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Surgery stands as the primary treatment for spheno-orbital meningiomas, following a symptoms-oriented approach. We discussed the decision-making process behind surgical strategies through a review of medical records from 80 patients who underwent surgical resection at the University of Naples Federico II. Different surgical approaches were employed based on the tumor’s location relative to the optic nerve’s long axis, categorized into lateral (type I), medial (type II), and diffuse (type III). We examined clinical, neuroradiological, surgical, pathological, and outcome factors. Proptosis emerged as the most frequent symptom (97%), followed by visual impairment (59%) and ocular motility issues (35%). Type I represented 20%, type II 43%, and type III 17%. Growth primarily affected the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%). The resection outcomes varied, with Simpson grades I and II achieved in all type I cases, 67.5% of type II, and 18% of type III. Recurrence rates were highest in type II (41.8%) and type III (59%). Improvement was notable in proptosis (68%) and visual function (51%, predominantly type I). Surgery for spheno-orbital meningiomas should be tailored to each patient, considering individual characteristics and tumor features to improve quality of life by addressing primary symptoms like proptosis and visual deficits.

Mutated in melanomas Mutations in BRAF and NRAS that lead to constitutive activation of MAPK signalling have been found at high frequencies in many melanomas, both benign and malignant. However, they have not been found in uveal melanomas (arising from in the cells that give colour to the eye) or in blue naevi melanomas (a type of benign blue–black mole). Now a genetic screen of biopsy samples shows that these melanoma subtypes instead show frequent activating mutations in the G protein α-subunit GNAQ , also leading to the activation of the MAPK pathway. This identifies signalling components downstream of GNAQ as potential therapeutic targets. Mutations in BRAF and NRAS that lead to constitutive activation of MAP kinase signalling have been found at high frequencies in many melanomas. However, they have not been found in the uveal melanomas and blue nevi subtypes of melanoma. This paper shows that these subtypes instead show frequent activating mutations in the G protein α-subunit GNAQ , also leading to the activation of the MAP kinase pathway. BRAF and NRAS are common targets for somatic mutations in benign and malignant neoplasms that arise from melanocytes situated in epithelial structures, and lead to constitutive activation of the mitogen-activated protein (MAP) kinase pathway 1 , 2 . However, BRAF and NRAS mutations are absent in a number of other melanocytic neoplasms in which the equivalent oncogenic events are currently unknown 3 . Here we report frequent somatic mutations in the heterotrimeric G protein α-subunit, GNAQ , in blue naevi (83%) and ocular melanoma of the uvea (46%). The mutations occur exclusively in codon 209 in the Ras-like domain and result in constitutive activation, turning GNAQ into a dominant acting oncogene. Our results demonstrate an alternative route to MAP kinase activation in melanocytic neoplasia, providing new opportunities for therapeutic intervention.

Aneurysmal bone cyst （ABC） is an uncommon benign lesion with a reported incidence rate of 0.14 cases for every 1,000,000 people.ABC can occur in any part of the skeletal system but is mainly detected in the long bones.Orbit involvement is rare （〈1% of all ABCs）.In this article,we presented two female patients aged 49 and 33 years old respectively who suffered from ABC.After successful surgical removal of the cyst via frontal craniotomy and simple curettage,the patients recovered well and have been in good health throughout the 2.5-4.5 years of follow-up.

To report late recurrence of orbital cavernous hemangioma in a patient ten years after complete resection of the primary tumor. A 32-year-old woman with a history of progressive visual loss and proptosis underwent lateral orbitotomy for resection of a large cavernous hemangioma. Ten years later, proptosis recurred and the patient developed progressive ocular deviation. Imaging studies were in favor of a recurrent cavernous hemangioma and the tumor was excised via the previous incision site. Reassessment of previous orbital images suggested the presence of two separate tumors, only one of which had been excised at the time of initial surgery. Recurrent orbital cavernous hemangioma may follow incomplete excision of multiple orbital lesions with gradual growth of unidentified residual tumors. Accordingly, when an encapsulated cavernous hemangioma is removed, exploration is recommended to rule out multiple lesions.

Neuroblastoma is an undifferentiated malignancy of primitive neuroblasts. Neuroblastoma is among the most common solid tumors of childhood. Orbital neuroblastoma is typically a metastatic tumor. In this case report, we describe a 2-year-old child with a rapidly progressing orbital tumor. Computed tomography revealed an orbital mass lesion with extraocular and intraocular components. An incisional biopsy was done, and a histopathological examination showed features suggestive of neuroblastoma. Systemic workup including ultrasonography of the abdomen, chest roentgenogram, whole body computed tomography, and bone scintigraphy showed no evidence of systemic involvement. The diagnosis of primary orbital neuroblastoma was made, and the child was subjected to chemotherapy followed by rapid melting of the tumor. Neuroblastoma should be considered in the differential diagnosis of childhood orbital tumors.

Purpose Gamma knife radiosurgery (GKRS) for orbital cavernous hemangioma (OCH) has emerged as a promising method due to its significant clinical improvement and low incidence of complications. This study aimed to evaluate the safety and efficacy of GKRS for the treatment of OCH. Methods In accordance with the PRISMA framework, we searched PubMed, Cochrane Central, and Embase for studies reporting outcomes of GKRS for OCH. Studies reporting complications, visual improvement, proptosis, tumor reduction rate, and tumor progression rate for OCH following GKRS were included. Results Six studies, out of 1856 search results, with 100 patients were included. Among them, only 5 minor complications were related to GKRS, including 3 with orbital pain and 2 with periorbital chemosis. Thus, the complication rate was 13% (95% CI, 7–25%). Visual acuity and visual field improvement rates after GKRS were 80% (95% CI, 63–96%) and 71% (95% CI, 47–95%) respectively. Proptosis improved in 94% of cases (95% CI, 83–100%). The tumor reduction rate was 77% after GKRS (95% CI, 69–85%). Conclusion GKRS for OCH appears to be a safe technique, as evidenced by the rate of clinical improvement and radiological improvement. However, studies are limited by an absence of a control group. Additional studies are needed to evaluate the relative efficacy of GKRS as compared with alternative surgical modalities for OCH.

Orbital exenteration (OE) is a disfiguring procedure most commonly performed for locally advanced and potentially life-threatening periorbital malignancies. We retrospectively reviewed records of 11 consecutive HIV patients who underwent OE for invasive orbital malignancy at our institution from January 2005 to December 2015. Patient demographic and clinic data and histopathology of the tumor were analyzed. There were eight (72.72%) female and three (27.28%) male participants ranging in age from 31 to 52 years with an mean of 39.4 years. Nine patients had been known to be HIV-positive for at least 2 years, and HIV-positive status was revealed at presentation for two patients. The mean CD4 cell count was 154.4 cells/mm . Histopathological examination showed invasive orbital squamous cell carcinomas in nine patients (81.81%), achromic orbital melanoma in one patient (9.09%), and adenoid cystic carcinoma in one patient (9.09%). None of the patients underwent primary orbital reconstruction. The mean follow-up time was 3.4 months. Only one patient who underwent adjuvant radiotherapy was seen after 12 months. Oculo-orbital malignancies are very aggressive in HIV-positive individuals, especially in untreated patients. Routine screening for suspected ocular surface lesions and early surgical removal of all these lesions could help to avoid the need to perform the radical and disfiguring OE procedure.

Background It is well-known that the lacrimal gland (LG) may be affected in IgG4-related ophthalmic disease (IgG4ROD). Recently, IgG4-related ophthalmic lesions other than those of the lacrimal gland have been reported. However, no study to date has revealed the details of these lesions. This study was conducted to evaluate the location and frequency of lesions found in conjunction with IgG4ROD using radiological imaging. Methods Radiological images and clinical records of 65 patients collected from seven institutions in Japan were reviewed retrospectively. All patients had been pathologically diagnosed with IgG4ROD. Patients of mucosa-associated lymphoid tissue lymphoma associated with IgG4-related lesions were excluded. Orbital magnetic resonance imaging or computed tomography findings were evaluated. Results Of the 65 patients, 31 (47.7 %) had lesions involving the LG alone, whereas 34 (52.3 %) had lesions involving the areas other than LG, including eight patients who had lesions without any LG involvement. IgG4-related ophthalmic lesions included LG enlargement in 57 patients (87.7 %), trigeminal nerve branch enlargement in 25 (38.5 %), extraocular muscle enlargement in 16 (24.6 %), diffuse orbital fat lesions in 15 (23.1 %), orbital mass lesions in 11 (16.9 %), eyelid lesions in eight (12.3 %), and nasolacrimal duct lesion in one (1.5 %). Six patients (9.2 %) presented with visual disturbance due to optic nerve disturbance, eight (12.3 %) with a restriction of ocular movement, and 19 (29.2 %) with exophthalmos. Conclusions Thirty-four (52.3 %) of the 65 IgG4ROD patients had lesions in areas other than LG. Lesions were found in the trigeminal nerve branch including pterygopalatine fossa, extraocular muscles, orbital fat, eyelid, and nasolacrimal duct.

Background Mesenchymal chondrosarcoma (MC) is an uncommon type of malignant soft tissue tumor. The skeleton is the most common site of MC. Extraosseous sarcoma is rare, especially that of the orbit. Fusion gene HEY1–NCOA2 has diagnostic significance for MC, but there is a lack of research on its related pathways. To analyze the characteristics of orbital mesenchymal chondrosarcoma (MC), and search for HEY1-NCOA2-related pathways in orbital MCs, four cases of orbital MC were included in the study. Methods From January 2018 to December 2022, four MC patients hospitalized at Tianjin Medical University Eye Hospital were collected for a retrospective series of case studies. HEY1-NCOA2 of the assay specimens was detected by fluorescence in situ hybridization, and immunohistochemical staining was performed for representative proteins of the relevant pathways. For figure modification and statistical analysis, GraphPad Prism 8.0 (La Jolla, CA, USA) was utilized. Results Four orbital MC were reported. Among the 4 ligible MC specimens, two were HEY1-NCOA2-positive and two were HEY1-NCOA2-negative. Immunohistochemistry showed stronger expression of COL2A1, APC, CD99, and Bcl2 in HEY1-NCOA2-positive samples than in HEY1-NCOA2-negative MCs. Conclusion We summarized the clinical features, treatments, and prognosis of orbital MC, including our cases and the literature. The expression of COL2A1 and Bcl2 is elevated in HEY1-NCOA2-positive tissues, and they promote tumor cell growth by regulating cell proliferation, apoptosis, epithelial–mesenchymal transition, and drug resistance. For HEY1-NCOA2-positive patients, given the high expression of CD99, drugs targeting the MAPK pathway may be an effective treatment.

Sarcoidosis is a systemic disease of still-unknown etiology in which characteristic granulomas develop in various parts of the body. Symptoms depend on the localization of the formations. The isolated orbital form, in which granulomas develop only in the orbit of the eye, is very rare and presents a challenge in terms of differential diagnosis. Treatment for the orbital form may include a conservative, surgical, or combined approach. The goal of surgical treatment is to completely remove the tumour formation, which achieves excellent results in many cases. The current article presents a clinical case of an isolated orbital form of sarcoidosis, in which surgical treatment was undertaken with complete removal of the granulomatous formation. In the postoperative follow-up, a complete reversal of the symptoms was observed without residual disturbances in vision or eye movements.