Hatem Krema

Stereotactic Radiotherapy for Treatment of Juxtapapillary Choroidal Melanoma: 3-Year Follow-up.

Aims: To report the treatment outcomes and complication rates of stereotactic radiotherapy in the management of patients with juxtapapillary choroidal melanoma. Methods: A retrospective review of 64 consecutive patients with juxtapapillary choroidal melanoma, located within 2 mm of the optic disc, treated with stereotactic radiotherapy at Princess Margaret Hospital between October 1998 and January 2006. Results: The median age was 63 years. The median tumour height was 4.2 mm, and median largest basal diameter was 9.8 mm. The prescribed radiation dose was 70 Gy in five fractions over 10 days, and the median follow-up was 37 months. Post-treatment, the actuarial rates of local tumour control, metastases and survival at 37 months were 94%, 15% and 90%, respectively. Actuarial rates of radiation-induced complications at 37 months were neovascular glaucoma 42%, cataract 53%, retinopathy 81% and optic neuropathy 64%. Secondary enucleation was necessary for 10 patients (16%), in four patients for tumour recurrence and in six for painful neovascular glaucoma. Conclusions: Stereotactic radiotherapy offers a non-invasive alternative to enucleation and brachytherapy in the management of juxtapapillary choroidal melanoma with a high tumour control rate, however, at the expense of a significant rate of long-term ocular complications.

Orthovoltage radiotherapy in the management of medial canthal basal cell carcinoma.

Aims To report the local control and complication rates of orthovoltage radiotherapy in the management of medial canthal basal cell carcinoma (BCC). Methods The medical records of all patients treated with medial canthal BCC between 1998 and 2010, with orthovoltage radiotherapy as primary treatment, adjuvant treatment after incomplete surgical excision, or for tumour recurrence following surgical excision, were retrospectively studied. The actuarial rates of tumour control and complications were calculated using Kaplan–Meier estimates. Main outcome measures were rates of tumour control and radiation complications. Results 90 patients were included with a median follow-up of 80 months. Tumour control rate at 10 years for the entire cohort was 94% (95% CI 84% to 98%). Tumour control rates showed no statistically significant differences among different treatment intents or treatment radiation energies. Radiation-related complication rates included loss of eyelashes in 59% (95% CI 48% to 66%), epiphora 51% (95% CI 39% to 62%), dry eye 14% (95% CI 3% to 35%) and conjunctival scarring 11% (95% CI 1% to 33%). No patient developed long-term corneal complications. Conclusions Orthovoltage radiotherapy can be a reliable therapeutic alternative for selected medial canthal BCCs, which can be contained within the prescribed radiation field, with anticipated radiation-related toxicities.

Failure of Systemic Propranolol Therapy for Choroidal Hemangioma of Sturge-Weber Syndrome: A Report of 2 Cases

Propranolol was incidentally discovered to induce accelerated involution of infantile cutaneous hemangioma. Thereafter, reports have demonstrated a favorable response to propranolol as a first-line treatment for cutaneous, orbital, and ocular hemangioma in different ages. We report the outcome of oral propranolol therapy in 2 cases with choroidal hemangioma of SturgeWeber syndrome (SWS).

Stereotactic radiotherapy in the treatment of juxtapapillary choroidal melanoma: 2-year follow-up

OBJECTIVE To evaluate the efficacy and complications of stereotactic radiotherapy in the management of patients with juxtapapillary choroidal melanoma. DESIGN Retrospective review. PARTICIPANTS 64 patients with juxtapapillary choroidal melanoma. METHODS Consecutive patients with juxtapapillary choroidal melanomas located within 2 mm of the optic nerve, treated with stereotactic radiotherapy at Princess Margaret Hospital from October 1998 to January 2006, were reviewed for treatment effect and complication rates. RESULTS Median age was 63 years. Median tumor height was 4.2 mm, and median maximum tumor diameter was 9.8 mm. The prescribed radiation dose was 70 Gy in 5 fractions over 10 days, and the median follow-up was 26 months. After treatment, there was local tumor recurrence in 3 patients, and in 8 patients there was systemic progression. Actuarial rates of local tumor control, metastases, and survival at 26 months were 94%, 12%, and 94%, respectively. Rates of radiation-induced neovascular glaucoma, cataract, retinopathy, and optic neuropathy at 26 months were 28%, 45%, 80%, and 52%, respectively. Enucleation was necessary for 7 patients. CONCLUSIONS Stereotactic radiotherapy offers a noninvasive alternative with acceptable ocular toxicity rates to enucleation and brachytherapy in the management of juxtapapillary choroidal melanoma.

Spectral-domain optical coherence tomography versus ultrasound biomicroscopy for imaging of nonpigmented iris tumors.

PURPOSE To evaluate the use of spectral-domain optical coherence tomography (SDOCT) for imaging of nonpigmented iris tumors, through its comparison with ultrasound biomicroscopy (UBM). DESIGN Retrospective observational case series. METHODS Consecutive patients with non-pigmented iris tumors, not extending to the ciliary body, who were concurrently evaluated with SD-OCT and UBM were included. Demographics, anterior segment clinical photographs, images obtained with SD-OCT (Cirrus HD-OCT, Zeiss Meditec, Dublin, California, USA) with 5.1.1 anterior segment software upgrade, and UBM (Humphrey Instruments, San Leandro, California, USA) were reviewed. The images produced were compared regarding the degree of anterior and posterior tumor surface resolution, internal structures, tumor thickness measurement, image artefacts, and overall tumor visualization. RESULTS Thirty-seven patients with nonpigmented iris tumors were included. Comparing SDOCT to UBM, the image definitions of anterior tumor surface and internal tumor heterogeneity were equivalent. Posterior tumor surface was well defined in 54% of SDOCT vs 100% in UBM images. Full tumor thickness measurement was possible in 86% of SDOCT vs 100% with UBM. The maximum measurable tumor thickness with SDOCT was 1.34 mm. SDOCT images showed optical aberrations such as shadowing and ghost images in 22 tumors (59%), which encroached on the tumor image in 8 patients (22%). The overall tumor visualization with SDOCT was possible in 65% of the iris tumors. CONCLUSIONS UBM generally provides superior imaging quality and reproducible measurements of nonpigmented iris tumors. Nevertheless, SDOCT, being a noncontact technique, can be a reliable alternative in imaging and following some selected nonpigmented iris tumors.

Factors predictive of radiation retinopathy post 125Iodine brachytherapy for uveal melanoma

OBJECTIVE To report the host, tumour, and radiation-related predictive factors for developing radiation retinopathy post (125)Iodine brachytherapy for uveal melanoma. DESIGN A retrospective clinical case series. PARTICIPANTS Three hundred consecutive patients with uveal melanoma treated with (125)Iodine brachytherapy. METHODS Electronic chart review of demographic, clinical, treatment, and follow-up data. Proliferative and (or) nonproliferative radiation retinopathy patients were included. Cumulative incidence rates were calculated using Kaplan-Meier estimates. Univariate and multivariate statistical regression analyses were performed to identify factors predictive of radiation retinopathy. RESULTS The mean follow-up period was 48 months. Radiation retinopathy occurred in 107 patients (36%). Actuarial incidence of radiation retinopathy was 30% (CI 24%-36%) at 24 months post-treatment. In the multivariate model, the predictive factors were a younger age (hazard ratio [HR] 0.98, p < 0.03), diabetes (HR 2.17, p < 0.007), and hypertension (HR 2.17, p < 0.004). Tumour-related factors were proximity to optic disc (HR 0.95, p < 0.02) and proximity to foveola (HR 0.96, p < 0.02). Pretreatment tumour dimensions, other tumour characteristics, and total radiation dose did not demonstrate statistically significant risks for developing radiation retinopathy. CONCLUSIONS Radiation retinopathy affects 30% of patients 2 years following (125)Iodine brachytherapy for uveal melanoma. The predictive factors for radiation retinopathy are a younger age, comorbidity with diabetes or hypertension, and proximity of the tumour margin to the optic disc or foveola. Identifying the predictive factors for developing radiation retinopathy can modify follow-up for patients at risk, which may permit earlier management of the developing radiation-induced ischemic retinal changes.

A comparison between 125Iodine brachytherapy and stereotactic radiotherapy in the management of juxtapapillary choroidal melanoma

Aims To compare the treatment efficacy and radiation complications between 125Iodine brachytherapy and stereotactic radiotherapy in the management of juxtapapillary choroidal melanoma. Methods Consecutive juxtapapillary melanoma patients treated with radiotherapy were included. Patients were divided into two cohorts: patients treated with 125Iodine brachytherapy and patients with stereotactic radiotherapy. Comparison included the rates postradiotherapy local recurrence, secondary enucleation, metastasis and radiotherapy complications. Kaplan–Meier estimates were used to determine the actuarial rates, and logrank test to compare between the estimates. Results We included 94 patients with juxtapapillary melanoma treated with radiotherapy. The brachytherapy cohort included 30 patients and stereotactic radiotherapy was 64. The median follow-up was 46 months in both cohorts. No statistically significant differences existed between the two cohorts on comparing pretreatment clinical data and tumour characteristics. On comparing treatment efficacy, the actuarial rates at 50 months for tumour recurrence were 11% and 7% (p=0.61), secondary enucleation was 11% and 21% (p=0.30) and for metastasis were 4% and 16% (p=0.11), respectively. On comparing treatment complications, the actuarial rates at 50 months for cataracts were 62% and 75% (p=0.1), for neovascular glaucoma 8% and 47% (p=0.002), for radiation retinopathy 59% and 89% (p=0.0001), and for radiation papillopathy 39% and 74% (p=0.003), respectively. Conclusions Both 125Iodine brachytherapy and stereotactic radiotherapy demonstrate comparable efficacy in the management of juxtapapillary choroidal melanoma. However, stereotactic radiotherapy shows statistically significant higher radiation-induced ocular morbidities at 4 years postradiotherapy.

Cancer-associated nummular loss of RPE: expanding the clinical spectrum of bilateral diffuse uveal melanocytic proliferation.

This report describes a case of cancer-associated nummular retinal pigment epithelium loss associated with uterine cancer. The patient had progressive visual loss despite treatment with plasmapheresis, intravenous immunoglobulin, and local injection of corticosteroids. Clinical deterioration was corroborated by extension of the areas of retinal pigment epithelium loss, progression of cataracts, and growth of pigmented choroidal and iris lesions. Previously published cases of cancer-associated nummular retinal pigment epithelium loss did not describe the presence of cataracts or uveal melanocytic lesions. This case expands the clinical spectrum of bilateral diffuse uveal melanocytic proliferation.

PIMASERTIB AND SEROUS RETINAL DETACHMENTS

This is the first case that describes bilateral, multifocal central serous retinopathy appearing 2 days after starting pimasertib for ovarian cancer and rapidly resolving 3 days after stopping pimasertib. Pimasertib is an orally bioavailable MEK 1 and 2 inhibitor with potential antineoplastic activity, that is, currently used in clinical trials for ovarian cancer. Multifocal serous retinal detachments have been reported with the use of MEK 1 and 2 inhibitors. The authors report the development of multifocal serous retinal detachments with the use of pimasertib in a 26-year-old woman with metastatic low-grade serous ovarian cancer that was initially treated in 2010, with laparoscopic right oophorectomy. The tumor showed evidence of progression in 2011 for which a hysterectomy, left salpingo-oophorectomy, omentectomy, and removal of the metastatic disease from the diaphragm was performed. This was followed by six cycles of carboplatin and taxol, along with one cycle of maintenance bevacizumab. There was no other medical history. The pathophysiology of this complication is still poorly understood.

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

PURPOSE To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI. DESIGN Retrospective, multicenter observational study. PARTICIPANTS Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults. METHODS Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression. MAIN OUTCOME MEASURES Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality. RESULTS Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival. CONCLUSIONS This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.