Concordancia clínica y patológica en tumores conjuntivales: Análisis de 195 casos / Clinic-pathological agreement in the diagnosis of conjunctival tumours: Analysis of 195 cases

Propósito Evaluar la concordancia entre el diagnóstico clínico y patológico en tumores conjuntivales en una unidad especializada en oncología ocular. Métodos Estudio retrospectivo de pacientes consecutivos con tumores conjuntivales diagnosticados en la Unidad de Oncología Ocular del Hospital Universitario de Valladolid desde 1992 hasta 2017. Los tumores se clasificaron según su origen (epitelial, melanocítico, linfoide y otros) y grado de malignidad (benigno, premaligno, maligno). Se realizó biopsia en los casos de lesiones sintomáticas o en crecimiento. Como indicador de concordancia entre el diagnóstico clínico y el patológico se utilizo el estadístico kappa (κ) de Cohen. Resultados Cuatrocientos sesenta y dos pacientes fueron atendidos de manera consecutiva, requiriendo biopsia en 195 (42,2%). La concordancia con el diagnóstico anatomopatológico fue satisfactoria en 154 (79%) casos. El análisis según el grado de malignidad mostró la menor tasa de concordancia en las lesiones benignas (n = 83; 91,6%) y premalignas (n = 62; 90,3%), con una concordancia total en las lesiones malignas (n = 50; 100%); el valor κ fue de 0,90. Los mayores índices de concordancia se encontraron en las lesiones epiteliales, melanocíticas y de partes blandas, con valores κ de 1, 0,8 y 1 respectivamente. El peor índice de concordancia se observó en lesiones linfoides, con un valor κ de 0,3. Conclusiones La mayoría de los tumores conjuntivales fueron correctamente identificados clínicamente. Las lesiones benignas y malignas mostraron la mayor tasa de precisión; sin embargo, las lesiones premalignas pueden ocultar enfermedad microinvasiva que puede pasar desapercibida en el examen clínico. La biopsia es esencial para un diagnóstico y un tratamiento precisos (AU)

Purpose The present study aims to assess the agreement between clinical and pathological diagnosis in conjunctival tumours in a specialist ocular oncology unit. Methods A retrospective study of consecutive patients with conjunctival tumours diagnosed at the Ocular Oncology Unit of the University Hospital of Valladolid was performed from 1992 to 2017. Tumours were classified according to their origin (epithelial, melanocytic, lymphoid, and others) and degree of malignancy (benign, premalignant, and malignant). A biopsy was performed in cases of symptomatic or growing lesions. Cohen's kappa (κ) statistics was used as an indicator of agreement between clinical and pathological diagnosis. Results Of 462 consecutive patients, a biopsy was required in 195 (42.2%). The agreement with the pathological diagnosis was successful in 154 (79%) cases. Analysis according to the grade of malignancy showed the lowest rate of agreement among benign (n = 83; 91.6%) and premalignant (n = 62; 90.3%) lesions, with a total agreement in malignant lesions (n = 50; 100%); the Cohen's kappa coefficient (κ) was 0.90. The highest rates of concordance were found in epithelial, melanocytic and soft tissue lesions with κ values of 1, 0.8 and 1, respectively. The worst rate of concordance was found in lymphoid lesions with a κ value of 0.3. Conclusions Most of the conjunctival tumours were correctly identified clinically; benign and malignant lesions showed the highest rate of accuracy; however, premalignant tumours can hide micro-invasive diseases that can go unnoticed on clinical examination. The biopsy is essential for accurate diagnosis and treatment (AU)

Clinic-pathological agreement in the diagnosis of conjunctival tumours: Analysis of 195 cases.

PURPOSE: The present study aims to assess the agreement between clinical and pathological diagnosis in conjunctival tumours in a specialist ocular oncology unit. METHODS: retrospective study of consecutive patients with conjunctival tumours diagnosed at the Ocular Oncology Unit of the University Hospital of Valladolid was performed from 1992 to 2017. Tumours were classified according to their origin (epithelial, melanocytic, lymphoid and others) and degree of malignancy (benign, premalignant, malignant). A biopsy was performed in cases of symptomatic or growing lesions. Cohen´s kappa (κ) statistics was used as an indicator of agreement between clinical and pathological diagnosis. RESULTS: Of 462 consecutive patients, a biopsy was required in 195 (42.2%). The agreement with the pathological diagnosis was successful in 154 (79.0%) cases. Analysis according to the grade of malignancy showed the lowest rate of agreement among benign (n = 83; 91.6%) and premalignant (n = 62; 90.3%) lesions, with a total agreement in malignant lesions (n = 50; 100%); the Cohen´s kappa coefficient (κ) was 0.90. The highest rates of concordance were found in epithelial, melanocytic and soft tissue lesions with κ values of 1, 0.8 and 1 respectively. The worst rate of concordance was found in lymphoid lesions with a κ value of 0.3. CONCLUSION: Most of the conjunctival tumours were correctly identified clinically; benign and malignant lesions showed the highest rate of accuracy; however, premalignant tumours can hide micro-invasive diseases that can go unnoticed on clinical examination. The biopsy is essential for accurate diagnosis and treatment.

[Impact of COVID-19 on a rare disease (uveal melanoma) in a national reference unit of intraocular tumors in Spain]. / Impacto del COVID-19 en una enfermedad rara (melanoma uveal) en una unidad de referencia nacional de tumores intraoculares en España.

Objective: To analyze the impact of the COVID-19 pandemic on the diagnosis and management of uveal melanoma (a tumor included in the Orphanet catalog of rare diseases) in a Spanish national reference unit for intraocular tumors during the first year of the pandemic. Material and methods: An observational retrospective study of patients with uveal melanoma in the National Reference Unit for Adult Intraocular Tumors of the Hospital Clínico Universitario de Valladolid (Spain) was performed, analyzing the pre- and post-COVID-19 periods: from March 15, 2019 to March 15, 2020 and from March 16, 2020 to March 16, 2021. Demographic data, diagnostic delay, tumor size, extraocular extension, treatment and evolution were collected. A multivariable logistic regression model was used to identify factors that were associated with the variable: enucleation. Results: Eighty-two patients with uveal melanoma were included, of which 42(51.21%) belonged to the pre-COVID-19 period and 40(40.78%) to the post-COVID-19 period. An increase in tumor size at diagnosis and in the number of enucleations was observed during the post-COVID-19 period (p < 0.05). Multivariable logistic regression demonstrated that both medium-large tumor size and patients diagnosed in the post-COVID-19 period were independently related to an increased risk of enucleation (OR 250, 95%CI, 27.69-2256.37; p < 0.01 and OR 10; 95% CI,1.10-90.25; p = 0.04, respectively). Conclusions: The increase in tumor size observed in uveal melanomas diagnosed during the first year of the COVID-19 pandemic may have favored the increase in the number of enucleations performed during that period.

Impacto del COVID-19 en una enfermedad rara (melanoma uveal) en una unidad de referencia nacional de tumores intraoculares en España / Impact of COVID-19 on a rare disease (uveal melanoma) in a national reference unit of intraocular tumors in Spain

Objetivo Analizar el impacto de la pandemia por COVID-19 en el diagnóstico y manejo del melanoma uveal (tumor incluido en el catálogo de enfermedades raras por Orphanet), en una unidad de referencia nacional española de tumores intraoculares, durante el primer año de pandemia. Materiales y métodos Se realizó un estudio retrospectivo observacional de pacientes con melanoma uveal en la Unidad de Referencia Nacional de Tumores Intraoculares del Adulto del Hospital Clínico Universitario de Valladolid (España), analizando los periodos pre- y pos-COVID-19: del 15 de marzo de 2019 al 15 de marzo de 2020 y del 16 marzo de 2020 al 16 de marzo de 2021. Se recogieron datos demográficos, demora diagnóstica, tamaño del tumor, extensión extraocular, tratamiento y evolución. Se utilizó un modelo de regresión logística multivariable para identificar los factores que se asociaron a la variable: enucleación. Resultados Se incluyeron 82 pacientes con melanoma uveal, de los cuales 42(51,21%) pertenecían al periodo pre-COVID-19 y 40(40,78%) al periodo pos-COVID-19. Se observó un aumento del tamaño tumoral al diagnóstico y del número de enucleaciones durante el periodo pos-COVID-19 (p<0,05). La regresión logística multivariable demostró que tanto el tamaño tumoral mediano-grande como los pacientes diagnosticados en el periodo pos-COVID-19 estaban relacionados de forma independiente con un riesgo mayor de enucleación (OR 250, IC95%, 27,69-2256,37; p<0,01 y OR 10; IC95%,1,10-90,25; p=0,04, respectivamente). Conclusiones El incremento del tamaño tumoral observado en los melanomas uveales diagnosticados durante el primer año de pandemia por COVID-19 pudo favorecer el aumento de las enucleaciones realizadas en dicho periodo (AU)

Objective To analyze the impact of the COVID-19 pandemic on the diagnosis and management of uveal melanoma (a tumor included in the Orphanet catalog of rare diseases) in a Spanish national reference unit for intraocular tumors during the first year of the pandemic. Material and methods An observational retrospective study of patients with uveal melanoma in the National Reference Unit for Adult Intraocular Tumors of the Hospital Clínico Universitario de Valladolid (Spain) was performed, analyzing the pre- and post-COVID-19 periods: from March 15, 2019 to March 15, 2020 and from March 16, 2020 to March 16, 2021. Demographic data, diagnostic delay, tumor size, extraocular extension, treatment and evolution were collected. A multivariable logistic regression model was used to identify factors that were associated with the variable: enucleation. Results Eighty-two patients with uveal melanoma were included, of which 42(51.21%) belonged to the pre-COVID-19 period and 40(40.78%) to the post-COVID-19 period. An increase in tumor size at diagnosis and in the number of enucleations was observed during the post-COVID-19 period (p<0.05). Multivariable logistic regression demonstrated that both medium-large tumor size and patients diagnosed in the post-COVID-19 period were independently related to an increased risk of enucleation (OR 250, 95%CI, 27.69-2256.37; p<0.01 and OR 10; 95% CI,1.10-90.25; p=0.04, respectively). Conclusions The increase in tumor size observed in uveal melanomas diagnosed during the first year of the COVID-19 pandemic may have favored the increase in the number of enucleations performed during that period (AU)

Impact of COVID-19 on a rare disease (uveal melanoma) in a national reference unit of intraocular tumors in Spain.

OBJECTIVE: To analyse the impact of the COVID-19 pandemic on the diagnosis and management of uveal melanoma (a tumour included in the Orphanet catalogue of rare diseases) in a Spanish national reference unit for intraocular tumours during the first year of the pandemic. METHOD: An observational retrospective study of patients with uveal melanoma in the National Reference Unit for Adult Intraocular Tumors of the Hospital Clínico Universitario de Valladolid (Spain) was performed, analysing the pre- and post-COVID-19 periods: from March 15, 2019 to March 15, 2020 and from March 16, 2020 to March 16, 2021. Demographic data, diagnostic delay, tumour size, extraocular extension, treatment and evolution were collected. A multivariable logistic regression model was used to identify factors that were associated with the variable: enucleation. RESULTS: Eighty-two patients with uveal melanoma were included, of which 42 (51.21%) belonged to the pre-COVID-19 period and 40(40.78%) to the post-COVID-19 period. An increase in tumour size at diagnosis and in the number of enucleations was observed during the post-COVID-19 period (p < 0.05). Multivariable logistic regression demonstrated that both medium-large tumour size and patients diagnosed in the post-COVID-19 period were independently related to an increased risk of enucleation (OR 250, 95%CI, 27.69-2256.37; p < 0.01 and OR 10; 95%CI, 1.10-90.25; p = 0.04, respectively). CONCLUSIONS: The increase in tumour size observed in uveal melanomas diagnosed during the first year of the COVID-19 pandemic may have favored the increase in the number of enucleations performed during that period.

Metastatic involvement of optic nerve in patient with breast and lung neoplasia.

Metastases are the most common adult intraocular tumors. However, those located in the optic nerve are very uncommon and are usually associated with spread to other locations such as the central nervous system, which darkens the prognosis. There is a case of a 67-year-old woman who reports progressive vision loss in the right eye of 15 days of evolution. The ophthalmological examination shows a relative afferent pupil defect in this eye and a pseudoedema of the papilla with retinal hemorrhages in the fundus. Personal history and characteristics of the optic nerve suggest the diagnosis of metastatic infiltration.

Afectación metastásica del nervio óptico en paciente con neoplasia de mama y pulmón / Metastatic involvement of optic nerve in patient with breast and lung neoplasia

Las metástasis son los tumores intraoculares más frecuentes del adulto. Sin embargo, aquellas localizadas en el nervio óptico son muy infrecuentes y suelen asociarse a diseminación en otras localizaciones como el sistema nervioso central, lo que ensombrece el pronóstico.Se presenta un caso de una mujer de 67 años que refiere pérdida de visión progresiva en el ojo derecho de 15 días de evolución. En la exploración oftalmológica se observa un defecto pupilar aferente relativo en dicho ojo y un pseudoedema de papila con hemorragias retinianas en el fondo de ojo. Los antecedentes personales y las características del nervio óptico apuntan al diagnóstico de infiltración metastásica. (AU)

Metastases are the most common adult intraocular tumors. However, those located in the optic nerve are very uncommon and are usually associated with spread to other locations such as the central nervous system, which darkens the prognosis.There is a case of a 67-year-old woman who reports progressive vision loss in the right eye of 15 days of evolution. The ophthalmological examination shows a relative afferent pupil defect in this eye and a pseudoedema of the papilla with retinal hemorrhages in the fundus. Personal history and characteristics of the optic nerve suggest the diagnosis of metastatic infiltration (AU)

Intraocular metastasis as a systemic presentation of neuroendocrine differentiation of prostate carcinoma.

A 68 year-old male was referred for assessment of an amelanotic lesion in the right eye (RE) that was associated with a gradual loss of visual acuity (VA), of 2 months onset, as the main symptom. It was noted in his medical history, that 6 years ago, he had prostate cancer treated with prostatectomy, lymphadenectomy, and coadjuvant local radiotherapy (RT). He was asymptomatic until 6 months ago, when a metastasis was discovered in the left femur, which was treated with radiotherapy. There were no findings of interest in the left eye (LE). His AV was very low in his RE, and in the eye fundus examination a mass without pigment was observed in the posterior pole with an adjacent exudative retinal detachment. Due to his personal history and results of the complementary tests such as ultrasound and magnetic resonance, the most likely diagnostic option was metastasis of prostate carcinoma, subsequently being confirmed with the histopathology results. Despite 4 cycles of chemotherapy, the patient did not show any clinical or radiological response, worsening until his death 3 months later.

Metástasis intraocular como presentación sistémica de un adenocarcinoma de próstata con diferenciación neuroendocrina / Intraocular metastasis as a systemic presentation of neuroendocrine differentiation of prostate carcinoma

Varón de 68 años derivado para valoración de una lesión amelanótica en el ojo derecho (OD) que asociaba como síntoma principal pérdida de agudeza visual (AV) progresiva de 2 meses de evolución. Entre sus antecedentes destaca un adenocarcinoma de próstata tratado con prostatectomía, linfadenectomía y radioterapia local (RT) coadyuvante hace 6 años. Asintomático hasta hace 6 meses, cuando se descubrió una metástasis en fémur izquierdo, tratada con radioterapia. La exploración del ojo izquierdo (OI) no tenía hallazgos de interés. En el OD su AV era muy baja y en el fondo de ojo (FO) se encontró una masa sin pigmento en polo posterior con un desprendimiento de retina (DdR) exudativo adyacente. Por sus antecedentes personales y características de las pruebas complementarias como ecografía o resonancia, la opción diagnóstica más probable era metástasis de adenocarcinoma de próstata, certificándose posteriormente con los resultados anatomopatológicos. A pesar de los 4 ciclos de quimioterapia (QT) recibidos, el paciente no obtuvo respuesta clínica ni radiológica, empeorando hasta su fallecimiento 3 meses después (AU)

A 68 year-old male was referred for assessment of an amelanotic lesion in the right eye (RE) that was associated with a gradual loss of visual acuity (VA), of 2 months onset, as the main symptom. It was noted in his medical history, that 6 years ago, he had prostate cancer treated with prostatectomy, lymphadenectomy, and coadjuvant local radiotherapy (RT). He was asymptomatic until 6 months ago, when a metastasis was discovered in the left femur, which was treated with radiotherapy. There were no findings of interest in the left eye (LE). His VA was very low in his RE, and in the eye fundus examination a mass without pigment was observed in the posterior pole with an adjacent exudative retinal detachment. Due to his personal history and results of the complementary tests such as ultrasound and magnetic resonance, the most likely diagnostic option was metastasis of prostate carcinoma, subsequently being confirmed with the histopathology results. Despite 4 cycles of chemotherapy, the patient did not show any clinical or radiological response, worsening until his death 3 months later (AU)

Melanomalytic glaucoma secondary to iris melanoma. / Glaucoma melanomalítico secundario a melanoma de iris.

CLINICAL CASE: A 35-year-old male with unilateral ocular hypertension in the right eye (RE). Anterior segment examination of the RE showed a melanotic lesion. Intraocular pressure (IOP) was 40mmHg in RE. Gonioscopy revealed extension into the anterior chamber angle. Excisional biopsy was performed and consistent with iris melanoma and secondary melanomalytic glaucoma. Brachytherapy was performed as adjuvant therapy. DISCUSSION: In patients presenting with unilateral glaucoma and increased pigmentation in the trabecular meshwork, it is crucial to perform imaging studies. Resection by iridectomy with adjuvant episcleral brachytherapy is effective in controlling the disease.

Tumor pigmentado del cuerpo ciliar: ¿benigno o maligno? / Pigmented ciliary body tumours: benign or malignant?

Casos clínicos: Se presentan dos casos de mujeres con una tumoración pigmentada en cuerpo ciliar, un melanoma y un melanocitoma, con diferentes manifestaciones clínicas. La primera refiere disminución de agudeza visual asociada a la tumoración de reciente aparición, así como subluxación y opacificación sectorial cristaliniana. La segunda está asintomática y ha permanecido en observación más de 30 años. Discusión: Aunque al diagnóstico definitivo de un tumor pigmentado del cuerpo ciliar solo se llega a través del estudio anatomopatológico, el conjunto de características clínicas es determinante cuando se plantea un tratamiento conservador (AU)

Cases report: We report the cases of 2 women with a pigmented tumour in the ciliary body, one a melanocytoma and the other a melanoma, with different clinical manifestations. The first one presented with decreased visual acuity associated with recent growth of the tumour, as well as sectorial opacities of the lens and subluxation. The second one is asymptomatic and has been kept under observation for more than 30 years. Discussion: Although the definitive diagnosis of a pigmented tumour of the ciliary body is only achieved by the histopathology study, the group of clinical features is a determining factor when a conservative treatment is indicated (AU)

[Pigmented ciliary body tumours: benign or malignant?]. / Tumor pigmentado del cuerpo ciliar: ¿benigno o maligno?

CASES REPORT: We report the cases of 2 women with a pigmented tumour in the ciliary body, one a melanocytoma and the other a melanoma, with different clinical manifestations. The first one presented with decreased visual acuity associated with recent growth of the tumour, as well as sectorial opacities of the lens and subluxation. The second one is asymptomatic and has been kept under observation for more than 30 years. DISCUSSION: Although the definitive diagnosis of a pigmented tumour of the ciliary body is only achieved by the histopathology study, the group of clinical features is a determining factor when a conservative treatment is indicated.

[Primary localized conjunctival amyloidosis: two case reports]. / Amiloidosis primaria conjuntival: análisis de dos casos.

CASE REPORTS: Two male patients, 21 and 61 years old presenting with hyperemia resistant to topical treatment. Ocular examination showed reddish conjunctival mass, painless, highly vascularized in bulbar conjunctiva. The conjunctival biopsy revealed the presence of amyloid within the conjunctiva itself. CONCLUSIONS: Primary localized conjunctival amyloidosis is a rare disease. Diagnosis consists of biopsy in order to detect amyloid material in the conjunctival tissue together with a systemic evaluation in order to rule out the presence of primary systemic amyloidosis.

Amiloidosis primaria conjuntival: análisis de dos casos / Primary localized conjunctival amyloidosis: two case report

Caso clínico: Se presentan dos pacientes varones de 21 y 61 años de edad con hiperemia resistente al tratamiento tópico. En el examen ocular se observó una masa rosada, no dolorosa y muy vascularizada, en conjuntiva bulbar. La biopsia excisional diagnóstica reveló la presencia de amiloide en la sustancia propia conjuntival.Conclusiones: La amiloidosis conjuntival primaria es una enfermedad rara. El diagnóstico consiste la realización de una biopsia detectando material amiloide en el tejido conjuntival junto con un examen sistémico que descarte la presencia de una amiloidosis primaria sistémica

Case Reports: Two male patients, 21 and 61 years old presenting with hyperemia resistant to topical treatment. Ocular examination showed reddish conjunctival mass, painless, highly vascularized in bulbar conjunctiva. The conjunctival biopsy revealed the presence of amyloid within the conjunctiva itself.Conclusions: Primary localized conjunctival amyloidosis is a rare disease. Diagnosis consists of biopsy in order to detect amyloid material in the conjunctival tissue together with a systemic evaluation in order to rule out the presence of primary systemic amyloidosis

[Sympathetic ophthalmia post-vitrectomy following trauma and perfluorocarbon liquid tamponade]. / Oftalmía simpática postvitrectomía tras traumatismo y taponamiento con perfluorocarbono líquido.

CASE REPORT: We report a case of sympathetic ophthalmia (SO) developed after blunt trauma and vitrectomy (complicated with a massive suprachoroidal hemorrhage) using perfluorocarbon liquid (PFCL) for tamponade. DISCUSSION: The presentation of SO in a vitrectomized patient after severe blunt trauma with extended intra-ocular tamponade with PFCL could support the hypothesis that chronic inflammation caused by PFCL could have contributed to the development of SO. Nevertheless, there are other possible causal factors such as the trauma, the vitrectomy itself or the choroidal detachment with possible uveal incarceration at wound sites.

[Symptomatic ciliary body melanocytoma: melanocytomalytic glaucoma]. / Melanocitoma de cuerpo ciliar sintomático: glaucoma melanocitomalítico.

CASE REPORT: A 33 year-old female with an asymptomatic pigmented mass in the iridocorneal angle of her right eye, arising from the ciliary body is presented. Ciliary body melanocytoma was suspected and conservative management recommended. After 36 months of follow-up the patient developed pain, inflammatory reaction and uncontrollable ocular hypertension, which was diagnosed as melanocytomalytic glaucoma. Tumor was removed by external iridecyclectomy and the histopathologic findings revealed necrotic melanocytoma. DISCUSSION: Ciliary body melanocytoma is a rare benign pigmented tumor that may present extension to the anterior chamber. Differential diagnosis mainly includes ciliary body melanoma, which carries a different prognosis and treatment.

[Experimental study on usefulness of silicone-fluorsilicone copolymer oil (SiFO) in vitreoretinal surgery]. / Estudio experimental sobre la utilidad del aceite de copolímero de silicona-fluorsilicona (SiFO) en la cirugía vitreorretiniana.

PURPOSE: To evaluate the usefulness of silicone-fluorsilicone copolymer oil (SiFO) as an intraoperative tool and a vitreous substitute in vitreoretinal surgery. METHODS: Handling properties of SiFO were tested and compared with those of perfluorooctane (PFO). The transparency of both substances was measured by spectrophotometry and subjectively assessed. Their tendency to dispersion was observed during injection in balanced saline solution (BSS) and after mechanized and manual shaking. Ease of injection and aspiration through small-gauge instruments was evaluated. Ocular tolerance to SiFO and PFO was studied after intravitreal injection in rabbit eyes: intraocular pressure, anterior segment inflammatory response and dispersion were evaluated, and a histopathological study was performed. RESULTS: Injection and aspiration of SiFO were more difficult than those of PFO because of its higher viscosity. PFO dispersed progressively into small droplets as early as two days after intravitreal injection, whereas SiFO remained as a single bubble for 14 days. Histopathologically both substances induced an inflammatory response over the inferior retina, with microvacuolated macrophages and foreign body giant cells, which were larger in eyes wearing SiFO. CONCLUSIONS: SiFO may be useful as an intraoperative tool, although its main drawback is a more difficult injection and aspiration compared to PFO. It has been well tolerated as a short-term vitreous substitute, but further clinical studies are needed.