Faculty Bibliography

Fundus autofluorescence imaging (FAF) in a case of diffuse uveal melanocytic proliferation is described in this study. It is a rare chorioretinopathy associated with systemic cancer, for which the exact pathological mechanisms are poorly understood. FAF-imaging revealed a diffuse background of hyper-autofluorescence associated with diffuse orange pigment deposition and islands of persistent hypo-fluorescence corresponding to loss of retinal pigment epithelium (RPE). In this disorder, increasingly smaller spots of FAF hypo-fluorescence were found from the center to the periphery of the affected retina. Fluorescein angiography demonstrated a negative of the FAF-images. FAF hypo-autofluorescence corresponded to optical coherence tomography (OCT) thinning or absence of the RPE-layer. Conversely, FAF hyper-autofluorescence correlated to thickening of the RPE-layer on OCT. The case demonstrates that FAF can be useful for the diagnosis of diffuse uveal melanocytic proliferation and offers greater insight into the pathophysiology of this disease

OBJECTIVE: To correlate combined optical coherence tomography (OCT) and scanning laser ophthalmoscope (SLO) imaging to clinical and histopathologic characteristics of optic disc melanocytoma (ODM). DESIGN: Retrospective, consecutive clinical case analysis. PARTICIPANTS: Twenty patients with ODM were evaluated. TESTING: All underwent ophthalmic examinations including a best-corrected visual acuity, slit-lamp examination, dilated ophthalmoscopy, fundus photography with angiography, B-scan ultrasonography, and combined OCT and SLO imaging. MAIN OUTCOME MEASURES: One eye, treated by enucleation, was evaluated by pathologic analysis and was correlated to its combined OCT and SLO images. These findings were correlated to clinical and OCT information from 19 patients with ODM. RESULTS: Histopathologic and combined OCT and SLO features included: disruption of the internal limiting membrane, disorganization of the retina (overlying the tumor), and visualization of the tumor's subretinal surface. Compressive and infiltrative changes in the optic nerve, peripapillary choroid, and retina were correlated directly to combined OCT and SLO images. To varying degrees, these histopathologic findings were seen on combined OCT and SLO. However, the pigment of the ODMs blocked OCT reflectance deep to the tumor's surface. CONCLUSIONS: This study demonstrated that OCT and SLO findings could be correlated directly with histopathologic characteristics. Imaging with OCT can be used to diagnose and correlate secondary retinal, superficial, and subretinal tumor characteristics. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article

PURPOSE: To determine the reliability and role of conjunctival exfoliative cytologic and histopathologic diagnosis of biopsied tissue in ocular surface squamous neoplasia. DESIGN: Retrospective review of an interventional case series of patients biopsied and treated for squamous conjunctival and corneal neoplasia. METHODS: Forty-nine patients who underwent conjunctival cytologic analysis (n = 36), conjunctival biopsy (n = 35), or both were evaluated. For the purposes of this study, three ocular pathologists reviewed the results of cytologic and biopsied tissue in a masked fashion. RESULTS: Evaluation of cytologic smears revealed a 91% concordance in interpretation of conjunctival cytologic material as no dysplasia vs dysplasia. The concordance dropped to 59% in grading the degree of dysplasia. The cytologic material was found to be inadequate for interpretation in 1 case. Evaluation of subsequent biopsy revealed a 98% concordance between the pathologists in interpretation of biopsied tissue as no dysplasia vs any degree of dysplasia. The concordance decreased to 83% in grading the degree of dysplasia. Cytologic evaluation was capable of distinguishing a neoplastic from nonneoplastic process before tissue biopsy in 80% of cases. CONCLUSIONS: Ocular surface cytologic analysis is a simple, safe, and relatively noninvasive diagnostic tool. It was found helpful in detecting dysplasia before surgical resection. It was used in the settings of recurrent tumor and for follow-up care of patients treated with topical chemotherapy. Although cytologic smears cannot replace incisional or excisional biopsy for definitive diagnosis, exfoliative cytologic analysis can play an important role in the diagnosis and management of patients with ocular surface squamous neoplasia

PURPOSE: To examine the incidence of retinoblastoma for regional variations by race. METHODS: Age-adjusted incidence rates, rate ratios, and 95% confidence intervals were determined in 109 regions from 1993 to 1997 using compiled data from the International Agency for Research on Cancer. RESULTS: The rate ratio was 1.12 (range: 0.35 to 4.15) between white populations in the United States and Europe/Australia, 0.98 (range: 0.37 to 2.65) between Hispanic populations in Spain and the United States, and 1.44 (range: 0.29 to 1.79) between Hispanic populations in Uruguay and the United States. The rate ratio between Hispanic and white populations within the United States was 0.94 (range: 0.33 to 2.56). No differences were significant. CONCLUSIONS: Because retinoblastoma is a genetic disease, its incidence is similar among varied populations

Radiation therapy is widely used for the treatment of orbital tumors and inflammatory disease. Both external beam teletherapy and implant brachytherapy radiation techniques are employed. External beam radiation therapy is the most common. It involves directing an external radiation source towards the eye, sinuses, and orbit. Whereas most patients are treated with linear accelerator-derived external beam radiation therapy, proton, neutron stereotactic radiosurgery, gamma knife, and intensity-modulated radiation therapy have become more available in developed countries. Radiation can be used alone or together with surgery or chemotherapy. Implant radiation therapy (brachytherapy) is also used to treat orbital tumors. Brachytherapy involves surgical placement of radiation sources within the tumor or targeted volume. Characteristically conformal, brachytherapy increases the dose within the target while maximally decreasing exposure of normal tissues. Orbital brachytherapy can be used to boost the dose to the target volume prior to orbital external beam radiation therapy. Herein, I explore the unique challenges associated with irradiation of the orbit, basic radiobiology, doses, indications, and results of treatment. The tolerances of normal ocular and orbital tissues are reported. This review of the literature offers a unique perspective, synthesizing the world's experience on the use of orbital radiation therapy

CONTEXT: The ocular adnexal lymphomas (OAL) arise in the conjunctiva, orbit, lacrimal gland, and eyelids. To date, they have been clinically staged using the Ann Arbor staging system, first designed for Hodgkin and later for nodal, non-Hodgkin lymphoma. The Ann Arbor system has several shortcomings, particularly when staging extranodal non- Hodgkin lymphomas, such as OAL, which show different dissemination patterns from nodal lymphomas. OBJECTIVE: To describe the first TNM-based clinical staging system for OAL. DESIGN: Retrospective literature review. RESULTS: We have developed, to our knowledge, the first American Joint Committee on Cancer-International Union Against Cancer TNM-based staging system for OAL to overcome the limitations of the Ann Arbor system. Our staging system defines disease extent more precisely within the various anatomic compartments of the ocular adnexa and allows for analysis of site-specific factors not addressed previously. It aims to facilitate future studies by identifying clinical and histomorphologic features of prognostic significance. This system is for primary OAL only and is not intended for intraocular lymphomas. CONCLUSIONS: Our TNM-based staging system for OAL is a user-friendly, anatomic documentation of disease extent, which creates a common language for multicenter and international collaboration. Data points will be collected with the aim of identifying biomarkers to be incorporated into the staging system

CONTEXT: The American Joint Committee on Cancer (AJCC) and the International Union Against Cancer commissioned the Ophthalmic Oncology Task Force to modify and update the ophthalmic chapters of the 7th edition of the AJCC Cancer Staging Manual. OBJECTIVE: To review the existing eyelid carcinoma chapter in the 6th edition of the AJCC Cancer Staging Manual for its clinical and research utility and to seek evidence-based revisions with the strongest medical foundation to use in updating the anatomically based TNM cancer staging system manual. DATA SOURCES: The 4-year Ophthalmic Oncology Task Force consisted of 45 tumor specialists from 10 countries and an extensive internal and external peer review process. The 10-member Carcinoma of the Eyelid team included a diverse group of international authors. The group included extensive representation by clinicians, pathologists, surgeons, radiation therapists, and cancer registrars, all with advanced, ophthalmic cancer-related areas of subspecialty. Data sources included the above expertise applying a worldwide medical literature search, with no discrimination based on language, country of origin, discipline source, specialty source, or surgical practice. CONCLUSIONS: Revisions were made to the TNM classification in areas with the strongest basis in evidence and practical effect. Lymph node staging data were expanded markedly to reflect its significant prognostic value. T3 and T4 were redefined and stage groupings were added that applied current understanding in tumor biology, respected site-specific risk factors, and provided greater correlation with the common language of the overall AJCC Cancer Staging Manual. Evidence-based biomarkers and data-field modifiers were included to capture additional pathologically and clinically substantiated prognostic factors