Mean Tumor Basal Diameter Research Articles

Neoadjuvant chemotherapy for advanced eyelid and periocular sebaceous gland carcinoma: a study of 25 cases.

To report the outcomes of platinum-based neoadjuvant chemotherapy (NACT) for eyelid and periocular sebaceous gland carcinoma (eSGC). Retrospective study of 25 patients. The mean age at presentation of eSGC was 59years. The mean tumor basal diameter was 46mm. By the 8th edition of AJCC classification, tumors belonged T2 (n = 2, 8%), T3 (n = 6, 24%), and T4 (n = 17, 68%); N1 (n = 12,48%); and M1 (n = 1, 4%). NACT with 5-fluorouracil (5-FU) and cisplatin/carboplatin was administered in 21 (84%)/4 (16%) patients, respectively. The mean number of cycles of neoadjuvant systemic chemotherapy per patient was 2 (median, 3). The mean percentage reduction of tumor basal volume after neoadjuvant chemotherapy was 65% (median, 60%). After NACT, 12 (48%) patients underwent surgical treatment, 6 (12%) patients underwent EBRT, and 4 (8%) underwent adjuvant chemotherapy. A total of 11 (44%) patients were lost to follow-up during the course of treatment, of whom 3 died from metastatic disease. In 16 patients followed up for ≥ 3months, complete tumor control was achieved in 11 (69%) patients, local tumor control in 14 (88%), and globe salvage in 7 (44%) at a mean follow-up of 25months (median, 7months; range, 3 to 110months). No tumor recurrence was seen in any case. One (4%) serious adverse event of cardiotoxicity was noted. Platinum-based NACT is a suitable option for eSGC with advanced tumors and locoregional metastasis. Adverse events are rare and in patients compliant with treatment, NACT-based combination therapy offers globe salvage and systemic tumor control.

Correlation of fitzpatrick skin type and iris color with tumor size in 823 patients with uveal melanoma

PurposeTo determine the correlation of Fitzpatrick Skin Type (FST) and iris color with tumor size (tumor thickness and basal diameter) in patients with uveal melanoma. DesignRetrospective Cohort MethodsRetrospective cohort from a single ocular oncology center of 823 patients with uveal melanoma and documented FST, iris color, and tumor size. Patients were classified by FST (type I, II, and III–V) and iris color (blue, green, and brown) on the basis of external facial photography. There were no FST type VI patients. Tumor thickness was classified into small [< 3 millimeter (mm)], medium (3.1–8.0 mm), or large (> 8.0 mm), and basal diameter into small (< 10 mm), medium (10.1–15 mm) or large (> 15 mm). The correlation of FST and iris color with tumor thickness and basal diameter was evaluated using the Kruskal-Wallis H test. ResultsThe FST classification was type I (n = 92, 11%), type II (n = 643, 78%), or III–V (n = 88, 11%), and iris color was blue (n = 472, 57%), green (n = 102, 12%), or brown (n = 249, 30%). A comparison of FST revealed differences in mean tumor thickness (P = 0.04) and basal diameter (P = 0.006). Iris color showed no difference for mean tumor thickness (P = 0.41) or basal diameter (P = 0.48). There was a statistically significant difference with brown iris color relative to FST III–V for mean tumor thickness (P = 0.003) and basal diameter (P = 0.001) but no difference with blue or green iris color (P > 0.05). ConclusionsIris color alone showed no difference in tumor size, but those with brown iris color and FST type III–V demonstrated larger tumor thickness and basal diameter.

Retinoblastoma associated with total exudative retinal detachment: Treatment and outcomes.

To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma (RB) and total retinal detachment (RD). Retrospective study of 50 eyes in 43 patients. Mean age at presentation was 15 months (median, 9 months). RB associated total RD was unilateral (n=36; 84%) or bilateral (n=7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm) and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n=26; 52%), vitreous seeds (n=13; 26%), and subretinal exudation in (n=4; 8%). Based on 8th edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n=19; 38%), cT2b (n=28; 56%), or cT3c (n=3; 6%). Primary treatment included intravenous (n=48; 96%) or intra-arterial chemotherapy (n=2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7 to 62 months), RD resolved in 41 (82%) eyes, tumor recurrence was noted in 39 (78%) eyes, globe salvage was achieved in 35 (70%) eyes and one (2%) patient died due to metastasis. Relatively high tumor recurrence rate is noted in RB presenting with total RD. With appropriate treatment, 70% of the globes can be salvaged.

Yttrium-90 (90Y) Brachytherapy for Small Iridociliary Melanomas

<h3>Purpose/Objective(s)</h3> Beta irradiation has been useful in treating numerous ocular tumors and benign growths. However, strontium-90 applicators are no longer supported and ruthenium-106 is not available for high dose rate (HDR) brachytherapy. Herein we present the first clinical use of a new HDR ⁹⁰Y brachytherapy source that is FDA 510(k) cleared for the treatment of ocular tumors and benign growths. <h3>Materials/Methods</h3> The iWand A® is a new single-use ophthalmic hand-held brachytherapy applicator, with a surface contact diameter of 10 mm. In addition, the applicator's translucent polymer backing both attenuates radiation exposure towards the surgeon and ancillary staff and allows visualization of the disc-shaped ⁹⁰Y source during treatment. A well-shaped recess on the anterior surface of the applicator accommodates a 6-mm disc-shaped source, where it is flush-mounted and affixed by biocompatible adhesive. The titanium shell of the source provides rigid encapsulation of the radioactive ⁹⁰Y source. Though the LV Y-90 Disc is FDA cleared to be utilized at up to 20 millicuries (mCi); the activity used for these 2 treatments was mean 12.4 mCi. For example, a 20 mCi LV ⁹⁰Y Disc which decays by β-emission with an end point energy of 2.28 MeV (0.98 MeV average), yields 30.0, 21.6, 16.2, and 4.8 Gy/minute at depths of 0.0, 0.6, 1.0, and 2.6 mm, respectively. While planning tool simulations were used to demonstrate the treatment volume and penumbra characteristics of the ⁹⁰Y disc, NIST-traceable preoperative calibrations were performed by an independent authorized medical physicist. <h3>Results</h3> Two patients (mean age=74 years) were diagnosed with AJCC 8^th edition T1-sized, biopsy-proven iridociliary melanoma. High-frequency ultrasound was used to measure tumor size, basal dimensions and distance from the overlying sclera. Measurements revealed a mean maximum tumor thickness of 0.7 mm (0.6-0.8) and a mean maximum largest tumor basal diameter of 3.0 mm (2.9-3.1). Surgery required a minor conjunctival recession to expose the episcleral target zone. The 10 mm application site was marked with tissue dye on the cornea and sclera. Then the ⁹⁰Y source, affixed within the iWand A® applicator, was applied to deliver HDR episcleral brachytherapy to doses of 25 - 30 Gy to a 2.5-mm depth. Treatment durations ranged from 5 to 13 minutes. During treatment, the acrylic of the iWand A® applicator was found capable of shielding the patient, surgeon and operating-room personnel. No acute or post-operative ⁹⁰Y-radiation-related complications were noted. The ⁹⁰Y source's 64-hour half-life allowed for single-use, decay in storage, and return for disposal. <h3>Conclusion</h3> A novel, FDA-cleared, HDR ⁹⁰Y beta source was used to treat 2 patients with uveal melanoma. It was affixed within a hand-held, single-use applicator that aids guidance, targeting and facilitates radiation safety (as measured during both surgeries). Future research is needed to explore additional applications for this novel ⁹⁰Y HDR brachytherapy source.

Primary Macular Retinoblastoma: Clinical Presentation and Treatment Outcomes.

Purpose: To describe the clinical features and treatment outcomes in eyes with primary macular retinoblastoma (RB). Methods: Patients with primary macular RB were analyzed. Results: Of 41 patients (47 eyes), 20 (49%) were boys and 21 (51%) were girls. The mean age at diagnosis was 16 months (range, 1-60 months). The RB was bilateral in 6 patients (15%). At presentation, the macula was completely covered with the tumor in 22 eyes (47%) and partially covered with the fovea spared in 13 eyes (28%) and the fovea involved 12 eyes (25%). Based on the International Classification of Intraocular Retinoblastoma, 25 (53%), 15 (32%), and 7 (15%) of tumors were in Group B, Group C, and Group D, respectively. The tumor had exophytic features in 36 eyes (77%). The mean tumor basal diameter was 10.0 mm and the mean thickness, 5.6 mm. Associated features included subretinal seeds (10 eyes; 21%) and surrounding subretinal fluid (16 eyes; 34%). Forty-three eyes (92%) were treated with intravenous chemotherapy, 2 (4%) with intra-arterial chemotherapy, and 2 (4%) with transpupillary thermotherapy. Local tumor control was achieved in 45 eyes (96%), with 33 eyes (70%) showing a type III regression pattern. Over a mean follow-up of 23 months (range, 3-48 months), the macular tumor recurred in 5 eyes (11%), the globe was salvaged in all eyes with associated foveal atrophy (36 eyes; 77%), and 1 patient (2%) died. Conclusions: Macular RB has a good prognosis for globe salvage while vision salvage might be compromised as a result of associated foveal atrophy.

Retinoblastoma in older patients: A retrospective comparative analysis of 100 consecutive patients based on age

PurposeTo describe comparative clinical features, treatment, and outcomes of retinoblastoma in patients initially diagnosed at age 4 or older. MethodsRetrospective case series. ResultsThere were 101 eyes in 100 consecutive patients age ≥4 years diagnosed with retinoblastoma. Mean patient age at diagnosis was 6.6 years (median 5.3, range 4.0–41.0 years). Tumors were predominantly classified (International Classification of Retinoblastoma) as group D (31%) or E (65%). Patients were divided by age into 3 groups: young (4–6 years [65%]), middle (>6–8 years [23%]), and older (>8 years [12%]). Comparing by age group (young vs. middle vs. older), mean tumor basal diameter (19.9 vs. 17.3 vs. 17.0 mm, p = 0.05) and mean tumor thickness (11.0 vs. 9.4 vs. 7.0 mm, p < 0.01) were greatest in the youngest group. Distance to the optic nerve (1.5 vs. 1.7 vs. 5.0 mm, p = 0.01) and foveola (1.9 vs. 1.8 vs. 6.0 mm, p < 0.01) were greatest in the oldest age group. Objective findings of leukocoria and strabismus were more common in younger patients, while older patients complained of subjective findings, like decreased vision (19% vs. 30% vs. 60%, p < 0.01) and floaters (3% vs. 4% vs. 17%, p = 0.05). Primary treatment included enucleation (76%) and other modalities (24%). Globe salvage rate was 13%, with no significant difference by age. Comparison of globe salvage by revealed significant improvement between 1974–2008 (6%) and 2009–2017 (38%, p < 0.01). ConclusionRetinoblastoma in older patients (>8 years) tends to be smaller and more peripherally located, with more subjective presenting symptoms.

Conjunctival Melanoma in Asian Indians: A Study of 42 Patients

ABSTRACTPurpose: To describe the clinical features, treatment, and outcomes of conjunctival melanoma in Asian Indians.Methods: Retrospective study of 42 patients.Results: The mean age at presentation of conjunctival melanoma was 43 years (median, 45 years; range, 9–78 years). There were 20 (48%) males and 22 (52%) females. Nineteen patients (45%) had a known history of a preexisting pigmented conjunctival lesion. Bulbar conjunctiva (n = 28; 67%) was the most common tumor epicenter, and medial ocular surface quadrant (n = 15; 36%) was more commonly involved. The mean tumor basal diameter was 12 mm (median, 10 mm; range, 4–30 mm), and the mean tumor thickness was 4 mm (median, 2 mm; range, 1–30 mm). Majority of the patients had a pigmented tumor (n = 33; 79%). The tumors arose de novo (n = 17, 41%) or were associated with conjunctival nevus (n = 9; 21%) or primary acquired melanosis (n = 16, 38%). Wide excisional biopsy, adjunctive cryotherapy, and amniotic membrane grafting were performed in 27 (71%) patients, 11 (29%) underwent orbital exenteration, and 4 were lost to follow-up prior to definitive treatment. Over a mean follow-up period of 24 months (median, 9 months; range, <1 to 136 months), four (11%) patients had tumor recurrence, seven (18%) had locoregional lymph node metastasis, and four (11%) developed systemic metastasis and died due to metastatic disease.Conclusion: Conjunctival melanoma predominantly occurs in middle-aged Asian Indians and is associated with a high rate of systemic metastasis and death.

Choroidal Nevus with Retinal Invasion in 8 Cases

Purpose: Choroidal nevus can cause overlying chronic retinal pigment epithelium (RPE) degenerative features, but frank retinal invasion is exquisitely rare. Procedures: This is a retrospective review of 8 cases of choroidal nevus with retinal invasion with evaluation of clinical and imaging features. Results: At the time of diagnosis of choroidal nevus with retinal invasion, mean patient age was 65 years. Mean tumor basal diameter was 7 mm, and mean thickness was 2.3 mm. Retinal invasion was ophthalmoscopically visible in all eyes. Related features included drusen (n = 4/8) and RPE fibrous metaplasia (n = 2/8). Overlying lipofuscin, subretinal fluid, RPE detachment, and retinal edema were absent. On B-scan ultrasonography, the lesion was dome-shaped (n = 7/7) and echo-dense (n = 6/7). Optical coherence tomography demonstrated outer retinal invasion (n = 8/8) with additional inner retinal invasion (n = 3/8). The tissue was hypoautofluorescent at the site of invasion (n = 6/7). Over a mean follow-up of 40 months, tumor enlargement was detected in 2 eyes and managed with observation (< 1 mm enlargement) or plaque radiotherapy (5 mm enlargement). Nevus hypoautofluorescence was correlated with nevus stability (p = 0.035). Conclusion: Retinal invasion of the choroidal nevus is rare. In this series of 8 cases, only 1 demonstrated transformation to melanoma over a mean interval of 40 months. Long-term monitoring of such lesions is warranted.

Cytogenetic results of choroidal nevus growth into melanoma in 55 consecutive cases

PurposeTo investigate the cytogenetic results of choroidal nevus with photographically-documented transformation into choroidal melanoma. MethodsRetrospective analysis of 55 consecutive patients who underwent fine needle aspiration biopsy (FNAB) for DNA isolation and whole genome array based assay for chromosomes 3, 6, and 8 analysis prior to plaque radiotherapy. Tumors with abnormalities in chromosomes 3 and 8 were considered high-risk for metastasis. ResultsAt diagnosis of choroidal nevus the mean patient age was 57 years (median 57, range 10–83 years). All patients were Caucasian and 36 (65%) were female. At the time of nevus diagnosis, the mean tumor basal diameter was 7.4 mm (median 6.5, range 1.5–18.0 mm) and tumor thickness was 2.2 mm (median 2.2, range 0.5–3.9 mm). The mean interval between diagnosis of choroidal nevus and transformation into choroidal melanoma was 58 months (median 42, range 3–238 months). At the time of melanoma diagnosis, the mean tumor basal diameter was 9.7 mm (median 9.0, range 5.0–19.0) and tumor thickness was 3.5 mm (median 3.4, range 1.3–8.1). Cytogenetic analysis of FNAB-isolated melanoma revealed 25 patients (45%) with high-risk and 30 (55%) with low-risk cytogenetic findings. The rate of tumor growth into melanoma was inversely related to high-risk cytogenetic profile (p = 0.03) as those with fast transformation ≤ 1 year showed high-risk in 80% compared to those with slow transformation > 1 year whoshowed high-risk profile in only 38%. Fast transformation into melanoma conferred a relative risk (RR) of 2.116 for high-risk cytogenetic profile, compared to slow transformation. ConclusionsChoroidal nevus with rapid transformation into melanoma within 1 year is significantly more likely to demonstrate high-risk cytogenetic profile, at risk for metastatic disease, compared to those with slow transformation.

Vitrectomy and Vitrector Port Needle Biopsy of Choroidal Melanoma for Gene Expression Profile Testing Immediately before Brachytherapy

Transvitreal and transscleral needle biopsy can result in complications including vitreous hemorrhage and retinal detachment. This study evaluated a technique using 25-gauge vitrectomy as an adjunct to needle biopsy immediately before brachytherapy to minimize these complications and preserve good visual acuity. Retrospective, observational case series. Fifty-seven consecutive eyes of 57 patients with treatment-naïve medium choroidal melanomas without extraocular extension from July 2012 through September2015. Fifty-seven consecutive eyes of 57 patients with a clinically diagnosed choroidal melanoma underwent complete 25-gauge posterior vitrectomy followed by transvitrector port fine-needle aspiration biopsy of the tumor immediately before implantation of a radioactive iodine 125 plaque as treatment for the tumor. Cytopathologic analysis was not performed on the tumor aspirates in this study. Best-corrected postoperative visual acuity, postoperative complications of the reported technique, implantation tumor development, local tumor recurrence, presence of metastatic disease after surgery, and sufficiency of the tumor aspirates obtained by the reported technique for successful gene expression profile testing and prognostic classification. Mean preoperative and postoperative visual acuities were similar (20/60 vs. 20/80, respectively). Mean tumor thickness was 5.0 mm (range, 2.5-10 mm) and mean tumor basal diameter was 13.1 mm (range, 7-22 mm). Only 1 of 57 eyes (1.8%) showed a transient vitreous hemorrhage, biopsy yield was 100% for genetic analysis, and no patients showed recurrence or implantation tumor at the vitrector site. Combined 25-gauge posterior vitrectomy and 25-gauge trans-vitrector port needle aspiration biopsy immediately before brachytherapy is excellent for obtaining tumor aspirate for gene expression profiling while controlling for hemostasis, resulting in few complications.

Ocular surface squamous neoplasia as the initial presenting sign of human immunodeficiency virus infection in 60 Asian Indian patients.

To study the importance of routine human immunodeficiency virus (HIV) screening in patients with ocular surface squamous neoplasia (OSSN) and describe their clinical features and management. Retrospective study. Of 228 cases of OSSN screened for HIV by enzyme-linked immunosorbent assay, 86 (38%) patients were HIV positive. Of these 86 patients, 60 (70%) were unaware of their HIV-positive status prior to HIV screening. These 60 (26%) patients with newly detected HIV-positive status were included in this study. Ocular surface squamous neoplasia was the sole presenting feature of HIV infection in these patients. Mean age at presentation was 41years. Bilateral involvement occurred in 9 (15%) cases. The mean tumor basal diameter was 11mm. Orbital involvement was noted in 6 (9%) cases, and intraocular tumor extension occurred in 1 (1%) case. Based on American Joint Committee Classification, T2 (n=35, 51%) was most common. The primary treatment for OSSN included excision biopsy (n=52, 75%), topical chemotherapy with Mitomycin-C (n=5, 7%), extended enucleation (n=4, 6%), and orbital exenteration (n=8, 12%). Tumor recurrence occurred in 23% cases during a mean follow-up period of 9months. On histopathology, invasive squamous cell carcinoma was more common (n=38, 55%). OSSN was the presenting sign of underlying HIV infection in 26% cases, and 70% were unaware of their HIV-positive status prior to HIV screening. In this study, T2 tumor was most common, and 26% cases required extended enucleation/orbital exenteration to achieve complete tumor resection.

Neoadjuvant Systemic Chemotherapy in the Management of Extensive Eyelid Sebaceous Gland Carcinoma: A study of 10 Cases.

To report the efficacy of neoadjuvant systemic chemotherapy in the management of eyelid sebaceous gland carcinoma (SGC). Retrospective study of 10 patients that received neoadjuvant systemic chemotherapy (Cisplatin/Carboplatin and 5-Fluorouracil) for eyelid SGC. The mean age at presentation of eyelid SGC was 58 years (median, 55 years; range, 45 to 72 years). There were 6 females and 4 males. The mean tumor basal diameter was 36 mm (median, 31 mm, range, 20 to 65 mm), with orbital tumor extension in 9 cases. On the basis of TNM Classification, the tumors were classified as T3 (n = 10), N1 (n = 6), and M1 (n = 2). The mean number of cycles of neoadjuvant systemic chemotherapy per patient was 3 (median, 3; range, 3 to 4). The mean percentage reduction of tumor basal diameter after neoadjuvant chemotherapy was 74% (median, 80%; range, 30% to 100%). None of them had any major systemic side-effects of neoadjuvant chemotherapy. Postchemotherapy, surgical treatment for residual tumor was performed in 7 cases. Five cases underwent excision biopsy and 2 cases with residual orbital component underwent eyelid-sparing orbital exenteration. No tumor recurrence was noted in any of the 7 cases at a mean follow-up period of 18 months (median, 14 months; range, 3 to 63 months). One patient died due to systemic metastasis. Neoadjuvant systemic chemotherapy is effective and safe in the management of eyelid sebaceous gland carcinoma.

Management of diffuse choroidal hemangioma in Sturge-Weber syndrome with Ruthenium-106 plaque radiotherapy

To evaluate Ruthenium-106 plaque radiotherapy in the treatment of diffuse choroidal hemangioma (DCH) associated with serous retinal detachment. A retrospective analysis was performed in five patients treated for DCH associated with Sturge-Weber syndrome (SWS). In all cases, Ruthenium-106 plaque therapy with a target apex dose of 30.98-47.36 Gy (mean:38.9 Gy) was performed. The outcomes of treatment were regression of DCH, assessed by B-scan ultrasonography; resolution of serous retinal detachment, measured by B-scan ultrasonography and optical coherence tomography (OCT); changes in best corrected visual acuity (BCVA) and the development of radiation-related complications. All investigations were repeated 3 months after treatment and then at six monthly intervals within 22-122 months (mean: 62 months) of follow-up. The initial BCVA of the affected eyes ranged from counting fingers at 1 m to 0.1 by the Snellen chart. Mean tumor basal diameter was 16.7 mm (range: 13.8 to 18.5 mm) and mean tumor thickness was 4.4 mm (range: 2.4 to 5.8 mm). Tumor regression was found in all cases with the prompt resolution of subretinal fluid. In three patients, BCVA improved and in two it remained stable. During the follow-up period, in one case secondary glaucoma was treated with transscleral cyclophotocoagulation, and in another case, recurrence of the hemangioma was treated with repeated Ruthenium-106 plaque irradiation and transpupillary therapy. Ruthenium-106 plaque radiotherapy is an effective and safe treatment option for DCH associated with SWS. Brachytherapy led to tumor regression and resolution of serous retinal detachments, and visual stabilization was achieved in most cases.

Sebaceous gland carcinoma of the eyelid: clinicopathological features and outcome in Asian Indians.

To study the clinical and histopathological features of eyelid sebaceous gland carcinoma (SGC) and to evaluate the prognosis in the Asian-Indian population. This is a retrospective study of 191 patients with SGC. The mean age at presentation of eyelid SGC was 57 years (median, 56 years). The tumor epicenter was most commonly located in the upper eyelid (n=125, 65%). The mean tumor basal diameter was 15 mm (median, 10 mm). There was evidence of tumor extension into the orbit (n=30, 16%), paranasal sinuses (n=3, 2%), and brain (n=1, 1%). Wide excision biopsy (n=146, 78%) was the most common treatment modality. Tumor recurrence was noted in 42 (24%) patients over a mean follow-up period of 29 months (median, 20 months). On the basis of the Kaplan-Meier estimate, lymph node metastasis occurred in 18%, systemic metastasis was detected in 10%, and death occurred in 2% of patients at 10 years. On multivariate analysis, the factors predicting locoregional lymph node and systemic metastasis were medial canthal involvement (P=0.004; P=0.013), lateral canthal involvement (P=0.013; P=0.025), tumor basal diameter >10 mm (P=0.002; P=0.002), and perivascular invasion (P=0.043; P<0.001), respectively. The factors predicting death due to metastasis on multivariate analysis were medial canthal involvement (P=0.012) and tumor basal diameter >10 mm (P=0.001). Advanced eyelid SGC is a tumor associated with poor prognosis. In this study, canthal involvement, larger tumor diameter, and perivascular invasion were poor prognostic factors.

Enhanced depth imaging optical coherence tomography of choroidal metastasis in 14 eyes.

To describe the imaging features of choroidal metastasis using enhanced depth imaging optical coherence tomography (EDI-OCT). This retrospective observational case series included 31 eyes with choroidal metastasis. Spectral domain EDI-OCT was performed using Heidelberg Spectralis HRA + OCT. The main outcome measures were imaging features by EDI-OCT. Of 31 eyes with choroidal metastasis imaged with EDI-OCT, 14 (45%) eyes displayed image detail suitable for study. The metastasis originated from carcinoma of the breast (n = 7, 50%), lung (n = 5, 36%), pancreas (n = 1, 7%), and thyroid gland (n = 1, 7%). The mean tumor basal diameter was 6.4 mm, and mean thickness was 2.3 mm by B-scan ultrasonography. The tumor location was submacular in 6 (43%) eyes and extramacular in 8 (57%) eyes. By EDI-OCT, the mean tumor thickness was 987 μm. The most salient EDI-OCT features of the metastasis included anterior compression/obliteration of the overlying choriocapillaris (n = 13, 93%), an irregular (lumpy bumpy) anterior contour (n = 9, 64%), and posterior shadowing (n = 12, 86%). Overlying retinal pigment epithelial abnormalities were noted (n = 11, 78%). Outer retinal features included structural loss of the interdigitation of the cone outer segment tips (n = 9, 64%), the ellipsoid portion of photoreceptors (n = 8, 57%), external limiting membrane (n = 4, 29%), outer nuclear layer (n = 1, 7%), and outer plexiform layer (n = 1, 7%). The inner retinal layers (inner nuclear layer to nerve fiber layer) were normal. Subretinal fluid (n = 11, 79%), subretinal lipofuscin pigment (n = 1, 7%), and intraretinal edema (n = 2, 14%) were identified. The EDI-OCT of choroidal metastasis shows a characteristic lumpy bumpy anterior tumor surface and outer retinal layer disruption with preservation of inner retinal layers.

Cutaneous Vitiligo Following Management of Uveal Melanoma in 6 Patients

The relationship of vitiligo to cutaneous melanoma is believed to be due to an immune response generated to melanoma antigens that cross-react with normal skin. There is little in the literature on the relationship between cutaneous vitiligo and uveal melanoma. To describe the clinical profile, treatment, and outcome in patients with uveal melanoma who subsequently developed cutaneous vitiligo. Retrospective case series of 6 patients with uveal melanoma who had developed cutaneous vitiligo and were examined at a tertiary eye care institution. Development of cutaneous vitiligo. The mean age at presentation was 62 years (range, 39-85 years). No patient had a personal history of cutaneous melanoma, autoimmune disease, or cutaneous vitiligo. The mean tumor basal diameter was 12.9 mm (median, 12.7 mm; range, 7-19 mm), with a mean thickness of 9.5 mm (median, 8.4 mm; range, 3-19 mm). Treatment included plaque radiotherapy in 4 patients and enucleation in 1 patient; 1 patient refused therapy. No patient had local tumor recurrence at the 71-month mean follow-up, but of the 3 patients who developed metastases at the 52-month mean follow-up, 2 were treated with a melanoma vaccine. The mean interval from initial presentation to onset of vitiligo was 77 months (range, 5-168 months). The vitiligo developed bilaterally with multiple well-defined lesions, affecting 5% to 40% of the cutaneous surface, generally in the upper body. During the 71-month mean follow-up (range, 4-205 months), there was 1 death. Patients with uveal melanoma can develop vitiligo spontaneously or following vaccine therapy. Involvement is multiple and bilateral, predominantly affecting the upper body.

Reply re

PURPOSE The aim of this study was to evaluate conjunctival melanoma outcomes based on American Joint Committee on Cancer classification. The study design constituted a nonrandomized interventional case series. METHODS This was a retrospective chart review comprising 343 participants, and the main outcome measures were melanoma local recurrence, lymph node metastasis, distant metastasis, and death. RESULTS On the basis of the American Joint Committee on Cancer classification (seventh edition), conjunctival melanoma was classified as T1 (196 [57%]), T2 (110 [32%]), T3 (37 [11%]), and T4 (0). The mean tumor basal diameter increased with tumor staging with 8.5 mm for T1, 12.7 mm for T2 (p = 0.0003), and 16 mm for T3 (p < 0.0001). The melanoma arose from primary acquired melanosis (T1 = 71%; T2 = 84%; T3 = 81%), preexisting nevus (T1 = 8%; T2 = 5%; T3 = 3%), or de novo (T1 = 21%; T2 = 12%; T3 = 16%). Outcomes at 5 years (Kaplan-Meier) revealed melanoma local recurrence/new tumor in 44% T1, 78% T2 (p < 0.0001), and 76% T3 (P=0.0044); regional lymph node metastasis in 17% T1, 52% T2 (p < 0.0001), and 49% T3 (p = 0.0092); melanoma-related distant metastasis in 11% T1, 35% T2 (p < 0.0001), and 42% T3 (p = 0.0018); and melanoma-related death in 5% T1, 20% T2 (p = 0.0655), and 23% T3 (p = 0.0526). Based on American Joint Committee on Cancer classification, factors predictive of melanoma recurrence included T2 stage (p < 0.0001), and T3 stage (p = 0.0061). After adjusting for tumor origin, factors predictive of regional lymph node metastasis, melanoma-related distant metastasis, and melanoma-related death included melanoma arising de novo (p < 0.0001; p < 0.0001; p < 0.0001), T2 stage (p < 0.0001; p < 0.0001; p = 0.007), and T3 stage (p = 0.005; p = 0.0014; p = 0.0342). CONCLUSION The American Joint Committee on Cancer staging predicts prognosis of conjunctival melanoma. Melanoma classified as T2 and T3 (compared with T1) showed significantly higher rates of local recurrence, regional lymph node metastasis, distant metastasis, and death.

Ciliary Body Medulloepithelioma: Analysis of 41 Cases

To describe the clinical features, histopathology, treatment, and outcomes of ciliary body medulloepithelioma. Retrospective study. Forty-one patients with medulloepithelioma. Cryotherapy, plaque radiotherapy, external beam radiotherapy, tumor removal by partial lamellar sclerouvectomy (PLSU), or enucleation. Metastasis and death. Of 41 patients with ciliary body medulloepithelioma, the median age at diagnosis was 5 years. The mean tumor basal diameter was 11 mm, and the mean tumor thickness was 7 mm. Related features included secondary glaucoma (n = 18, 44%), iris neovascularization (n = 21, 51%), cataract (n = 19, 46%), lens subluxation (n = 11, 27%), lens coloboma (n = 8, 20%), retrolental neoplastic cyclitic membrane (n = 21, 51%), intratumoral cysts (n = 25, 61%), and extraocular extension (n = 4, 10%). There was systemic association with pleuropulmonary blastoma in 2 cases (5%). Primary tumor treatment included enucleation (n = 21, 60%), tumor removal by PLSU (n = 8, 23%), plaque radiotherapy (n = 3, 9%), external beam radiotherapy (n = 1, 3%), cryotherapy (n = 1, 3%), or palliative chemotherapy (n = 1, 3%). In 1 case, medulloepithelioma was diagnosed histopathologically after inadvertent evisceration for blind painful eye. Subsequent treatment for residual or recurrent tumor in cases treated conservatively/inappropriately (n = 15) was necessary in 7 cases (47%). Histopathology disclosed benign features in 6 cases (20%), malignant features in 24 cases (80%), teratoid features in 11 cases (37%), and nonteratoid features in 19 cases (63%). In the 26 enucleated eyes, other features included retrolental neoplastic cyclitic membrane (n = 18, 69%), neoplastic epiretinal membrane (n = 6, 23%), and persistent hyaloid artery (n = 6, 23%). Systemic metastasis occurred in 3 cases (8%) over a mean follow-up of 49 months, all of whom presented with extrascleral extension of tumor due to mean delay in diagnosis by 39 months. Medulloepithelioma most commonly occurs in children. Systemic association with pleuropulmonary blastoma rarely is found. Patients with extrascleral medulloepithelioma are at risk for metastasis.

Iris Nevus Growth into Melanoma: Analysis of 1611 Consecutive Eyes: The ABCDEF Guide

To determine clinical features predictive of growth of iris nevus into melanoma. Retrospective, comparative case series. A total of 1611 consecutive patients referred to an ocular oncology center with iris nevus. Observation and photographic documentation. Growth into melanoma. The mean age at referral for iris nevus was 51 years (median, 54; range, <1-94 years). At presentation, the mean tumor basal diameter was 3 mm (median, 3 mm; range, <1-12 mm) and mean tumor thickness was 0.8 mm (median, 0.5 mm; range, 0-5 mm). All patients were initially diagnosed with benign iris nevus. Growth of iris nevus to melanoma was confirmed in 2% of eyes (n = 27) over a mean follow-up of 68 months (median, 46 months; range, 3-465 months). By Kaplan-Meier estimates, iris nevus growth to melanoma occurred in <1%, 3%, 4%, 8%, and 11% at 1, 5, 10, 15, and 20 years, respectively. Factors predictive of iris nevus growth to melanoma by multivariable analysis included age ≤ 40 years at presentation (hazard ratio [HR], 3), episode of hyphema (HR, 9), 4:00 to 9:00 clock hour location of tumor (HR, 9), diffuse tumor (involving entire iris surface) (HR, 14), ectropion uveae (HR, 4), and feathery tumor margins (HR, 3). Additional important factors by univariable analysis included tumor seeding on the iris or in the anterior chamber angle, feeder vessels, and nodule formation. These factors can be remembered using the mnemonic ABCDEF, representing A = age young, B = blood, C = clock hour inferior, D = diffuse, E = ectropion, and F = feathery margin. In an analysis of 1611 cases of iris nevus referred for evaluation at an ocular oncology center, growth into melanoma occurred in 8% by 15 years. Risk factors for growth, identified by ABCDEF included Age young, Blood (hyphema), Clock hour inferior, Diffuse configuration, Ectropion uveae, and Feathery tumor margin.

American Joint Committee on Cancer (AJCC) Clinical Classification Predicts Conjunctival Melanoma Outcomes

The aim of this study was to evaluate conjunctival melanoma outcomes based on American Joint Committee on Cancer classification. The study design constituted a nonrandomized interventional case series. This was a retrospective chart review comprising 343 participants, and the main outcome measures were melanoma local recurrence, lymph node metastasis, distant metastasis, and death. On the basis of the American Joint Committee on Cancer classification (seventh edition), conjunctival melanoma was classified as T1 (196 [57%]), T2 (110 [32%]), T3 (37 [11%]), and T4 (0). The mean tumor basal diameter increased with tumor staging with 8.5 mm for T1, 12.7 mm for T2 (p = 0.0003), and 16 mm for T3 (p < 0.0001). The melanoma arose from primary acquired melanosis (T1 = 71%; T2 = 84%; T3 = 81%), preexisting nevus (T1 = 8%; T2 = 5%; T3 = 3%), or de novo (T1 = 21%; T2 = 12%; T3 = 16%). Outcomes at 5 years (Kaplan-Meier) revealed melanoma local recurrence/new tumor in 44% T1, 78% T2 (p < 0.0001), and 76% T3 (P=0.0044); regional lymph node metastasis in 17% T1, 52% T2 (p < 0.0001), and 49% T3 (p = 0.0092); melanoma-related distant metastasis in 11% T1, 35% T2 (p < 0.0001), and 42% T3 (p = 0.0018); and melanoma-related death in 5% T1, 20% T2 (p = 0.0655), and 23% T3 (p = 0.0526). Based on American Joint Committee on Cancer classification, factors predictive of melanoma recurrence included T2 stage (p < 0.0001), and T3 stage (p = 0.0061). After adjusting for tumor origin, factors predictive of regional lymph node metastasis, melanoma-related distant metastasis, and melanoma-related death included melanoma arising de novo (p < 0.0001; p < 0.0001; p < 0.0001), T2 stage (p < 0.0001; p < 0.0001; p = 0.007), and T3 stage (p = 0.005; p = 0.0014; p = 0.0342). The American Joint Committee on Cancer staging predicts prognosis of conjunctival melanoma. Melanoma classified as T2 and T3 (compared with T1) showed significantly higher rates of local recurrence, regional lymph node metastasis, distant metastasis, and death.