Spitz nevus arising in the eyelid of a teenager.

A 16-year-old boy developed over a 2-month interval a lightly pigmented left upper eyelid lesion measuring 1.5 mm in greatest diameter that, when excised, microscopically was hypercellular and composed almost exclusively of nonpigmented epithelioid cells that created florid, large intraepidermal junctional nests and sheets and nests of subepidermal cells. The diagnosis was a Spitz nevus. HMB-45, MART-1, and microphthalmia-associated transcription factor were all positive and established the melanocytic nature of the benign tumor. The Ki-67 proliferation index (5%) and 2 mitoses/mm(2) were both low; p16 protein was immunohistochemically identified in the nevoid cells. We review the clinical, histopathologic, and other immunohistochemical features of this entity and provide a brief differential diagnosis (including separation from a Spitzoid melanoma). This is only the third eyelid Spitz nevus reported in the literature and is the most fully characterized immunohistochemically. At their present stage of development, contemporary immunohistochemical biomarkers, while providing supplemental information, nonetheless remain less than definitive in terms of reliably distinguishing benign from malignant Spitz lesions.

CLASSIFICATION OF SCLEROCHOROIDAL CALCIFICATION BASED ON ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY "MOUNTAIN-LIKE" FEATURES.

PURPOSE: To describe distinct enhanced depth optical coherence tomography patterns of sclerochoroidal calcification and their correlation to clinical features. METHODS: Retrospective chart review of 67 eyes of 46 patients with spectral domain optical coherence tomography imaging. RESULTS: The mean patient age at diagnosis was 68 years. There were 20 (43%) men and 26 (57%) women of white (n = 45, 98%) or Hispanic (n = 1, 2%) heritage. The most prominent sclerochoroidal calcification lesions were located in the superotemporal quadrant (n = 57, 85%) between the temporal arcades and the equator (n = 58, 87%). On enhanced depth optical coherence tomography, the sclerochoroidal calcification was located within the sclera in all cases and the inner surface topography assumed specific "mountain-like" patterns, including flat (Type 1) (n = 9) at median thickness of 1.2 mm, rolling (Type 2) (n = 28) at 1.4 mm thickness, rocky-rolling (Type 3) (n = 21) at 2.1 mm thickness, and table mountain (Type 4) (n = 9) at a thickness of 1.9 mm. The retinal layers were undisturbed in flat lesions, and outer retinal abnormalities were found in all other types. A comparison of the 4 types revealed that Type 3 lesions were thickest (P < 0.001), showing abnormalities in the retinal pigment epithelium, ellipsoid region, and external limiting membrane most commonly (P < 0.05) and demonstrating the most dramatic thinning of the overlying choroid (P < 0.01) and retina (P < 0.05). Type 4 lesions showed greatest basal diameter (P < 0.01) and least outer retinal abnormalities (P < 0.05) or choroid thinning (P < 0.05). CONCLUSION: In this report, enhanced depth optical coherence tomography has demonstrated that sclerochoroidal calcification is a scleral-based disease and can be classified based on four "mountain-like" topographic patterns, associated with variable effects on the choroid and retina.

Sclerochoroidal calcification: clinical features, outcomes, and relationship with hypercalcemia and parathyroid adenoma in 179 eyes.

PURPOSE: To describe the clinical features and long-term ophthalmic and systemic findings in patients with sclerochoroidal calcification (SCC). METHODS: Retrospective non-interventional clinical chart review of 179 eyes of 118 patients with SCC to evaluate for the relationship of SCC with systemic calcium metabolic abnormalities. RESULTS: The mean patient age at diagnosis was 69 years. There were 47 (40%) men and 71 (60%) women of Caucasian (n = 116, 98%) and Hispanic (n = 2, 2%) heritage. The condition was unilateral in 57 patients (48%) and bilateral in 61 (52%), with a mean of 1.6 lesions per eye (range, 1-7 lesions per eye). The referring diagnosis was choroidal nevus (n = 23, 20%), melanoma (n = 15, 13%), lymphoma (n = 12, 10%), metastasis (n = 6, 5%), osteoma (n = 4, 3%), SCC (n = 6, 5%), and no diagnosis (n = 51, 43%). Of 277 SCC lesions, the most common location was superotemporal quadrant (n = 191, 69%). The largest lesion per eye demonstrated mean basal diameter of 3.6 mm and thickness of 1.8 mm, with yellow or white color (n = 150 lesions, 84%) and located superiorly (n = 105, 61%) at the retinal vascular arcade or near the equator (n = 161, 94%). The lesion demonstrated overlying focal choroidal atrophy (n = 63, 35%) and retinal pigment epithelium atrophy (n = 88, 49%). There was no case of subretinal fluid, hemorrhage, or choroidal neovascular membrane. At mean 4 years follow up, there was no lesion enlargement, decalcification, or related subretinal fluid/hemorrhage, choroidal neovascularization, or vision loss. Ocular treatment was not necessary in any case. Systemic outcomes revealed hyperparathyroidism (n = 9/33, 27%) with parathyroid adenoma (n = 5/33, 15%), Bartter syndrome (n = 1/53, 2%), or Gitelman syndrome (n = 6/53, 11%). CONCLUSIONS: Sclerochoroidal calcification is a stable deposition of calcium in the sclera that, unlike choroidal osteoma, has minimal risk for vision loss. All patients with SCC should be evaluated for underlying systemic calcium disorders, especially parathyroid and renal metabolic conditions.

Review of cystic and solid tumors of the iris.

Iris tumors are broadly classified into cystic or solid lesions. The cystic lesions arise from iris pigment epithelium (IPE) or iris stroma. IPE cysts classically remain stable without need for intervention. Iris stromal cyst, especially those in newborns, usually requires therapy of aspiration, possibly with alcohol-induced sclerosis, or surgical resection. The solid tumors included melanocytic and nonmelanocytic lesions. The melanocytic iris tumors include freckle, nevus (including melanocytoma), Lisch nodule, and melanoma. Information from a tertiary referral center revealed that transformation of suspicious iris nevus to melanoma occurred in 4% by 10 years and 11% by 20 years. Risk factors for transformation of iris nevus to melanoma can be remembered using the ABCDEF guide as follows: A=age young (<40 years), B=blood (hyphema) in anterior chamber, C=clock hour of mass inferiorly, D=diffuse configuration, E=ectropion, F=feathery margins. The most powerful factors are diffuse growth pattern and hyphema. Tumor seeding into the anterior chamber angle and onto the iris stroma are also important. The nonmelanocytic iris tumors are relatively uncommon and included categories of choristomatous, vascular, fibrous, neural, myogenic, epithelial, xanthomatous, metastatic, lymphoid, leukemic, secondary, and non-neoplastic simulators. Overall, the most common diagnoses in a clinical series include nevus, IPE cyst, and melanoma. In summary, iris tumors comprise a wide spectrum including mostly iris nevus, IPE cyst, and iris melanoma. Risk factors estimating transformation of iris nevus to melanoma can be remembered by the ABCDEF guide.

Clinical survey of 3680 iris tumors based on patient age at presentation.

OBJECTIVE: To report the spectrum of iris lesions based on patient age at presentation. DESIGN: Retrospective, nonrandomized, single-center case series. PARTICIPANTS: We included 3680 iris tumors in 3451 patients. METHODS: Chart review. MAIN OUTCOME MEASURES: Diagnostic category based on age. RESULTS: The mean age at presentation was 48 years and there were 449 (12%) tumors in children (≤20 years), 788 (21%) in young adults (21-40 years), 1308 (36%) in mid adults (41-60 years), and 1135 (31%) in senior adults (>60 years). Of 3680 tumors, the diagnostic category was cystic (n = 768; 21%) or solid (n = 2912; 79%). The cystic tumors originated from iris pigment epithelium (IPE; n = 672; 18%) or iris stroma (n = 96; 3%). The solid tumors included melanocytic (n = 2510; 68%) and nonmelanocytic (n = 402; 11%). The melanocytic tumors comprised nevus (n = 1503; 60%), melanocytoma (n = 68; 3%), melanoma (n = 645; 26%), and melanocytosis (n = 64; 3%). Of 2510 melanocytic tumors, the first and second most common diagnoses by age (children, young adult, mid adult, senior adult) were nevus (53%, 57%, 63%, and 63%, respectively) and melanoma (17%, 27%, 26%, and 27%, respectively). The nonmelanocytic tumors included categories of choristomatous (n = 4; <1%), vascular (n = 57; 2%), fibrous (n = 2; <1%), neural (n = 3; <1%), myogenic (n = 2;, <1%), epithelial (n = 35; 1%), xanthomatous (n = 8; <1%), metastasis (n = 67; 2%), lymphoid (n = 12; <1%), leukemic (n = 2; <1%), secondary (n = 12; <1%), and nonneoplastic simulators (n = 198; 5%). The median age (in years) at diagnosis included cystic (39), melanocytic (52), choristomatous (0.7), vascular (56), fibrous (53), neural (8), myogenic (42), epithelial (63), xanthomatous (1.9), metastasis (60), lymphoid (57), leukemic (25.5), secondary (59), and nonneoplastic simulators (49). Overall, the 3 most common specific diagnoses (children, young adult, mid adult, senior adult) were nevus (25%, 36%, 47%, and 47%, respectively), IPE cyst (28%, 30%, 15%, and 14%, respectively), and melanoma (8%, 16%, 20%, and 19%, respectively). CONCLUSIONS: In an ocular oncology practice, the spectrum of iris tumors includes cystic (21%) and solid (79%) tumors. The solid tumors were melanocytic (68%) or nonmelanocytic (11%). At all ages, the most common specific diagnoses were nevus (42%), IPE cyst (19%), and melanoma (17%).