectively. (2) In 92 cases, 83.69% (77/92) of the patients were unilateral RB and 16.31% (15/92) were bilateral RB. (3) Leukocoria was the most common manifestation in the diagnosis early RB, accounting for 55.43% (51/92) .Secondary symptoms were blurred vision, eye redness and photophobia, accounting for 23.91% (22/92). Other symptoms included strabismus, proptosis, glaucoma and the abnormal in fundus examination, accounting for 20.64% (19/92) .No family history of RB was found in all of cases studied. (4) Among 107 eyes with RB, 76 eyes were enucleated up to February 2013. The rate of enucleation was 71.02%. (5) By February 2013, the average following-up time was 39 months, during the time 1 case was lost, 6 cases were dead, the overall survival rate was 93.46% (85/92) . The result of Kaplan-Meier curve showed that the estimates of 5-year expected average total survival time was (72.61 ± 1.89) months(95%confidence interval was 68.9-76.3 months). CONCLUSIONS: Unilateral RB are the most common cases in the age of 5 years or above, and the most common symptom is Leukocoria.Overall survival rate of older RB patient is high with comprehensive treatment.

Multidisciplinary collaborative therapy for 30 children with orbital rhabdomyosarcoma.

OBJECTIVE: To explore clinical experience and propose new ideas for treating children diagnosed with orbital rhabdomyosarcoma (RMS). METHODS: We retrospectively analyzed the clinical data for30 patients (16 males and 14 females, with a median age of 6.2 years) with primary orbital RMS who were enrolled in the Department of Eye Oncology and Pediatrics of our hospital from November 2004 to December 2012. International Rhabdomyosarcoma Organization Staging Standards indicated that among the 30 patients, 4 cases were in phase II, 20 were in phase III, and 6 were in phase IV. All patients underwent a multidisciplinary collaborative model of comprehensive treatment (surgery, chemotherapy, external radiotherapy, 125I radioactive particle implantation, and autologous peripheral blood stem-cell transplantation). RESULTS: Follow-up was conducted until March 2013, with a median follow-up time of 47.2 months (5 to 95 months), and 7 deaths occurred. The 2-year estimated survival rate reached 86.1%, the ≥3-year estimated survival rate was 77%, and the 5-year estimated survival rate was 70.6%. CONCLUSIONS: The multidisciplinary collaborative model can be a safe and effective approach to the comprehensive treatment of children with orbital RMS. It has clinical significance in improving the tumor remission rate.

[Transnasal endoscopic removal of orbital apex cavernous hemangioma].

OBJECTIVE: To explore the strategy and indications of endoscopic transnasal resection of orbital apex cavernous hemangiomas (OACH). METHODS: Eleven patients aged from 30 to 62 years-old diagnosed as OACH bypostoperative histopathology were reviewed retrospectively. Four males and 7 females were included. Both ophthalmological examination and rhinologic evaluation were adopted preoperatively. The surgeries were carried out under general anesthesia endoscopically by the same senior surgeon. After ethmoidectomy, the orbital lamina papyracea was opened, and the orbital fat and muscles were pushed back into the orbit by using the brain cotton, and then the orbital tumor was removed. The patients were kept follow-up both in ophthalmologic and rhinologic departments. RESULTS: As suggested by preoperative imagings, 4 lesions located in the extraconal space (nasal side), 6 between the optic nerve and the internal rectus muscle of the intraconal space, and 1 outside the optic nerve in the intraconal space. Total resection was achieved in 9 cases, and orbital decompressions were done in 2 cases. Meanwhile, orbital wall reconstruction was done in 7 cases. The follow up ranged from 6 to 47 months. Seven patients achieved visual acuity improvement and no deteriorations were found in other 4 patients. Defects of vision field in 3 patients disappeared after 2 weeks. No operative or postoperative complications occurred. CONCLUSIONS: The OACH located in the nasal side of extraconal space and between the optic nerve and the internal rectus muscle of the intraconal space can be accessed endoscopically by intranasal approach. Using the brain cotton to push the orbital fat and muscles back into the orbit and an experienced endoscopic surgeon are important to access a successful intranasal endoscopic removal of orbital apex tumor.

Role of B lymphoma Mo-MLV insertion region 1 in the oncogenic behavior of retinoblastomas.

PURPOSE: This study investigated the relationship between B lymphoma Mo-MLV insertion region 1 (BMI-1)--a polycomb protein for stem cell self-renewal and proliferation--and the clinicopathological parameters of human retinoblastomas, including differentiation status and retinal tissue invasion, as well as the effects of BMI-1 on retinoblastoma Y79 cells. METHODS: Thirty-four archived human retinoblastoma samples were recruited for BMI-1 immunohistochemistry. The percentage of BMI-1-expressing cells was scored by independent pathologists and the data were correlated with the clinical features. Y79 cells were transfected to overexpress or specifically inhibit BMI-1 for cell proliferation, propidium iodide cell cycle and terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) apoptosis analyses, multicellular sphere formation assay, and gene expression study. RESULTS: BMI-1 was widely expressed in human retinoblastomas. Higher percentages of BMI-1-expressing cells were selectively limited to undifferentiated tumors and those tumors undergoing invasion to the optic nerve and choroid. However, there was no difference in BMI-1 expression in retinoblastoma retinas with or without tumor invasion. In Y79 cells, BMI-1 stimulated cell proliferation and suppressed apoptosis with reduced p14ARF and p16INK4 expression, along with upregulation of proliferating cell nuclear antigens cyclin D1 and D2. In contrast, silencing BMI-1 reversed these changes. It also upregulated CHX10 and Rx, but not other retinal development-related genes, including nestin and neurofilament M. CONCLUSIONS: Our work indicates that BMI-1 might render important oncogenic property of retinoblastomas and it could be a therapeutic target for the cancer treatment.