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Objective To investigate the efficacy and safety of cranio - orbital approach for the removal of cavernous sinus - orbital communication trigeminal schwannomas. Methods and Results A total of 23 patients with cavernous sinus-orbital communication trigeminal schwannoma were included from January 2013 to January 2019, the combined cranio-orbital approach (modified Dolenc approach combined with supraorbital approach) was used for surgical resection of tumors. Total tumor resection was performed in 20 cases and nearly total resection in 3 cases. Thirteen patients with preoperative visual loss, 7 of them further decreased visual acuity; 5 cases with preoperative eye movement disorder and/or ptosis, 3 cases with residual eye movement disorder, and 11 cases appeared new eye movement disorders after surgery. None of the 23 cases occurred cerebrospinal fluid leakage, intracranial hematoma or intracranial infection occurred, no death and no tumor recurrence case. Conclusions Cavernous sinus-orbital communication trigeminal schwannoma, due to the special tumor occurrence site and surrounding anatomical structure, was easy to cause complications such as visual loss and eye movement disorder during operation. The combined cranio-orbital approach was used, it may increase the total resection rate, and also show relatively low surgical complications rate. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the survival outcomes of patients with lacrimal gland adenoid cystic carcinoma who underwent eye-sparing surgery combined with 125 I 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: 125 I 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., (International Journal of Ophthalmology Press.)

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.

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.

Retinoblastoma is the most common childhood primary intraocular malignancy, with the majority of cases being diagnosed before 5 years of age. Retinoblastoma in adults is extremely rare. Here, we report the case of a 20-year-old man who presented with a 3 year history of blurred vision in the right eye. Imaging did not reveal the typical presentation of retinoblastoma. After considering Coats' disease, a diagnosis of late-presenting retinoblastoma was made through cytological analysis. Diagnosis of retinoblastoma should be considered in the presence of uncertain mass lesions in the fundus of an adult.

Unlabelled: Xeroderma pigmentosum is a rare autosomal recessive disease characterised by hypersensitivity to sunlight, and is associated with a high incidence of skin cancer. We report a case of xeroderma pigmentosum with malignant neurilemoma in a 46-year-old woman which is unique due to its presentation, which was confirmed histopathologically., Trial Registration Number: 31095.

Objective: To investigate the influence of enucleation with or without orbital implant on the occurrence of apoptosis of orbital osteocytes and to explore the mechanism of orbital implant in the prevention and treatment of orbital malformations., Methods: It was an experimental research. Twenty-one age and weight-matched New Zealand rabbits were divided into three groups: enucleation group, enucleation with implant group and the control group. At one-month-old, the left eyes of the rabbits were enucleated in the enucleation group; an orbital implant was inserted after enucleation in the implant group. The left orbits of rabbits in the control group were served as the controls. At two-month-old, all rabbits were sacrificed, apoptosis of osteocytes of the zygomatic bones was observed by photomicroscopy, electron microscopy and TUNEL staining technology. The ratios of apoptosis osteocytes were calculated and analyzed in these three groups., Results: Classical apoptosis of osteocytes was found with photomicroscopy and electron microscopy. The distribution of apoptosis of osteocytes was irregular in bone sections. The ratio of apoptosis cells in the enucleation group was significantly different from that in the other two groups (P<0.01). There was no significant difference in ratios of apoptosis cells between the implant group and the controls (P>0.05)., Conclusions: Apoptosis of osteocytes participates in the normal development of bony orbit. The results of this study indicate that apoptosis of osteocytes plays a role in the development of orbital malformation after enucleation and orbital implant can prevent the orbital malformation after enucleation.

Objective: To investigate the clinical manifestations of lacrimal gland tumor involving the anterior and middle cranial fossae and the effect of transcranial-orbital approach in treatment of such tumor., Methods: A retrospective study was conducted on the clinical data of 23 cases lacrimal gland tumor involving the anterior and middle cranial fossae confirmed by radiological examination, including 11 cases of adenoid cystic carcinoma, 6 cases of pleomorphic adenocarcinoma (malignant mixed tumor), 2 cases of adenocarcinoma, 1 case of squamous cell carcinoma, 1 case of ductal carcinoma, 1 case of mucoepidermoid carcinoma, and 1 case of benign mixed tumor, 15 males and 8 females, aged 42.5 (2 - 76), with a case history of 43 months (2 months to 27 years), with the chief complaints of progressive proptosis, disgenesia of the eye ball, and orbit pain, all undergoing transcranial-orbital operation from August 1998 to February. 2006. Follow up was conducted for 1 month to 7 years., Results: Postoperatively, ophthalmoplegia was found in 4 cases, and blindness in 1 case. There was no operative death or other significant complication. Recurrence of tumor occurred in 4 cases, and 1 case died from distant metastasis of adenocarcinoma., Conclusions: Malignant lacrimal gland tumors, mainly adenoid cystic carcinomas, incline to involve the anterior and middle cranial fossae. Adequate orbital apex decompression and exposure of the tumor can result from suitable transcranial-orbital approach. However, complete surgical excision is difficult, and the tumor has a tendency to recur post-operatively. Suitable treatment strategy should by combination of operation with irradiation or chemotherapy. Prognosis is poor.

Objective: To investigate the histopathological classification and clinical features of ocular adnexal lymphoproliferative lesions., Methods: The clinical, histomorphological and immuno-histochemical features of 112 cases of ocular adnexal lymphoproliferative lesions (116 paraffin specimens) were studied retrospectively. The lesions were classified according to the World Health Organization classification of tumors of haematopoietic and lymphoid tissues (2001)., Results: This group of patients' ages averaged at 49. The mean duration between the onset of the symptoms and the time of presentation was 22 months. Sixteen patients (14.3%) had bilateral lesions. Proptosis or local orbital mass was presented in 69 cases (61.6%). Reactive lymphoid hyperplasia was diagnosed in 11 cases (9.8%) and atypical lymphoid hyperplasia in 10 cases (8.9%). The rest 91 cases (81.3%) were diagnosed as lymphoma, among which 74 cases (81.3%) were extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-EMZL)., Conclusions: MALT-EMZL is the most frequent type of ocular lymphoproliferative lesions in ocular adnexa. The ocular adnexal lymphoproliferative lesions show an indolent course clinically, tending to affect the old. Bilateral case is not uncommon.

Objective: To investigate the clinical effect of early stage operation for optic nerve sheath meningioma., Methods: Retrospective study The clinical data of 21 cases of optic nerve sheath meningioma, 6 males and 25 females, aged 39 (12-60), with the chief complaints of loss of vision in 13 eyes (including 6 blind eyes) and proptosis in 8 eyes who underwent resection of the tumors via transcranio-orbital approach were analyzed retrospectively. Radiology showed 15 tumors growing along the optic nerve and enclosing it (flat type), 5 of which showed intracranial invasion; and other 6 of which had the tumors located on one side of optic nerve with spherical shape (nodular type) without intracranial invasion. The patients were followed up for 2-6 years., Results: Total resection was accomplished in 15 cases, and partial removal in 6 cases. Function of oculomotor nerve was preserved in 17 cases, and function of abducent nerve was preserved in 18 cases, visual function was successfully preserved in the 2 cases of small tumor. Tumor recurred in two cases., Conclusion: Resection of optic nerve sheath meningioma via transcranio-orbital approach has the advantage of totally removing the tumor and preserving the function of the oculomotor and abducent nerves, preventing tumor from intracranial invasion. Early stage operation is recommended. Visual function may be preserved in the cases of small tumors.

Objective: A retrospective study of histopathological and clinical aspects of 261 cases of lacrimal tumors (273 paraffin specimens) from Ophthalmic Pathology Laboratory, Beijing Tongren Hospital (Dec. 1961 to Jun. 2002) was performed to investigate the pathological classification and clinical features of these tumors., Methods: Clinical features were analyzed from 261 patients of lacrimal tumors, including patient's history, age, gender, laterality, visual acuity, ultrasound B scan, X-ray, CT, MRI, pre- and post-operation condition, follow-up data and others. These data were compared with the pathological features., Results: Pleomorphic adenoma was the most common tumor in epithelial tumors of lacrimal gland, followed by adenoid cystic carcinoma, pleomorphic adenocarcinoma and adenocarcinoma. Recurrence was observed in 12 cases. The recurrence rate is 4.6%. Four cases was dead, the mortality was 1.5%., Conclusion: Pleomorphic adenoma shows a low mortality and a relatively high recurrence rate. The main treatment for pleomorphic adenoma is surgical treatment. It is important to have a correct clinical diagnosis, to select the proper surgical method, drug treatment and postoperative management based on the pathological diagnosis, which can result in a decrease of recurrence rate.

Objective: To investigate the relationship between the histopathology and the imaging analysis of orbital pseudotumor to judge the histopathological types of these tumors before treatment according to imaging analysis, in order to search for different therapeutic approaches for different pathologic types and to predicate prognosis., Methods: Fifty-six cases (58 eyes) of orbital pseudotumor that had been operated in Tongren hospital were studied. The clinical manifestations of these patients were reviewed. Changes in the imaging analysis and histopathologic examination were observed and their relationship was studied. Methods of treatment and their prognosis were also analyzed., Results: Historically, the lymphocytic infiltrated type of orbital pseudotumor composed of large amounts of chronic inflammatory cells, only a small amount of collagen was found, so the imaging features of the lymphocytic infiltrated type presented as low or none reflecting areas with a clear posterior demarcation on B scan, and the signals were low or equal on T(1)WI and high on T(2)WI, which could be enhanced by contrast. Oppositely, the fibrotic type was composed of large amounts of fibrotic tissue, and few chronic inflammatory cells were found, so that only the anterior border of the lesion could be identified and the posterior demarcation was blur on the B scan, and the signals were low or equal on T(1)WI and low on T(2)WI, which could not be enhanced or could be enhanced only slightly by changing the contrast. The mixed type presented as an admixture of chronic inflammatory cells and fibric hyperplasia, and the imaging of the mixed type was midway between the lymphocytic infiltrated type and the fibrotic type. Forty-two patients of lymphocytic and mixed types which were identified by histopathology and imaging analysis received a local and systemic corticosteroid therapy. The effect was unstable, and recurrence occurred easily. Therefore, operations were performed on these patients, thirteen of them were treated with low-dose radiotherapy (2 000 - 3 000 cGy) two weeks after operation. Fifteen cases recurred after the operation and 4 cases recurred after the radiotherapy. The fibrotic type of orbital pseudotumor was identified in 12 cases in which no effect could be observed after conservative therapy and operations were performed subsequently. Two cases recurred and were operated again. Exenteration was performed in 2 cases of 56 cases. The cure rate of the composite treatment in the present series was 68.5%., Conclusions: Different types of orbital pseudotumor present different imaging exhibition, and imaging features are closely related with histopathological changes. Lesions can be localized by CT scan, and also can be localized by B scan and MRI. While it is difficult to obtain a cure result in the orbital pseudotumor, the cure rate can be improved by composite treatment.