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Background/AimsTo validate the prognostic performance of the American Joint Committee on Cancer (AJCC) eighth edition classification for ocular adnexal lymphoma (OAL).MethodsWe performed a retrospective review of 140 consecutive patients treated for primary OAL between March 2010 and September 2017. Associations between T/N/M categories at presentation and disease-related outcomes, including relapse, progression-free survival (PFS) and overall survival (OS) were evaluated.ResultsSeventy-nine women and 61 men (median age, 52 (range 20–84) years; median follow-up, 57 (range 7–131) months) were included. Histological subtypes included mucosa-associated lymphoid tissue lymphoma (92.1%, n=129), diffuse large B-cell lymphoma (5.0%, n=7), follicular lymphoma (1.4%, n=2) and mantle cell lymphoma (1.4%, n=2). Patients with ≥T2 disease had significantly higher risks of overall relapse (unadjusted HR)=4.32, p=0.016), decreased PFS (uHR=5.19, p=0.004) and decreased OS (uHR=9.21, p=0.047). Patients with ≥N1 disease had significantly higher risks of overall relapse (uHR=9.17, p<0.001) and decreased PFS (uHR=9.24, p<0.001). M1 disease was significantly associated with higher risks of overall relapse (uHR=3.62, p=0.036), decreased PFS (uHR=5.13, p=0.001) and decreased OS (uHR=9.24, p=0.013). On considering TNM categories as continuous data, the uHRs for per level increase in T, N and M categories were 1.77, 1.83 and 2.30 for overall relapse and 1.72, 1.87 and 2.78 for decreased PFS, respectively (p<0.05 for each comparison).ConclusionThe T, N and M categories of the AJCC eighth edition classification have prognostic value for relapse and survival among patients with primary OAL. Particularly, nodal/metastatic involvement at presentation indicated less favourable outcome.

To investigate the clinical and radiologic characteristics, treatment outcomes, and management strategies for orbital incidentalomas. We retrospectively reviewed 43 patients with orbital tumors incidentally identified through imaging conducted for unrelated reasons between March 2015 and July 2023. Data on imaging indications, clinicoradiologic features, diagnoses, treatments, and outcomes were analyzed. Patients were divided into surgery ( n  = 14, 32.6%) and observation ( n  = 29, 67.4%) groups for comparison. Among the 43 cases, 20 patients (46.5%) were male, with a mean age of 57.1 years and a mean follow-up of 2.77 years. Initial imaging was most commonly conducted for health check-ups (48.8%), headaches (27.9%), or dizziness (14.0%). Common clinical signs included proptosis (41.9%), peripheral diplopia (21.4%), and hypoglobus (9.3%). Benign lesions, such as cavernous venous malformations (55.8%) and schwannomas (27.9%), predominated, with one case of lymphoma. The surgery group more frequently exhibited clinical signs and anterior tumor locations (71.4% vs. 13.8%, p  = 0.001). Surgical excision was successful without complications. In the observation group, tumor stability was noted in 96.6% without functional deficits. Orbital incidentalomas are often benign and asymptomatic. Observation is recommended for asymptomatic posterior orbital lesions, while surgical removal is indicated for anterior lesions with significant clinical signs.

Background Given the rarity orbital myoepithelial carcinoma (OMC) and the paucity of current evidence characterizing its clinical profile, this study aims to summarize the clinical features and prognostic outcomes of OMC. Methods This retrospective cohort study analyzed a consecutive series of 12 patients with histopathologically confirmed OMC managed at Beijing Tongren Hospital, Capital Medical University (January 1, 2000-December 31, 2024), with evaluation of demographic data, clinical features, imaging findings, histopathological features, treatment modalities, prognostic outcomes, and follow-up records, using progression-free survival and overall survival as primary outcome measures alongside clinical presentation and imaging study assessments. Results Twelve patients (58.3% male, 7/12) were enrolled, with a mean age of 55.08 ± 14.46 years (range: 28–82). The mean age at initial detection of the tumor was 44.83 ± 18.89 years (range: 19–80). The most common initial presentation was abnormal globe position (41.7%). Tumors were generally large, with a maximum diameter ranging from 9.00 to 73.00 mm (median: 25.50 mm, IQR 18.75–35.25). Invasion into adjacent structures (sinonasal or intracranial) was observed in 41.7% (5/12) of cases. A history of prior neoplasms was noted in 83.3% (10/12) of patients, with pleomorphic adenoma (PA) accounting for 50%. MRI findings predominantly demonstrated lobulated cystic lesions with iso-intensity on T1-weighted imaging (T1WI), iso- to hyper-intensity on T2-weighted imaging (T2WI), and heterogeneous enhancement. CT scans revealed soft-tissue density masses, with calcifications in 25% of cases and bone destruction of 66.7% cases. Histopathologically, hemorrhage/necrosis was identified in 63.6% (7/11), and perineural invasion in 18.2% (2/11). During a mean follow-up of 6.56 ± 3.36 years (range: 1–12) in 9 patients, the overall survival rate was 77.8%, with two disease-specific deaths at 6- and 8-years post-diagnosis. The recurrence rate was 55.6%, and all three patients with a history of PA experienced recurrence. The progression-free survival rate was 44.4%, with a median survival time of 3 years (IQR 2–6). Conclusions OMC predominantly affects males, presents with large tumors prone to sinonasal/intracranial invasion and bone destruction, and carries a poor prognosis. Patients with PA-associated OMC exhibit exceptionally high recurrence rates, emphasizing the necessity of long-term postoperative surveillance.

Purpose To evaluate the clinical features, pathological findings, treatment, and prognosis of patients with orbital natural killer/T-cell lymphoma (NKTCL). Methods This retrospective study collected clinical data from 11 patients diagnosed with orbital NKTCL by pathological examination at Beijing Tongren Hospital, Capital Medical University, between November 2005 and March 2024. The patients were followed up to assess their prognosis. Results The median age of patients with orbital NKTCL was 51 years, with a slight male predominance. All cases were unilateral, with clinical presentations of eyelid or periorbital swelling, and other symptoms including decreased vision (90.9%), conjunctival congestion (90.9%), ocular motility disorders (81.8%), proptosis (63.6%), and ptosis (36.4%). All patients had surrounding tissue involvement, with the most common sites being the eyelid (11 cases), eyeball wall (7 cases), and lacrimal gland (5 cases). Additionally, 63.6% of patients had concurrent soft tissue involvement of the nasal region, while 36.4% had isolated orbital NKTCL. Pathological examination revealed diffuse proliferation and infiltration of atypical lymphocytes, occasionally with necrosis. Immunohistochemical staining showed positive expression of GrB (11 cases), CD3 (10 cases), TIA1 (9 cases), CD56 (8 cases), and EBV/EBER (10 cases). All patients underwent ocular surgery, 7 received postoperative chemotherapy, and one received concurrent local radiotherapy. Follow-up (median duration: 120 months) revealed poor prognosis, with 5 patients lost to follow-up, 5 deaths, and only one survival without recurrence. Conclusions Orbital NKTCL is highly aggressive, rapidly progressive, and has a poor prognosis. Its clinical symptoms are similar to orbital cellulitis or inflammatory pseudotumor. When anti-inflammatory and corticosteroid therapies prove ineffective, early biopsy for histopathological confirmation is strongly recommended. Upon diagnosis, prompt chemotherapy and radiotherapy should be initiated to improve the patient’s prognosis.

Immunoglobulin G4-related ophthalmic disease is an autoimmune condition characterized histologically by dense lymphoplasmacytic infiltration, obliterative phlebitis, and storiform fibrosis. Orbital hemangioma is one of the most common primary orbital tumors in adults. The coexistence of immunoglobulin G4-related ophthalmic disease and orbital hemangioma is extremely rare. This report presents the case of a man in his early 30s diagnosed with immunoglobulin G4-related ophthalmic disease. He was treated with glucocorticoid therapy and gamma knife radiosurgery. Herein, we described the clinical presentation, imaging findings, histopathology, treatment, and outcomes of this rare case to provide a reference for future diagnosis and management.

Extraskeletal orbital mesenchymal chondrosarcoma is an extremely rare and highly aggressive tumor. We herein report a case involving a woman in her early 20s diagnosed with orbital mesenchymal chondrosarcoma. This case report aims to increase recognition and understanding of this condition.

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. Purposes 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. Highlights Imaging shows sphenoid wing hyperostosis and the dural tail sign. Complete tumor removal, followed by radiotherapy, reduces recurrence risk. The intracranial part exhibits a lower fibrous and higher bleeding intention.

Purpose The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma. Methods In this clinicopathologic series, clinical features were obtained from patient records. All imaging studies were reviewed. All specimens were reviewed with haematoxylin and eosin, and 10 were subject to a staining protocol including: Movat Pantachrome, periodic acid Schiff, D2-40, CD31, GLUT-1, Ki-67, vascular endothelial growth factor receptor 1 (VEGF-r1) (flt-1), VEGF-r2 (Flk-1), VEGF, anti-smooth muscle actin (SMA), CD20, CD4, CD8 and CD68. Imaging and pathology were reviewed in a systematic fashion. Results Clinically, lesions were more common in middle-aged females presenting with axial proptosis and pain. One-third demonstrated signs of optic nerve dysfunction. Dynamic imaging revealed focal early and diffuse late enhancement. Lesions demonstrated slow growth at 0.2 cm3/year. Histologically, all lesions demonstrated large vascular channels with mature-appearing endothelium and abundant stroma. Three salient features were noted and characterised: thrombosis, nests of perivascular hypercellularity and expanded stromal elements. Acute thrombosis was a feature of each specimen (<10% of channels). Fibrin clots were lined by a layer of CD31+ endothelium. Perivascular hypercellular areas stained uniformly with CD31 and less so with VEGFr2. Additionally, focal areas of Ki67+ and CD68+ cells were found in these regions. Expanded stroma contained CD31+ microcapillary networks and stained diffusely with anti-SMA. Conclusions Cavernous haemangioma demonstrate clinical features and growth characteristics of a benign mass. Dynamic imaging highlights their slow flow vascular nature. Histologically, the hypercellularity and stromal changes identified can be understood within the pathogenic context of thrombosis and recanalisation in an organised lesion.

Orbital neurofibromas are benign tumors originating from the peripheral nerve sheath, often linked to neurofibromatosis type 1 (NF1) [1], although they account for less than 1% of all orbital tumors [2, 3]. These tumors can cause symptoms such as proptosis, vision impairment, and ocular misalignment [4]. While typically linked to NF1, multiple isolated orbital neurofibromas in the absence of a definitive NF1 diagnosis remain exceedingly rare, warranting clinical attention. A 56-year-old female presented with ptosis and dystopia on the right side. MRI revealed multiple intraorbital and extraconal masses, with the largest being excised via anterior orbitotomy. Histopathological analysis confirmed the diagnosis of neurofibroma. The patient had no cutaneous or systemic signs suggestive of NF1. In adults, multiple orbital tumors should prompt suspicion for neurofibromas, even when NF1 is not confirmed. Furthermore, recurrence is possible, emphasizing the importance of long-term follow-up. This case highlights the diagnostic challenge posed by orbital neurofibromas without NF1 and the need for comprehensive systemic evaluation in such presentations.

Purpose To investigate the possibility of distinguishing between IgG4-related ophthalmic disease (IgG4-ROD) and orbital MALT lymphoma using artificial intelligence (AI) and hematoxylin–eosin (HE) images. Methods After identifying a total of 127 patients from whom we were able to procure tissue blocks with IgG4-ROD and orbital MALT lymphoma, we performed histological and molecular genetic analyses, such as gene rearrangement. Subsequently, pathological HE images were collected from these patients followed by the cutting out of 10 different image patches from the HE image of each patient. A total of 970 image patches from the 97 patients were used to construct nine different models of deep learning, and the 300 image patches from the remaining 30 patients were used to evaluate the diagnostic performance of the models. Area under the curve (AUC) and accuracy (ACC) were used for the performance evaluation of the deep learning models. In addition, four ophthalmologists performed the binary classification between IgG4-ROD and orbital MALT lymphoma. Results EVA, which is a vision-centric foundation model to explore the limits of visual representation, was the best deep learning model among the nine models. The results of EVA were ACC = 73.3% and AUC = 0.807. The ACC of the four ophthalmologists ranged from 40 to 60%. Conclusions It was possible to construct an AI software based on deep learning that was able to distinguish between IgG4-ROD and orbital MALT. This AI model may be useful as an initial screening tool to direct further ancillary investigations.