Epidemiology and tumor microenvironment of ocular surface and orbital tumors on growth and malignant transformation.

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.

Orbital metastasis of myxofibrosarcoma.

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.

Orbital Adipose Tissue: The Optimal Control for Back-Table Fluorescence Imaging of Orbital Tumors.

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.

Concurrent delayed recurrence of peripheral primitive neuroectodermal tumors in orbital and sellar/suprasellar regions in an older adult.

BACKGROUND: Peripheral primitive neuroectodermal tumors (pPNETs) are rare and aggressive small round cell tumors, tending to occur in the thoracic and paravertebral soft tissues in children and young adults. This report describes an exceptionally rare case of concurrent delayed recurrence of pPNET in the orbital and sellar/suprasellar regions in an older adult, with a discussion supported by a literature review. CASE PRESENTATION: We report an 82-year-old woman with a history of orbital pPNETs resection at age 62, followed by gamma knife radiosurgery for local recurrence at age 66. She presented left eye pain, left eye protrusion, decreased vision in the right eye, and right homonymous hemianopia. MRI revealed extensive lesions in the left orbital cavity and sellar/suprasellar region, contiguous through the optic canal. The recurrent tumor was treated through a two-stage resection via transcranial and transsphenoidal approaches, which resulted in symptom improvement and a pathologic diagnosis of pPNETs. CONCLUSION: This case highlights a highly rare instance of late-onset orbital pPNETs recurrence in an elderly patient, with evidence suggesting tumor progression into the sellar/suprasellar regions through the optic canal.

Rare Case of Non-Keratinizing Squamous Cell Carcinoma in the Lacrimal Sac Treated with Surgery.

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.

Intraosseous venous malformation of the frontal bone with extension into the frontal sinus and orbit.

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.

Global incidence and prevalence of malignant orbital tumors.

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.

Gamma knife radiosurgery for orbital cavernous hemangioma: a systematic review and single-arm meta-analysis.

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.

Rare Orbital Involvement Originating from Extranodal Marginal Zone Lymphoma.

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.

Orbital aspergillosis in an immunocompromised man with no history of trauma: a case report.

Fungal orbital cellulitis is usually seen in immunocompromised individuals, and opportunistic pathogens are the main etiology. We herein report a case of fungal orbital cellulitis due to Aspergillus in a patient with no history of trauma. A 48-year-old man presented to the emergency room of our hospital with a 2-week history of periorbital swelling, conjunctival hyperemia, and chemosis of his right eye. The visual acuity of his right eye was 6/20, and the intraocular pressure was 44 mmHg. The main clinical findings were proptosis of the right ocular globe with conjunctival hyperemia and a palpable infratemporal orbital mass. Laboratory testing failed to detect the presence of a pathogenic infection, and the lesions on computed tomography images resembled those of a malignant tumor of the orbit. The diagnosis was finally confirmed by postoperative pathological examination, and the patient responded favorably to debridement combined with antifungal therapy. Histopathological examination may help to reveal the nature of this disease. Surgical removal of inflammatory lesions can serve as an important diagnostic and treatment method for fungal orbital cellulitis.

Surgical treatment of orbital tumors in a single center: Analysis and results.

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.

Extramedullary plasmacytoma of the orbit complicating the evolution of multiple myeloma in complete remission.

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.

MR Imaging Characteristics of Solitary Fibrous Tumors of the Orbit : Case Series of 18 Patients.

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.

Superior orbital rim grinding as a novel surgical technique to approach superior and posterior orbital tumors.

PURPOSE: To describe a novel surgical technique aimed to approach those orbital lesions located superior and posterior to the equator of the globe. METHODS: We describe a novel surgical technique that was performed in four patients to approach intraorbital tumors superiorly and posteriorly located. This technique was completed through an upper eyelid skin crease followed by grinding the superior orbital rim to achieve complete removal of the lesions. RESULTS: Complete removal of the lesion was accomplished in every case. No intraoperative complications were observed in any of the patients. During the follow-up period, one patient presented with frontal hypersensitivity and one with diplopia. CONCLUSIONS: In the case series presented, the upper eyelid skin crease approach with grinding of the superior orbital rim proved to be a safe and effective surgical technique to remove lesions located superior and posterior within the orbit in our series. More studies are needed to further evaluate the efficacy and long-term results of this approach.

[Pediatric orbital Rosai-Dorfman disease: An unusual case]. / Maladie de Rosai-Dorfman à localisation orbito-palpébrale de l'enfant : à propos d'une observation exceptionnelle.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a sinus histiocytosis with massive lymphadenopathy. This rare condition is a benign disease of unknown etiology. Bilateral orbital localization of RDD is rare. OBSERVATION: The authors report the case of a 6-year-old child who presented with bilateral orbital-palpebral masses associated with chronic cervical lymphadenopathy. There were no laboratory signs of inflammation. Serological and tuberculosis screening tests were negative. Histopathological examination of a lymph node biopsy established a diagnosis of Rosai-Dorfman disease. The patient underwent surgical excision of the orbital lesions followed by long-term corticosteroid therapy. A favorable course was observed, with no sign of recurrence after one year of follow-up. CONCLUSION: Rosai-Dorfman disease is very rare in its bilateral orbital presentation. Histopathological diagnosis remains challenging. In Africa, the presence of chronic oculo-palpebral tumor associated with or without cervical lymphadenopathy must raise the suspicion of Rosai-Dorfman disease after ruling out tuberculosis and lymphoma.

Multi-scale consistent self-training network for semi-supervised orbital tumor segmentation.

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.

Unilateral orbital schwannoma arising from the supraorbital nerve: Report of a rare case.

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.

Fat-forming solitary fibrous tumor of the orbit with typical imaging findings.

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.

Can 18F-FDG PET/CT findings be used to predict orbital tumor histology?

PURPOSE: To investigate whether 18F-FDG PET/CT might be useful to predict the histology of various orbital tumors based on the maximum standard uptake value (SUVmax) and the OMSUV (orbital max SUV)/MLSUV (mean liver SUV) ratio. PATIENTS AND METHODS: A retrospective single-center study was conducted between May 2019 and December 2020. Patients with an orbital mass who underwent preoperative 18F-FDG PET/CT followed by an orbital biopsy were included. Tumor histology was classified as follows: orbital inflammation, solid tumor, low-grade lymphoid tumor, and high-grade lymphoid tumor. Orbital tumors were also classified as indolent or aggressive. Data recorded included the orbital SUVmax, OMSUV/MLSUV ratio and additional extra-orbital SUV sites. RESULTS: Forty-five patients (24 men) were included. There were 15 (33.3%), 14 (31.1%), 9 (20%), and 7 (15.5%) cases of orbital inflammation, solid tumor, low-grade lymphoid tumor, and high-grade lymphoid tumor, respectively. No correlation was found between the OMSUV/MLSUV ratio and orbital SUVmax and tumor histology (Z = -0.77, Z = -0.6, Z = -1.6, and Z = 0.94, all P > 0.05, respectively). No correlation was found between the OMSUV/MLSUV ratio (Z = -1.42, P > 0.05) and orbital SUVmax (Z = -0.82, P > 0.05) and tumor aggressiveness (indolent versus aggressive). Subgroup analyses showed that SUVmax was predictive of lymphoma aggressiveness (P = 0.05) and was able to distinguish orbital cancers (all lymphomas+solid tumors) from benign tumors (P = 0.02). CONCLUSION: 18F-FDG PET/CT could not be used to predict the underlying orbital tumor histology. However, more aggressive tumors, especially high-grade lymphomas and cancers, tended to have a higher orbital SUVmax compared to indolent lesions.