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The goal of radiotherapy in the treatment of eyelid and ocular surface tumors is to eradicate tumor burden in a manner that maintains visual function and preserve surrounding sensitive ocular tissue. Interventional radiotherapy (IRT-brachytherapy) is a radiotherapy technique associated with a highly focal dose distribution, with the advantage of boosting limited size target volumes to very high dose while sparing normal tissue. The reduction in the ocular and adnexal complications that result from this form of therapy, has led in recent years, to an increase in the use of IRT for the treatment of eyelid and ocular surface tumors. For eyelid malignancies, IRT is used as an independent treatment in small eyelids tumors, in postoperative treatment of high-risk patients and as well as salvage therapy in local recurrences. In the treatment of conjunctival malignancies, due to the high risk of local recurrence, the use of adjuvant therapies as IRT has shown to improve outcomes. In this review, we focus on eyelid and ocular surface IRT techniques and provide an overview of indication, outcomes and toxicity of IRT for the treatment of naïve and recurrent eyelid and conjunctival tumors.
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Braquiterapia , Carcinoma Basocelular , Neoplasias Palpebrais , Carcinoma Basocelular/patologia , Carcinoma Basocelular/radioterapia , Neoplasias Palpebrais/patologia , Neoplasias Palpebrais/radioterapia , Pálpebras/patologia , Humanos , RecidivaRESUMO
PURPOSE: The complexity of multimodal approaches in cancer management has lately led to the establishment of multidisciplinary tumor boards (MDTBs) to define targeted, patient-centered treatment strategies. However, few data are available regarding the application of this approach in Ocular Oncology. Hereby, the Authors analyze the implementation and outcomes of a trained MDTB in a tertiary ocular oncology referral center. METHODS: A retrospective descriptive analysis of MDTB meetings discussing patients with ocular and periocular cancers, over a 12-months period, was carried out. Data were grouped by main site involved, topics discussed and final clinical decisions therefore taken. Meetings were held by a constant 'Core team' or - when required - by a broader 'Extended team'. RESULTS: During the observational period 86 cases were discussed. In 27 patients ocular surface tissues were involved (31%), in 25 patients orbital tissues (29%), in 22 patients eyelids (26%), and in 12 patients intraocular tissues (14%). In 13 cases (15%) naïve or referred new patients, in 34 cases (40%) imaging or histopathologic reports and in 39 cases (45%) treatment plans were discussed. Regarding final decisions, a treatment plan was scheduled in 47 cases (55%) and a diagnostic ascertainment was required in 27 patients (31%); locally advanced and/or systemic diseases were referred or teamed up with other specialists in 12 cases (14%). CONCLUSIONS: Ocular Oncology multidisciplinary team, by sharing expertise of different specialists, ensures a comprehensive evaluation of patients improving the accuracy of diagnosis and staging upon which planning a proper treatment. Further studies are needed to assess if this approach may also improve the outcomes and prognosis of patients.
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Neoplasias , Equipe de Assistência ao Paciente , Humanos , Centros de Atenção Terciária , Estudos Retrospectivos , Neoplasias/terapia , OncologiaRESUMO
PURPOSE: To analyze the clinical characteristics and long-term follow-up of patients with advanced ocular surface squamous cell carcinoma (OSSC) involving periocular tissues and/or orbit. Primary outcomes were overall survival (OS), disease-free survival (DFS), and overall recurrence rate (RR). Secondary outcomes were a correlation between primary outcomes and tumor location, American Joint Committee on Cancer Classification (AJCC) staging system, histological results, surgical margins, and type of treatment. STUDY DESIGN: a retrospective case series. METHODS: The medical records of patients affected by OSSC involving periocular tissues and/or orbit referring, from 01/2011 to 01/2020, to our tertiary referral center were reviewed. RESULTS: Thirty-six eyes of 36 patients were included. The mean age was 68.2 years; 18 (50%) patients were males. The mean follow-up was 40 months. The RR was 64%. The OS at 12, 24, 36, and 60 months was respectively 97.1%, 92.7%, 92.7%, and 92.7%. The DFS at 12, 24, 36, and 60 months was respectively 62.9%, 50.8%, 41.6%, and 29.7%. Multicentric disease (p = 0.0039), inferior tarsus localization (p = 0.0428), histological diagnosis of high-risk SSCs (p = 0.0264), positive surgical margins (p = 0.0434), and excisional biopsy (EB) alone (p = 0.0005) were associated with an increased risk of recurrence. A shorter OS was observed in patients who underwent EB alone (p = 0.0049). CONCLUSION: OSCC involving periocular tissues and/or orbit is an aggressive disease with a high recurrence rate. Multicentric disease, positive surgical margins, inferior tarsus localization, and surgery without adjuvant therapies are strong predictors of recurrence and are the main factors affecting prognosis.
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Carcinoma de Células Escamosas , Recidiva Local de Neoplasia , Idoso , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/cirurgia , Seguimentos , Humanos , Masculino , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Estudos RetrospectivosRESUMO
BACKGROUND: No standard treatment has been defined for metastatic uveal melanoma (mUM). Although clinical trials testing Nivolumab/Pembrolizumab for cutaneous melanoma did not include mUM, anti PD-1 agents are commonly used for this disease. PATIENTS AND METHODS: In this prospective observational cohort single arm study, we investigated efficacy and safety of Pembrolizumab as first-line therapy for mUM. The efficacy was evaluated in terms of progression-free survival (PFS), response rate and overall survival (OS). Toxicity was also assessed. RESULTS: Seventeen patients were enrolled. A median of 8 cycles were administered (range 2-28). Two patients achieved partial response (11.7%), 6 a disease stabilization (35.3%), whereas 9 (53%) had a progression. No complete response was observed. PFS of the overall population was 3.8 months. PFS was 9.7 months for patients with an interval higher than 5 years from diagnosis of primary tumor to metastatic disease and 2.6 months for patients with an interval lower than 5 years [p = 0.039, HR 0.2865 (95% CI 0.0869-0.9443)]. Median OS was not reached. The two responding patients were still on treatment with Pembrolizumab at the time of data analysis. Survival was 12.8 months for patients with clinical benefit, while OS for progressive patients was 3.1 months. PD-L1 expression and genomic abnormalities predictive of relapse after diagnosis of primary tumor were not associated with PFS. Toxicity was mild, without grade 3-4 side effects. CONCLUSIONS: The efficacy of Pembrolizumab does not seem particularly different when compared to other agents for mUM, but responding patients had a remarkable disease control.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Melanoma/tratamento farmacológico , Neoplasias Uveais/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Melanoma/imunologia , Melanoma/mortalidade , Pessoa de Meia-Idade , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Receptor de Morte Celular Programada 1/imunologia , Intervalo Livre de Progressão , Estudos Prospectivos , Neoplasias Uveais/imunologia , Neoplasias Uveais/mortalidadeRESUMO
Conjunctival melanoma (CjM) is a rare, primary cancer of the ocular region. Genetic and epigenetic characteristics of conjunctival melanoma have not been completely elucidated yet. Conjunctival melanoma presents similarities with cutaneous melanoma, with substantial differences in the biological behavior. We reviewed the genetic and epigenetic insights of CjM involved in invasion and metastatic spread. CjM is commonly characterized by mutations of v-raf murine sarcoma viral oncogene homolog B1 (BRAF), neurofibromin 1 (NF1) and telomerase reverse transcriptase (TERT), high expression of mammalian target of rapamycin (mTOR) and heat shock protein 90 (HSP90), frequent phosphatase and tensin homolog (PTEN) loss and upregulation of specific miRNAs. These features should identify CjM as a distinct subset of melanoma with its own profile, which is more similar to cutaneous melanoma than mucosal melanoma and remarkably different from uveal melanoma.
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Neoplasias da Túnica Conjuntiva/genética , Epigênese Genética/genética , Predisposição Genética para Doença/genética , Melanoma/genética , Mutação , Humanos , Neurofibromina 1/genética , PTEN Fosfo-Hidrolase/genética , Proteínas Proto-Oncogênicas B-raf/genética , Telomerase/genéticaRESUMO
PURPOSE: This study was done to evaluate the role of high-resolution magnetic resonance (MR) imaging with special surface coils in selecting the correct therapeutic approach (eye enucleation or follow-up) in patients with suspected extrascleral extension of uveal melanoma. MATERIALS AND METHODS: This prospective study involved 12 patients with suspected extrascleral extension of uveal melanoma on orbital ultrasonography. All patients were studied with thin-section MR imaging of the eye using surface coils. RESULTS: High-resolution MR imaging of the eye excluded extrascleral extension of disease in 8/12 patients: in 4/8 cases it revealed vascular ectasia and in the other 4/8 cases the linear hypointensity of the sclera was unbroken. Seven of these eight patients were followed up by ultrasound, which showed stability of melanoma for at least 2 years, while the last patient underwent enucleation, and the histological examination confirmed the MR diagnosis. In 4/12 patients, high-resolution MR suggested a diagnosis of extrascleral extension of melanoma, which was confirmed at histological examination after enucleation. CONCLUSION: High-resolution MR imaging of the eye with surface coils allowed us to evaluate extrascleral extension of uveal melanoma and choose the correct therapeutic approach, avoiding unnecessary enucleation in 7/12 patients.
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Amplificadores Eletrônicos , Imageamento por Ressonância Magnética/instrumentação , Melanoma/patologia , Neoplasias Uveais/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Desenho de Equipamento , Enucleação Ocular , Feminino , Humanos , Aumento da Imagem/instrumentação , Imageamento por Ressonância Magnética/métodos , Masculino , Melanoma/cirurgia , Pessoa de Meia-Idade , Invasividade Neoplásica , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Neoplasias Uveais/cirurgiaRESUMO
PURPOSE: The main purpose of treatment of advanced ocular surface and periocular malignant tumors is to eradicate the tumor while trying to preserve visual function and aesthetics. Our purpose is to describe the outcome of a retrospective case series of 10 patients with advanced ocular surface and periocular tumors treated surgically in first instance and then with postoperative interventional radiotherapy (IRT/Brachiterapy). MATERIALS AND METHODS: We describe the clinicopathological features, treatments and outcome, in a retrospective case series of 10 patients with advanced tumors involving ocular surface (staging ≥ T2) and eyelids (staging ≥ T3), with involvement of periocular and/or orbit tissues. Patients were first surgically treated, most of them with incomplete excision, and then underwent a post-operative interventional radiotherapy (IRT/Brachytherapy) as an alternative to more invasive and disfiguring surgical retreatment. Tumor location, risk factors, staging, histological features, and follow-up timing were analyzed. RESULTS: Three patients had advanced eyelid basal cell carcinomas, 2 patients were diagnosed with eyelid and conjunctival squamous cell carcinomas, 3 as sebaceous carcinomas, and 2 as primary conjunctival melanomas. The mean follow-up time from IRT to last clinical follow-up was 58.6 weeks, range 28.4-168 (median 43.65, IQR 28.9-72.9). Two patients - one with ocular surface SCC, the other with conjunctival melanoma - had a local recurrence 23.4 and 40,9 weeks after IRT, respectively. An overview of the current knowledge on adjuvant or post-operative IRT is also provided. CONCLUSIONS: IRT can be considered an effective therapeutic option to avoid more invasive surgical retreatment in advanced tumors involving eyelids and ocular surface.
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Braquiterapia , Carcinoma de Células Escamosas , Neoplasias da Túnica Conjuntiva , Neoplasias Palpebrais , Humanos , Estudos Retrospectivos , Masculino , Braquiterapia/métodos , Feminino , Neoplasias Palpebrais/radioterapia , Neoplasias Palpebrais/cirurgia , Neoplasias Palpebrais/patologia , Idoso , Pessoa de Meia-Idade , Neoplasias da Túnica Conjuntiva/radioterapia , Neoplasias da Túnica Conjuntiva/cirurgia , Neoplasias da Túnica Conjuntiva/patologia , Carcinoma de Células Escamosas/radioterapia , Carcinoma de Células Escamosas/cirurgia , Carcinoma de Células Escamosas/patologia , Melanoma/radioterapia , Melanoma/cirurgia , Melanoma/patologia , Idoso de 80 Anos ou mais , Carcinoma Basocelular/radioterapia , Carcinoma Basocelular/cirurgia , Carcinoma Basocelular/patologia , Procedimentos Cirúrgicos Oftalmológicos , Seguimentos , Estadiamento de Neoplasias , Adulto , Neoplasias das Glândulas Sebáceas/cirurgia , Neoplasias das Glândulas Sebáceas/radioterapia , Neoplasias das Glândulas Sebáceas/patologiaRESUMO
BACKGROUND: We report a case of personalized exclusive brachytherapy treatment for the management of a highly recurrent squamous cell conjunctival carcinoma with corneal invasion. CASE DESCRIPTION: This is a case of a Caucasian 81-years-old man who presented 10 years ago to our clinic with a pink-white para-limbal mass with dilated feeder arteries and mild leukoplakia. Excisional biopsy confirmed the presence of conjunctival intraepithelial neoplasia (CIN). Successively, he underwent two 4-weeks cycles of Mytomicin C topical therapy and a second excisional surgery, due to several recurrences of the lesion. At the last relapse, the pink-white peri-limbic mass which invaded the corneal limbus, determining corneal opacification from 5- to 7-clock hours, was confirmed by anterior segment optical coherence tomography (AS-OCT). Due to resistance to MMC therapy and chronic epitheliopathy, an AS-OCT guided exclusive radiotherapy plan was set: a Rhutenium-106 CCD plaque was applied directly over the afflicted corneal surface, the corneal limbus and the neighboring sclera for 24 hours. The remission of both conjunctival and corneal malignancy was complete 2 months after surgery and no signs of recurrence were highlighted at AS-OCT analysis at the 2-year follow up. CONCLUSION: Brachytherapy treatment showed optimal management of both corneal and conjunctival involvement, with a free-of-disease follow-up of 24-months. This result suggests that, in specific conditions, Ru-106 brachytherapy could be an effective option of treatment even if not associated with surgical excision.
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Braquiterapia , Carcinoma de Células Escamosas , Neoplasias da Túnica Conjuntiva , Recidiva Local de Neoplasia , Radioisótopos de Rutênio , Humanos , Masculino , Braquiterapia/métodos , Neoplasias da Túnica Conjuntiva/radioterapia , Neoplasias da Túnica Conjuntiva/diagnóstico por imagem , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas/radioterapia , Carcinoma de Células Escamosas/diagnóstico por imagem , Carcinoma de Células Escamosas/patologia , Recidiva Local de Neoplasia/radioterapia , Radioisótopos de Rutênio/uso terapêutico , Tomografia de Coerência ÓpticaRESUMO
The migration of silicone oil into the suprachoroidal region is a rare complication of vitrectomy. A 70-year-old man was admitted to our ocular oncology unit with a choroidal elevation in the nasal area, as well as signs of pars plana vitrectomy surgery performed about 5 years ago for retinal detachment. The patient underwent ocular ultrasound, visual field test, fluorangiography, and magnetic resonance imaging, which led to the diagnosis of silicone oil migration in the suprachoroidal space. Silicone oil may move into the suprachoroidal area, mimicking choroidal neoformation. It is important to have a comprehensive diagnosis and then set up the appropriate treatment. [Ophthalmic Surg Lasers Imaging Retina 2024;55:104-108.].
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Efusões Coroides , Neoplasias , Descolamento Retiniano , Masculino , Humanos , Idoso , Óleos de Silicone/efeitos adversos , Achados Incidentais , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , Vitrectomia/métodos , Neoplasias/complicações , Neoplasias/cirurgia , Efusões Coroides/complicações , Efusões Coroides/cirurgiaRESUMO
BACKGROUND: Primary and metastatic leiomyosarcomas (LMS) involving the orbital region are well known to occur however, the conjunctiva represents an extremely rare site of occurrence. METHODS: A 97-year-old male was referred to the Ocular Oncology Unit due to a rapidly growing painful mass (16×12×20 mm) in the nasal conjunctiva of his left eye. Wide excision followed by radiotherapy was performed. RESULTS: Based on the microscopic features (hypercellular neoplasm composed of spindle cells with cigar shaped and blunt ended nuclei with brightly eosinophilic fibrillary cytoplasm) and immunohistochemical findings (positive staining for Vimentin, Desmin, Caldesmon, and SMA and negative staining for AE1/AE3, EMA, CD117, S100, MelanA, SOX10, HMB45, TLE1, CD99, EMA and AE1 / AE3) the final diagnosis of grade 2 leyomiosarcoma was rendered. Moreover, 'in deep' DNA sequencing (>500 genes analysis) revealed a neoplasm with high TMB: 64 muts/Mb and numerous VUS and several pathogenic/oncogenic molecular alterations, including CNV loss or gain in > 10 genes. At the last follow-up visit, residual disease was observed in the superior fornix, at the nasal limbus and the cornea. At the time of writing, after a follow-up of 2 month the patients is still alive without evidence of metastatic disease. CONCLUSION: An uncommon molecular finding observed in our case was the presence of TSC1 gene mutation usually associated with soft tissue and gynecological PEComas. Our finding may harbor important therapeutic implications since the inactivation of the tumor suppressor genes TSC1 and TSC2 lead to upregulation of mTOR signaling, providing the rationale for target therapy with mTOR inhibitors. Additional studies on larger series are needed to validate our findings.
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Leiomiossarcoma , Neoplasias Cutâneas , Masculino , Humanos , Idoso de 80 Anos ou mais , Leiomiossarcoma/genética , Leiomiossarcoma/patologia , Imuno-Histoquímica , Proteínas de Ligação a Calmodulina , Núcleo Celular/patologia , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análiseRESUMO
PURPOSE: Since there is a lack of clear information regarding the benefit to combine supportive therapies (such as artificial tears) to mitomycin C (MMC) in the treatment of ocular surface neoplasia, the primary purpose of the study was to evaluate hyaluronic acid eye drops and hyaluronic acid-conjugated lactobionic acid (LACTOyal FREE) eye drops as supportive therapy. METHODS: Retrospective evaluation of patients with ocular surface squamous neoplasia or conjunctival melanocytic tumor treated with MMC, who had used also artificial tears as supportive treatment. A 6-month follow-up with evaluation of subjective and objective tests for ocular surface integrity was conducted. RESULTS: A total of 35 patients were analyzed, most of them with squamous disease (71.4%). The break-up time (BUT), Ocular Surface Disease Index (OSDI) and Schirmer test values showed a significant difference at any time point with overall population. No statistical difference was found among subgroups (Lactoyal vs No Lactoyal). CONCLUSION: The use of an ancillary therapy based on hyaluronic acid allows to improve both subjective and objective ocular parameters, reducing MMC induced adverse effects. Meantime, hyaluronic acid-conjugated lactobionic acid eye drops highlighted the same advantages with a more positive trend in OSDI results.
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Carcinoma de Células Escamosas , Neoplasias da Túnica Conjuntiva , Neoplasias Oculares , Humanos , Mitomicina/farmacologia , Mitomicina/uso terapêutico , Lubrificantes Oftálmicos/uso terapêutico , Ácido Hialurônico/uso terapêutico , Estudos Retrospectivos , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/patologia , Neoplasias da Túnica Conjuntiva/tratamento farmacológico , Neoplasias da Túnica Conjuntiva/induzido quimicamente , Neoplasias da Túnica Conjuntiva/patologia , Neoplasias Oculares/tratamento farmacológico , Neoplasias Oculares/induzido quimicamente , Neoplasias Oculares/patologiaRESUMO
Uveal melanoma is the most common ocular tumor with frequent metastatic spread to the liver. Immune checkpoint inhibitors have demonstrated poor results in this disease. The addition of hepatic radiotherapy to anti-PD-1 could enhance the sensitivity to immunotherapy. In this study, patients treated with pembrolizumab and who have undergone hepatic radiotherapy have been retrospectively evaluated. Twenty-two patients have been considered. Six patients (27.3%) achieved a partial response and 3 (13.6%) a stable disease. Disease control rate was 40.9%. Thirteen patients (59.1%) had progression as best response. The median PFS was 4.8 months and 6 months PFS rate 45.4%. The median OS was 21.2 months, while 1 year OS rate was 72.7%. Longer survival was observed in patients who achieved a partial response on irradiated metastases (HR 0.23, 95% CI 0.06-0.83) or progressed after 6 months (HR 0.12-95% CI 0.03-0.44). No radiotherapy-related or grade 3-4 adverse events were reported. This study demonstrates that the addition of hepatic radiotherapy to anti-PD-1 treatment can be a valid option for the treatment of metastatic uveal melanoma, particularly for HLA A 02:01 negative patients. Prospective studies should be conducted to confirm these data.
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BACKGROUND: Periocular malignancies may be clinically different from the examples arising at other sites, with possible delayed diagnosis and greater challenges for treatment and repair. Line-field confocal optical coherence tomography (LC-OCT) is a recently developed technique characterized by an unprecedented capacity to acquire high-definition images in vertical and horizontal modes. In this study, we aimed to investigate the LC-OCT morphological features of a series of eyelid skin lesions, correlating them to histopathological findings. METHODS: Patients with biopsy-proven equivocal skin lesion in the eyelid area, previously investigated by means of LC-OCT, were included in the study. Percentage overall agreement was estimated for LC-OCT and histopathological diagnosis for study cases. RESULTS: A total of 51 patients (28 women, 23 men; mean age 66.4 years old), for a total of 51 skin lesions, were assessed. The histopathological diagnosis consisted of 30 malignant and 21 benign tumors. Different entities were characterized by peculiar findings in LC-OCT, alike to histopathological features, allowing for an accurate "in vivo" classification in almost all cases, with a diagnostic concordance with histopathology of 92.1% (47/51). CONCLUSIONS: By integrating this new imaging technique into the assessment of suspicious tumors in this area, diagnostic accuracy may increase, improving strategies adopted in multidisciplinary meetings and patient-centered care.
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PURPOSE: To report a case of macular choroidal osteoma treated with photodynamic therapy. OBSERVATIONS: A 34-years old woman with decreased visual acuity in her left eye came to our observation for assessment of an amelanotic choroidal tumor in the left eye. On the basis of ophthalmoscopic and echographic features the tumor was diagnosed as choroidal osteoma. Imaging examination revealed subretinal fluid involving the foveal area associated with alterations of outer neuroepithelial layers and photoreceptors without evidence of choroidal neovascularization. Foveal sparing standard fluence rate photodynamic therapy was performed. After treatment, subretinal fluid reabsorption and visual acuity recovery was noted with progressive restoration of foveal architecture. Due to the relapse of fluid and visual impairment, 1 year after treatment, a second PDT session was made using the same parameters and protocol of treatment. Despite a complete subretinal fluid reabsorption and visual acuity recovery the second treatment was complicated by the development of subretinal fibrosis. CONCLUSIONS: PDT is effective to induce subretinal fluid reabsorption and visual recovery in choroidal osteoma located in the macular area. However, the risk of possible complications related to the treatment have to be considered.
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Neovascularização de Coroide , Osteoma , Fotoquimioterapia , Adulto , Neovascularização de Coroide/tratamento farmacológico , Feminino , Angiofluoresceinografia , Humanos , Recidiva Local de Neoplasia , Osteoma/complicações , Osteoma/diagnóstico , Osteoma/tratamento farmacológico , Fotoquimioterapia/métodos , Tomografia de Coerência ÓpticaRESUMO
Primary malignant epithelial tumours arising from accessory lacrimal glands (ALGs) are extremely rare, with only few cases reported in literature. They generally appear as gradually increasing masses of the upper or the lower eyelid. Only one case of primary adenocarcinoma or adenocarcinoma not otherwise specified (ACNOS) from ALGs has been reported in literature. Herein, we describe a case of ACNOS arising from ALGs with an atypical clinical presentation and review prior cases of ALGs epithelial malignancies reported in the literature. A 78-year-old man referred to our Ocular Oncology Unit for adjuvant therapy after the excision of a conjunctival tumour of the left eye with a histological diagnosis of squamous cell carcinoma. He underwent topical chemotherapy with MMC and during follow up he presented a multinodular iris mass in his left eye. The MRI of the orbit showed an ocular mass infiltrating orbital soft tissues of the inferior palpebral region with an involvement of the corresponding zygomatic cutis. We performed orbital exenteration and histological studies revealed an epithelial neoplasm with a solido-glandular growth pattern with lumens containing an eosinophilic material positive for PAS and PAS-D. The immunohistochemical findings confirmed the diagnosis of adenocarcinoma NOS from ALGs. Although ALGs epithelial malignancies are extremely uncommon, they should be considered in the differential diagnosis of ocular tumours. A vigilant approach towards these entities is required, since they can be clinically insidious and locally aggressive.
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Adenocarcinoma , Neoplasias da Túnica Conjuntiva , Neoplasias Oculares , Doenças do Aparelho Lacrimal , Aparelho Lacrimal , Adenocarcinoma/diagnóstico , Idoso , Neoplasias da Túnica Conjuntiva/patologia , Neoplasias Oculares/diagnóstico , Neoplasias Oculares/patologia , Humanos , Aparelho Lacrimal/diagnóstico por imagem , Aparelho Lacrimal/patologia , Doenças do Aparelho Lacrimal/diagnóstico , Doenças do Aparelho Lacrimal/patologia , Doenças do Aparelho Lacrimal/cirurgia , MasculinoRESUMO
Uveal melanoma (UM) is the most common intra-ocular tumor in Caucasian adults and may derive from melanocytes. Historically, the first therapeutic approach to treat UM has been surgical removal of the eye, with obvious consequences in terms of function, cosmesis, and psychological impact on patients. Collaborative Ocular Melanoma Study (COMS) introduced the first uniform approach to perform interventional radiotherapy (IRT - brachytherapy) procedure in a standardized way that allowed to demonstrate equivalence of IRT with enucleation in terms of overall survival. Since this milestone, several international guidelines have been issued with regard to several technical aspects of this procedure, which has become the mainstream therapy worldwide. However, nowadays, there is still a lack of consensus about the criteria to definitively assess UM response after IRT. We present a collection of paradigmatic cases treated in our institution, and then discuss in detail the latest available evidence on the topic.
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PURPOSE: To evaluate the effect of photodynamic therapy (PDT) as adjuvant treatment, after brachytherapy, in posterior amelanotic choroidal melanomas. METHODS: Six patients with posterior amelanotic choroidal melanoma underwent brachytherapy treatment. Tumour response was assessed by fundus examination, fundus photography and A-B scan ultrasonography. The residual tumours were treated with adjuvant PDT performed with infusion of verteporfin intravenously at 6â mg/m2 body surface area. Five minutes after infusion, a 689â nm laser was applied with a light dose of 100 J/cm2 over an interval of 166 s. RESULTS: At a median follow-up after brachytherapy of 17.5 months (IQR 16.2-22.5, range 5-42 months), tumours showed a partial reduction of tumour thickness (22.5% as compared to baseline value) and persistent low internal reflectivity at A-B scan ultrasonography. Supplementary photodynamic treatment resulted in complete resolution of the lesion with marked decrease of elevation . Mean decrease in thickness after PDT was 49.9% with respect to previous brachytherapy treatment 22.5% (p = 0.007). The results was achieved within a median period of 4 months (range 2-4 months) after PDT, and there has been no recurrence after a median follow-up of 84.7 months ± 18.7 (range 59 to 107 months). CONCLUSION: Combined treatment of brachytherapy and adjuvant PDT in amelanotic uveal melanoma seems to be favourable with regard to complete and rapid tumour regression.
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PURPOSE: Orbital pseudotumor (or pseudotumor orbitae) is a benign entity, also known as idiopathic orbital inflammatory syndrome (IOIS), which encompasses a wide range of non-tumoral, space occupying lesions of the orbit. In selected cases of refractory disease or presence of side effects or even comorbidities that limit systemic therapies, radiotherapy could play a role in the management because it has been demonstrated that irradiation is effective in suppressing the inflammatory process. METHODS: A systematic review of the literature about the main scientific databases was launched and the time interval included all published articles present in the databases from their inception until September 2020. RESULTS: We were able to identify 19 studies eligible for inclusion in this review from 1978 to 2018. Overall the data of 241 patients were collected and are presented in this systematic review. The response rate varied between 74% and 100% with a median recurrence rate of 10%. The median total dose was 20 Gy whereas the mean total dose was 21 Gy with a range from 4 to 36 Gy. Regarding the fractionation, 2 Gy/fraction daily was the most widely used. CONCLUSIONS: Radiotherapy seemed to achieve good response rates however, in most of the studies, inclusion criteria and outcome parameters are not uniform and therefore the results are difficult to compare. Often important parameters such as chronic pain and permanent functional deficits are not assessed in the outcome. Therefore, prospective studies, with good cohort characteristics and a clear definition of the outcome, are required.
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Pseudotumor Orbitário , Humanos , Órbita/patologia , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/radioterapia , Estudos Prospectivos , Estudos RetrospectivosRESUMO
Metastatic uveal melanoma (UM) is a poor prognosis malignancy. Immunotherapy is commonly employed, despite the low activity, considering the lack of other effective systemic treatments. In this study, the prognostic and predictive role of soluble immune checkpoints and inflammatory cytokines/chemokines in 22 metastatic UM patients was evaluated. Baseline levels of these molecules were assessed, as well as their changes during anti-PD-1 therapy. The correlation between soluble immune checkpoints/cytokines/chemokines and survival was analyzed. A comparison between circulating immune profile of metastatic cutaneous melanoma (CM), for which immunotherapy is a mainstay of treatment, and UM during anti-PD-1 therapy was also performed. Three immune molecules resulted significantly higher in metastatic UM patients with survival <6 months versus patients with survival ≥6 months: IL-8, HVEM and IDO activity. Considering these three molecules, we obtained a baseline score able to predict patients' survival. The same three molecules, together with soluble(s) CD137, sGITR and sCD27, resulted significantly lower in patients with survival >30 months. We also observed an increase of sCD137, sCD28, sPD-1, sPD-L2 sLAG3, sCD80 and sTim3 during anti-PD-1 treatment, as well as IDO activity, IP-10 and CCL2. Several of these molecules were significantly higher in UM compared to CM patients during anti-PD-1 therapy. The analysis of circulating immune molecules allows to identify patients with poor prognosis despite immunotherapy and patients with long survival treated with an anti-PD-1 agent. The different serum concentration of these molecules during anti-PD-1 therapy between UM and CM reflects the different efficacy of immune checkpoint inhibitors.
Assuntos
Melanoma , Neoplasias Cutâneas , Neoplasias Uveais , Citocinas , Humanos , Melanoma/tratamento farmacológico , Neoplasias Uveais/patologiaRESUMO
Melanoma is an extremely aggressive tumor and is considered to be an extremely immunogenic tumor because compared to other cancers it usually presents a well-expressed lymphoid infiltration. The aim of this paper is to perform a multidisciplinary comprehensive review of the evidence available about the combination of radiotherapy and immunotherapy for melanoma. Radiation, in fact, can increase tumor antigens visibility and promote priming of T cells but can also exert immunosuppressive action on tumor microenvironment. Combining radiotherapy with immunotherapy provides an opportunity to increase immunostimulatory potential of radiation. We therefore provide the latest clinical evidence about radiobiological rationale, radiotherapy techniques, timing, and role both in advanced and systemic disease (with a special focus on ocular melanoma and brain, liver, and bone metastases) with a particular attention also in geriatric patients. The combination of immunotherapy and radiotherapy seems to be a safe therapeutic option, supported by a clear biological rationale, even though the available data confirm that radiotherapy is employed more for metastatic than for non-metastatic disease. Such a combination shows promising results in terms of survival outcomes; however, further studies, hopefully prospective, are needed to confirm such evidence.