BACKGROUND: Spheno-orbital meningiomas (SOMs) pose a challenge to the skull base neurosurgeon because of their variable presentation and involvement of critical structures within the orbit. There is no consensus on optimal management of these patients and how to achieve maximal safe resection. The authors share an illustrative case with an accompanying video to demonstrate their aggressive approach to resect SOMs and their intraorbital components.
METHODS: A 75-year-old-woman presented with progressive vision loss and proptosis. Magnetic resonance imaging was consistent with a large, left-sided sphenoid wing meningioma with extension to the orbital wall and compression of the optic nerve medially. The patient elected to undergo surgical excision and optic nerve decompression. She did well postoperatively with resolution of proptosis and good resection margins on follow-up imaging.
CONCLUSIONS: Aggressive resection of SOMs is possible with an understanding of the underlying anatomy. Familiarity with the orbit can facilitate a maximal safe resection with optic nerve decompression.

Rhabdomyoma of the orbit is a rare tumor with very few cases reported in the literature. We herein describe a 5-year-old boy who presented to us with a deviation of his left eye. Magnetic Resonance Imaging (MRI) showed a well-defined homogeneous intraconal mass in the superomedial aspect compressing the optic nerve. An excision biopsy was performed and the diagnosis of rhabdomyoma was confirmed on histopathology and immunohistochemistry with a coincidental finding of Trichinella spiralis larvae within the excised specimen. We report this phenomenon in two rare diseases with a predilection for striated muscle occurring simultaneously in a single patient.

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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.

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BACKGROUND: Sinonasal malignancies with orbital invasion have dismal prognosis even when treated with orbital exenteration (OE). Sugawara et al. developed a surgical strategy called \"extended-OE (EOE),\" showing encouraging outcomes. We hypothesized that a similar resection is achievable under endoscopic guidance through the exenterated orbit (endoscopic-EOE).
METHODS: The study was conducted in three institutions: University of Vienna; Mayo Clinic; University of Insubria; 48 orbital dissections were performed. A questionnaire was developed to evaluate feasibility and safety of each step, scoring from 1 to 10, (\"impossible\" to \"easy,\" and \"high risk\" to \"low risk,\" respectively), most likely complication(s) were hypothesized.
RESULTS: The step-by-step technique is thoroughly described. The questionnaire was answered by 25 anterior skull base surgeons from six countries. Mean, median, range, and interquartile range of both feasibility and safety scores are reported.
CONCLUSIONS: Endoscopic-EOE is a challenging but feasible procedure. Clinical validation is required to assess real-life outcomes.

BACKGROUND: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm.
METHODS: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy.
METHODS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.