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11 pages, 689 KB  
Review
Use of Robotic Surgery for the Management of Orbital Diseases: A Comprehensive Review
by Riccardo Nocini, Lorenzo Marini, Luca Michelutti, Chiara Zilio, Stefania Troise, Salvatore Sembronio, Giovanni Dell’Aversana Orabona, Massimo Robiony and Alessandro Tel
Medicina 2025, 61(6), 1081; https://doi.org/10.3390/medicina61061081 - 12 Jun 2025
Viewed by 859
Abstract
Background and Objectives: Robotic surgery represents one of the most significant innovations in the field of surgery, offering new opportunities for the treatment of complex pathologies that require greater accuracy and precision. It is a technology that has become widely used in [...] Read more.
Background and Objectives: Robotic surgery represents one of the most significant innovations in the field of surgery, offering new opportunities for the treatment of complex pathologies that require greater accuracy and precision. It is a technology that has become widely used in general, urologic, gynecologic, and cardio-thoracic surgery, but has a limited evidence in the head and neck region. This review explores the use of robotic surgery in orbital pathology, focusing on its applications, benefits, and limitations. Materials and Methods: A cross-sectional search method was performed in multiple databases to answer the following question: “What are the applications of robotic surgery in the management of orbital pathologies?” Studies were carefully reviewed by two simultaneous researchers, and, in case of disagreement, a third researcher was engaged. Care was taken to identify the surgical hardware (robotic station) used to perform the surgical procedure. Results: Out of 491 records, eight studies met the inclusion criteria. These included cadaveric, preclinical, in vitro, and early clinical investigations assessing robotic approaches for fronto-orbital advancement, tumor resection, orbital decompression, and other surgical procedures such as lacrimal gland dissection and biopsy, medial and lateral orbital wall dissections, enucleation, and lid-sparing orbital exenteration. The robotic systems evaluated included the Da Vinci Xi, Da Vinci SP, Medineering Robotic Endoscope Guiding System, and a modular multi-arm concentric tube robot, each with specific advantages and limitations. Conclusions: Robotic surgery provides significant advantages for orbital pathologies such as improved precision, visualization, and tissue preservation, with reduced complications and faster recovery, although some limitations still exist. Future advancements, such as smaller instruments and AI integration, promise to improve outcomes, making robotic surgery more effective in treating orbital conditions. Full article
(This article belongs to the Special Issue New Trends and Advances in Oral and Maxillofacial Surgery)
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11 pages, 1273 KB  
Article
General and Treatment-Specific Outcomes with Osseointegrated Implants in Auricular, Nasal, and Orbital Prosthetic Reconstruction
by Morgan M. Sandelski, Deema Martini, Todd M. Kubon, Greg G. Gion and Amy L. Pittman
Craniomaxillofac. Trauma Reconstr. 2025, 18(1), 16; https://doi.org/10.3390/cmtr18010016 - 18 Feb 2025
Cited by 1 | Viewed by 1428
Abstract
Background: Osseointegrated implants outside of dental restoration remain an integral area of facial reconstruction in which more outcomes data is needed. We aimed to describe our 13-year experience using osseointegrated implants for orbital, nasal, and auricular reconstruction, looking at general outcomes, including radiated [...] Read more.
Background: Osseointegrated implants outside of dental restoration remain an integral area of facial reconstruction in which more outcomes data is needed. We aimed to describe our 13-year experience using osseointegrated implants for orbital, nasal, and auricular reconstruction, looking at general outcomes, including radiated and surgically manipulated bone. Methods: This retrospective chart review covered demographics and outcomes from January 2008 to August 2021 in patients who underwent an orbital exenteration, partial or total rhinectomy, and partial or total auriculectomy with subsequent osseointegrated implant placement. We hypothesized radiation would increase the failure rate of implants and prostheses. Results: There were 79 implants placed in 27 patients, with over half of the patients requiring implants for reconstruction because of malignancy. The success rate was 86%. Complications were uncommon. Only 2 (7.4%) patients were unable to use their prosthesis. Prior radiation and surgery to the bone were associated with an increased risk of loss of implant (p = 0.008 and p = 0.007, respectively) but not associated with other complications or prosthesis non-viability. Conclusions: Osseointegrated implants are a reliable, permanent option for a realistic prosthesis. Radiation and prior surgery are significantly associated with an increased risk of implant failure but not associated with the inability to use the prosthesis. Regardless of prior treatments, bone-retained implants should be considered in facial reconstruction, especially after failing autologous repair or with concerns for cosmetic outcomes. Full article
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14 pages, 1434 KB  
Article
Long-Term Outcomes After Multidisciplinary Treatment for Pediatric Orbital Rhabdomyosarcoma
by Nur Khatib, Johannes H. M. Merks, Jeroen E. Markenstein, Brian V. Balgobind, Cemile. D. Savci-Heijink, Michele Morfouace, Bradley R. Pieters and Peerooz Saeed
Cancers 2025, 17(4), 615; https://doi.org/10.3390/cancers17040615 - 11 Feb 2025
Viewed by 1338
Abstract
(1) Background: Orbital rhabdomyosarcoma is a rare and aggressive soft tissue tumor that primarily occurs in the eye socket (orbit) of children. Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy, aiming to remove the tumor and prevent metastasis. (2) Methods: [...] Read more.
(1) Background: Orbital rhabdomyosarcoma is a rare and aggressive soft tissue tumor that primarily occurs in the eye socket (orbit) of children. Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy, aiming to remove the tumor and prevent metastasis. (2) Methods: An institutional retrospective study was conducted with data from 39 patients with primary orbital RMS treated between 1995 and 2016 at the Amsterdam University Medical Centers/Emma Children Hospital. (3) Results: The median age at presentation was 7 years (range, 9 months to 16 years). The median follow-up period was 9.4 years (range, 3 to 25 years). Ten underwent chemotherapy and excision without additional radiotherapy. A total of 29 patients received additional local treatment: Ablative surgery MOld technique with after loading brachytherapy and surgical REconstruction (AMORE) (N = 21), proton (N = 4) or external beam radiation treatment (EBRT; N = 4). We found 14 cases with recurrences, 9 of which underwent exenteration and two of which died. The 10-year overall survival rate was 95% and the EFS was 63%. (4) Conclusions: long-term follow-up with 10-year survival rate of orbital RMS in this series was 95% achieved by local tumor control and eye preservation in 77% of our study population. Full article
(This article belongs to the Special Issue Current Progress and Research Trends in Ocular Oncology)
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9 pages, 1103 KB  
Article
Proton Beam Therapy for Advanced Periocular Skin Cancer: An Eye-Sparing Approach
by Yingying Zhang, Isabela C. S. Lima, Alessandra A. Woo, Stephen Zieminski, Judith A. Adams, Megan A. Hughes and Annie W. Chan
Cancers 2025, 17(2), 327; https://doi.org/10.3390/cancers17020327 - 20 Jan 2025
Cited by 1 | Viewed by 1598
Abstract
Background/Objectives: The management of periocular skin malignancies presents a unique challenge. Proton beam therapy, due to its sharp dose fall-off, allows for the delivery of a tumoricidal dose to the tumor while sparing adjacent normal tissues. Methods: Thirteen patients with a median age [...] Read more.
Background/Objectives: The management of periocular skin malignancies presents a unique challenge. Proton beam therapy, due to its sharp dose fall-off, allows for the delivery of a tumoricidal dose to the tumor while sparing adjacent normal tissues. Methods: Thirteen patients with a median age of 76.5 years received protons at our institution to a median dose of 66.6 Gy (RBE). Sixty-four percent of the lesions were basal cell carcinoma, and 22% were squamous cell carcinoma. Eighty-six percent of patients underwent biopsy only or partial resection. Fifty-seven percent of the lesions were located in the medial or lateral canthus. There was orbital invasion in 93% of the cases. Locoregional control probability and overall survival were estimated with the Kaplan–Meier method. Treatment toxicity was scored using the CTCAE 4.0. Results: At a median follow-up of 96 months, there was no local recurrence. The rate of orbital preservation was 100%. Functional vision was maintained in all the patients. There was no acute or late grade 3 or higher toxicity. Conclusions: Protons allow for long-term tumor control with eye preservation in patients with locally advanced periocular skin cancers. Larger prospective multi-institutional trials with standardized ophthalmological assessments are needed to confirm our findings. Full article
(This article belongs to the Special Issue Advances in Proton Pencil Beam Scanning Therapy)
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11 pages, 1785 KB  
Article
The Comprehensive Management of Patients with Rhino-Orbito-Cerebral Mucormycosis; A Perspective from Antifungal Treatment to Prosthetic Rehabilitation: A Descriptive Cohort Study
by Angélica Julián Castrejón, Rosa Marene Hernández Martínez, Diana Rivero Méndez, Israel Nayensei Gil Velázquez, Juan Heriberto Rodríguez Piña, Juan Manuel Salgado Camacho, Nicolás Teyes Calva, Sayuri I. Espíndola Chavarría, Patricia A. Meza-Meneses and Carlos Alberto Castro-Fuentes
Trop. Med. Infect. Dis. 2024, 9(7), 158; https://doi.org/10.3390/tropicalmed9070158 - 12 Jul 2024
Cited by 1 | Viewed by 1835
Abstract
Surgical intervention is a key element in the management of patients diagnosed with mucormycosis. A retrospective cohort study was carried out, in which patients with a proven diagnosis of mucormycosis were evaluated over a period of 10 years, according to the MSGERC criteria. [...] Read more.
Surgical intervention is a key element in the management of patients diagnosed with mucormycosis. A retrospective cohort study was carried out, in which patients with a proven diagnosis of mucormycosis were evaluated over a period of 10 years, according to the MSGERC criteria. A descriptive analysis of the clinical characteristics, comorbidities, imaging, and microbiology studies, as well as medical and surgical treatment and the type of prosthesis was carried out. A total of 22 cases were identified, of which 54.5% (n = 12) of the population were men. Furthermore, 77.2% (n = 17) of the population had diabetes mellitus. The main antifungal treatment implemented was liposomal amphotericin B (77.2%, n = 17). The most affected structures in our patients were the paranasal sinuses (n = 18; 81%), followed by the maxilla and orbit (n = 15; 68%), nose (n = 12; 54%), central nervous system (n = 11; 50%), and skin and soft tissues (n = 2; <1%). Of the total population, 59.09% (n = 13) of patients underwent maxillofacial surgery, of which 61.53% (n = 8) required some type of prosthetic rehabilitation. Orbital exenteration and maxillectomy were the most frequent surgeries, accounting for 69.23% (n = 9), while skull base drainage was performed in four patients (30.76%). Of the total number of patients (n = 22), eight died (36.36%). Appropriate surgical management according to the affected structures, considering not only increasing the patient’s survival, but also considering the aesthetic and functional consequences, will require subsequent rehabilitation. Full article
(This article belongs to the Special Issue Monitoring and Diagnosis of Invasive Fungal Infections)
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17 pages, 4803 KB  
Review
Advances in Microvascular Reconstruction of the Orbit and Beyond: Considerations and a Checklist for Decision-Making
by Gian Battista Bottini, Veronika Joos, Christoph Steiner, Katharina Zeman-Kuhnert and Alexander Gaggl
J. Clin. Med. 2024, 13(10), 2899; https://doi.org/10.3390/jcm13102899 - 14 May 2024
Cited by 1 | Viewed by 1534
Abstract
This paper aims to discuss the microvascular reconstruction of the orbit and adjacent regions and to propose a checklist to aid the reconstructive surgeon in this challenging undertaking. The discussion is based on a literature review that includes 32 retrospective case series, 5 [...] Read more.
This paper aims to discuss the microvascular reconstruction of the orbit and adjacent regions and to propose a checklist to aid the reconstructive surgeon in this challenging undertaking. The discussion is based on a literature review that includes 32 retrospective case series, 5 case reports published in the last 34 years in PubMed, and 3 textbook chapters. Additionally, it relies on the senior author’s expertise, described in a case series, and two case reports published elsewhere. Classifications and treatment algorithms on microvascular orbit reconstruction generally disregard patient-related factors. A more holistic approach can be advantageous: patient-related factors, such as age, comorbidities, prognosis, previous interventions, radiotherapy, and the wish for maximal dental rehabilitation and a prosthetic eye, have the same importance as defect-related considerations and can inform the choice of a reconstructive option. In this manuscript, we examine defect- and patient-related factors and new technologies, provide a checklist, and examine future directions. The checklist is intended as a tool to aid in the decision-making process when reconstructing the orbital region with microvascular flaps. Full article
(This article belongs to the Special Issue New Advances in Oral and Facial Surgery)
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9 pages, 511 KB  
Article
Risk Factors for Orbital Invasion in Malignant Eyelid Tumors, Is Orbital Exenteration Still Necessary?
by Krzysztof Gąsiorowski, Michał Gontarz, Tomasz Marecik, Paweł Szczurowski, Jakub Bargiel, Jan Zapała and Grażyna Wyszyńska-Pawelec
J. Clin. Med. 2024, 13(3), 726; https://doi.org/10.3390/jcm13030726 - 26 Jan 2024
Cited by 3 | Viewed by 2253
Abstract
Basal cell carcinoma is the most common malignant skin tumor of the eyelids in Caucasians, followed by squamous cell carcinoma and sebaceous gland carcinoma. The primary treatment for these tumors is radical excision. In cases where malignant eyelid tumors are advanced and have [...] Read more.
Basal cell carcinoma is the most common malignant skin tumor of the eyelids in Caucasians, followed by squamous cell carcinoma and sebaceous gland carcinoma. The primary treatment for these tumors is radical excision. In cases where malignant eyelid tumors are advanced and have invaded the orbit, orbital exenteration is necessary. In this retrospective study, we aimed to determine the correlation between the risk of orbital infiltration and various factors like tumor location, size, histological type, and patient age. This study revealed that tumors in multiple regions increased the risk of orbital infiltration by 3.75 times. Tumors with a diameter of 21–30 mm raised the likelihood of requiring exenteration by 15.5 times compared to smaller tumors (up to 10 mm). Age was also associated with the likelihood of orbital invasion in periocular tumors. Interestingly, no correlation was found between the histological type of the tumor and the risk of orbital infiltration. Notably, the conjunctiva of the eyeball was the most commonly infiltrated orbital structure, followed by the orbital fat. Timely treatment and well-planned procedures are crucial for patients with malignant periocular skin tumors to avoid multiple reoperations and the potential need for orbital exenteration. Full article
(This article belongs to the Section Dermatology)
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12 pages, 2725 KB  
Article
Orbital Exenteration in Recurrence Cancer: 5 Years Experience
by Giorgio Barbera, Mattia Todaro, Gianmarco Saponaro, Giulio Gasparini and Alessandro Moro
J. Clin. Med. 2023, 12(19), 6180; https://doi.org/10.3390/jcm12196180 - 25 Sep 2023
Cited by 2 | Viewed by 1538
Abstract
Introduction: The purpose of this study was to assess the overall survival (OS) and disease-free survival (DFS) of patients who underwent orbital exenteration for periorbital, conjunctival, and primary intraorbital carcinomas. Additionally, we assessed the outcomes of anterior retrograde temporalis muscle flap restoration. Methods: [...] Read more.
Introduction: The purpose of this study was to assess the overall survival (OS) and disease-free survival (DFS) of patients who underwent orbital exenteration for periorbital, conjunctival, and primary intraorbital carcinomas. Additionally, we assessed the outcomes of anterior retrograde temporalis muscle flap restoration. Methods: For all patients who had orbital exenteration in the previous five years, a non-comparative retrospective assessment of their medical records, histology, and radiographic imaging was carried out. We investigated the relationships between the various qualitative factors using Cramer’s V Kaplan–Meier (KM) analysis. For each of the patient’s categorical factors that were of relevance, estimates of the survival distribution were displayed, and log-rank tests were used to determine whether the survival distributions were equal. Results: This study looks at 19 participants. The sample is made up of 13 men (68%) and 6 women (32%). The degree of relationship (Cramer’s V index) between lymph node metastases (N) and the existence of distant metastases (M) is high, at 64%, and is statistically significant because the p-value is 0.0034 < 0.005. Lymph node metastases had a statistically significant impact on overall survival (p = 0.04 < 0.05). Thirteen of the nineteen patients tested had no palsy (68%). There was no one presenting a CSF leak. Conclusion: Our findings show how crucial it is to identify any lymph node involvement that orbital neoplasms may have. In patients who have received many treatments, sentinel lymph node biopsy (SLNB) may be used to determine the stage and spread of the cancer. To determine whether additional tumor characteristics may be explored, more expertise in the SLNB field for patients with orbital cancer who have received many treatments may be helpful. To prevent additional scarring and to be comparable to previous techniques for facial nerve lesions, the anterior retrograde approach and the transorbital procedure for temporal muscle flap in-setting are both effective methods. Full article
(This article belongs to the Special Issue Oral and Maxillofacial Surgery in 2023 and Beyond)
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16 pages, 2794 KB  
Systematic Review
Orbital Exenteration for Craniofacial Lesions: A Systematic Review and Meta-Analysis of Patient Characteristics and Survival Outcomes
by Jumanah Qedair, Ali S. Haider, Kishore Balasubramanian, Paolo Palmisciano, Taimur Hassan, Ataollah Shahbandi, Mohammadmahdi Sabahi, Abdurrahman F. Kharbat, Hussam Abou-Al-Shaar, Kenny Yu, Aaron A. Cohen-Gadol, Tarek Y. El Ahmadieh and Othman Bin-Alamer
Cancers 2023, 15(17), 4285; https://doi.org/10.3390/cancers15174285 - 27 Aug 2023
Cited by 4 | Viewed by 2124
Abstract
Background: The outcomes of orbital exenteration (OE) in patients with craniofacial lesions (CFLs) remain unclear. The present review summarizes the available literature on the clinical outcomes of OE, including surgical outcomes and overall survival (OS). Methods: Relevant articles were retrieved from Medline, Scopus, [...] Read more.
Background: The outcomes of orbital exenteration (OE) in patients with craniofacial lesions (CFLs) remain unclear. The present review summarizes the available literature on the clinical outcomes of OE, including surgical outcomes and overall survival (OS). Methods: Relevant articles were retrieved from Medline, Scopus, and Cochrane according to PRISMA guidelines. A systematic review and meta-analysis were conducted on the clinical characteristics, management, and outcomes. Results: A total of 33 articles containing 957 patients who underwent OE for CFLs were included (weighted mean age: 64.3 years [95% CI: 59.9–68.7]; 58.3% were male). The most common lesion was squamous cell carcinoma (31.8%), and the most common symptom was disturbed vision/reduced visual acuity (22.5%). Of the patients, 302 (31.6%) had total OE, 248 (26.0%) had extended OE, and 87 (9.0%) had subtotal OE. Free flaps (33.3%), endosseous implants (22.8%), and split-thickness skin grafts (17.2%) were the most used reconstructive methods. Sino-orbital or sino-nasal fistula (22.6%), flap or graft failure (16.9%), and hyperostosis (13%) were the most reported complications. Regarding tumor recurrences, 38.6% were local, 32.3% were distant, and 6.7% were regional. The perineural invasion rate was 17.4%, while the lymphovascular invasion rate was 5.0%. Over a weighted mean follow-up period of 23.6 months (95% CI: 13.8–33.4), a weighted overall mortality rate of 39% (95% CI: 28–50%) was observed. The 5-year OS rate was 50% (median: 61 months [95% CI: 46–83]). The OS multivariable analysis did not show any significant findings. Conclusions: Although OE is a disfiguring procedure with devastating outcomes, it is a viable option for carefully selected patients with advanced CFLs. A patient-tailored approach based on tumor pathology, extension, and overall patient condition is warranted. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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11 pages, 261 KB  
Review
Induction Chemotherapy for Locoregionally Advanced Sinonasal Squamous Cell Carcinoma and Sinonasal Undifferentiated Carcinoma: A Comprehensive Review
by Katie L. Melder and Mathew Geltzeiler
Cancers 2023, 15(15), 3798; https://doi.org/10.3390/cancers15153798 - 26 Jul 2023
Cited by 9 | Viewed by 3211
Abstract
Sinonasal squamous cell carcinoma (SNSCC) and sinonasal undifferentiated carcinoma (SNUC) are two of the most common, high-grade malignancies of the sinonasal cavity. The standard of care for resectable lesions per the National Comprehensive Cancer Network (NCCN) guidelines includes surgical resection with negative margins [...] Read more.
Sinonasal squamous cell carcinoma (SNSCC) and sinonasal undifferentiated carcinoma (SNUC) are two of the most common, high-grade malignancies of the sinonasal cavity. The standard of care for resectable lesions per the National Comprehensive Cancer Network (NCCN) guidelines includes surgical resection with negative margins plus adjuvant radiation therapy. However, surgery for locally advanced disease with both orbital and intracranial involvement is associated with significant morbidity and poor overall survival. Over the last decade, induction chemotherapy (IC) has emerged as part of a multimodal treatment strategy to optimize locoregional disease control and minimize substantial surgical morbidity such as orbital exenteration without compromising rates of overall survival. The response to IC both guides additional therapy and helps prognosticate a patient’s disease. This narrative review examines the data surrounding the management of patients with SNSCC and SNUC. The pros and cons of upfront surgical management plus adjuvant therapy will be explored, and the case for IC will be presented. The IC-specific regimens and treatment paradigms for SNSCC and SNUC will each be explored in detail. Organ preservation, treatment morbidity, and survival data will be presented, and evidence-based recommendations will be presented for the management of these patients. Full article
(This article belongs to the Special Issue Skull Base Tumors)
20 pages, 11285 KB  
Article
Spectral Domain Optical Coherence Tomography Findings in Vision-Threatening Rhino-Orbital Cerebral Mucor Mycosis—A Prospective Analysis
by Ankur Singh, Preeti Diwaker, Akanksha Agrawal, Aniruddha Agarwal, Jolly Rohatgi, Ramandeep Singh, Gopal Krushna Das, Pramod Kumar Sahoo and Vinod Kumar Arora
Diagnostics 2022, 12(12), 3098; https://doi.org/10.3390/diagnostics12123098 - 8 Dec 2022
Cited by 1 | Viewed by 2025
Abstract
Rhino-orbital cerebral mucor mycosis is a rare disease entity, where retinal involvement is described in the literature mostly as CRAO. However, pathological studies have shown mucor invading the choroid and retina with a neutrophilic reaction. So, it is pertinent that retinal inflammation secondary [...] Read more.
Rhino-orbital cerebral mucor mycosis is a rare disease entity, where retinal involvement is described in the literature mostly as CRAO. However, pathological studies have shown mucor invading the choroid and retina with a neutrophilic reaction. So, it is pertinent that retinal inflammation secondary to invading mucor has some role in microstructural changes seen in the vitreous and retina of these patients. This novel study aims to describe the vitreal and retinal features of patients with vision-threatening rhino-orbital cerebral mucor mycosis and how they evolve on spectral domain optical coherence tomography (SD-OCT). This study shall also provide insight into the pathophysiology of these vitreoretinal manifestations by in vitro analysis of the exenterated orbital content. Fifteen eyes of fifteen patients with vision-threatening ROCM treated with standard care were enrolled in this study and underwent complete ophthalmic examination, serial colour fundus photography, and SD-OCT for both qualitative and quantitative analysis, at baseline and follow-up visits. SD-OCT on serial follow-up revealed thickening and increased inner-retinal reflectivity at presentation followed by thinning of both, other features such as the loss of the inner-retinal organized layer structure, external limiting membrane (ELM) disruption, necrotic spaces in the outer retina, and hyperreflective foci. Vitreous cells with vitreous haze were also seen. There was a significant reduction in CMT, inner and outer retinal thickness, total retinal thickness (all p < 0.05) with time, the quantum of reduction concentrated primarily to the inner retina. In summary, in vivo and in vitro analysis revealed that early microstructural changes were primarily a result of retinal infarctions secondary to thrombotic angioinvasion. With the late microstructural changes, there was possible sequelae of retinal infarction with some contribution from the inflammation, resulting from mucor invading the choroid and retina. Full article
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18 pages, 3069 KB  
Systematic Review
Orbital Metastases: A Systematic Review of Clinical Characteristics, Management Strategies, and Treatment Outcomes
by Paolo Palmisciano, Gianluca Ferini, Christian Ogasawara, Waseem Wahood, Othman Bin Alamer, Aditya D. Gupta, Gianluca Scalia, Alexandra M. G. Larsen, Kenny Yu, Giuseppe E. Umana, Aaron A. Cohen-Gadol, Tarek Y. El Ahmadieh and Ali S. Haider
Cancers 2022, 14(1), 94; https://doi.org/10.3390/cancers14010094 - 24 Dec 2021
Cited by 42 | Viewed by 7019
Abstract
Background: Orbital metastases often lead to severe functional impairment. The role of resection, orbital exenteration, and complementary treatments is still debated. We systematically reviewed the literature on orbital metastases. Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched upon PRISMA guidelines to identify studies [...] Read more.
Background: Orbital metastases often lead to severe functional impairment. The role of resection, orbital exenteration, and complementary treatments is still debated. We systematically reviewed the literature on orbital metastases. Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched upon PRISMA guidelines to identify studies on orbital metastases. Clinical characteristics, management strategies, and survival were analyzed. Results: We included 262 studies comprising 873 patients. Median age was 59 years. The most frequent primary tumors were breast (36.3%), melanoma (10.1%), and prostate (8.5%) cancers, with median time interval of 12 months (range, 0–420). The most common symptoms were proptosis (52.3%) and relative-afferent-pupillary-defect (38.7%). Most metastases showed a diffuse location within the orbit (19%), with preferential infiltration of orbital soft tissues (40.2%). In 47 cases (5.4%), tumors extended intracranially. Incisional biopsy (63.7%) was preferred over fine-needle aspiration (10.2%), with partial resection (16.6%) preferred over complete (9.5%). Orbital exenteration was pursued in 26 patients (3%). A total of 305 patients (39.4%) received chemotherapy, and 506 (58%) received orbital radiotherapy. Post-treatment symptom improvement was significantly superior after resection (p = 0.005) and orbital radiotherapy (p = 0.032). Mean follow-up was 14.3 months, and median overall survival was 6 months. Fifteen cases (1.7%) demonstrated recurrence with median local control of six months. Overall survival was statistically increased in patients with breast cancer (p < 0.001) and in patients undergoing resection (p = 0.024) but was not correlated with orbital location (p = 0.174), intracranial extension (p = 0.073), biopsy approach (p = 0.344), extent-of-resection (p = 0.429), or orbital exenteration (p = 0.153). Conclusions: Orbital metastases severely impair patient quality of life. Surgical resection safely provides symptom and survival benefit compared to biopsy, while orbital radiotherapy significantly improves symptoms compared to not receiving radiotherapy. Full article
(This article belongs to the Special Issue New Perspectives of Ocular Oncology)
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26 pages, 3347 KB  
Review
Small but Challenging Conjunctival Melanoma: New Insights, Paradigms and Future Perspectives
by Sacha Nahon-Estève, Corine Bertolotto, Alexandra Picard-Gauci, Lauris Gastaud, Stéphanie Baillif, Paul Hofman, Anaïs Groulier, Célia Maschi, Jean-Pierre Caujolle, Sandra Lassalle and Arnaud Martel
Cancers 2021, 13(22), 5691; https://doi.org/10.3390/cancers13225691 - 14 Nov 2021
Cited by 17 | Viewed by 6470
Abstract
Although its incidence has increased over the last decades, conjunctival melanoma (CM) remains a rare but challenging periocular malignancy. While there is currently no recognized standard of care, “no-touch” surgical excision followed by adjuvant treatments is usually recommended. Despite its small size, managing [...] Read more.
Although its incidence has increased over the last decades, conjunctival melanoma (CM) remains a rare but challenging periocular malignancy. While there is currently no recognized standard of care, “no-touch” surgical excision followed by adjuvant treatments is usually recommended. Despite its small size, managing CM is challenging for clinicians. The first challenge is the high risk of tumour local recurrence that occurs in about one third of the patients. The management of locally advanced CM (≥T2) or multiple recurrences may require mutilating surgeries such as orbital exenteration (OE). The second challenge is the metastatic spread of CM that occurs in about one quarter of patients, regardless of whether complete surgical excision is performed or not. This highlights the infiltrative and highly aggressive behaviour of CM. Recently, attention has been directed towards the use of eye-sparing strategies to avoid OE. Initially, wide conservative surgeries followed by customized brachytherapy or radiotherapy have appeared as viable strategies. Nowadays, new biological insights into CM have revealed similarities with cutaneous melanoma. These new findings have allowed clinicians to reconsider the management of locally advanced CM with “medical” eye-sparing treatment as well as the management of metastatic spread. The aim of this review was to summarize the current and future perspectives of treatment for CM based on recent biological findings. Full article
(This article belongs to the Special Issue Metastatic Progression of Human Melanoma)
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13 pages, 4249 KB  
Review
New Targeted Therapies and Immunotherapies for Locally Advanced Periocular Malignant Tumours: Towards a New ‘Eye-Sparing’ Paradigm?
by Arnaud Martel, Sandra Lassalle, Alexandra Picard-Gauci, Lauris Gastaud, Henri Montaudie, Corine Bertolotto, Sacha Nahon-Esteve, Gilles Poissonnet, Paul Hofman and Stephanie Baillif
Cancers 2021, 13(11), 2822; https://doi.org/10.3390/cancers13112822 - 5 Jun 2021
Cited by 13 | Viewed by 4042
Abstract
The management of periocular skin malignant tumours is challenging. Surgery remains the mainstay of treatment for localised eyelid cancers. For more locally advanced cancers, especially those invading the orbit, orbital exenteration has long been considered the gold standard; however, it is a highly [...] Read more.
The management of periocular skin malignant tumours is challenging. Surgery remains the mainstay of treatment for localised eyelid cancers. For more locally advanced cancers, especially those invading the orbit, orbital exenteration has long been considered the gold standard; however, it is a highly disfiguring and traumatic surgery. The last two decades have been marked by the emergence of a new paradigm shift towards the use of ‘eye-sparing’ strategies. In the early 2000s, the first step consisted of performing wide conservative eyelid and orbital excisions. Multiple flaps and grafts were needed, as well as adjuvant radiotherapy in selected cases. Although being incredibly attractive, several limitations such as the inability to treat the more posteriorly located orbital lesions, as well as unbearable diplopia, eye pain and even secondary eye loss were identified. Therefore, surgeons should distinguish ‘eye-sparing’ from ‘sight-sparing’ strategies. The second step emerged over the last decade and was based on the development of targeted therapies and immunotherapies. Their advantages include their potential ability to treat almost all tumours, regardless of their locations, without performing complex surgeries. However, several limitations have been reported, including their side effects, the appearance of primary or secondary resistances, their price and the lack of consensus on treatment regimen and exact duration. The aim of this article was to review the evolution of the management of locally advanced periocular malignant tumours over the last three decades and highlight the new paradigm shift towards the use of ‘eye-sparing’ strategies. Full article
(This article belongs to the Special Issue Molecular Mechanisms of Skin Cancer)
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10 pages, 1631 KB  
Case Report
Primary Signet Ring Cell/Histiocytoid Carcinoma of the Eyelid: Somatic Mutations in CDH1 and Other Clinically Actionable Mutations Imply Early Use of Targeted Agents
by Lei-Chi Wang, Tai-Chi Lin, Yi-Chen Yeh, Hsiang-Ling Ho, Chieh-Chih Tsai and Teh-Ying Chou
Curr. Oncol. 2021, 28(1), 918-927; https://doi.org/10.3390/curroncol28010090 - 16 Feb 2021
Cited by 5 | Viewed by 3843
Abstract
Primary signet ring cell/histiocytoid carcinoma of the eyelid is a rare ocular malignancy and its diagnosis is often delayed. This neoplasm presents as an insidious, diffusely infiltrative mass in the periocular area that later infiltrates the orbit. An exenteration is usually indicated; however, [...] Read more.
Primary signet ring cell/histiocytoid carcinoma of the eyelid is a rare ocular malignancy and its diagnosis is often delayed. This neoplasm presents as an insidious, diffusely infiltrative mass in the periocular area that later infiltrates the orbit. An exenteration is usually indicated; however, nearly one-third of patients develop local recurrence or metastasis. Morphologically, it resembles signet ring cell carcinoma of the stomach and breast, raising the possibility of mutations in CDH1, the gene encoding E-cadherin. To determine whether primary signet ring cell/histiocytoid carcinoma harbors the CDH1 mutation or other actionable mutations, we analyzed the tumor tissue via next-generation sequencing. We identified only one case of primary signet ring cell carcinoma of the eyelid with adequate DNA quality for sequencing from the pathological archive during the period 2000 to 2020. A comprehensive evaluation including histopathology, immunohistochemistry, and next-generation sequencing assay was performed on tumor tissue. Immunohistochemically, the tumor exhibited E-cadherin membranous staining with the aberrant cytoplasmic staining of β-catenin. Using next-generation sequencing, we demonstrated the mutation in the CDH1 gene. In addition, other clinically actionable mutations including ERBB2 and PIK3CA were also detected. The alterations in other actionable genes indicate a need for larger studies to evaluate the pathogenesis and potential therapies for primary signet ring cell/histiocytoid carcinoma of the eyelid. Full article
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