Aim To describe the 5-year profile of anatomic critical diagnoses from an ophthalmic pathology laboratory and raise awareness of the challenges of establishing guidelines for these diagnoses. Methods Medical records of patients who had consecutively submitted surgically removed globes or eviscerated eyes from 1 October 2009 to 31 October 2014 were examined for a critical diagnosis, as defined by a verbal communication for a serious, unanticipated diagnosis.Important discordant anatomic and clinical diagnoses were reviewed to determine whether the anatomic finding was truly unanticipated. Results During the study period, 313 eyes were submitted to the laboratory as primary specimens. https://www.selleckchem.com/products/danicopan.html Twenty (6.4%) had critical (alert) diagnoses. Six of the 20 anatomic diagnoses (30%) were known or suspected prior to surgery but were not communicated on the pathology request form. Five diagnoses (25%) were not clinically suspect before surgery. In 9 cases (45%) medical-care providers were alerted to the critical findings but insufficient clinical information was provided about preoperative conditions. Conclusions The proportion of critical diagnoses among surgically removed eyes is small, but not inconsequential. Some "critical alerts" would be unnecessary if relevant clinical information was provided when the tissue is submitted to the laboratory. Laboratory guidelines for critical values in surgical pathology should be flexible since they need to anticipate the vicissitudes of clinical practice. Surgeons need to appreciate that relevant clinical information must be provided to pathologists because it can play a role in formulating anatomic diagnoses. Copyright © 2019 by S. Karger AG, Basel.Neoplasms of the retinal pigment epithelium (RPE) are rare tumors that can simulate choroidal melanoma, but clinical and imaging characteristics often differentiate these lesions. We report a 70-year-old male with an abruptly elevated pigmented lesion that arose at the site of congenital hypertrophy of the RPE and demonstrated associated exudation, as well as feeding and draining vessels, suggestive of RPE adenoma. Optical coherence tomography showed retinal elevation with serous retinal detachment adjacent to the mass, and ultrasonography revealed an abruptly elevated, moderately echodense mass of 6.4-mm thickness. Fluorescein -angiography showed early tumor hypofluorescence, late -tumor hyperfluorescence with staining and leakage, and -retinal vessels buried under the mass, suggestive of a retinal tumor. The patient was monitored with the presumed diagnosis of RPE adenoma, but 3 months later, the growth was documented and fine-needle aspiration biopsy revealed choroidal melanoma. Management with I-125 plaque radiotherapy was performed leading to tumor regression and a thickness of 4.6 mm. Copyright © 2019 by S. Karger AG, Basel.Background Conjunctival melanoma is a potentially lethal malignancy of the ocular surface. There have been no therapeutic advancements made in the past several decades despite increasing prevalence of the disease. Methods The authors report the case of a 52-year-old Caucasian male with unresectable, recurrent conjunctival melanoma with V600 BRAF mutation who was treated with systemic BRAF/MEK inhibition. Results There was complete regression of local disease within the first 9 months. The patient remains without local recurrence or systemic metastasis at 1 year. Conclusion This is the first reported case of conjunctival melanoma with complete response to BRAF/MEK inhibition. As long as targeted therapy remains an option, patients with conjunctival melanoma should undergo mutational profiling of their tumor. Copyright © 2019 by S. Karger AG, Basel.Background There are limited reports of uveal "melanocytic" lesions in patients with oculocutaneous albinism, with no reports from Asia. Objectives In this study, we report 3 eyes with uveal "melanocytic" lesions in Asian patients with oculocutaneous albinism. Methods Retrospective small case series. Three eyes of 2 Asian patients with oculocutaneous albinism were included. Case 1 was a 54-year-old female who presented with juxtapapillary choroidal melanoma and underwent enucleation. Case 2 was a 39-year-old male with pigmented choroidal nevus in the right eye and suspicious nevus/choroidal variegation in the left eye. Results For case 1, metastatic workup including ultrasound of the abdomen, liver function test, and chest X-ray was unremarkable. Magnetic resonance imaging of the cranium ruled out extraocular extension. The left eye was enucleated due to the large tumor size and poor visual potential, and histopathological examination confirmed the diagnosis of choroidal melanoma with mixed cell type. At the last follow-up 6 months after enucleation, there was no evidence of metastasis. Case 2 was observed. Conclusion Pigmented choroidal nevus and melanoma are rarely observed in patients with oculocutaneous albinism. Copyright © 2019 by S. Karger AG, Basel.A 20-year-old boy presented with a mass superotemporal to the right eyebrow of 1 month's duration without a history of recent trauma. Complete examination and workup, including laboratory analysis, Doppler ultrasound imaging, and magnetic resonance imaging, suggested a diagnosis of juvenile temporal arteritis. Excisional biopsy and histopathology contradicted the preoperative workup and yielded the final diagnosis of angiolymphoid hyperplasia with eosinophilia, a rare eosinophilic dermatosis of unknown etiology. This case highlights the need for better characterization of temporal vascular tumors in the pediatric population. Copyright © 2019 by S. Karger AG, Basel.Choroidal melanoma (CM) is the most commonly diagnosed primary intraocular malignancy in adults. Gamma knife radiosurgery (GKR) has demonstrated reliable results in the treatment of CM, but it is limited by the risk of radiation-induced ocular complications. To optimize the dose of radiation focused on the tumor, and limit side effects, the eye should be properly immobilized before treatment. A recently released ultra-widefield imaging instrument (Optomap California; Optos, Dunfermline, Scotland, UK) allows for an interactive three-dimensional (3D) virtual reconstruction of the globe, called 3D Wrap. The authors share their experience with this rapid, reliable, and relatively easy support in planning globe positioning before GKR treatment. Copyright © 2019 by S. Karger AG, Basel.
Aim To describe the 5-year profile of anatomic critical diagnoses from an ophthalmic pathology laboratory and raise awareness of the challenges of establishing guidelines for these diagnoses. Methods Medical records of patients who had consecutively submitted surgically removed globes or eviscerated eyes from 1 October 2009 to 31 October 2014 were examined for a critical diagnosis, as defined by a verbal communication for a serious, unanticipated diagnosis.Important discordant anatomic and clinical diagnoses were reviewed to determine whether the anatomic finding was truly unanticipated. Results During the study period, 313 eyes were submitted to the laboratory as primary specimens. https://www.selleckchem.com/products/danicopan.html Twenty (6.4%) had critical (alert) diagnoses. Six of the 20 anatomic diagnoses (30%) were known or suspected prior to surgery but were not communicated on the pathology request form. Five diagnoses (25%) were not clinically suspect before surgery. In 9 cases (45%) medical-care providers were alerted to the critical findings but insufficient clinical information was provided about preoperative conditions. Conclusions The proportion of critical diagnoses among surgically removed eyes is small, but not inconsequential. Some "critical alerts" would be unnecessary if relevant clinical information was provided when the tissue is submitted to the laboratory. Laboratory guidelines for critical values in surgical pathology should be flexible since they need to anticipate the vicissitudes of clinical practice. Surgeons need to appreciate that relevant clinical information must be provided to pathologists because it can play a role in formulating anatomic diagnoses. Copyright © 2019 by S. Karger AG, Basel.Neoplasms of the retinal pigment epithelium (RPE) are rare tumors that can simulate choroidal melanoma, but clinical and imaging characteristics often differentiate these lesions. We report a 70-year-old male with an abruptly elevated pigmented lesion that arose at the site of congenital hypertrophy of the RPE and demonstrated associated exudation, as well as feeding and draining vessels, suggestive of RPE adenoma. Optical coherence tomography showed retinal elevation with serous retinal detachment adjacent to the mass, and ultrasonography revealed an abruptly elevated, moderately echodense mass of 6.4-mm thickness. Fluorescein -angiography showed early tumor hypofluorescence, late -tumor hyperfluorescence with staining and leakage, and -retinal vessels buried under the mass, suggestive of a retinal tumor. The patient was monitored with the presumed diagnosis of RPE adenoma, but 3 months later, the growth was documented and fine-needle aspiration biopsy revealed choroidal melanoma. Management with I-125 plaque radiotherapy was performed leading to tumor regression and a thickness of 4.6 mm. Copyright © 2019 by S. Karger AG, Basel.Background Conjunctival melanoma is a potentially lethal malignancy of the ocular surface. There have been no therapeutic advancements made in the past several decades despite increasing prevalence of the disease. Methods The authors report the case of a 52-year-old Caucasian male with unresectable, recurrent conjunctival melanoma with V600 BRAF mutation who was treated with systemic BRAF/MEK inhibition. Results There was complete regression of local disease within the first 9 months. The patient remains without local recurrence or systemic metastasis at 1 year. Conclusion This is the first reported case of conjunctival melanoma with complete response to BRAF/MEK inhibition. As long as targeted therapy remains an option, patients with conjunctival melanoma should undergo mutational profiling of their tumor. Copyright © 2019 by S. Karger AG, Basel.Background There are limited reports of uveal "melanocytic" lesions in patients with oculocutaneous albinism, with no reports from Asia. Objectives In this study, we report 3 eyes with uveal "melanocytic" lesions in Asian patients with oculocutaneous albinism. Methods Retrospective small case series. Three eyes of 2 Asian patients with oculocutaneous albinism were included. Case 1 was a 54-year-old female who presented with juxtapapillary choroidal melanoma and underwent enucleation. Case 2 was a 39-year-old male with pigmented choroidal nevus in the right eye and suspicious nevus/choroidal variegation in the left eye. Results For case 1, metastatic workup including ultrasound of the abdomen, liver function test, and chest X-ray was unremarkable. Magnetic resonance imaging of the cranium ruled out extraocular extension. The left eye was enucleated due to the large tumor size and poor visual potential, and histopathological examination confirmed the diagnosis of choroidal melanoma with mixed cell type. At the last follow-up 6 months after enucleation, there was no evidence of metastasis. Case 2 was observed. Conclusion Pigmented choroidal nevus and melanoma are rarely observed in patients with oculocutaneous albinism. Copyright © 2019 by S. Karger AG, Basel.A 20-year-old boy presented with a mass superotemporal to the right eyebrow of 1 month's duration without a history of recent trauma. Complete examination and workup, including laboratory analysis, Doppler ultrasound imaging, and magnetic resonance imaging, suggested a diagnosis of juvenile temporal arteritis. Excisional biopsy and histopathology contradicted the preoperative workup and yielded the final diagnosis of angiolymphoid hyperplasia with eosinophilia, a rare eosinophilic dermatosis of unknown etiology. This case highlights the need for better characterization of temporal vascular tumors in the pediatric population. Copyright © 2019 by S. Karger AG, Basel.Choroidal melanoma (CM) is the most commonly diagnosed primary intraocular malignancy in adults. Gamma knife radiosurgery (GKR) has demonstrated reliable results in the treatment of CM, but it is limited by the risk of radiation-induced ocular complications. To optimize the dose of radiation focused on the tumor, and limit side effects, the eye should be properly immobilized before treatment. A recently released ultra-widefield imaging instrument (Optomap California; Optos, Dunfermline, Scotland, UK) allows for an interactive three-dimensional (3D) virtual reconstruction of the globe, called 3D Wrap. The authors share their experience with this rapid, reliable, and relatively easy support in planning globe positioning before GKR treatment. Copyright © 2019 by S. Karger AG, Basel.
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