Novel Insights in Ocular and Orbital Oncology: From Molecular Biology to Treatment Strategies

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Molecular Cancer Biology".

Deadline for manuscript submissions: 30 November 2024 | Viewed by 6599

Special Issue Editors


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Guest Editor
Department of Medical Surgical Sciences and Advanced Technologies “G.F. Ingrassia” – Radiology I Unit, University Hospital Policlinico “G. Rodolico-San Marco", Catania, Italy
Interests: magnetic resonance imaging; diffusion-weighted MR imaging; quantitative MRI; uveal melanoma; head and neck imaging; ocular oncology; ocular imaging; women's imaging; gynecologic oncology; gastrointestinal radiology

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Guest Editor
Department of Medical, Surgical and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, Italy
Interests: immunohistochemistry; dermatopathology; neuropathology; uveal melanoma; head and neck pathology
Special Issues, Collections and Topics in MDPI journals

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Guest Editor
Department of Medical Surgical Sciences and Advanced Technologies “G.F. Ingrassia” – U.O. Radioterapia Oncologica, A.O.U. Policlinico “G. Rodolico—San Marco” Catania, Via Santa Sofia 78, 95123 Catania, Italy
Interests: ocular oncology; head and neck cancer; uveal melanoma; radiation therapy; proton therapy; integrated radio-chemotherapy
Special Issues, Collections and Topics in MDPI journals

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Guest Editor
Department of Ophthalmology, University of Catania, 95100 Catania, Italy
Interests: ocular oncology; uveal melanoma; vitreoretinal surgery; cataract surgery; medical retina
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Special Issue Information

Dear Colleagues,

Ocular and orbital tumors are rare pathologic entities that can originate from all structures located in the orbital region (eye, orbit and eyelids) and encompass a wide spectrum of diseases ranging from benign tumors to primary and secondary malignancies.

Ocular oncology is a subspeciality of ophthalmology, dedicated to the research, diagnosis and treatment of neoplastic lesions affecting the eye and its adnexa, including the orbit. In recent years, progress in the identification of new intraocular biomarkers of eye neoplasia, new diagnostic imaging technologies and some new insights into treatment modalities have contributed to the advancement of this subspeciality.

Both the diagnosis and treatment of ocular tumors represent a multidisciplinary team effort involving practitioners of different medical specialties. Along with currently available ophthalmological and radiologic imaging techniques, promising novel techniques have recently been developed.

Imaging plays an important role in lesion detection, characterization and staging and avails of the application of many diagnostic methods. High-resolution computed tomography (CT) is irreplaceable to assess the bony anatomy. Due to its excellent soft tissue contrast and intrinsic multiparametric capabilities, magnetic resonance imaging (MRI) makes a pivotal contribution to narrowing down differential diagnoses and provides crucial information for treatment planning. Furthermore, functional MRI techniques plat a role in predicting and detecting therapeutic outcomes.

New prognostic and genetic features were recently identified in orbital tumors and must be reported in the pathology report.

Radiotherapy plays a pivotal role in the management of uveal melanoma, the most common primary intraocular tumor in adults, and is widely employed in other less frequent primitive or secondary tumors of the orbital region. Modern advanced radiotherapy treatments, such as proton therapy, stereotactic ablative or radiosurgery techniques and interventional brachytherapy, are frequently applied in order to achieve local disease control. The prevention and treatment of damage related to radiotherapy, in particular, radiation retinopathy, deserves special attention, mostly because personalized therapy may contribute to vision salvage. Intraocular tumors in patients of pediatric age have also received special attention from the genetic and pathophysiologic perspectives.

In the field of surgery, endoscopic transnasal approaches to the orbit may represent a safe alternative option to traditional external craniofacial approaches in the treatment of intraconal orbital tumors.

Recently, interventional radiology has gained importance in the clinical management of patients with uveal melanomas through minimally invasive approaches, which have partially replaced open surgical techniques in the treatment of liver metastases.

For the abovementioned reasons, ocular oncology warrants attention from all medical specialties involved in the diagnosis and treatment of ocular and orbital tumors. This Special Issue is devoted to reporting on new aspects of this subspeciality, from any perspective.

Dr. Pietro Valerio Foti
Dr. Rosario Caltabiano
Dr. Corrado Spatola
Dr. Andrea Russo
Guest Editors

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Keywords

  • ocular oncology
  • orbital tumors
  • molecular biology
  • therapy
  • surgery

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Published Papers (4 papers)

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Research

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12 pages, 1148 KiB  
Article
Ocular and Periocular Metastasis in Breast Cancer: Clinical Characteristics, Prognostic Factors and Treatment Outcome
by Yacoub A. Yousef, Mona Mohammad, Hanan Khalil, Tala Khouri, Rand Alsweiti, Jakub Khzouz, Dima Abu Laban, Imad Jaradat, Ahmad Kh. Ibrahimi, Akram Al-Ibraheem, Mahmoud Al Masri, Ibrahim AlNawiaseh and Hikmat Abdel-Razeq
Cancers 2024, 16(8), 1518; https://doi.org/10.3390/cancers16081518 - 16 Apr 2024
Viewed by 1561
Abstract
Background: Breast cancer remains a leading cause of cancer-related mortality and morbidity worldwide. Ocular and periocular metastasis present as a rare but clinically significant manifestation. This study aims to explore demographics and clinical aspects of ocular and periocular metastasis in breast cancer patients. [...] Read more.
Background: Breast cancer remains a leading cause of cancer-related mortality and morbidity worldwide. Ocular and periocular metastasis present as a rare but clinically significant manifestation. This study aims to explore demographics and clinical aspects of ocular and periocular metastasis in breast cancer patients. Methods: A retrospective cohort study comprising 45 breast cancer patients with ocular or periocular metastasis treated between 2013 and 2023. Patient demographics, tumor characteristics, diagnostic methods, treatment modalities, visual outcomes, and survival data were analyzed. Results: Among 9902 breast cancer patients, 0.5% developed ocular or periocular metastasis, constituting 2.4% of metastatic cases. The median age was 50 years. Ocular metastasis timing varied: 5% before breast cancer, 24% concurrent, 22% within a year, and 49% after. The most common presentations included incidental MRI findings (42%) and vision decline (31%). Metastasis involved the orbit (47%), choroid (40%), optic nerve (11%), and iris (2%), with 44% having bilateral involvement. Predictive factors included invasive lobular carcinoma (ILC) (p < 0.0001) and brain metastasis (p < 0.0001), with ILC exhibiting a sixfold higher likelihood of ocular metastasis than invasive ductal carcinoma (IDC). Primary treatment was radiation therapy (89%), yielding a 55% maintenance of excellent vision (<0.5), with 93% developing dry eye disease. Patients with ocular metastasis faced an increased risk of disease-related mortality (p < 0.0001), with 71% succumbing within 10 months post-diagnosis. Conclusions: Ocular metastasis in breast cancer is rare (0.5%) but signifies poor outcome. It is linked to ILC and concurrent brain metastasis. Primary treatment involves radiation therapy, with a favorable visual prognosis. Full article
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18 pages, 13108 KiB  
Article
Quantitative Diffusion-Weighted MR Imaging: Is There a Prognostic Role in Noninvasively Predicting the Histopathologic Type of Uveal Melanomas?
by Pietro Valerio Foti, Corrado Inì, Giuseppe Broggi, Renato Farina, Stefano Palmucci, Corrado Spatola, Maria Chiara Lo Greco, Emanuele David, Rosario Caltabiano, Lidia Puzzo, Andrea Russo, Antonio Longo, Teresio Avitabile and Antonio Basile
Cancers 2023, 15(23), 5627; https://doi.org/10.3390/cancers15235627 - 29 Nov 2023
Cited by 2 | Viewed by 1013
Abstract
Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic [...] Read more.
Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic type of UMs in order to verify the role of diffusion-weighted magnetic resonance imaging (DWI) as a noninvasive prognostic marker. A total of 26 patients with UMs who had undergone MRI and subsequent primary enucleation were retrospectively selected. The ADC of the tumor was compared with the histologic type. The data were compared using both one-way analysis of variance (ANOVA) (assessing the three histologic types separately) and the independent t-test (dichotomizing histologic subtypes as epithelioid versus non-epithelioid). Histologic type was present as follows: the epithelioid cell was n = 4, and the spindle cell was n = 11, the mixed cell type was n = 11. The mean ADC was 1.06 ± 0.24 × 10−3 mm2/s in the epithelioid cells, 0.98 ± 0.19 × 10−3 mm2/s in the spindle cells and 0.96 ± 0.26 × 10−3 mm2/s in the mixed cell type. No significant difference in the mean ADC value of the histopathologic subtypes was found, either when assessing the three histologic types separately (p = 0.76) or after dichotomizing the histologic subtypes as epithelioid and non-epithelioid (p = 0.82). DWI-ADC is not accurate enough to distinguish histologic types of UMs. Full article
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14 pages, 1344 KiB  
Article
Long-Term Follow-Up of Patients with Conjunctival Lymphoma after Individualized Lens-Sparing Electron Radiotherapy: Results from a Longitudinal Study
by Christian Hoffmann, Toke Ringbaek, Anja Eckstein, Wolfgang Deya, Alina Santiago, Martin Heintz, Wolfgang Lübcke, Frank Indenkämpen, Wolfgang Sauerwein, Andrea Flühs, Claudia Le Guin, Andreas Huettmann, Julia von Tresckow, Sophia Göricke, Cornelius Deuschl, Sourour Moliavi, Christoph Poettgen, Thomas Gauler, Nika Guberina, Patricia Johansson, Nikolaos Bechrakis, Martin Stuschke and Maja Guberinaadd Show full author list remove Hide full author list
Cancers 2023, 15(22), 5433; https://doi.org/10.3390/cancers15225433 - 15 Nov 2023
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Abstract
Irradiation with electrons is the primary treatment regime for localized conjunctival low-grade lymphomas. However, radiation-induced cataracts are a major cause of treatment-related morbidity. This study investigates whether lens-sparing electron irradiation produces sufficient disease control rates while preventing cataract formation. All consecutive patients with [...] Read more.
Irradiation with electrons is the primary treatment regime for localized conjunctival low-grade lymphomas. However, radiation-induced cataracts are a major cause of treatment-related morbidity. This study investigates whether lens-sparing electron irradiation produces sufficient disease control rates while preventing cataract formation. All consecutive patients with strictly conjunctival, low-grade Ann Arbor stage IE lymphoma treated with superficial electron irradiation between 1999 and 2021 at our department were reviewed. A total of 56 patients with 65 treated eyes were enrolled with a median follow-up of 65 months. The median dose was 30.96 Gy. A lens-spearing technique featuring a hanging rod blocking the central beam axis was used in 89.2% of all cases. Cumulative incidences of 5- and 10-year infield recurrences were 4.3% and 14.6%, incidences of 5- and 10-year outfield progression were 10.4% and 13.4%. We used patients with involvement of retroorbital structures treated with whole-orbit photon irradiation without lens protection—of which we reported in a previous study—as a control group. The cumulative cataract incidence for patients treated with electrons and lens protection was significantly lower (p = 0.005) when compared to patients irradiated without lens protection. Thus, electrons are an effective treatment option for conjunctival low-grade lymphomas. The presented lens-sparing technique effectively prevents cataract formation. Full article
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Review

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15 pages, 497 KiB  
Review
Radiotherapy of Orbital and Ocular Adnexa Lymphoma: Literature Review and University of Catania Experience
by Madalina La Rocca, Barbara Francesca Leonardi, Maria Chiara Lo Greco, Giorgia Marano, Irene Finocchiaro, Arianna Iudica, Roberto Milazzotto, Rocco Luca Emanuele Liardo, Viviana Anna La Monaca, Vincenzo Salamone, Antonio Basile, Pietro Valerio Foti, Stefano Palmucci, Emanuele David, Silvana Parisi, Antonio Pontoriero, Stefano Pergolizzi and Corrado Spatola
Cancers 2023, 15(24), 5782; https://doi.org/10.3390/cancers15245782 - 10 Dec 2023
Cited by 3 | Viewed by 1874
Abstract
Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients [...] Read more.
Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients with confirmed histological diagnosis of lymphoma stage IE-IIE, treated between 2010 and 2021 with radiotherapy alone or in association with chemotherapy or immunotherapy. Eight patients were treated with primary radiotherapy only, while three received previous systemic treatments. Six patients were treated with Proton beam therapy (PBT), and five with external beam radiotherapy (EBRT). The five-year local control rate was 98%; only one patient developed an out-of-field recurrence. We also conducted a comprehensive literature review using electronic databases (PubMed, EMBASE, and Cochrane Library). Articles were selected based on their pertinence to treatment of the ocular and adnexal lymphoma focusing on radiotherapy techniques (electron beam radiotherapy, photon beam radiotherapy, or proton beam radiotherapy), treatment total dose, fractionation schedule, early and late radio-induced toxicities, and patient’s clinical outcome. Radiotherapy is an effective treatment option for orbital lymphoma, especially as standard treatment in the early stage of orbital lymphoma, with excellent local control rate and low rates of toxicity. Full article
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