Ectopic orbital meningioma: a retrospective case series.

BACKGROUND: To evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy. METHODS: Patient data from patients admitted to our institution during a 217-month period from August 1999 to September 2017 were included. Patient ophthalmic manifestations, radiographic features (CT and MRI), diagnosis, pathology, therapeutic regimens, and prognosis were retrospectively reviewed. RESULTS: Six patients with ectopic orbital meningioma were identified. The mean age at the first visit was 33.2 years (range, 7-56 years). All six patients displayed manifestations of exophthalmos, upper eyelid oedema, and motility impairment with a mean history of illness of 20.3 months (range 3-72 months). Optical lesions were located in the superonasal extraconal compartment (3/6, 50%), bitemporal extraconal compartment (1/6, 16.7%) and orbital intraconal compartment (2/6, 33%). Radiographic features were ill-defined, heterogeneous, enhancing soft tissue masses with extraocular muscular adhesion (6/6, 100%) and calcification (1/6, 16.7%), not adjacent to the optic nerve and not extending along the dura. Six cases were treated intraoperatively with complete surgical resection, indicating that all lesions were independent of the optic nerve and sphenoid ridge. The histopathologic classification was mostly of meningothelial cells (5/6, 83%). Immunohistochemistry revealed EMA and vimentin to have positive expression in all six cases, while two cases were calponin-positive and strongly expressed in the olfactory bulb. Postoperatively, lesions caused no visual impairment, and there were no cases of recurrence. CONCLUSIONS: Ectopic orbital meningiomas are rare tumours that are not easily diagnosed without postoperative histopathology. This report highlights some of the distinguishing features of isolated orbital lesions, especially around the location of frontoethmoidal suture. Accompanying upper eyelid oedema and eye mobility restriction were observed to be dissimilar to other orbital tumours. In these cases, a diagnosis of ectopic orbital meningioma should be considered.

Clinical analysis of transcranial orbitotomy approach on cranio-orbital tumors.

AIMS: The aims of this study were to investigate 21 cases of transcranial orbitotomy for cranio-orbital tumors and evaluate the clinical value of the surgical approach. METHODS: A retrospective study was done on 21 patients with cranio-orbital tumors. According to the tumors' location, shape, boundary, and invasion on the images, coronal scalp flap approach, subfrontal approach, or pterion approach for transcranial orbitotomy was performed. RESULTS: The surgical route was coronal scalp flap approach in 7 cases (including 3 cases combined with lateral orbitotomy), subfrontal approach in 6 cases, or pterion approach in 8 cases (including 1 case combined with evisceration of orbit). Tumors were en bloc resection in 4 cases, block resection or curettage in 13 cases, and incomplete resection in 4 cases. Postoperatively, the mean reduction in exophthalmos was 4 mm; maximum reduction was 11 mm. Ocular movement restriction was made better in 10 of 14 patients, whereas there was no change in 4 of 14 patients. Ophthalmoplegia occurred in 2 cases, complete ptosis occurred in 3 cases, and mydriasis occurred in 3 cases. No vision loss or cerebrospinal fluid leak was observed. Fourteen patients were followed up, including 2 patients with metastatic carcinoma who died dead because of systemic metastasis; 2 patients had a recurrence (schwannoglioma and meningioma). No recurrence was found in the other 10 patients. CONCLUSIONS: Cranio-orbital tumors can be removed effectively through transcranial orbitotomy, whereas operative approach is convenient to the surgical procedure and can offer better exposure. Being familiar with the tumors' location, adhesion, and invasion, coupled with the skilled surgical techniques, would improve the results and reduce serious complications.

End-to-End Deep-Learning-Based Diagnosis of Benign and Malignant Orbital Tumors on Computed Tomography Images.

Determining the nature of orbital tumors is challenging for current imaging interpretation methods, which hinders timely treatment. This study aimed to propose an end-to-end deep learning system to automatically diagnose orbital tumors. A multi-center dataset of 602 non-contrast-enhanced computed tomography (CT) images were prepared. After image annotation and preprocessing, the CT images were used to train and test the deep learning (DL) model for the following two stages: orbital tumor segmentation and classification. The performance on the testing set was compared with the assessment of three ophthalmologists. For tumor segmentation, the model achieved a satisfactory performance, with an average dice similarity coefficient of 0.89. The classification model had an accuracy of 86.96%, a sensitivity of 80.00%, and a specificity of 94.12%. The area under the receiver operating characteristics curve (AUC) of the 10-fold cross-validation ranged from 0.8439 to 0.9546. There was no significant difference on diagnostic performance of the DL-based system and three ophthalmologists (p > 0.05). The proposed end-to-end deep learning system could deliver accurate segmentation and diagnosis of orbital tumors based on noninvasive CT images. Its effectiveness and independence from human interaction allow the potential for tumor screening in the orbit and other parts of the body.

UBE2T/STAT3 Signaling Promotes the Proliferation and Tumorigenesis in Retinoblastoma.

Purpose: The purpose of this paper was to investigate the expression and function of Ubiquitin-conjugating enzyme 2T (UBE2T), a human E2 ubiquitin-conjugating enzyme, in human retinoblastoma. Methods: The expression of UBE2T in normal retina and retinoblastoma was analyzed using the Gene Expression Omnibus (GEO) databases, and its expression was immunohistochemically evaluated in 29 retinoblastoma sections and 5 normal retinas. Then CCK-8, flow cytometry, RNA-sequencing analysis, and in vivo assays were performed to explore the exact role of UBE2T in retinoblastoma. Results: We found that retinoblastoma showed higher UBE2T expression than normal retina in GEO datasets and tissues. The immunoreactive score of UBE2T ≥4 was associated with group E in IIRC, T2-T4b in pTNM staging, poorly differentiated retinoblastoma, and high-risk histopathological factors. Knockdown of UBE2T reduced the cell viability, increased the apoptosis cells and G0/G1 cells, and inhibited subcutaneous tumor growth in vivo. Mechanistic studies showed that UBE2T knockdown induced down-regulation of phosphorylation of STAT3 and its downstream genes in vitro and in vivo. Rescue assays confirmed that STAT3 signaling pathway was involved in the effect of reduced cell viability, elevated apoptosis cells, and G0/G1 cells mediated by UBE2T knockdown. Conclusions: Our data indicate that UBE2T significantly participates in the proliferation of retinoblastoma via the STAT3 signaling pathway, suggesting the potential of UBE2T as a therapeutic target for retinoblastoma treatment.

RhoA with Associated TRAb or FT3 in the Diagnosis and Prediction of Graves' Ophthalmopathy.

During Graves' disease (GD) treatment, Graves' ophthalmopathy (GO) is often ignored because only mild ocular symptoms are present in early GD. Therefore, we performed isobaric tags for relative and absolute quantification (iTRAQ) analysis and measured relevant endocrine hormones to identify predisposing factors of GO. Serum samples from 3 patients with mild GD and GO and 3 patients with GD but without GO were analyzed by iTRAQ. Based on their clinical data, 60 patients with GD were divided into the GO-free and GO groups. All patients were followed up for 7 months. Their eye conditions and changes in related biochemical indexes were recorded. The iTRAQ results showed that RhoA expression was upregulated and correlated significantly with the tight junction pathway and immunity. The changes in FT3 and RhoA from baseline to 7 months, the FT3 and RhoA baseline levels, and the TRAb titer levels in patients with GD significantly differed between the groups. ELISA and western blotting for RhoA, TRAb, and FT3 in the serum samples from GO patients showed significant upregulation, as well as elevated serum RhoA and TRAb levels in the mild stage of GO. At 7 months, the serum RhoA and FT3 levels were elevated. RhoA is a potential biomarker for mild GO. In GD patients, if an elevated serum RhoA level is accompanied by an elevated TRAb or FT3 level, GO is highly likely to occur, even when obvious ocular symptoms are absent.

Postoperative severe visual impairment: surgical outcome of 165 patients with orbital tumours in the muscle cone.

OBJECTIVE: This study aimed to evaluate the risk factors of postoperative severe vision impairment (PSVI) for a primary orbital tumour in the muscle cone. METHODS: A retrospective analysis of the patients who underwent orbitotomy for primary intraconal tumours at the Tianjin Medical University Eye Hospital from January 2010 to December 2015. RESULTS: A total of 165 cases of orbitotomy for primary orbital tumours in the muscle cone were included in the study. Postoperatively, 12 cases with vision acuity ≤20/400 or ≥4 rows of vision decline and without any corrected effect were analysed as PSVI, including no light perception (NLP) for 3 cases. The multivariate logistic regression indicated that the tumour in orbital apex (P = 0.048, OR = 4.912, 95% CI: 1.011-23.866), severe optic nerve displacement (P = 0.030, OR = 6.007, 95% CI: 1.184-30.473) and intraoperative tight adhesion (P = 0.003, OR = 12.031, 95% CI: 2.282-63.441) were the independent risk factors for PSVI. CONCLUSIONS: The incidence of PSVI for the intraconal tumour was 7.3%, and the incidence of NLP was 1.8%. The tumour in orbital apex, severe optic nerve displacement and intraoperative tight adhesion were independent risk factors for PSVI.

[Endoscopic transnasal orbital balanced decompression technique for severe Graves' orbitopathy].

OBJECTIVE: To determine the efficacy of endoscopic transnasal orbital balanced decompression technique for the treatment of severe Graves' orbitopathy. METHODS: Six cases (12 eyes) of severe Graves'orbitopathy were included in this study. All patients were undergone endoscopic transnasal orbital balanced decompression technique (orbital inner and lower wall decompression+ lateral orbital decompression) for severe Graves'orbitopathy and serious affecting visual function or appearance. RESULTS: The follow-up ranged from 12 months to 2 years and 4 months. Postoperatively, the visual acuity markly improved from 0.23 ± 0.08 to 0.48 ± 0.13 (t=-5.749, P<0.001). Proptosis measured by exophthalmoter distictly reduced from (22.08 ± 1.08) mm to (15.67 ± 1.44) mm (t=17.924, P< 0.001). The palpebral fissures obviously reduced from (12.03 ± 0.91) mm to (8.62 ± 0.75) mm (t=9.600, P< 0.001), and orbital volume evidently increased from (26.84 ± 0.62) cm³ to (31.56 ± 1.10) cm³ (t=-16.567, P<0.001). Compared with the preoperative result, the orbital pressure was significantly decreased after operation (t=-6.759 (Y=100 g), t=-11.850 (Y=200 g), t=-6.189 (Y=300 g), P<0.001). Diplopia was appeared in 3 cases, and disappeared 3 to 5 months after surgery. CONCLUSION: Endoscopic transnasal orbital balanced decompression technique is effective for the treatment of severe Graves'orbitopathy.