RESUMEN
Retinoblastoma (RB) is an intraocular malignancy initiated by loss of RB1 function and/or dysregulation of MYCN oncogene. RB is primarily treated with chemotherapy; however, systemic toxicity and long-term adverse effects remain a significant challenge necessitating the identification of specific molecular targets. Aurora kinase A (AURKA), a critical cell cycle regulator, contributes to cancer pathogenesis, especially in RB1-deficient and MYCN-dysregulated tumors. The current immunohistochemistry study in patient specimens (n = 67) indicated that AURKA is overexpressed in RB, and this elevated expression correlates with one or more histopathologic high-risk factors, such as tumor involvement of the optic nerve, choroid, sclera, and/or anterior segment. More specifically, AURKA is ubiquitously expressed in most advanced-stage RB tumors that show a suboptimal response to chemotherapy. shRNA-mediated depletion/pharmacologic inhibition studies in cell lines, patient-derived cells, in vivo xenografts, and enucleated patient specimens confirmed that RB cells are highly sensitive to a lack of functional AURKA. In addition, AURKA and N-myc proto-oncogene protein (MYCN) associate with each other to regulate their levels in RB cells. Overall, these results demonstrate a previously unknown up-regulation of AURKA in RB, facilitated by its crosstalk with MYCN. The elevated levels of this kinase may indicate unfavorable prognosis in tumors refractory to chemotherapy. This study provides a rationale and confirms that therapeutic targeting of elevated AURKA in RB could be a potential treatment approach.
Asunto(s)
Aurora Quinasa A , Proto-Oncogenes Mas , Neoplasias de la Retina , Retinoblastoma , Humanos , Retinoblastoma/patología , Retinoblastoma/metabolismo , Retinoblastoma/genética , Aurora Quinasa A/metabolismo , Aurora Quinasa A/genética , Animales , Neoplasias de la Retina/patología , Neoplasias de la Retina/metabolismo , Neoplasias de la Retina/genética , Neoplasias de la Retina/tratamiento farmacológico , Ratones , Femenino , Factores de Riesgo , Masculino , Terapia Molecular Dirigida , Línea Celular Tumoral , Regulación Neoplásica de la Expresión Génica , Proteína Proto-Oncogénica N-Myc/metabolismo , Proteína Proto-Oncogénica N-Myc/genética , Biomarcadores de Tumor/metabolismo , PreescolarRESUMEN
PURPOSE: To describe the clinical presentation and treatment outcomes of children who received a diagnosis of retinoblastoma in 2017 throughout Asia. DESIGN: Multinational, prospective study including treatment-naïve patients in Asia who received a diagnosis of retinoblastoma in 2017 and were followed up thereafter. PARTICIPANTS: A total of 2112 patients (2797 eyes) from 96 retinoblastoma treatment centers in 33 Asian countries. INTERVENTIONS: Chemotherapy, radiotherapy, enucleation, and orbital exenteration. MAIN OUTCOME MEASURES: Enucleation and death. RESULTS: Within the cohort, 1021 patients (48%) were from South Asia (SA), 503 patients (24%) were from East Asia (EA), 310 patients (15%) were from Southeast Asia (SEA), 218 patients (10%) were from West Asia (WA), and 60 patients (3%) were from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, < 1-261 months). The cohort included 1195 male patients (57%) and 917 female patients (43%). The most common presenting symptoms were leukocoria (72%) and strabismus (13%). Using the American Joint Committee on Cancer Staging Manual, Eighth Edition, classification, tumors were staged as cT1 (n = 441 [16%]), cT2 (n = 951 [34%]), cT3 (n = 1136 [41%]), cT4 (n = 267 [10%]), N1 (n = 48 [2%]), and M1 (n = 129 [6%]) at presentation. Retinoblastoma was treated with intravenous chemotherapy in 1450 eyes (52%) and 857 eyes (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, and 20% and 11% for WA (P < 0.0001 and P < 0.0001), respectively. CONCLUSIONS: At the conclusion of this study, significant heterogeneity was found in treatment outcomes of retinoblastoma among the regions of Asia. East Asia displayed better outcomes with higher rates of globe and life salvage, whereas Southeast Asia showed poorer outcomes compared with the rest of Asia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Asunto(s)
Neoplasias de la Retina , Retinoblastoma , Niño , Humanos , Masculino , Femenino , Lactante , Preescolar , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiología , Retinoblastoma/terapia , Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/epidemiología , Neoplasias de la Retina/terapia , Estudios Prospectivos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Resultado del Tratamiento , Asia/epidemiología , Estudios Retrospectivos , Enucleación del OjoRESUMEN
PURPOSE: To determine the efficacy of secondary salvage intravenous chemotherapy (IVC) for refractory/recurrent retinoblastoma. METHODS: Retrospective, nonrandomized interventional case series of 41 eyes of 33 patients with recurrent retinoblastoma. RESULTS: Of the 33 patients, mean age at the time of commencement of salvage IVC was 5 years (median, 5 years; range, 2-8 years). At presentation, recurrent retinoblastoma in 41 eyes of 33 patients was classified by the International Classification of Retinoblastoma as Group B (n = 7; 17%), Group C (n = 3; 7%), Group D (n = 16; 39%), and Group E (n = 15; 37%). All patients received 6 cycles of IVC as primary treatment. The indication for secondary salvage IVC with focal treatment included recurrent solid tumor (n = 36; 88%), subretinal seeds (n = 22; 54%), or persistent solid tumor (n = 2; 5%). Mean number of cycles of salvage IVC were 8 (median, 6; range, 6-18). Over a mean follow-up period of 43 months (median, 43 months; range, 12-96 months) after completion of salvage IVC, globe salvage was achieved in 22 (54%) eyes, 1 (3%) patient had histopathology-proven bone metastasis, and 1 (3%) patient died because of presumed metastasis. CONCLUSION: Secondary salvage IVC with appropriate focal treatment allows globe salvage in 54% eyes with refractory/recurrent retinoblastoma and thus serves as an alternative to intraarterial chemotherapy or enucleation.
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Neoplasias de la Retina , Retinoblastoma , Humanos , Lactante , Preescolar , Retinoblastoma/tratamiento farmacológico , Retinoblastoma/patología , Neoplasias de la Retina/tratamiento farmacológico , Neoplasias de la Retina/patología , Estudios Retrospectivos , Melfalán , Resultado del Tratamiento , Infusiones Intraarteriales , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéuticoRESUMEN
PURPOSE: To evaluate high-risk histopathological features (HRHF) following primary enucleation of eyes with retinoblastoma (RB) and assess the patient outcomes across continents. METHODS: Retrospective study of 1426 primarily enucleated RB eyes from five continents. RESULTS: Of all, 923 (65%) were from Asia (AS), 27 (2%) from Australia (AUS), 120 (8%) from Europe (EUR), 162 (11%) from North America (NA), and 194 (14%) from South America (SA). Based on the continent (AS vs. AUS vs. EUR vs. NA vs. SA), the histopathology features included massive choroidal invasion (31% vs. 7% vs. 13% vs. 19% vs. 27%, p=0.001), post-laminar optic nerve invasion (27% vs. 0% vs. 16% vs. 21% vs. 19%, p=0.0006), scleral infiltration (5% vs. 0% vs. 4% vs. 2% vs. 7%, p=0.13), and microscopic extrascleral infiltration (4% vs. 0% vs. <1% vs. <1% vs. 4%, p=0.68). Adjuvant chemotherapy with/without orbital radiotherapy was given in 761 (53%) patients. Based on Kaplan-Meier estimates in different continents (AS vs. AUS vs. EUR vs. NA vs. SA), the 6-year risk of orbital tumor recurrence was 5% vs. 2% vs. 0% vs. 0% vs. 12% (p<0.001), systemic metastasis was reported in 8% vs. 5% vs. 2% vs. 0% vs. 13% (p=0.001), and death in 10% vs. 3% vs. 2% vs. 0% vs. 11% (p<0.001) patients. CONCLUSION: There is a wide variation in the infiltrative histopathology features of RB across continents, resulting in variable outcomes. SA and AS had a higher risk of orbital tumor recurrence, systemic metastasis, and death compared to AUS, EUR, and NA.
RESUMEN
In the current era of global health awareness for retinoblastoma (RB), the challenge that lies ahead of us is providing optimal care for children affected with RB in underdeveloped nations. The understanding of similarities and disparities between various nations across the world aids in achieving comparable outcomes. With dissolving geographic barriers and evolving collaboration, global collaborative studies on RB are becoming increasingly common. They provide real-world, robust evidence on several aspects of RB. This review discusses insights gained from global RB studies regarding the demographics, certain aspects of etiopathogenesis and epidemiology, international travel burden, disparities in clinical presentations based on national income levels, management protocols, pathology, treatment outcomes, and the effect of COVID-19 on RB care across the world. These insights are likely to impact individual practice as well as inform policy reforms.
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Neoplasias de la Retina , Retinoblastoma , Niño , Humanos , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiología , Retinoblastoma/terapia , Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/epidemiología , Neoplasias de la Retina/terapia , Resultado del TratamientoRESUMEN
OBJECTIVE: To study the efficacy and side-effect profile of topical 5-Fluorouracil (5-FU) in the treatment of ocular surface squamous neoplasia (OSSN). METHODS: Retrospective study of 101 eyes of 100 patients treated with 5-FU with one week on and 3 weeks off regimen. RESULTS: Of the 100 patients (101 eyes), the mean age at diagnosis of OSSN was 49 (median, 52 years; range, 11-87 years). History of prior intervention was noted in 6 (6%) eyes. Tumor epicenter included bulbar conjunctiva (n = 54; 53%), limbus (n = 27; 27%), and cornea (n = 20;20%). Mean number of cycles of topical 5-FU administered was 3 (median, 3; range, 1-8). Complete tumor regression was achieved with topical 5-FU in 89 (88%) eyes with a mean number of 2 cycles (median, 2; range, 1-6) of 5-FU. The remaining 12 (12%) lesions underwent additional treatment including excisional biopsy (n = 7), extended enucleation (n = 3), and topical Interferon alpha 2b (n = 2) for complete tumor control. Over a mean follow-up period of 6 months (median, 5 months; range, 1-36 months) following treatment, tumor recurrence was noted in 2 (2%) patients, and side-effects were noted in 7 (7%) eyes including conjunctival hyperemia (n = 1), punctal stenosis (n = 1), sterile keratitis (n = 4), and limbal stem cell deficiency (n = 1). CONCLUSION: Topical 5-FU is an effective non-invasive therapy for OSSN with a minimal side-effect profile.
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Antimetabolitos Antineoplásicos , Carcinoma de Células Escamosas , Fluorouracilo , Soluciones Oftálmicas , Humanos , Fluorouracilo/administración & dosificación , Estudios Retrospectivos , Anciano , Masculino , Persona de Mediana Edad , Femenino , Adulto , Anciano de 80 o más Años , Adolescente , Antimetabolitos Antineoplásicos/administración & dosificación , Adulto Joven , Soluciones Oftálmicas/administración & dosificación , Niño , Resultado del Tratamiento , Carcinoma de Células Escamosas/tratamiento farmacológico , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/diagnóstico , Administración Tópica , Neoplasias del Ojo/tratamiento farmacológico , Neoplasias del Ojo/diagnóstico , Neoplasias de la Conjuntiva/tratamiento farmacológico , Neoplasias de la Conjuntiva/diagnóstico , Neoplasias de la Conjuntiva/patología , Enfermedades de la Córnea/tratamiento farmacológico , Enfermedades de la Córnea/diagnóstico , Estudios de SeguimientoRESUMEN
PURPOSE: To report the outcomes of platinum-based neoadjuvant chemotherapy (NACT) for eyelid and periocular sebaceous gland carcinoma (eSGC). METHODS: Retrospective study of 25 patients. RESULTS: The mean age at presentation of eSGC was 59 years. The mean tumor basal diameter was 46 mm. By the 8th edition of AJCC classification, tumors belonged T2 (n = 2, 8%), T3 (n = 6, 24%), and T4 (n = 17, 68%); N1 (n = 12,48%); and M1 (n = 1, 4%). NACT with 5-fluorouracil (5-FU) and cisplatin/carboplatin was administered in 21 (84%)/4 (16%) patients, respectively. The mean number of cycles of neoadjuvant systemic chemotherapy per patient was 2 (median, 3). The mean percentage reduction of tumor basal volume after neoadjuvant chemotherapy was 65% (median, 60%). After NACT, 12 (48%) patients underwent surgical treatment, 6 (12%) patients underwent EBRT, and 4 (8%) underwent adjuvant chemotherapy. A total of 11 (44%) patients were lost to follow-up during the course of treatment, of whom 3 died from metastatic disease. In 16 patients followed up for ≥ 3 months, complete tumor control was achieved in 11 (69%) patients, local tumor control in 14 (88%), and globe salvage in 7 (44%) at a mean follow-up of 25 months (median, 7 months; range, 3 to 110 months). No tumor recurrence was seen in any case. One (4%) serious adverse event of cardiotoxicity was noted. CONCLUSION: Platinum-based NACT is a suitable option for eSGC with advanced tumors and locoregional metastasis. Adverse events are rare and in patients compliant with treatment, NACT-based combination therapy offers globe salvage and systemic tumor control.
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Neoplasias de los Párpados , Terapia Neoadyuvante , Neoplasias de las Glándulas Sebáceas , Humanos , Persona de Mediana Edad , Masculino , Femenino , Estudios Retrospectivos , Neoplasias de los Párpados/tratamiento farmacológico , Neoplasias de los Párpados/diagnóstico , Neoplasias de los Párpados/patología , Neoplasias de las Glándulas Sebáceas/tratamiento farmacológico , Neoplasias de las Glándulas Sebáceas/cirugía , Neoplasias de las Glándulas Sebáceas/diagnóstico , Neoplasias de las Glándulas Sebáceas/patología , Terapia Neoadyuvante/métodos , Anciano , Adulto , Adenocarcinoma Sebáceo/tratamiento farmacológico , Adenocarcinoma Sebáceo/diagnóstico , Adenocarcinoma Sebáceo/patología , Adenocarcinoma Sebáceo/cirugía , Estudios de Seguimiento , Cisplatino/administración & dosificación , Cisplatino/uso terapéutico , Anciano de 80 o más Años , Fluorouracilo/administración & dosificación , Fluorouracilo/uso terapéutico , Quimioterapia Adyuvante/métodos , Carboplatino/administración & dosificación , Carboplatino/uso terapéutico , Estadificación de Neoplasias , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Resultado del TratamientoRESUMEN
PURPOSE: To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma and total retinal detachment. METHODS: A retrospective study of 50 eyes in 43 patients. RESULTS: The mean age at presentation was 15 months (median, 9 months). Retinoblastoma-associated total retinal detachment was unilateral (n = 36; 84%) or bilateral (n = 7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm), and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n = 26; 52%), vitreous seeds (n = 13; 26%), and subretinal exudation (n = 4; 8%). Based on eighth edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n = 19; 38%), cT2b (n = 28; 56%), or cT3c (n = 3; 6%). Primary treatment included intravenous (n = 48; 96%) or intraarterial chemotherapy (n = 2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7-62 months), retinal detachment resolved in 41 eyes (82%), tumor recurrence was noted in 39 eyes (78%), globe salvage was achieved in 35 eyes (70%), and one patient (2%) died due to metastasis. CONCLUSION: Relatively high tumor recurrence rate is noted in retinoblastoma presenting with total retinal detachment. With appropriate treatment, 70% of the globes can be salvaged.
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Desprendimiento de Retina , Neoplasias de la Retina , Retinoblastoma , Humanos , Lactante , Retinoblastoma/complicaciones , Retinoblastoma/diagnóstico , Retinoblastoma/tratamiento farmacológico , Neoplasias de la Retina/complicaciones , Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/terapia , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/tratamiento farmacológico , Desprendimiento de Retina/etiología , Estudios Retrospectivos , Recurrencia Local de Neoplasia/complicaciones , Resultado del TratamientoRESUMEN
PURPOSE: To describe the risk factors, clinical features and management outcomes of ocular surface squamous neoplasia (OSSN) with 360° of limbal involvement (360-OSSN) and compare with segmental limbal involvement (SL-OSSN). METHODS: Retrospective comparative study of 360-OSSN vs SL-OSSN. All 360-OSSN and every 10th patient with SL-OSSN during the study period (2012-2020) were included. Lesions with uncertain diagnosis were excluded. RESULTS: Of 1250 patients diagnosed with OSSN during the study period, 30 (2%) had 360-OSSN. A total of 100 patients of OSSN with SL-OSSN were included for comparison. 360-OSSN patients more often had longer duration of symptoms (mean, 17 vs 8 months; p, 0.003), prior misdiagnosis (17% vs 6%, p, 0.13) and prior intervention (47% vs 13%; p, 0.0002) than patients with SL-OSSN. 360-OSSN had higher incidence of scleral fixity (57% vs 16%; p < 0.0001), corneal/scleral melt (17% vs 0%; p, 0.0005), intraocular tumor extension (17% vs 0%; p, 0.003), orbital tumor extension (33% vs 1%; p < 0.0001), and advanced T stage at presentation (Tis: 37% vs 76%, T1: 0% vs 15%; T2: 7% vs 4%; T3: 27% vs 4%; T4: 30% vs 1%; p < 0.001). Over a mean follow-up of 14 months, lymph node metastasis (8% vs 0%; p, 0.05) and distant metastasis (4% vs 0%; p, 0.23) were more common in 360-OSSN group compared to SL-OSSN group. CONCLUSION: Risk factors of 360-OSSN include prolonged symptoms, prior misdiagnosis and prior intervention. It represents an advanced form of disease with propensity for corneo-scleral melt and invasive disease which requires aggressive management.
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Carcinoma de Células Escamosas , Neoplasias de la Conjuntiva , Úlcera de la Córnea , Neoplasias del Ojo , Enfermedades de la Esclerótica , Humanos , Neoplasias del Ojo/patología , Estudios Retrospectivos , Neoplasias de la Conjuntiva/diagnóstico , Neoplasias de la Conjuntiva/patología , Carcinoma de Células Escamosas/patologíaRESUMEN
PURPOSE: To study the clinical profile, treatment, and visual outcome of patients with Coats disease in India. METHODS: This was a cross-sectional, observational hospital-based study of patients diagnosed with Coats disease during a 10-year period using an electronic medical record system. RESULTS: We identified 675 patients with Coats disease with a prevalence rate of 0.025%. The mean age of the patients was 16.8 years (median, 12 years). Majority were males (75%) with unilateral presentation (98%) in first decade of life (n = 309, 46%). The most common presentation was foveal exudation (stage 2B, n = 161, 23.3%), followed by exudative retinal detachment-extrafoveal (stage 3A1, n = 143, 20.7%), and extrafoveal exudation (stage 2A, n = 136, 19.7%). Treatment modalities included observation (48 eyes, 17%), laser photocoagulation ± intravitreal bevacizumab/triamcinolone acetonide (n = 82, 29%), cryotherapy ± intravitreal bevacizumab/triamcinolone acetonide (n = 64, 23%), and surgical intervention (n = 86, 31%). Despite appropriate treatment, at mean follow-up of 16 months, there was no significant difference between presenting and final visual acuity (48% vs. 48%, p > 0.05). Using multivariate regression analysis, factors associated with poor visual outcome were younger age (< 0.001; - 0.02 to - 0.1), unilateral disease (0.04; - 0.68 to - 0.01), cataract (0.004; 0.13 to 0.69), retinal detachment (< 0.001; 0.49 to 0.82), and glaucoma (< 0.001; 0.34 to 0.94). CONCLUSION: The most common clinical presentation of Coats disease in India is foveal exudation in first and second decade. At initial presentation, about half of the affected eyes had blindness and one-third underwent surgical intervention.
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Desprendimiento de Retina , Telangiectasia Retiniana , Masculino , Humanos , Adolescente , Femenino , Telangiectasia Retiniana/diagnóstico , Telangiectasia Retiniana/epidemiología , Telangiectasia Retiniana/terapia , Bevacizumab/uso terapéutico , Triamcinolona Acetonida , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/epidemiología , Desprendimiento de Retina/terapia , Estudios Transversales , Coagulación con Láser , Estudios Retrospectivos , Estudios de SeguimientoRESUMEN
Exosomes are a subgroup of membrane-bound extracellular vesicles secreted by all cell types and present virtually in all biological fluids. The composition of exosomes in the same cell type varies in healthy and disease conditions. Hence, exosomes research is a prime focus area for clinical research in cancer and numerous age-related metabolic syndromes. Functions of exosomes include crucial cell-to-cell communication that mediates complex cellular processes, such as antigen presentation, stem cell differentiation, and angiogenesis. However, very few studies reported the presence and role of exosomes in normal physiological and pathological conditions of specialized ocular tissues of the eye and ocular cancers. The eye being a protected sense organ with unique connectivity with the rest of the body through the blood and natural passages, we believe that the role of exosomes in ocular tissues will significantly improve our understanding of ocular diseases and their interactions with the rest of the body. We present a review that highlights the existence and function of exosomes in various ocular tissues, their role in the progression of some of the neoplastic and non-neoplastic conditions of the eyes.
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Exosomas , Comunicación Celular , Exosomas/metabolismo , Ojo , Cara , Humanos , Órganos de los SentidosRESUMEN
PURPOSE: To describe the risk factors, clinical features, management, and outcomes in patients with metastatic conjunctival squamous cell carcinoma (mcSCC). METHODS: Retrospective comparative study. RESULTS: Of the 1192 cases with ocular surface squamous neoplasia during the study period, 654 (55%) patients were biopsied and 223 (19%) had invasive squamous cell carcinoma (cSCC). Of these 223 patients with cSCC, locoregional metastasis developed in 4 (2%) and distant metastasis in 1 (0.4%) patient. Mean age at diagnosis of OSSN was 48 years (median, 40 years; range 35-74 years). Tumors belonged to T2 (n = 2; 40%) and T4a (n = 3; 60%) at presentation. Primary treatment modalities included topical and subconjunctival interferon α 2B immunotherapy (n = 2; 40%), extended enucleation (n = 1; 20%) and orbital exenteration (n = 2; 40%). Metastases were noted after a mean period of 22 months of onset of primary tumor (median, 18 months; range 2-46 months). Death from metastatic disease occurred in all patients over a mean follow-up period of 21 months (median, 11 months; range 1-46 months). Bivariate regression analysis revealed smoking (p = 0.037, Odds Ratio (OR) = 0.13), tumor thickness ≥ 5 mm (p = 0.015, OR = 17.78), orbital invasion (p = 0.018, OR = 1.00), and poor histopathological differentiation (p = 0.031, OR = 10.44) to be significant risk factors for mcSCC. CONCLUSION: Metastatic disease in cSCC is rare and risk factors for metastasis include smoking, thicker tumors, orbital tumor extension, and poor tumor differentiation. mcSCC is associated with high mortality.
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Neoplasias Óseas , Carcinoma de Células Escamosas , Neoplasias de la Conjuntiva , Adulto , Anciano , Carcinoma de Células Escamosas/patología , Conjuntiva/patología , Neoplasias de la Conjuntiva/patología , Humanos , Interferón-alfa , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
PURPOSE: To study the correlation between retinoblastoma (RB) associated with orbital pseudocellulitis and high-risk histopathology features. METHODS: Retrospective study of 32 patients who underwent primary enucleation for RB presenting with orbital pseudocellulitis. RESULTS: All RB patients presented with orbital pseudocellulitis. The mean age at presentation of RB was 30 months (median, 24 months; range, 3-70 months). There were 14 (44%) males and 18 (56%) females. All patients were referred with a diagnosis of RB with orbital pseudocellulitis. Tumor was bilateral in 12 (38%) patients but orbital pseudocellulitis was unilateral in all cases. The pseudocellulitis features included proptosis (n = 9; 28%), eyelid edema (n = 22; 69%), conjunctival congestion (n = 23; 72%), and conjunctival chemosis (n = 15; 47%). Based on clinical features and orbital imaging, all patients were diagnosed to have group E intraocular RB. All patients received intravenous steroids prior to enucleation. On histopathology, tumor necrosis was present in all cases with a mean % necrosis of 60% (median, 60%; range, 10% to 90%). Most tumors (72%) were poorly differentiated. High-risk histopathology features were noted in 23 (72%) cases and adjuvant chemotherapy was advised for all these patients. The most common high-risk histopathology features included post-laminar optic nerve infiltration (34%) and scleral infiltration (22%). Over a mean follow-up period of 34 months (median, 9 months; range, < 1-188 months), there was no event of metastasis or death in any patient. CONCLUSION: RB presenting with orbital pseudocellulitis is associated with high incidence of high-risk histopathology features.
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Neoplasias de la Retina , Retinoblastoma , Ojo , Enucleación del Ojo , Femenino , Humanos , Lactante , Masculino , Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/epidemiología , Neoplasias de la Retina/cirugía , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiología , Retinoblastoma/cirugía , Estudios RetrospectivosRESUMEN
PURPOSE: To describe the clinical features, histopathology, treatment, and outcomes of patients with ocular surface squamous neoplasia (OSSN) presenting to a referral centre in India. METHODS: Retrospective interventional study. RESULTS: Of 438 patients, the mean age at presentation was 49 years. Human immunodeficiency virus infection was noted in 72 (16%), xeroderma pigmentosum in 22 (5%), hepatitis B virus infection in 14 (3%), and systemic cancer in 8 (2%) patients. Tumor pigmentation was noted in 243 (54%) tumors with a mean percentage of tumor pigmentation of 44% (median, 40%; range, 1 to 100%). Intraocular tumor extension was noted in 12 (3%), and orbital tumor extension in 16 (4%) eyes. Of the 381 treated lesions, excisional biopsy (n = 247; 65%) was the most common treatment modality. Of the 311 lesions with histopathology diagnosis of OSSN, invasive squamous cell carcinoma (n = 92; 30%) was the most common. Over a mean follow-up period of 11 months (median, 5 months; range, 1 to 108 months) in 368 patients, tumor recurrence was noted in 16 (4%) eyes, globe salvage was achieved in 341 (90%) eyes, vision salvage in 338 (89%) eyes, regional lymph node metastasis occurred in 9 (2%), and metastasis-related death in 9 (2%) patients. CONCLUSION: Pigmented OSSN is common in Asian Indian population. Appropriate management of OSSN is associated with good vision, globe, and life salvage rates in India.
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Carcinoma de Células Escamosas , Neoplasias de la Conjuntiva , Neoplasias del Ojo , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/epidemiología , Carcinoma de Células Escamosas/terapia , Neoplasias de la Conjuntiva/diagnóstico , Neoplasias de la Conjuntiva/epidemiología , Neoplasias de la Conjuntiva/terapia , Neoplasias del Ojo/diagnóstico , Neoplasias del Ojo/epidemiología , Neoplasias del Ojo/terapia , Humanos , India/epidemiología , Recurrencia Local de Neoplasia , Estudios RetrospectivosRESUMEN
The management of retinoblastoma is complex. With increasing treatment modalities and increasing experience with each treatment modality, the globe salvage rates have drastically improved with time. Aqueous seeding in an eye with retinoblastoma is classified as group E based on International Classification of Intraocular Retinoblastoma, and most group E eyes were enucleated a decade earlier. Newer modalities of treatment have improved the globe salvage rates in group E eyes including those with aqueous seeding. Various globe salvage treatment modalities attempted for the management of aqueous seeds include external beam radiotherapy, plaque radiotherapy, intra-arterial chemotherapy, periocular chemotherapy, intravitreal chemotherapy and intracameral chemotherapy. Of all treatment modalities, intracameral chemotherapy holds promise for the management of aqueous seeds in selected patients, but it should be done with the utmost care by a trained ocular oncologist. Enucleation is still the preferred modality of treatment for eyes with advanced retinoblastoma and anterior segment invasion. This review focuses on the current evidence on aqueous seeding, highlighting the pathogenesis, classification, clinical presentation, treatment and outcomes.
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Neoplasias de la Retina , Retinoblastoma , Enucleación del Ojo , Humanos , Lactante , Siembra Neoplásica , Neoplasias de la Retina/terapia , Retinoblastoma/terapia , Estudios Retrospectivos , Terapia RecuperativaRESUMEN
Phyllodes tumors are rare and account for 0.5% of all mammary neoplasms. They commonly present as lobulated breast tumors and have a variable clinical course. Histologically, phyllodes tumor is composed of 2 major elements: stromal element composed of spindle cells admixed with collagen and epithelial element in the form of epithelium lined cysts and clefts. Metastasis is rarely seen with phyllodes tumors. Sarcomatous component of phyllodes tumor metastasizes to lung, liver, adrenal, brain, and bones by hematogenous route. A thorough literature search did not reveal any report of orbital metastasis from malignant phyllodes tumor. Here, the authors describe a rare case of malignant phyllodes tumor of the breast with metastasis to the orbit in a 46-year-old female patient.
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Neoplasias de la Mama , Tumor Filoide , Animales , Femenino , Humanos , Persona de Mediana Edad , Órbita , Tumor Filoide/diagnósticoRESUMEN
PURPOSE: The aim of the study was to investigate the clinical resolution versus radiologic regression of orbital lymphatic malformations (LMs) following treatment with intralesional bleomycin sulfate sclerotherapy. METHODS: A retrospective interventional study of 24 eyes with orbital LMs treated with nonimage-guided bleomycin sclerotherapy. The clinical and radiologic outcomes were classified as excellent, good, fair, and poor. Regression was assessed clinically and by radiologic volumetrics. RESULTS: Mean age at presentation was 17 ± 18 years (median 11, range 5 months to 70 years). Lesion morphology was microcystic in 11 (46%), macrocystic in 8 (34%), and mixed in 5 (21%) eyes. Mean units of bleomycin injected per session were 4 ± 2 IU (median 5 IU, range 1-6 IU). Mean number of treatment sessions required was 2 ± 1 (median 2, range 1-6). Cumulative units of bleomycin injected were 11 ± 9 (median 9, range 1-38 IU). The clinical response was excellent in 19 (79%), good in 4 (17%), and fair in 1 (4%). The mean preoperative and postoperative lesion volumes were 7 ± 4 cm3 and 0.8 ± 1.2 cm3, respectively (p < 0.0001, 95% CI, -7.89 to -4.51). Radiologic resolution of LM was excellent in 6 (25%), good in 8 (33%), fair in 7 (29%), and poor in 3 (13%) eyes. Spearman's rank correlation coefficient for correlation between clinical and radiologic grading was 0.51 (p = 0.01, 95% CI, 0.13-0.75%). There was a sustained tumor resolution without recurrence over a mean follow-up duration was 2 years (median 18 months; range 12-60 months). CONCLUSIONS: Bleomycin sclerotherapy for orbital LMs gives an excellent to good clinical response in 93%. However, a parallel radiologic regression is seen only in 58%. The endpoint to assess response should be clinical. Treatment till complete radiologic resolution may not be necessary.
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Bleomicina , Anomalías Linfáticas , Bleomicina/uso terapéutico , Humanos , Lactante , Anomalías Linfáticas/diagnóstico , Anomalías Linfáticas/tratamiento farmacológico , Recurrencia Local de Neoplasia/tratamiento farmacológico , Estudios Retrospectivos , Soluciones Esclerosantes/uso terapéutico , Escleroterapia , Resultado del TratamientoRESUMEN
PURPOSE: To assess the relationship between age at presentation and outcomes of patients with periocular sebaceous gland carcinoma. METHODS: Retrospective case series of 241 patients. RESULTS: Of 241 cases, 29 (12%) were young adults (≤40 years), 122 (51%) were middle-aged adults (41-60 years), and 90 (37%) were older adults (>60 years). Based on the eighth edition of American Joint Committee on Cancer, most tumors belonged to T1 category (n = 78, 32%) on presentation. Wide excisional biopsy was the most common treatment modality (n = 183, 79%). There were no statistically significant differences in the clinical presentation, treatment approaches, and histopathology features amongst different age groups, except pagetoid spread which was higher in middle-aged adults (61%, p = 0.004). The incidence of tumor recurrence was higher in older age group compared to younger age groups, with 5-year, 10-year Kaplan-Meier estimate at 31%, 31% in young adults, 38%, 38% in middle-aged adults, and 45%, 100% in older adults (p = 0.03), respectively. The 10-year Kaplan-Meier estimate rate of locoregional lymph node metastasis, systemic metastasis, and death was higher in young adults (51%, 48%, and 48%, respectively) compared to middle-aged (30%, 17%, and 12%, respectively) and older adults (24%, 25%, and 27%, respectively), but the differences were not statistically significant. CONCLUSIONS: There is no difference in the clinical presentation of periocular sebaceous gland carcinoma based on age. However, the tumor recurrence rate is much higher in the older age group and death higher in younger patients despite the same treatment strategies in all age groups.
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Adenocarcinoma Sebáceo , Neoplasias de los Párpados , Neoplasias de las Glándulas Sebáceas , Adenocarcinoma Sebáceo/epidemiología , Anciano , Humanos , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Neoplasias de las Glándulas Sebáceas/epidemiología , Glándulas Sebáceas , Adulto JovenRESUMEN
Purpose: To evaluate the outcome of primary orbital polymethylmethacrylate (PMMA) implant following the primary enucleation for retinoblastoma. Methods: Retrospective study of 321 retinoblastoma patients who underwent unilateral enucleation and PMMA implant for retinoblastoma by myoconjunctival technique. Outcome measures included implant centration and extrusion. Results: The mean age at the time of enucleation of patients with retinoblastoma was 35 months (median, 30 months; range, <1 to 449 months). After primary enucleation, primary orbital PMMA implant was used in all cases. The mean diameter of implant was 18 mm (median, 18 mm; range, 12-20 mm) and the mean horizontal diameter of the socket conformer was 24 mm (median, 24 mm; range, 18 mm-26 mm). Post-enucleation and implant, seven (2%) patients underwent orbital external beam radiotherapy owing to microscopic extrascleral tumor extension or tumor infiltration of optic nerve transection. Over a mean follow-up period of 40 months (median, 34 months; range, 4-129 months), implant migration was noted in 28 (9%) patients, implant extrusion in 9 (3%), and implant exposure in 5 (2%), and contracted socket in 5 (2%) patients including grade 1 contraction in 3 (1%), grade 2 in 1 (<1%), and grade 4 in 1 (<1%) patient. Implant exchange for an improved prosthesis fit was performed in 4 (1%) cases. Stable customized ocular prosthesis was achieved in all but one patient. Conclusion: Primary orbital PMMA implant following primary enucleation for retinoblastoma is associated with minimal complications and provides acceptable cosmetic outcomes.
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Implantes Orbitales , Neoplasias de la Retina , Retinoblastoma , Enucleación del Ojo , Humanos , Polimetil Metacrilato , Implantación de Prótesis , Neoplasias de la Retina/cirugía , Retinoblastoma/cirugía , Estudios RetrospectivosRESUMEN
PURPOSE: To analyze the risk factors and estimate the risk period for tumor recurrence in intraocular retinoblastoma (RB). METHODS: Retrospective study of 60 RB patients. RESULTS: The mean age at presentation with RB was 16 months (median 11 months; range 1-84 months). Tumor was unilateral in 13 (22%) and bilateral in 47 (78%) patients. Of 83 eyes with intraocular RB, group B (n = 27; 33%) tumors were more common based on International Classification of Intraocular Retinoblastoma. All cases received intravenous chemotherapy as a primary treatment. Over a mean follow-up period of 57 months (median 38 months; range 12-185 months) post-primary treatment, 44 (73%) patients developed tumor recurrence. The mean interval between the completion of primary treatment and first tumor recurrence was 5 months (median 3 months; range 1-24 months). The total duration of treatment for complete tumor control including treatment of tumor recurrences was 20 months (median 19 months; 2-58 months). By multivariate analysis, the factors predictive of tumor recurrence were multiple tumors (p = 0.008) and retinal detachment (p = 0.003) at presentation. Kaplan-Meier estimate of tumor recurrence at 6 months, 1 year, 3 years, and 5 years was 20%, 31%, 68%, and 73%, respectively. There was no tumor recurrence beyond 5 years since primary treatment. CONCLUSION: Multiple tumors and retinal detachment at presentation are risk factors for tumor recurrence in RB. Close follow-up is mandatory for at least 5 years since the initiation of treatment for RB.