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The article "Adenoid Cystic Carcinoma of the Lacrimal Glands: A Single Institute Experience" published in the International Journal of Surgical Pathology discusses the rarity and complexity of lacrimal gland adenoid cystic carcinoma (ADCC). The study highlights the aggressive behavior, perineural invasion, and high local recurrence rate of this neoplasm. Surgical resection with clear margins, with or without adjuvant radiotherapy, is the standard management approach. Comprehensive diagnostic methods, including clinical history, immunohistochemistry, and genetic analysis, are crucial for accurate diagnosis and treatment. Ongoing research is needed to improve understanding, treatment, and patient outcomes for this rare neoplasm. [Extracted from the article]

Background: Orbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment. With the aim of clarifying the role of BE as a risk factor, we analyzed patients with orbital RMS included in the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol. Methods: We retrospectively analyzed the radiological reports of 199 patients with orbital RMS (PM or not) and defined three grades of BE: minimal (thinning of the bone), moderate (focal bone lysis), and extensive (complete cortical destruction). Results: BE was present in 55 of the 199 (27.6%) patients, which was classified as minimal in 27, moderate in 7, and extensive in 21. Tumors with extensive BE were more frequently large (>5 cm, p = 0.0008) and invasive (T2, p = 0.001). With a median follow-up of 70.4 months (range = 7.1–167.7), a total of 183 patients are alive, with 5-year event-free survival (EFS) and overall survival (OS) rates of 76% (95%CI = 69.2–81.3) and 92% (95%CI = 86.7–94.8), respectively. Patients without any BE had better OS (95% vs. 81%, p = 0.001), but not EFS. Patients with no/minimal/moderate BE had better EFS and OS compared with patients with extensive BE [EFS of 78.1 (95%CI = 71.1–83.5) vs. 57.1 (95%CI = 33.8–74.9), p = 0.0114, respectively, and OS of 94.0 (95%CI = 89.2–96.8) vs. 71.1 (95%CI = 46.6–85.9), p < 0.0001, respectively]. Events and metastatic relapses (in all cases CNS/meningeal) were more frequent in patients with extensive BE. Conclusions: Only those patients with orbital RMS and extensive BE should be considered as PM and should be treated accordingly. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Methods Results Conclusion Endoscopic endonasal techniques, initially developed for sinonasal tumor resection, have revolutionized the approach to orbital lesions. The emergence of endonasal orbital tumor surgery has prompted anatomical studies focusing on the medial orbit, yet there remains a lack of literature on maneuverability lateral to the optic nerve (ON), with current feasibility assessments relying primarily on the plane of resectability (POR).Bilateral anatomical dissections were conducted on four latex‐injected human cadaveric heads using an endoscopic medial and inferior orbitotomy and superomedial displacement of the inferior rectus muscle (IRM) to access the inferolateral intraconal quadrant. Measurements of distances, areas, angles of attack, and volumetric exposure were obtained using stereotactic points from an imaging‐based navigation system. Additionally, an illustrative case was presented to demonstrate the endoscopic management of laterally based intraconal lesions.The intraconal space was safely accessed through superomedial displacement of the IRM. The mean intraconal volumetric exposure attained through this maneuver was 2.78 cm3 (1.18 cm3). The most superolateral point reachable by the ipsilateral endoscopic endonasal approach was consistently lateral and superior to the ON at a mean absolute distance of 1.45 cm (0.37 cm). Maneuverability at this target point was superior in the sagittal plane, noted by a larger vertical angle of attack compared with the horizontal angle of attack.This study demonstrates that inferolateral intraconal dissection through an ipsilateral endoscopic endonasal approach is feasible via a medial orbitotomy and superomedial retraction of the IRM. Additionally, our findings reaffirm lesions below the POR are suitable for endoscopic endonasal resection. [ABSTRACT FROM AUTHOR]

Objective: The aim of this case report is to assess the clinicopathological characteristics and differential diagnosis of orbital granular cell tumor (GCT). Methods: Clinical and imaging data of a rare case of orbital GCT involving the superior rectus muscle were collected. Its clinical characteristics, imaging, and histopathological features were observed. Results: A 36-year-old female patient presented with a 2-year history of left eye proptosis. Magnetic resonance imaging (MRI) enhancement suggested a space-occupying lesion in the left superior rectus muscle region. On T1-weighted and T2-weighted MRI, the tumor was isointense to gray matter and significantly enhanced on the enhanced scan. Microscopic examination revealed that most tumor cells exhibited diffuse growth with unclear boundaries, and some cells were arranged in small nests. The tumor cells were large, with abundant, coarse eosinophilic granules in the cytoplasm. Occasional cells contained larger round eosinophilic droplets in the cytoplasm. Focal areas showed foamy cells, small and central round or oval nuclei with occasional nuclear enlargement and mild atypia, inconspicuous nucleoli, rare mitoses, and low proliferative activity. Immunohistochemistry results were Vimentin (+), S-100 (+), CD68 (+), Ki67 (2%+), Inhibin-a (−), CK (−), SMA (−), and Desmin (−). The pathological examination of a specimen harvested from the mass corresponded to a GCT. Conclusion: Orbital GCT is rare and should be considered in the differential diagnosis of orbital tumors. It is essential to distinguish it from thyroid-associated ophthalmopathy, inflammatory pseudotumor, and myohemangioma. Definitive diagnosis requires a comprehensive analysis of clinical, histopathological, and immunohistochemical findings. Surgical excision is the primary treatment for orbital GCTs. For patients with incomplete tumor resection, close follow-up is necessary. Proton beam radiation therapy can be considered to prevent recurrence or metastasis if needed. [ABSTRACT FROM AUTHOR]

Objective: Our aim is to present two cases of infantile sinonasal tract myxoma with orbital involvement and conduct a comprehensive literature review of the topic. We aim to provide a summary of the presentation of infantile sinonasal tract myxomas to effectively aid clinicians in considering this rare entity as a potential diagnosis. Methods: We present a case series and a retrospective review of the published literature in the English language. A search was conducted between 1945 and 2023 on sinonasal myxoma. Cases of infantile (<3 years) sinonasal myxoma in the literature were reported as well as two cases of our own. Data was collected from each of the identified articles on the age of presentation, sex, initial presentation, tumor location, imaging, pathology, and treatment. Results: Forty-eight cases of sinonasal myxoma in children <3 years of age were identified. These cases had a slight male preponderance (F:M, 1:1.39) and an average age of diagnosis of sixteen months. Of the cases included, the majority involved the maxilla and maxillary sinuses (83.3%) and commonly presented with a painless slow-growing mass in the region of the tumor. Characteristic CT findings are homogenous solid masses with heterogeneous contrast enhancement. On MRI, these lesions appear hypointense on T1 with highly variable contrast enhancement and hyperintense on T2. Conclusions: We present two cases of infantile sinonasal myxoma with secondary orbital involvement followed by a major review. Treatment of these cases with surgical excision (confirmed clear margins) provided 0% rates of recurrence in the reported cases. Due to the rarity of these tumors and recent histological reclassification, a comprehensive review of this condition will assist clinicians in their management of it. [ABSTRACT FROM AUTHOR]

The ocular surface and orbit constitute unique microenvironments in the human body. Current advances in molecular research have deepened our understanding of tumor development in these regions. Tumors exhibit greater heterogeneity compared to normal tissues, as revealed by pathological and histological examinations. The tumor microenvironment (TME) plays a crucial role in the proliferation and progression of cancer cells. Factors from the external environment or the body's own inflammation and microcirculation interact within the TME, maintaining a delicate balance. Disruption of this balance, through uncontrolled signal pathway activation, can transform normal or benign tissues into malignant ones. In recent years, various systemic immunotherapies have been developed for cancer treatment. This study reviews the epidemiology of ocular surface and orbital tumors include squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma and lymphoma in conjunction with their occurrence, growth, and underlying mechanisms. We propose that by examining clinical histopathological images, we can identify specific and shared microscopic features of tumors. By collecting, classifying, and analyzing data from these clinical histopathological images, we can pinpoint independent diagnostic factors characteristic of tumors. We hope this study provides a basis for future exploration of the mechanisms underlying different ocular diseases. [ABSTRACT FROM AUTHOR]

GLI1-altered mesenchymal tumors are an emerging entity in soft tissue pathology. In the head and neck region, they are most commonly in the tongue. Limited published data indicate a propensity for local recurrence, regional spread, and distant metastasis in both GLI1-rearranged and GLI1-amplified tumors. The purpose of this report is to present the rare case of a GLI1-amplified spindle cell tumor of the orbit and a focused review of the literature. A 54-year-old woman presented with proptosis, eye pain, and ocular motility restriction in the left eye. Imaging demonstrated a tumor occupying the superomedial intraconal orbit that was distinct from the extraocular muscles, optic nerve, and globe. The tumor was totally resected with a combined open transorbital and endoscopic, endonasal approach. Pathological analysis demonstrated a spindled and epithelioid mesenchymal tumor with diffuse nuclear GLI1 expression. PCR-based, next*-generation sarcoma fusion panel was negative for GLI1 fusions, including GLI1::ACTB fusions; however, DDIT3 breaks apart fluorescence in situ hybridization (FISH), which can be used as a surrogate for GLI1 alterations due to proximity to 12q13.3, showing amplification. Post-operatively, the patient had recovered visual acuity. She received adjuvant radiation therapy (60 Gy in 30 fractions). Surveillance for recurrence, regional spread, and distant metastasis has been negative at a 6-month follow-up. Ultimately, we report the first case of a GLI1-amplified mesenchymal neoplasm of the intraconal orbit managed with gross total resection via a combined approach followed by adjuvant radiation therapy. [ABSTRACT FROM AUTHOR]

Control tissue is essential for ensuring the precision of semiquantitative analysis in back-table fluorescence imaging. However, there remains a lack of agreement on the appropriate selection of control tissues. To evaluate the back-table fluorescence imaging performance of different normal tissues and identify the optimal normal tissue, a cohort of 39 patients with orbital tumors were enrolled in the study. Prior to surgery, these patients received indocyanine green (ICG) and following resection, 43 normal control tissues (34 adipose tissues, 3 skin tissues, 3 periosteal tissues, and 3 muscle tissues) were examined using back-table fluorescence imaging. The skin tissue demonstrated significantly elevated fluorescence intensity in comparison to the diseased tissue, whereas the muscle tissue exhibited a broad range and standard deviation of fluorescence signal intensity. Conversely, the adipose and periosteum displayed weak fluorescence signals with a relatively consistent distribution. Additionally, no significant correlations were found between the signal-to-background ratio (SBR) of adipose tissue and patients' ages, genders, weights, disease duration, tumor origins, dosing of administration of ICG infusion, and the time interval between ICG infusion and surgery. However, a positive correlation was observed between the SBR of adipose tissue and its size, with larger adipose tissues (>1 cm) showing an average SBR 27% higher than smaller adipose tissues (≤1 cm). In conclusion, the findings of this study demonstrated that adipose tissue consistently exhibited homogeneous hypofluorescence during back-table fluorescence imaging, regardless of patient clinical variables or imaging parameters. The size of the adipose tissue was identified as the primary factor influencing its fluorescence imaging characteristics, supporting its utility as an ideal control tissue for back-table fluorescence imaging. [ABSTRACT FROM AUTHOR]

Purpose: Solitary fibrous tumor (SFT) of the orbit is a rare tumor that was first described in 1994. We aimed to investigate its imaging characteristics that may facilitate the differential diagnosis between SFT and other types of orbital tumors. Material and Methods: Magnetic resonance imaging (MRI) data of patients with immunohistochemically confirmed orbital SFT from 2002 to 2022 at a tertiary care center were retrospectively analyzed. Tumor location, size, morphological characteristics, and contrast enhancement features were evaluated. Results: Of the 18 eligible patients 10 were female (56%) with a mean age of 52 years. Most of the SFTs were oval-shaped (67%) with a sharp margin (83%). The most frequent locations were the laterocranial quadrant (44%), the extraconal space (67%) and the dorsal half of the orbit (67%). A flow void phenomenon was observed in nearly all cases (94%). On the T1-weighted imaging, tumor signal intensity (SI) was significantly lower than that of the retrobulbar fat and appeared predominantly equivalent (82%) to the temporomesial brain cortex, while on T2-weighted imaging its SI remained equivalent (50%) or slightly hyperintense to that of brain cortex. More than half of the lesions showed a homogeneous contrast enhancement pattern with a median SI increase of 2.2-fold compared to baseline precontrast imaging. Conclusion: The SFT represents a rare orbital tumor with several characteristic imaging features. It was mostly oval-shaped with a sharp margin and frequently localized in the extraconal space and dorsal half of the orbit. Flow voids indicating hypervascularization were the most common findings. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract Precise resection of orbital tumors is a critically important but elusive issue. Fluorescence imaging in the near‐infrared II window (NIR‐II) holds the potential to provide the surgeons with real‐time identification for orbital tumors. Here, for the first time, we evaluated the feasibility and clinical value of NIR‐II fluorescence imaging in orbital tumor surgery. To establish the method of NIR‐II fluorescence imaging for orbital tumors, we developed a NIR‐II fluorescence imaging system and indocyanine green (ICG) served as the fluorescent contrast agent. Twenty‐two patients diagnosed with orbital tumors and scheduled for standard‐of‐care surgery were enrolled in this study. Time‐course NIR‐II fluorescence imaging of two patients with superficial orbital tumors showed the optimum imaging time was 2 h post injection of ICG. Fifteen patients were allocated for diagnostic test, which showed that both the in situ and ex vivo NIR‐II fluorescence imaging showed better sensitivity and specificity than the surgeon judgment. In the feasibility trial of the remaining five patients, the surgeon encountered 34 suspicious regions and surgical decisions were changed nine times due to NIR‐II fluorescence imaging. The resultant seven additional resections were justified by histopathology and the two conservative treatments did not result in recurrence. Based on these findings, we suggested that ICG‐based NIR‐II fluorescence imaging was feasible to guide precise resection of orbital tumors. A future randomized controlled trial with a larger cohort is encouraged to further verify the clinical value.

PurposeWe describe an unusual case of a rapidly progressive pilomatricoma along the left brow, which was initially misdiagnosed and treated as preseptal cellulitis. Although rare, pilomatricomas and other adnexal tumors should be considered in the differential diagnosis of a growing mass near the brow.Case presentationA 29-year-old male presented to the emergency department with a progressively enlarging left brow lesion, initially noted 3 weeks prior. Exam revealed an erythematous left subbrow mass that measured 2.5 x 2 cm, with resultant mechanical ptosis. The lesion was initially misdiagnosed and treated as preseptal cellulitis, with concern for abscess. The patient ultimately underwent excisional biopsy of the lesion and pathology revealed pilomatricoma.ConclusionsPilomatricoma has similarities to more common skin lesions. Lack of pain or tenderness are important clues against an infectious or inflammatory etiology. Complete surgical excision is therapeutic and allows for diagnostic confirmation. Histopathology is required to rule out pilomatrix carcinoma, a malignant variant.

BackgroundOrbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment. With the aim of clarifying the role of BE as a risk factor, we analyzed patients with orbital RMS included in the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol.MethodsWe retrospectively analyzed the radiological reports of 199 patients with orbital RMS (PM or not) and defined three grades of BE: minimal (thinning of the bone), moderate (focal bone lysis), and extensive (complete cortical destruction).ResultsBE was present in 55 of the 199 (27.6%) patients, which was classified as minimal in 27, moderate in 7, and extensive in 21. Tumors with extensive BE were more frequently large (>5 cm, p = 0.0008) and invasive (T2, p = 0.001). With a median follow-up of 70.4 months (range = 7.1–167.7), a total of 183 patients are alive, with 5-year event-free survival (EFS) and overall survival (OS) rates of 76% (95%CI = 69.2–81.3) and 92% (95%CI = 86.7–94.8), respectively. Patients without any BE had better OS (95% vs. 81%, p = 0.001), but not EFS. Patients with no/minimal/moderate BE had better EFS and OS compared with patients with extensive BE [EFS of 78.1 (95%CI = 71.1–83.5) vs. 57.1 (95%CI = 33.8–74.9), p = 0.0114, respectively, and OS of 94.0 (95%CI = 89.2–96.8) vs. 71.1 (95%CI = 46.6–85.9), p < 0.0001, respectively]. Events and metastatic relapses (in all cases CNS/meningeal) were more frequent in patients with extensive BE.ConclusionsOnly those patients with orbital RMS and extensive BE should be considered as PM and should be treated accordingly.

Purpose: Aims to provide an overview of the contemporary epidemiology of malignant orbital tumors by analyzing population-based incidence patterns across various regions worldwide. Methods: In this article, we retrieved orbital malignancy data from the MEDLINE database and analyzed the incidence and prevalence of orbital malignancies worldwide. We performed the literature search by searching on the Mesh terms for malignant orbital tumors (''orbital'', ''tumor'', ''lymphoma'', ''malignant'', ''cancer'', ''incidence'', and ''epidemiology''). All included studies were published between 1993 and 2023 and were written in English. Results: Ocular or ophthalmic lymphoma most frequently occurred in the orbit, with a prevalence ranging from 47% to 54%. The incidence of malignant orbital tumors was increasing in the USA (2.0 per million (1981–1993), Netherlands (0.86 (1981–1985) to 2.49 (2001–2005) per million) and South Korea (0.3–0.8 per million (1999–2016)), respectively. Ophthalmic lymphoma which includes orbit lymphoma was increasing in Canada (0.17–1.47 per million (1992–2010)), Denmark (0.86 per million (1981–1985) to 2.49 per million (2001–2005)), respectively. Conclusions: The predominant primary malignant orbital tumor in adults was lymphoma. Ocular or ophthalmic lymphoma most frequently occured in the orbit. The limited data available suggested an increasing trend in the incidence of malignant orbital tumors in each country included, which were mainly attributed to the increase in lymphoma. Generally, incidence rates were found to increase with advancing age, with no difference between males and females.

We present a novel case of intraosseous venous malformation of the frontal bone with dehiscence of the inner table of the frontal calvarium and extension into the frontal sinus and orbit. This case report discusses the surgical management of this intraosseous lesion achieved with a multidisciplinary approach involving otolaryngology and neurosurgical teams. We also present a review of the literature of the pathophysiology of venous malformations, the imaging modalities that aid in diagnosis of these lesions and the management options. [ABSTRACT FROM AUTHOR]

Background and Objectives: The objective of this study was to present a rare case of non-keratinizing squamous cell carcinoma (SCC) of the lacrimal sac (LS). Neoplasms of the lacrimal drainage system are extremely rare. These lesions are predominantly malignant and are associated with a high mortality rate. Case Presentation: A 51-year-old woman was referred to an ophthalmologist with a history of unilateral epiphora, presbyopia, and abnormal eye discharge. Antibiotic therapy was applied and modified later due to persisting symptoms. After five months, edema of the medial left eye angle occurred. A lacrimal sac incision was performed, and a subsequent magnetic resonance imaging (MRI) scan revealed a 2 cm, contrast-enhanced solid tumor. The patient was qualified for dacryocystectomy, which was conducted by the otolaryngology department. Postoperative histopathologic findings indicated the presence of non-keratinizing SCC. During a reoperation, margins were extended, and the surgery was found to be radical. Following the reoperation, no alarming symptoms were observed. However, a follow-up MRI and positron emission tomography (PET) scan six weeks later revealed metastases. Further treatment is being planned. Conclusions: LS tumors are life-threatening conditions that are challenging to diagnose at an early stage. Surgical excision is the preferred treatment option. Imaging studies play an important role in post-operative follow-up because of the possibility of recurrence and metastasis, even after radical surgery. [ABSTRACT FROM AUTHOR]

Purpose: Segmentation of orbital tumors in CT images is of great significance for orbital tumor diagnosis, which is one of the most prevalent diseases of the eye. However, the large variety of tumor sizes and shapes makes the segmentation task very challenging, especially when the available annotation data is limited. Methods: To this end, in this paper, we propose a multi‐scale consistent self‐training network (MSCINet) for semi‐supervised orbital tumor segmentation. Specifically, we exploit the semantic‐invariance features by enforcing the consistency between the predictions of different scales of the same image to make the model more robust to size variation. Moreover, we incorporate a new self‐training strategy, which adopts iterative training with an uncertainty filtering mechanism to filter the pseudo‐labels generated by the model, to eliminate the accumulation of pseudo‐label error predictions and increase the generalization of the model. Results: For evaluation, we have built two datasets, the orbital tumor binary segmentation dataset (Orbtum‐B) and the orbital multi‐organ segmentation dataset (Orbtum‐M). Experimental results on these two datasets show that our proposed method can both achieve state‐of‐the‐art performance. In our datasets, there are a total of 55 patients containing 602 2D images. Conclusion: In this paper, we develop a new semi‐supervised segmentation method for orbital tumors, which is designed for the characteristics of orbital tumors and exhibits excellent performance compared to previous semi‐supervised algorithms. [ABSTRACT FROM AUTHOR]

Myxofibrosarcoma (MFS), a common sarcoma in adults, typically affects the extremities. Primary orbital involvement is uncommon, and orbital metastasis is exceedingly rare. We describe a case of orbital MFS due to metastasis and provide an updated literature review. A 56-year-old woman presented with 2 months of progressive right eye proptosis. Relevant history included left palmar MFS excised 2 years prior. Examination demonstrated proptosis and restriction in abduction of her right eye without optic nerve compromise. Imaging revealed a well-circumscribed right orbital mass. Initial biopsy showed glandular tissue with chronic inflammation without an evidence of malignancy. She subsequently developed worsening proptosis, complete ophthalmoplegia, and a new afferent pupillary defect. Repeat orbital excisional biopsy with deeper dissection demonstrated high-grade MFS consistent with metastasis. She underwent chemoradiation with resolution of her lesion. Literature review identified 12 previously reported cases of MFS, only two of which involved metastasis to the orbit and were associated with worse prognosis. This report highlights a rare case of orbital metastasis of MFS with high-grade features confirmed on histopathology and provides an updated literature review. Prompt diagnosis and complete removal of MFS located both systemically and in the orbit are essential due to the metastatic potential. [ABSTRACT FROM AUTHOR]

Merkel cell carcinoma of the eyelid (MCCE) is a rare yet aggressive neuroendocrine tumour associated with significant morbidity and mortality. This study aimed to investigate survival trends and demographic factors related to MCCE, 2000–2019, using the Surveillance, Epidemiology, and End Results (SEER) Program. Cases were analysed by demographic parameters, disease properties, and survival. Statistical analyses were performed via a dedicated computerized software package. A total 349 cases of MCCE were identified, accounting for 2% of all MCC cases in the United States during that time. Of note, the incidence of MCCE remained stable over the study period (p =.35). Female patients accounted for 56% of the cases, and males for 44%. White patients accounted for 90.8% of the the cases, and Black patients for 2.9%. MCCE incidence increased with age, with the majority of patients age 85+ (p <.05). Incidence was greatest in metropolitan areas and among those with median incomes >$75,000/year. Income correlated with likelihood of MCCE diagnosis (p <.05). Analysis of 5-year survival data showed 20% of the patients died due to MCCE within 5 years of diagnosis. Of these, the majority died within one year of diagnosis. Consistent with previous reports, most patients were white, female, and age 85+.Incidence correlated with metropolitan environments and median income. While most patients did not die from MCCE, majority of recorded deaths occurred within one year of diagnosis. [ABSTRACT FROM AUTHOR]

ObjectiveThe aim of this case report is to assess the clinicopathological characteristics and differential diagnosis of orbital granular cell tumor (GCT).MethodsClinical and imaging data of a rare case of orbital GCT involving the superior rectus muscle were collected. Its clinical characteristics, imaging, and histopathological features were observed.ResultsA 36-year-old female patient presented with a 2-year history of left eye proptosis. Magnetic resonance imaging (MRI) enhancement suggested a space-occupying lesion in the left superior rectus muscle region. On T1-weighted and T2-weighted MRI, the tumor was isointense to gray matter and significantly enhanced on the enhanced scan. Microscopic examination revealed that most tumor cells exhibited diffuse growth with unclear boundaries, and some cells were arranged in small nests. The tumor cells were large, with abundant, coarse eosinophilic granules in the cytoplasm. Occasional cells contained larger round eosinophilic droplets in the cytoplasm. Focal areas showed foamy cells, small and central round or oval nuclei with occasional nuclear enlargement and mild atypia, inconspicuous nucleoli, rare mitoses, and low proliferative activity. Immunohistochemistry results were Vimentin (+), S-100 (+), CD68 (+), Ki67 (2%+), Inhibin-a (−), CK (−), SMA (−), and Desmin (−). The pathological examination of a specimen harvested from the mass corresponded to a GCT.ConclusionOrbital GCT is rare and should be considered in the differential diagnosis of orbital tumors. It is essential to distinguish it from thyroid-associated ophthalmopathy, inflammatory pseudotumor, and myohemangioma. Definitive diagnosis requires a comprehensive analysis of clinical, histopathological, and immunohistochemical findings. Surgical excision is the primary treatment for orbital GCTs. For patients with incomplete tumor resection, close follow-up is necessary. Proton beam radiation therapy can be considered to prevent recurrence or metastasis if needed.

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. [ABSTRACT FROM AUTHOR]

Orbital tumors are the most common eye tumors that affect people all over the world. Early detection prevents the progression to other regions of the eye and the body. Also, early identification and treatment could reduce mortality. A computer-assisted diagnosis (CAD) system to help physicians diagnose tumors is in great demand in ophthalmology. In recent years, deep learning has demonstrated promising outcomes in computer vision systems. This work proposes a CAD system for detecting various forms of orbital tumors using convolutional neural networks. The system has three stages: preprocessing, data augmentation and classification. The proposed system was evaluated on two datasets of magnetic resonance imaging (MRI) images containing 1404 MRI T1-weighted images and 1560 MRI T2-weighted images. The results have shown that the system is capable of detecting and classifying the tumor in each image type, and the recognition rate for the T1-weighted image is 98% and for the T2-weighted image is 97%. [ABSTRACT FROM AUTHOR]

Purpose: Orbital tumors are an interdisciplinary disease, and surgery is one of the main treatment methods. The oculocardiac reflex (OCR) is a condition of surgery for orbital tumors. The aim of this study was to investigate whether there is an association between many surgical factors and the incidence of OCR in orbital tumor surgery. Methods: Comparisons were made between patients with and without OCR using the Mann–Whitney test, Fisher's exact test, and Chi-square test. When comparing multiple groups (groups > 2), to explain which two groups had differences, post hoc testing was used for analysis, and the differences between groups were judged according to the adjusted standardized residuals. Results: The results showed that the incidence of intraoperative OCR was different based on the different exposed operative field locations (p = 0.021). The OCR incidence in those with lesions involving the orbital apex and lesions adhering to extraocular muscles was higher than that of others (p < 0.001 and p = 0.003). In addition, multivariate logistic regression analysis revealed that orbital apex involvement and extraocular muscle adhesion were highly associated with a higher incidence of OCR (p < 0.001 and p = 0.013), while the operative field located in the lateral-superior orbit was highly associated with a lower incidence of OCR (p = 0.029). Conclusion: In orbital tumor surgery under general anesthesia, lesions involving the orbital apex and lesion adhesion to the extraocular muscles were independent risk factors for OCR, and an operative field located in the lateral-superior orbit was a protective factor for OCR. [ABSTRACT FROM AUTHOR]

A 59-year-old male presented with a 1-year history of proptosis in the right eye. Ocular examination revealed proptosis, global displacement, limited adduction and infraduction and slight conjunctival hyperemia in the right eye with increased orbital compartment pressure (T+2). The rest of the physical examination was unremarkable. Radiological examinations demonstrated that a soft-tissue mass involving both intraconal and extraconal space was located behind the globe and at the nasal and downward side of the optic nerve, which was tightly adjacent to the optic nerve, medial rectus and inferior rectus. The internal signal of the tumor was inhomogeneous. The signals of the upper and under part were different in the MRI: the upper half of tumor body was enhanced obviously and the lower half was not enhanced. Thus, an orbital tumorectomy through lower eyelids was performed after hospital admission, and a diagnosis of solitary fibrous tumor was confirmed by histopathological examination. Discussion and experience: for orbital solitary fibrous tumor, incomplete resection led to the recurrence of tumor. There is a tendency of malignant transformation, even threatening the patient’s life in partial cases. For orbital tumors with atypical imaging features, differential diagnosis before operation, appropriate surgical approach and complete resection are important to the prevention of tumor recurrence. [ABSTRACT FROM AUTHOR]

Objectives: To assess the usefulness of ultrasonography in the diagnosis and evaluation of extraocular intra‐orbital lesions in pediatric patients. Methods: Twenty‐three pediatric patients with intra‐orbital lesions who underwent both ultrasound and computed tomography/magnetic resonance imaging (CT/MRI) were included. The following parameters were evaluated using ultrasound: 1) lesion detection rate (presence or absence of lesions), 2) lesion characteristics, 3) lesion location (extraconal or intraconal), and 4) the lesion longest linear dimensions, and these were compared using Fisher's exact test and Mann–Whitney U test. Results: Two lesions could not be detected using ultrasound; in the other 21 cases, the lesion characteristics diagnosed by ultrasound were correct. Diagnostic accuracy of detection and characteristics assessment using ultrasound were 91.3% and 91.3%, respectively. The lesion location was not significantly different between the two groups (intraconal/extraconal in those detected using ultrasound versus those in the absence on ultrasound = 7/14 versus 0/2, P >.999); however, in two cases that were not detected on ultrasound, the lesions were located at extraconal. Lesions that were small in longest linear dimensions on CT/MRI were not detected using ultrasound (the longest linear dimensions in lesions detected using ultrasound versus that in the absence of ultrasound: 29.5 ± 8.2 [range, 13–46] versus 10 and 11 mm, P =.043). Conclusions: Ultrasonography proved to be useful for visualizing and evaluating intra‐orbital lesions except for lesions that were relatively small in size. Therefore, although ultrasound could not detect lesions located behind bone and bone invasion, it could be used for diagnosing and selecting treatment strategies for intra‐orbital lesions. [ABSTRACT FROM AUTHOR]

Objective: We report a case of a scleral buckle mimicking an orbital tumor 28 years after a retinal detachment repair. Methods: This is a case report. Results: A 75-year-old male consulted for progressive, painless blurred vision of the right eye. He had a history of scleral buckling surgery for retinal detachment on the right eye in 1990 that restored his vision. Examination showed right eye ophthalmoplegia and inferior displacement of the globe. Imaging revealed a right superotemporal orbital mass. Excision of the encapsulated mass was performed. Histopathology revealed an acellular, amorphous, granular and eosinophilic material with no evidence of malignancy. These were consistent with a foreign body. Postoperatively, there were improvements in ocular motility and hypoglobus. Conclusion: In patients who present with limited ocular motility and have undergone scleral buckling, hydrogel scleral buckle overexpansion should be considered. Excision of such implants is warranted to resolve the signs and symptoms and confirm the etiology.

A 42-year-old non-menopausal, non-pregnant woman presented with an incidentally noted right orbital apex lesion producing mild compressive optic neuropathy. Imaging revealed an apical, intraconal, inhomogenously enhancing mass consistent with a cavernous venous malformation displacing the optic nerve. The patient was monitored over a 15-year period with serial orbital imaging and clinical examinations and ultimately demonstrated significant regression in the size of the lesion, accompanied by the complete resolution of optic neuropathy. She was non-menopausal when the regression was noted and remained non-pregnant throughout the follow-up period. [ABSTRACT FROM AUTHOR]

Purpose: We describe a case of fat-forming solitary fibrous tumor (SFT) of the orbit with typical findings on imaging that may improve the awareness of orbital fat-forming SFT. Observations: An 88-year-old female presented with exophthalmos and pain in her right eye. Preoperative imaging showed an oval, well-defined mass with soft-tissue density, interspersed with a well-circumscribed lesion. The lesion showed low-density in computed tomography (CT) scans, hyperintense in T1/T2 weighted images of magnetic resonance imaging (MRI) scans and hypointense in fat-suppressed images of MRI scans. The tumor was removed en bloc and diagnosed as low-grade malignant fat-forming SFT by pathological examination. There was no evidence of recurrence 9-month postoperatively. Conclusions: The imaging feature of orbital fat-forming SFT is a well-defined solid tumor interspersed with adipose tissue. Such findings are vital for the preoperative diagnosis and the choice of the treatment.

A 60-year-old female presented with a large, left upper eyelid mass that had rapidly expanded in the 3 months prior to presentation. She had a presumed chalazion excised from the same area 1.5 years ago, but no pathology was investigated. On examination, she had a palpebral mass measuring 4.5 cm x 3.5 cm that abutted the globe with extensive conjunctival involvement. Neuroimaging demonstrated lesions concerning for parotid gland metastases. An incisional biopsy demonstrated synaptophysin-positive small blue cells concerning for neuroendocrine carcinoma. The patient underwent orbital exenteration with parotidectomy and radical neck dissection. The excised mass was found to have distinct neuroendocrine carcinoma cells intermingled with sebaceous carcinoma cells, a combination not previously reported. [ABSTRACT FROM AUTHOR]

Heterotopic brain tissues are a group of extracerebral neuroglial tissues. Heterotopic brain tissue in the orbit remains a rare clinical entity. This article presents a 7-year-old male child who presented with severe globe displacement, proptosis, and vision loss in the right eye. The orbital imaging showed a huge orbital cystic mass displacing the globe. The cyst was excised entirely from the orbit. The histopathological investigations revealed the presence of a cystic lesion containing brain tissue that was immune reactive for S-100 and glial fibrillary acidic proteins. The diagnosis was confirmed to be heterotopic brain tissue due to the lack of visible bony defect. The relevant literature was also reviewed. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Schwannomas are rare tumors in the orbit, typically originating from various nerves and presenting diagnostic challenges. We present a unique case of a unilateral orbital schwannoma arising from the supraorbital nerve. A 55‐year‐old female presented with a painless, slowly growing mass in the right superior orbit, causing proptosis. Visual acuity remained unimpaired, and clinical examination revealed a well‐defined mass in the superior orbit. A provisional diagnosis of an orbital dermoid or cyst was made, leading to excision biopsy. The histopathological examination confirmed a diagnosis of benign schwannoma. Schwannomas in the orbit, particularly those arising from the supraorbital nerve, are uncommon and often challenging to diagnose. Early surgical intervention is crucial to prevent complications associated with tumor growth. This case underscores the need to consider schwannomas as a differential diagnosis for slow‐growing orbital masses in adults and emphasizes the importance of timely management to prevent vision‐threatening complications. [ABSTRACT FROM AUTHOR]

Background: Orbital tumors, arising within the bony orbit and its contents, present diverse challenges due to their varied origins and complex anatomical context. These tumors, classified as primary, secondary, or metastatic, are further subdivided into intraconal and extraconal based on their relationship with the muscle cone. This classification significantly influences surgical approach and management. This study highlights surgical experiences with orbital tumors, underscoring the importance of tailored surgical approaches based on the lesion's site and its proximity to the optic nerve. Methods: This retrospective study at the National Institute of Cancer's Head and Neck Department (2005-2014) analyzed 29 patients with orbital tumors treated with surgery, radiotherapy, chemotherapy, or combinations of them. Patient demographics, tumor characteristics, and treatment responses were evaluated using computed tomography (CT), magnetic resonance imaging, and positron emission tomography-CT imaging. Malignant tumors often required orbital exenteration and reconstruction, highlighting the study's commitment to advancing orbital tumor treatment. Results: 29 patients (18 females and 11 males, age 18-88 years, mean 53.5 years) with orbital tumors exhibited symptoms such as decreased vision and exophthalmos. Tumors included primary lesions like choroidal melanoma and secondary types like epidermoid carcinoma. Treatments varied, involving a multidisciplinary team for surgical approaches like exenteration, with follow-up from 1 to 9 years. Radiotherapy and chemotherapy were used for specific cases. Conclusion: Our study underscores the need for a multidisciplinary approach in treating orbital tumors, involving various surgical specialists and advanced technologies like neuronavigation for tailored treatment. The integration of surgery with radiotherapy and chemotherapy highlights the effectiveness of multidimensional treatment strategies. [ABSTRACT FROM AUTHOR]

Adenoid cystic carcinoma (ACC) is the most common malignant neoplasm involving the lacrimal glands, with high rates of recurrence and metastasis. During the pregnancy, reports of recurrence of ACC of the salivary glands and trachea have previously been published, but no lacrimal gland ACC recurrence has been reported. We present a 35-year-old woman with lacrimal gland ACC who was initially treated by surgical resection and adjunctive radiotherapy, but her cancer recurred during pregnancy, with rapid progression to cavernous sinuses and brain. Estrogen and progesterone receptors have been detected on lacrimal glands and ACCs of salivary glands. Thus, hormonal changes during pregnancy might contribute to the recurrence of ACC. However, the inherent invasive and recurrent nature of ACC could also account for the regrowth in this patient and further molecular studies can provide more accurate explanations. [ABSTRACT FROM AUTHOR]

AIM: To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma. METHODS: The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital, from 2006 to 2021 were collected and analyzed. The data includes age, gender, affected orbital side, course of disease, status of disease (primary or recurrent), clinical manifestations, preoperative visual acuity, operative treatment, the relations between liposarcoma and surrounding tissue, longest diameter of liposarcoma, histological subtype, immunohistochemical indicators, follow-up treatment and prognosis. RESULTS: The initial symptoms are diverse. Proptosis is the most frequent chief complaint and the others included vision loss, epiphora, diplopia, and eyelid palpable mass. Results of imaging examination [computed tomography (CT) or magnetic resonance imaging (MRI)] showed orbital mass. In terms of treatment, 10 patients received tumor resection, and the mean longest diameter of the tumor was 3.39±1.36 cm. The other 3 patients had optic nerve invaded, so they received orbital exenteration. Pathological examination results confirmed the diagnose of liposarcoma for 13 patients. Six patients displayed as myxoid type, and three patients in each type of dedifferentiated and well-differentiated type. One patient was verified as pleomorphic, which was a rare type of liposarcoma. All of the patients showed Vimentin positive, and most showed CD34 and S-100 positive. Besides, four patients showed smooth muscle actin positive. All thirteen patients were alive. CONCLUSION: Orbital liposarcoma is a rare disease and it has no specific clinical manifestation. The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit. It is recommended to perform pathological examination to achieve early detection and early treatment.

Orbital plasmacytoma is rare and has only been reported in the context of the initial diagnosis of multiple myeloma. Moreover, isolated orbital plasmacytoma without any signs of multiple myeloma is extremely rare.We report the case of a 59-year-old female patient diagnosed with IgA Kappa multiple myeloma. It was stage I ISS (International Staging System) and stage I R-ISS (Revised ISS). According to the Tunisian national protocol, the patient was included in the standard-risk group and was eligible for four cycles of CTD (Cyclophosphamide, Thalidomide, Dexamethasone) followed by autologous stem cell transplantation. Taking into account the partial response after the CTD cycles, the patient has benefited from two VTD cycles (Bortezomib, Thalidomide, Dexamethasone). Thus, complete remission was obtained. The patient refused autologous stem cell transplantation. Therefore, maintenance treatment based on Thalidomide only was started and received over a twelve-month period.Five months after the end of maintenance treatment, she reported frontal headaches that were resistant to symptomatic treatment, with ptosis in the right eye in physical examination. Brain MRI revealed the presence of a right cranio-orbital tissue mass with intra-orbital and extra-axial cerebral components. The mass measured 32/36 mm on axial sections and 47 mm in height. The patient underwent a complete resection of the cranio-orbital mass with cranioplasty. The histopathological examination of the mass with Immunohistochemistry staining confirmed the diagnosis of orbital plasmocytoma.An update of the multiple myeloma assessment did not reveal any biological, cytological or radiological signs in favor of multiple myeloma. Therefore the diagnosis of isolated orbital plasmacytoma without signs of multiple myeloma was made.Post-operative brain MRI showed complete disappearance of the right cranio-orbital tissue lesion. There was only a persistent meningeal enhancement of the dura mater at the surgical site, suggestive of post-operative changes. The patient was then referred for cranio-orbital radiotherapy.

Background: The Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system has become the gold standard for outcomes reporting in endoscopic orbital surgery for orbital cavernous hemangiomas (OCHs). A recent systematic review demonstrated similar outcomes between OCHs and other primary benign orbital tumors (PBOTs). Therefore, we hypothesized that a simplified and more comprehensive classification system could be developed to predict surgical outcomes of other PBOTs. Methods: Patient and tumor characteristics as well as surgical outcomes from 11 international centers were recorded. All tumors were retrospectively assigned an Orbital Resection by Intranasal Technique (ORBIT) class and stratified based on surgical approach as either exclusively endoscopic or combined (endoscopic and open). Outcomes based on approach were compared using chi‐squared or Fisher's exact tests. The Cochrane‐Armitage test for trend was used to analyze outcomes by class. Results: Findings from 110 PBOTs from 110 patients (age 49.0 ± 15.0 years, 51.9% female) were included in the analysis. Higher ORBIT class was associated with a lower likelihood of gross total resection (GTR). GTR was more likely to be achieved when an exclusively endoscopic approach was utilized (p < 0.05). Tumors resected using a combined approach tended to be larger, to present with diplopia, and to have an immediate postoperative cranial nerve palsy (p < 0.05). Conclusion: Endoscopic treatment of PBOTs is an effective approach, with favorable short‐term and long‐term postoperative outcomes as well as low rate of adverse events. The ORBIT classification system is an anatomic‐based framework that effectively facilitates high‐quality outcomes reporting for all PBOTs. [ABSTRACT FROM AUTHOR]

Orbital tumors are the most common type of tumor affecting the orbit. Some factors, such as technical causes relating to imaging quality and human error, contribute to radiologists misdiagnosing eye tumors. Computer-aided detection systems (CADs) are being developed to address these limitations and have recently been used in numerous imaging modalities for eye tumor diagnosis. CAD technologies increase radiologists' ability to detect and distinguish between normal and diseased tissues. These techniques are only conducted as a second opinion, but the radiologist makes the final decisions. This article presents the contemporary CAD method for detecting orbital tumors on magnetic resonance imaging (MRI) utilizing Convolutional Neural Networks (CNN). Pre-processing, Data Augmentation, Classification, and Evaluation are the four stages that involve our CAD system. Two datasets were used for MRI images: 1404 MRI T1-weighted images and 1560 MRI T2-weighted images. The system was evaluated by many evaluation metrics including the recognition rate which gives 95% for T1-weighted images and 94% for T2-weighted images. [ABSTRACT FROM AUTHOR]

Abstract Purpose: In this study, we describe different orbital space-occupying lesions (SOLs) from a referral center in Iran. Methods: In this retrospective case series, all records of “orbital tumors” with a definite histopathologic diagnosis at a referral center in Iran were reviewed from April 2008 to May 2020. Results: A total of 375 orbital SOLs were included. The study population consisted of 212 (56.5%) female and 163 (43.5%) male subjects with overall mean age of 31.09 ± 21.80 years. The most common clinical presentation was proptosis and the superotemporal quadrant was the most frequent site of involvement. Extraconal lesions (276 cases, 73.6%) outnumbered intraconal lesions (99 cases 26.4%). The great majority of SOLs (344, 91.7%) were primary, while 24 (6.4%) were secondary and 7 (1.9%) were metastatic. Benign lesions (309, 82.4%) were much more common than malignant SOLs (66, 17.6%). Overall, dermoid cysts and malignant lymphoma were the most prevalent benign and malignant orbital SOLs, respectively. The malignant to benign lesion ratio was 0.46 in children ( ≤ 18 years), 0.81 in middle-aged subjects (19–59 years), and 5.9 in older ( ≥ 60 years) cases. The most common type of malignancy was rhabdomyosarcoma in children, lymphoma in middle-aged subjects, and invasive basal cell carcinoma in older age group. Conclusion: Over the 12-year study period, benign, primary, extraconal orbital SOLs were more frequent than malignant, secondary, and intraconal lesions. The ratio of malignant lesions increased with age in this cohort of patients.

AIM: To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and to compare the treatment outcome between postoperative radiotherapy (RT) and chemotherapy in a retrospective analysis nearly 20y. METHODS: A retrospective cohort study of 56 patients with orbital NRSTS were reviewed, 34 of whom received postoperative RT, and 22 received postoperative chemotherapy. The clinicopathological features, local recurrence, metastases, and survival data were recorded. Survival analysis was performed using the Kaplan-Meier method. RESULTS: During follow-up (111.8mo, ranged 8-233mo) for 56 patients, 19 patients of them developed local recurrence, and 7 patients developed distant metastases. Fifteen patients died during follow-up period. Overall survival rates considering the whole study group was 78.57% at 5y, and 72.16% at 10y after the initial diagnosis. Compared with chemotherapy, RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy, 0.263, 95% confidence interval (CI), 0.095-0.728, P=0.0015]; with lower risk of distant metastasis (hazard ratio for RT vs chemotherapy, 0.073, 95%CI, 0.015-0.364, P=0.0014); and with lower risk of death from disease (hazard ratio for RT vs chemotherapy, 0.066, 95%CI, 0.022-0.200, P

Phantaraporn Tangtammaruk,1 Mongkol Uiprasertkul,2 Mingkwan Lumyongsatien1 1Department of Ophthalmology, Mettapracharak Hospital, Nakhon Pathom, Thailand; 2Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Mingkwan Lumyongsatien, Department of Ophthalmology, Mettapracharak Hospital, Nakhon Pathom, Thailand, Tel +66814958985, Fax +6634388744, Email mingkwan@metta.go.thAbstract: Extraocular muscle lipoma is a rare benign mesenchymal tumor of the orbit. We report a case of a 37-year-old woman who presented with chronic progressive proptosis and inferior globe displacement of left eye. External eye examination revealed a yellowish mass at the superior bulbar conjunctiva. Magnetic resonance imaging showed a well-circumscribed mass confined in the superior rectus muscle belly and tendon with a fat signal. Debulking surgery was performed using the transconjunctival and vertical lid split approach. A pathological study demonstrated matured adipose tissue cells encapsulated by a thin layer of fibrous tissue, in addition to the chronic non-specific inflammation of the tenon capsule tissue sample. Histopathological findings of the mass were consistent with a well-circumscribed intramuscular lipoma. The symptoms of the patient were significantly improved 3 months after surgical and short-course systemic steroid treatments. However, long-term surveillance is needed.Keywords: intramuscular lipoma, soft tissue tumor, orbital tumor, extraocular muscle, well-circumscribed lipoma

Orbital venous malformations are low-flow lesions resulting from vascular dysgenesis during development. Patients may present with vision loss, proptosis accentuated by Valsalva, and/or painful spontaneous thrombosis. The preferred treatment for symptomatic lesions is embolization combined with excision. A 34-year-old male presented to our institution from an outside emergency department with a diagnosis of presumed idiopathic orbital inflammation. For the prior month, he had been experiencing left orbital pressure, subjective eye bulging, and both diplopia and blurry vision when in peripheral gaze or when bending over. Despite initial improvement with steroids, his symptoms recurred with tapering. Visual acuity was reduced to 20/25, but pupils and motility remained normal. Biopsy demonstrated a vascular lesion characterized by fibroadipose tissue with histologically unremarkable blood vessels, and cerebral arteriography showed no high-flow components. A diagnosis of orbital venous malformation was made. He then underwent intraoperative angiography and Onyx embolization followed by excision via a transcaruncular approach. Two prior reports have described the use of Onyx in venolymphatic malformations. This report highlights a detailed approach to defining flow characteristics pre- and intraoperatively and expands upon our understanding of the use of Onyx for such cases.

Myxofibrosarcoma (MFS), a common sarcoma in adults, typically affects the extremities. Primary orbital involvement is uncommon, and orbital metastasis is exceedingly rare. We describe a case of orbital MFS due to metastasis and provide an updated literature review. A 56-year-old woman presented with 2 months of progressive right eye proptosis. Relevant history included left palmar MFS excised 2 years prior. Examination demonstrated proptosis and restriction in abduction of her right eye without optic nerve compromise. Imaging revealed a well-circumscribed right orbital mass. Initial biopsy showed glandular tissue with chronic inflammation without an evidence of malignancy. She subsequently developed worsening proptosis, complete ophthalmoplegia, and a new afferent pupillary defect. Repeat orbital excisional biopsy with deeper dissection demonstrated high-grade MFS consistent with metastasis. She underwent chemoradiation with resolution of her lesion. Literature review identified 12 previously reported cases of MFS, only two of which involved metastasis to the orbit and were associated with worse prognosis. This report highlights a rare case of orbital metastasis of MFS with high-grade features confirmed on histopathology and provides an updated literature review. Prompt diagnosis and complete removal of MFS located both systemically and in the orbit are essential due to the metastatic potential.