Maria Antonietta Blasi

PHOTODYNAMIC THERAPY FOR VASOPROLIFERATIVE RETINAL TUMORS

Purpose: To report our experience with photodynamic therapy (PDT) with verteporfin for patients with vasoproliferative retinal tumors (VPRTs). Methods: Three patients with VPRTs who presented with macular exudative changes were treated with one session of PDT with 6 mg/m2 body surface area of verteporfin and a light dose of 100 J/cm2 at 689 nm delivered in 166 seconds. Biomicroscopy, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and ultrasonography were performed before treatment and 1 month, 3 months, 6 months, and 1 year after treatment; visual acuity was measured using Early Treatment Diabetic Retinopathy Study criteria. Results: At the 1-year follow-up, all tumors responded with a reduction in size (mean height: pretreatment, 2.96 mm; posttreatment, 1.32 mm), and optical coherence tomography showed complete resolution of macular exudates. For all patients, fluorescein angiography evidenced reduction of leakage from the lesion, and indocyanine green angiography verified nonperfusion of the vascular channels. An improvement in visual acuity (average, 4.7 Early Treatment Diabetic Retinopathy Study letters) was observed. No retreatment was needed. Conclusion: PDT may represent an effective and safe modality of treatment for VPRTs because of its selectivity. Our study supports the application of a light dose of 100 J/cm2, although further studies with larger numbers of cases and longer follow-ups are required.

Photodynamic Therapy with Verteporfin for Symptomatic Circumscribed Choroidal Hemangioma: Five-Year Outcomes

OBJECTIVE To evaluate the long-term efficacy of verteporfin photodynamic therapy (PDT) as the primary treatment for symptomatic circumscribed choroidal hemangioma (CCH). DESIGN Prospective consecutive, 2-centered, noncomparative, interventional case series. PARTICIPANTS Twenty-five subjects with symptomatic CCH. All patients had recent onset of visual symptoms and evidence of exudative macular changes on fluorescein angiography (FA) and optical coherence tomography (OCT). METHODS Verteporfin 6 mg/m(2) body surface area was administered intravenously over a 10-minute interval. Five minutes after infusion, a 689 nm laser was applied with a light dose of 50 J/cm(2) for the first 3 patients and a light dose of 100 J/cm(2) for all the other patients. Retreatments were performed in case of persistent exudation found on OCT. Evaluation of best-corrected visual acuity (BCVA) using Early Treatment of Diabetic Retinopathy Study (ETDRS) criteria, FA, indocyanine green angiography (ICGA), OCT, and ultrasound were performed before PDT and on follow-up examinations. All patients were followed for at least 5 years. MAIN OUTCOME MEASURES Primary outcome measures were changes in BCVA and foveal center thickness (FCT) between baseline and month 60. Secondary measures were tumor thickness decrease, absence of leakage on FA, and adverse events. RESULTS Twenty-two patients received 1 PDT session at 100 J/cm(2), and no recurrences were detected. Three eyes, treated with 50 J/cm(2), received a second PDT session at 100 J/cm(2) 1 month after the first session. After a follow-up of 60 months, BCVA improved an average of 18.5 ETDRS letters (P<0.001); BCVA improved by > or =2 lines in 19 eyes (76%). The FCT decreased from a mean of 386.20 microm to 179.2 microm, and OCT showed the complete resolution of macular exudation in all cases. All tumors responded with a reduction in size. No treatment-related adverse events or complications were identified. CONCLUSIONS The 5-year results of PDT in treating symptomatic CCH support treatment with a light dose of 100 J/cm(2) after slow intravenous infusion of verteporfin to stabilize or improve visual acuity and resolution of macular exudation.

Intralesional administration of rituximab for treatment of CD20 positive orbital lymphoma: Safety and efficacy evaluation.

B-cell lymphomas constitute the most frequent malignant neoplasm of the ocular adnexal, often presenting with localized disease. Five patients with primary localized CD20 positive B cell non Hodgkin ocular adnexal lymphomas received intralesional rituximab at the dose of 5mg once a week for one month, followed by 10mg weekly in case of incomplete response. Four of five patients obtained regression of symptoms and 2 of them showed complete response. No patients experienced side effects besides pain on the site of the injection. Local treatment with Rituximab for OAL is a safe and useful first-line therapeutic option.

Evaluation of intraorbital injection of rituximab for treatment of primary ocular adnexal lymphoma. A pilot study.

An interventional pilot study to assess the tolerability and activity of the intralesional injection of rituximab, a chimeric mAb that targets the CD20 antigen, in patients with orbital B‐cell lymphoma. Five patients received four intralesional injections (one injection a week) of rituximab together with ropivicaine 2%. Side‐effects and tumor response were assessed after each injection and during the follow‐up (20 months). Two patients obtained complete remission of the intraorbital lesion. Two patients showed incomplete response after induction therapy and received planned escalating rituximab doses, obtaining regression of subjective symptoms. One patient did not achieve tumor regression after the first injection and underwent systemic treatment. This small exploratory study suggests that intralesional rituximab is a well‐tolerated treatment for patients with primary ocular adnexal lymphoma. These preliminary findings suggest that intralesional rituximab is a well‐tolerated strategy in anterior intraorbital lesion localization of lymphoma. (Cancer Sci 2011; 102: 1565–1567)

Intralesional interferon-α for conjunctival mucosa-associated lymphoid tissue lymphoma: long-term results

OBJECTIVE To report the long-term therapeutic results for patients with conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma who were treated with intralesional injections of interferon-α (IFN-α). DESIGN Prospective, nonrandomized, interventional case series. PARTICIPANTS Twenty eyes of 16 patients with histologically proven conjunctival MALT lymphoma in the absence of systemic disease. METHODS Patients were given 1,500,000 international units (IU) of IFN-α (Roferon-A; Roche s.p.a., Milano, Italy) subconjunctivally inside the lesion 3 times weekly for 4 weeks. If there was even a minimal response, a further cycle of 1,000,000 IU 3 times weekly for 4 weeks was administered. MAIN OUTCOME MEASURES Patients were followed up clinically using slit-lamp examination to determine evidence of tumor disappearance or recurrence. In 10 eyes, an incisional biopsy was performed 6 months after therapy to verify the histologic absence of the lesion. RESULTS A complete response was obtained in 15 eyes (75%) at the end of first cycle treatment, and in 5 eyes (25%) after further cycles. Seventeen eyes (85%) showed no local recurrence after a median follow-up of 65 months (range, 15-136 months). Three eyes (15%) demonstrated recurrence at variable points after treatment. One patient with stage IIA lymphoma exhibited systemic lymphoma progression. CONCLUSIONS Local immunotherapy with IFN-α seems to be an effective and lasting treatment method and provides an alternative to radiotherapy for conjunctival MALT lymphomas. Very few transient side effects were detected.

Lymphoepithelioma-like carcinoma involving the lacrimal gland and infiltrating the eyelids.

Purpose TO report a case of a lymphoepithelioma-like carcinoma (LELC), which is a variant of carcinoma most commonly involving the nasopharynx and rarely occurring in a variety of sites outside its typical location. Methods We present the case of a 79-year-old Caucasian man with LELC of the lacrimal gland undergoing surgical resection of the mass. Results The patient had remained disease-free for 2 years, and then he developed homolateral metastases in latero-cervical and parotid lymph nodes, detected by computed tomography and confirmed by fine-needle aspiration biopsy. He did not receive any surgical treatment, radiotherapy, or chemotherapy because of the advanced stage of the disease and poor health condition. Conclusions This case report suggests that a diagnosis of lacrimal gland LELC makes combined early and radical therapy necessary.

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.

25-Gauge Pars Plana Vitrectomy for Retained Lens Fragments in Complicated Cataract Surgery

Purpose: To verify the efficacy of 25-gauge pars plana vitrectomy (PPV) for the management of posteriorly dislocated lens material after complicated cataract extraction and to determine in what patients this approach offers the optimal benefit in terms of efficacy and safety, considering the amount of retained nuclear material and the duration of surgery. Methods: Forty eyes of 40 patients with retained lens fragments undergoing early (within 1 week) or late (within >1 week) 25-gauge PPV were retrospectively reviewed. The amount of dislocated nuclear material was graded by the surgeon intraoperatively, and the patients were divided into two groups according to the nuclear grading: group A (≤50% dropped nucleus) and group B (>50% dropped nucleus). The presence of brunescent nuclear pieces was considered. The outcomes measured included best-corrected visual acuity (BCVA) and postoperative complications such as retinal detachment, cystoid macular edema (CME) and postoperative ocular hypertension or hypotonia. Results: The patients had a mean age of 78 years. The mean preoperative logarithm of the minimum angle of resolution (logMAR) BCVA was 0.57 ± 0.24 (20/80). A significant positive correlation was found between nuclear material grade and PPV duration (R2 = 0.81, p < 0.0001). None of the patients had dislocation of brunescent nuclear pieces. On postoperative day 1, the mean postoperative intraocular pressure was 16.75 ± 2.7 mm Hg, with no case of ocular hypotonia. At 6 months of follow-up, the mean logMAR BCVA improved to 0.23 ± 0.3 (20/32). Retinal detachment developed in 4 patients (10%), occurring only in patients of group B (p < 0.002). Four patients with late PPV developed postoperative CME, with no case of CME among patients with early vitrectomy (p = 0.014). Conclusion: Removal of dislocated lens fragments after complicated cataract surgery can be effectively managed with 25-gauge PPV, although it appears to be most efficient for cases with a limited amount of dislocated lens material. In consideration of the higher rate of retinal detachment observed in cases of prolonged PPV time, the expected duration of surgery should be taken into account when choosing the best surgical approach. Visual outcomes are not affected by the timing of PPV, whereas early vitrectomy seems to prevent the onset of inflammatory macular edema.

BRACHYTHERAPY ALONE or with NEOADJUVANT PHOTODYNAMIC THERAPY for AMELANOTIC CHOROIDAL MELANOMA: Functional Outcomes and Local Tumor Control

Purpose: To compare visual outcomes and local tumor control between two groups of patients with amelanotic choroidal melanoma treated with brachytherapy alone, or neoadjuvant photodynamic therapy before brachytherapy. Methods: Patients diagnosed with amelanotic choroidal melanoma were recruited for the study and divided into two groups: brachytherapy alone (Group A) and photodynamic therapy preceding brachytherapy (Group B). Patients of both groups were selected to be comparable. Results: Twenty-six patients with amelanotic choroidal melanoma were enrolled in the study. Within Group B, 1 month after photodynamic therapy, ultrasonography showed reduction of tumor height in 11 patients (73.4%). The mean doses of irradiation to macula and optic nerve, at baseline were 74.37 and 52.07 Gy, whereas after photodynamic therapy there was a decrease of 17.26% (P = 0.008) and 21.22% (P = 0.025), respectively. In terms of visual acuity, a mean decrease of 14 ETDRS letters and 5 ETDRS letters was observed at 24 months follow-up, in Groups A and B, respectively (P = 0.001). Conclusion: Photodynamic therapy as neoadjuvant therapy before brachytherapy reduces tumor thickness in 73.4% of cases. As a result, a decrease of radiation toxic effects on visual function could be obtained, without compromising disease control.

Intravitreal dexamethasone implant for acute exudative polymorphous vitelliform maculopathy

Purpose Acute exudative polymorphous vitelliform maculopathy is a rare retinal disease characterized by bilateral serous macular detachment and subretinal accumulation of yellowish deposits resembling Best dystrophy lesions. Corticosteroid systemic therapy has been used empirically in the attempt to treat this retinal disorder with mixed results. Thus, the benefit of corticosteroid remains undetermined. To our knowledge, we report the first case of acute exudative polymorphous vitelliform maculopathy (AEPVM) treated in one eye with intravitreal dexamethasone implant (Ozurdex; Allergan, Inc., Irvine, California, USA). Methods A 28-year-old man with AEPVM underwent intravitreal dexamethasone implantation in the left eye. Results Compared with the fellow eye, intravitreal dexamethasone implant did not significantly modify the clinical course of the disease. No implant-related complication was experienced during follow-up. Conclusions The lack of response to intravitreal dexamethasone seems to suggest that corticosteroids may be ineffective for the treatment of AEPVM.