BACKGROUND: Spheno-orbital meningioma (SOM) represents a unique variant of sphenoid wing meningiomas, distinguished by its propensity for bone infiltration and cranio-orbital involvement. SOM exhibits a considerable incidence of misdiagnosis and recurrence.
OBJECTIVE: To elucidate the clinical, radiological, and pathological characteristics of SOM.
METHODS: Review of electronic medical records, histopathology, radiological images and follow-up information of 100 SOM patients.
RESULTS: Of the 100 patients (28 males, 72 females) with SOM, mean age was 46.8 ± 12.6 years and prevalent symptoms were proptosis (99%). All the CT scans showed hyperostosis with 89.3% of the hyperostosis having an irregular edge. In MRI scans, dural tail sign was observed across all patients and the cranio-orbital tumors often penetrated temporal muscle (74.1%), extraocular muscle (74.1%) and lacrimal gland (63%). All the 100 patients underwent surgical intervention, and among them, 62 individuals received postoperative radiotherapy. Grade I resections had a lower recurrence rate(16.7%), which further decreased with the addition of radiotherapy(13.9%). In contrast, all patients with grade II or higher grade resections without radiotherapy experienced recurrence, indicating a higher risk associated with less complete tumor removal. The pathological examination revealed that intraorbital sections exhibited comparable tumor density to intraorbital SOM tumors, along with increased fibrous density but decreased vascular distribution.
CONCLUSIONS: Radiological characteristics of SOM included cranio-orbital tumors, hyperostosis of the sphenoid wing with an irregular edge, and dural tail sign. Combination of gross total resection and adjuvant radiotherapy was recommended to minimize recurrence rate. Intracranial SOM tumors tended to be softer and more bleed-prone than intraorbital sections, necessitating surgical precision.

Background: This report aims to present the case of a pediatric patient with a recurrent tumor in the superolateral orbit. Clinical Presentation: An 8-year-old patient was initially treated for a tumor in the superolateral orbit via a transconjunctival approach. The histopathological diagnosis was epidermoid cyst. Postoperatively, chronic inflammation and fistula developed in the lateral canthus area. Magnetic resonance imaging revealed a residual tumor posterior to the original tumor location. The patient was treated via a modified orbitozygomatic (mOZ) craniotomy approach that was originally applied in neurosurgery, and complete tumor removal was achieved. A temporary paralysis of the frontotemporal branch of the facial nerve was observed and fully resolved within one month following surgery. At the 18th month of follow-up, the visual, neurological, and cosmetic results were found to be satisfactory. Conclusions: mOZ craniotomy can be used to access and operate on recurrent orbital tumors in pediatric patients where other more aggressive surgical approaches should be avoided.

OBJECTIVE: 18F-N-(2-(Diethylamino)ethyl)-5-(2-(2-(2-fluoroethoxy)ethoxy)ethoxy) picolinamide (18F-PFPN) is a novel positron emission tomography (PET) probe designed to specifically targets melanin. This study aimed to evaluate the diagnostic feasibility of 18F-PFPN in patients with ocular or orbital melanoma.
METHODS: Three patients with pathologically confirmed ocular or orbital melanoma (one male, two females; age 41-59 years) were retrospectively reviewed. Each patient underwent comprehensive 18F-PFPN and 18F-fluorodeoxyglucose (18F-FDG) PET scans. The maximum standardized uptake value (SUVmax) of the lesion and the interference caused by background tissue were compared between 18F-PFPN and 18F-FDG PET imaging. In addition, the effect of intrinsic pigments in the uvea and retina on the interpretation of the results was examined. The contralateral non-tumorous eye of each patient served as a control.
RESULTS: All primary tumors (3/3) were detected using 18F-PFPN PET, while only two primary tumors were detected using 18F-FDG PET. Within each lesion, the SUVmax of 18F-PFPN was 2.6 to 8.3 times higher than that of 18F-FDG. Regarding the quality of PET imaging, the physiological uptake of 18F-FDG PET in the brain and periocular tissues limited the imaging of tumors. However, 18F-PFPN PET minimized this interference. Notably, intrinsic pigments in the uvea and retina did not cause abnormal concentrations of 18F-PFPN, as no anomalous uptake of 18F-PFPN was detected in the healthy contralateral eyes.
CONCLUSIONS: Compared to 18F-FDG, 18F-PFPN demonstrated higher detection rates for ocular and orbital melanomas with minimal interference from surrounding tissues. This suggests that 18F-PFPN could be a promising clinical diagnostic tool for distinguishing malignant melanoma from benign pigmentation in ocular and orbital melanomas.

OBJECTIVE: The objective is to analyze the radiological diagnosis of orbital lesions and their correlation with the final histopathological findings. We compared the initial reports by extramural radiologists and an in-house radiologist specialized in orbital imaging to evaluate the diagnostic accuracy in the interpretation of orbital imaging.
METHODS: This was a retrospective chart review of forty patients referred to a Tertiary Eye Care Center in South India over a period of 7 years. These patients already had their imaging done elsewhere. The imaging was re-evaluated by an in-house radiologist. The radiological findings were correlated with the histopathological diagnosis. The diagnostic accuracy between the two radiologists was compared. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value in differentiating malignant from benign lesions were calculated in both groups. The sensitivity and PPV of the radiological diagnosis for neoplastic and inflammatory lesions in both groups were analyzed.
RESULTS: The accuracy in differentiating malignant from benign and inflammatory lesions by our in-house radiologist and extramural radiologists was 95% (κ = 0.9 [0.764, 0.997]) and 50% (κ = 0.036 [-0.160, 0.232]), respectively. The sensitivity and PPV of the radiological diagnosis by our in-house radiologist were 93.31% and 100% for benign lesions and 95.24% and 95.24% for malignant lesions. On the contrary, reports from the extramural radiologists showed a sensitivity and PPV of 76.92% and 66.67% for benign lesions and 14.28% and 60% for malignant lesions.
CONCLUSIONS: A high radiological diagnostic accuracy is possible when analyzed by radiologists experienced in orbital imaging.

BACKGROUND Orbital metastasis originating from hepatocellular carcinoma (HCC), particularly as an initial manifestation in patients without a known history of HCC, is rare. Few reports exist on the treatment of patients having HCC with orbital metastasis using targeted therapy or immunotherapy. CASE REPORT We report a case of advanced-stage HCC in a 65-year-old man who first presented with progressive, painless blurred vision and proptosis of the right eye for 2 weeks. The patient had no history of chronic liver disease or cancer. Computed tomography revealed an enhancing hyperdense extraconal mass in the right orbit; a biopsy revealed metastatic HCC. Abdominal CT, which was performed to investigate the primary cancer, revealed a 1.2×1.6-cm arterial-enhancing nodule with venous washout in hepatic segment 5, associated with liver cirrhosis. The patient\'s serum alpha-fetoprotein level was 70.27 ng/dL. Chest computed tomography revealed lung metastasis. Thus, first-line systemic therapy combining durvalumab and tremelimumab was initiated alongside palliative radiotherapy targeting the right orbit, which began 1 week after the first dose of dual immunotherapy. The patient had significant clinical improvement, reduced proptosis, and serum alpha-fetoprotein levels. CONCLUSIONS Although orbital metastasis is a rare manifestation of HCC, physicians should recognize and consider aggressive investigations for early diagnosis, especially in patients with existing risk factors for HCC. Dual immunotherapy with durvalumab and tremelimumab in combination with radiotherapy can be considered a potential treatment option for managing advanced HCC with orbital metastasis.

Ocular adnexa region (OAR) primary lymphomas are uncommon, accounting for 1-2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Extranodal marginal zone lymphoma (EMZL) originates from several epithelial tissues, including the stomach, salivary gland, lung, small intestine, thyroid gland, and ocular adnexa region. Here, we report a 66-year-old female patient who was diagnosed with EMZL of OAR. In consideration of the possible side effect of radiotherapy, such as conjunctivitis, visual acuity impairment, and even retinal complications, she received six cycles of triweekly targeted chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) without radiotherapy. Then, she remained in complete remission up to the present day.

Introduction Orbital lipoma is an extremely rare tumor, representing less than 1% of all orbital tumors. We review the literature and describe the presentation, the differential diagnosis and the management of this tumor. Case report We report the case of a 63-year-old patient who was referred for a diplopia with recent hemi-cranial headache. Physical examination showed no exophthalmos nor decrease in visual acuity. The patient complained of diplopia on elevation and oculomotricity examination showed limited elevation of the right eye. The Hess Lancaster test was in favor of a limited course of the right inferior rectus muscle. Magnetic resonance imaging revealed a fusiform tissue process in the right inferior rectus muscle with a fatty signal. A complete excision of the tumor was performed by a trasncunjonctival approach. Cytopathological examination was consistent with a pleomorphic lipoma. The postoperative period was uneventful. The definitive histopathologic diagnosis was a lipoma. The postoperative Magnetic resonance imaging showed the complete disappearance of the lesion. With 3 years of follow up, there is no sign of recurrence or ocular motility trouble. Conclusion: Lipomas are rare tumors in the orbit. The clinic is variable depending on the size and the site. The clinical diagnosis is difficult to make. Only histology allows the final diagnosis.

BACKGROUND: Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature.
METHODS: A 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after.
CONCLUSIONS: Bilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging.

To investigate the Raman spectral features of orbital rhabdomyosarcoma (ORMS) tissue and normal orbital tissue in vitro, and to explore the feasibility of Raman spectroscopy for the optical diagnosis of ORMS. 23 specimens of ORMS and 27 specimens of normal orbital tissue were obtained from resection surgery and measured in vitro using Raman spectroscopy coupled to a fiber optic probe. The important spectral differences between the tissue categories were exploited for tissue classification with the multivariate statistical techniques of principal component analysis (PCA) and linear discriminant analysis (LDA). Compared to normal tissue, the Raman peak intensities located at 1450 and 1655 cm-1 were significantly lower for ORMS (p < 0.05), while the peak intensities located at 721, 758, 1002, 1088, 1156, 1206, 1340, 1526 cm-1 were significantly higher (p < 0.05). Raman spectra differences between normal tissue and ORMS could be attributed to the changes in the relative amounts of biochemical components, such as nucleic acids, tryptophan, phenylalanine, carotenoid and lipids. The Raman spectroscopy technique together with PCA-LDA modeling provides a diagnostic accuracy of 90.0%, sensitivity of 91.3%, and specificity of 88.9% for ORMS identification. Significant differences in Raman peak intensities exist between normal orbital tissue and ORMS. This work demonstrated for the first time that the Raman spectroscopy associated with PCA-LDA diagnostic algorithms has promising potential for accurate, rapid and noninvasive optical diagnosis of ORMS at the molecular level.

The transnasal endoscopic approach is increasingly used for resection of tumors that are located inferiorly and medially within the orbit. However, this usually requires multiple-handed manipulations, which demand a second corridor for an assistant. Here, we introduce a simple transseptal corridor from the contra-nare, to facilitate assistant instrument maneuverability. This simple, minimally invasive skill greatly improves operation efficiency and deserves greater attention in endoscopic orbital surgery.