CLINICALLY INVISIBLE RETINAL HEMANGIOBLASTOMAS DETECTED BY SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY AND FLUORESCEIN ANGIOGRAPHY IN TWINS.

PURPOSE: To report subclinical retinal hemangioblastoma detected by enhanced depth imaging optical coherence tomography and fluorescein angiography in at-risk twins. METHODS: Case report. RESULTS: A set of twins, age 7 years, (Twin A and Twin B) with known family history of von Hippel-Lindau disease (gene test positive) and no systemic manifestations were evaluated. Visual acuity was 20/20 in both eyes of both twins. Anterior segment examination and intraocular pressures were unremarkable in both eyes. Twin A showed no clinically visible tumor in the right eye, and a clinically evident 4-mm hemangioblastoma in the superior retina of the left eye. The enhanced depth imaging optical coherence tomography demonstrated normal fovea in both eyes. However, imaging at the inferonasal juxtapapillary region in the right eye documented an intraretinal mass from nerve fiber layer to outer plexiform layer on enhanced depth imaging optical coherence tomography and with hyperfluorescence on fluorescein angiography, consistent with retinal hemangioblastoma. Twin B demonstrated no clinically visible tumors in both eyes, but the left eye showed a small hyperreflective lesion in the parafoveal region on spectral domain optical coherence tomography from inner to outer nuclear layers, with no cystoid changes or subretinal fluid. The lesion was slightly hyperfluorescent on fluorescein angiography, consistent with hemangioblastoma. The optical coherence tomography angiography showed no vascularity within the lesion. Twin A was treated with laser photocoagulation to the larger hemangioblastoma in the left eye, and the asymptomatic juxtapapillary tumor was observed. Twin B was managed with cautious observation as treatment to the left eye could lead to vision loss. CONCLUSION: Patients at risk for retinal hemangioblastoma should have routine imaging with fundus photography, fluorescein angiography, and enhanced depth imaging optical coherence tomography for subclinical detection of asymptomatic tumors.

Clinical Features Differentiating Benign From Malignant Conjunctival Tumors in Children.

IMPORTANCE: Conjunctival tumors in children are usually benign and rarely malignant. OBJECTIVE: To evaluate clinical features of conjunctival tumors in children by comparing benign tumors with their malignant counterparts. DESIGN, SETTING, AND PARTICIPANTS: This retrospective case series reviewed 806 cases of conjunctival tumor in children (aged <21 years) who were evaluated at a tertiary referral center between November 1, 1975, and July 1, 2015. This study included 262 children who were part of a published review. MAIN OUTCOMES AND MEASURES: Features of benign and malignant tumors were compared. Data were collected on patient demographics, tumor features, and specific diagnoses to determine findings related to each tumor. RESULTS: Among the 806 patients with conjunctival tumor, the top 5 diagnoses included nevus (492 [61%]), benign reactive lymphoid hyperplasia (BRLH) (38 [5%]), nodular conjunctivitis (31 [4%]), dermoid (30 [4%]), and primary acquired melanosis (27 [3%]). Overall, conjunctival tumors were benign (779 [97%]) or malignant (27 [3%]), including melanoma (18 [2.2%]) and lymphoma (9 [1.1%]). The mean age at detection was 11 years for benign tumors and 14 years for malignant tumors (P = .005), with mean difference of 3 years (95% CI, 1.2-4.6). The relative frequency of any malignancy (per all conjunctival tumors) by age bracket (0-5 years, >5-10 years, >10-15 years, and >15-<21 years) was 1%, 2%, 3%, and 7%, respectively. A comparison between nevus and melanoma found differences with melanoma in the 10 to 15 years age bracket (29% vs 61%; difference of 32% [95% CI, 10%-55%]; P = .006), mean tumor thickness (1.1 mm vs 1.5 mm; difference of 0.4 mm [95% CI, -0.29 mm to 1.12 mm]; P = .04), tumor base of 10 mm or greater (relative risk [RR] = 4.92; 95% CI, 1.73-13.97; P = .003), tumor hemorrhage (RR = 25.30; 95% CI, 11.91-53.78; P < .001), and lack of intrinsic cysts (RR = 5.06; 95% CI, 1.84-13.98; P = .002). A comparison between BRLH and lymphoma revealed lymphoma with a larger base (RR = 5.16; 95% CI, 1.19- 22.19; P = .002) and diffuse location (RR = 16.50; 95% CI, 4.31-63.22; P < .001) and inferior (RR = 12.38; 95% CI, 2.88-53.16; P < .001) or superior vs nasal (RR = 8.25; 95% CI, 1.56-43.51; P = .01). The small cohort of malignant lesions precluded determining if these features were independent of one another. CONCLUSIONS AND RELEVANCE: These data, from an ocular tertiary referral center, suggest that conjunctival tumors in children are nearly always benign. The few malignant tumors included melanoma and lymphoma. Melanoma, compared with nevus, was associated with older children (aged >10-15 years) with larger tumor, hemorrhage, and lack of cyst. Lymphoma, compared with BRLH, was associated with larger size and diffuse involvement.

Conjunctival Tumors in 5002 Cases. Comparative Analysis of Benign Versus Malignant Counterparts. The 2016 James D. Allen Lecture.

PURPOSE: To evaluate frequency of conjunctival tumors in all ages and compare benign vs malignant counterparts. DESIGN: Retrospective series. METHODS: setting: Tertiary referral center. STUDY POPULATION: Total of 5002 patients. OBSERVATION: Clinical features. MAIN OUTCOME MEASURE: Differentiation of benign from malignant counterparts. RESULTS: The tumor was benign (52%), premalignant (18%), or malignant (30%). Malignant tumors included melanoma (12%), squamous cell carcinoma (SCC) (9%), lymphoma (7%), and others. Comparison of primary acquired melanosis vs melanoma revealed melanoma with greater median patient age (54 vs 61 years, P < .0001), male sex (35% vs 49%, P < .0001), location in fornix (2% vs 6%, P = .0016) and tarsus (1% vs 4%, P = .0018), larger median basal diameter (6 vs 8 mm, P < .0001) and thickness (<1 vs 1 mm, P < .0001), and intralesional cysts (0% vs 7%, P < .0001), feeder vessels (10% vs 48%, P < .0001), intrinsic vessels (4% vs 33%, P < .0001), and hemorrhage (<1% vs 3%, P = .0001). Comparison of conjunctival intraepithelial neoplasia (CIN) vs SCC revealed SCC with greater diffuse involvement (1% vs 8%, P < .0001) and larger median basal diameter (7 vs 8 mm, P < .0001) and thickness (1 mm vs 2 mm, P < .0001). Comparison of benign reactive lymphoid hyperplasia vs lymphoma revealed lymphoma with greater median patient age (50 vs 61 years, P < .0001), fornix location (32% vs 54%, P < .0001), larger median basal diameter (10 vs 20 mm, P < .0001), and less involvement of nasal region (50% vs 23%, P < .0001). CONCLUSION: In an ocular oncology practice, conjunctival tumors are benign (52%), premalignant (18%), or malignant (30%). Malignant tumors tend to occur in older patients and demonstrate greater basal diameter and thickness, compared with benign counterparts.