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Methods. A retrospective chart review of 26 eyes of 25 patients receiving three-drug (melphalan, topotecan, and carboplatin) multi agent SSIAC was conducted between May 2006 and June 2011. Results. Twenty-six eyes, Reese-Ellsworth group 5b (22) 5a (1) 4a (2) and 3a (1), received 61 infusions of 3 drug multiagent chemotherapy for rescue of eyes with advanced retinoblastoma. The dose range for melphalan was 2.5 to 7.5 mg, 0.3 to 0.6 mg for topotecan, and 30 to 50 mg for carboplatin. The median number of multi drug infusions was 2 with a maximum of 4 and minimum of 1 averaging 2.3 per eye. Fourteen of 25(56%) patients presented after failing intravenous chemotherapy (IVC), 2/25(8%) after failing IVC and external beam radiotherapy and 1/25(4%) after failing IVC and plaque brachytherapy. Twenty-four /26 (92)% of eyes were salvage over a mean follow up period of 14 mo. (1-43 mo.) Electroretinogram (ERG) showed improvement greater than in 25mv in 4/26 eyes (15%), greater than 25-mv loss in 12/26 eyes(46%), no change greater than 25mv in 10/26 eyes(39%). Conclusions. We have successfully used three-drug multiagent SSIAC to rescue eyes that have failed IVC and/ or single or double agent SSIAC. A significant portion of eyes avoided enucleation and retained ERG function. . Further investigation into multiagent SSIAC is required to determine its role in treatment of advanced retinoblastoma. Financial disclosure. None 1550 RB27 OCULAR COMPLICATIONS OF DIRECT INTRA-OPH- THALMIC ARTERY MELPHALAN TREATMENT FOR RE- FRACTORY RETINOBLASTOMA M Ashwin Reddy1,2, Wisam J. Muen1, Judith Kingston1,3, John Hungerford2, Fergus Robertson4, Stefan Brew4, Mandeep Sagoo1,2, Dorothy Thompson5 (mashwinreddy@hotmail.com) 1. Retinoblastoma Unit, Barts & the London NHS Trust, Royal London Hospital, Whitechapel Road 2. Moorfields Eye Hospital, City Road 3. Oncology Department, Great Ormond Street Children’s Hospital 4. National Hospital for Neurology and Neurosurgery, Queens Square 5. Ophthalmology Department, Great Ormond Street Children’s Hospital, London, UK Purpose. We report on patients receiving Intra-ophthalmic Artery Melphalan (IAM), which was delivered via direct catheterisation of the ophthalmic artery in cases of retinoblastoma refractory to systemic chemotherapy +/- radiation . Methods. All cases undergoing IAM between May 09 to August 10 were included and the results of ocular complications including reduced vision were recorded. Minimum follow-up was 11 months. Results. 15 eyes of 14 patients were treated. 5 developed IIIrd nerve palsies which resolved within 6 months. 5 patients with prior radiation developed more severe ocular complications. 2 patients demonstrated good ERG function post-treatment but a subtle deterioration of patternonset VEPs. 1 child had her vision reduce from 20/20 to 20/40 with treatment. 10 of 15 eyes (66%) showed a good tumour response. 2 patients with vitreous seeding required enucleation despite systemic chemotherapy and intraarterial melphalan. Conclusions. Potentially amblyogenic complications may occur when using direct IAM. Complications are exacerbated with prior radiation. Families undergoing this experimental treatment need to be counselled accordingly. Financial disclosure. None RETINOBLASTOMA Abstracts 44 438 RB28 LIMITATIONS OF THE INTERNATIONAL CLASSIFICA- TION IN PREDICTING SUCCESS OF INTRA-ARTERIAL CHEMOTHERAPY FOR GROUP D/E INTRAOCULAR RETINOBLASTOMA Sotiria Palioura, MD, PhD, Y. Pierre Gobin, MD, Scott E. Brodie, MD, PhD, Brian P. Marr, MD, Ira J. Dunkel, MD, and David H. Abramson, MD (sotiria. palioura@gmail.com) Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York. Currently, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston. Division of Interventional Neuroradiology, Departments of Radiology, Neurosurgery and Neurology, Weill Cornell Medical College, New York Presbyterian Hospital, New York; Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York; and Department of Ophthalmology, Mount Sinai School of Medicine, New York; Brian P. Marr: Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York; Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York; David H. Abramson: Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. Purpose. To report the success rate of superselective ophthalmic artery chemotherapy for International Classification group D and E retinoblastoma eyes and to compare it to reported success rates of systemic chemoreduction. Methods. Retrospective review of 41 group D (n=33) and E (n=8) eyes of 40 retinoblastoma patients who were treated (May 2006-June 2011) with intra-arterial chemotherapy as primary treatment. Kaplan Meier data analysis on ocular event-free (enucleation or external beam radiation) survival is reported. The PubMed database was searched through June 2011 for studies reporting success rates of systemic chemoreduction for Group D and E eyes. Results. The Kaplan-Meier estimates of ocular event-free survival at 2 years were 75.5% (95% confidence interval, 58.2%-92.9%) for group D eyes and 100% (95% confidence interval, 62.5%-100%) for group E eyes. The success rates of systemic chemoreduction for group D eyes reported in the literature range from 23% to 47%. According to our literature search, all group E eyes were enucleated either at presentation or after failed systemic chemoreduction and/or external beam radiation. Conclusions. The high success rate of intra-arterial chemotherapy for group D and E eyes suggests that the International Classification falls short when predicting intra-arterial treatment success. It seems that clinical features used by the International Classification that would deem an eye “hopeless” for chemoreduction are not predictors of treatment failure when intra-arterial chemotherapy is used instead. Financial disclosure. None 2330 RB29 INTRA-ARTERIAL MELPHALAN DOSING REGIMENS FOR THE TREATMENT OF RETINOBLASTOMA Timothy G. Murray1, Samuel K. Houston1, Mohammad A. Aziz-Sultan2, Christina E. Fernandes3, Christina Decatur1, Yolanda Pina1 (TMurray@ med.miami.edu) 1. Bascom Palmer Eye Institute; 2. University of Miami, Department of Neurosurgery 3. University of Miami, Department of Pediatrics
Purpose. The purpose of this study is to demonstrate dosing protocols for intraarterial chemotherapy for the treatment of children with retinoblastoma. Methods. his is an interventional case series of 21 patients with advanced retinoblastoma (Reese-Ellsworth Vb) treated with intraarterial chemotherapy and focal laser ablation. Dosing regimens included 3 different strategies. Patients were treated with either: (1) single dose of 7.5mg if unilateral disease; (2) repetitive treatment with 5mg or 7.5mg for 3 sessions; (3) tandem, bilateral therapy with 7.5mg to the more advanced eye for a total of 12.5mg. Six patients were treated with 3mg initially, then retreated at higher doses. Patients were evaluated by indirect ophthalmoscopy and wide-field photography for tumor response. Results. Patients tolerated treatment well, without severe side effects of stroke or death. Mild local toxicities were observed, as well 4 cases of vitreous hemorrhage. However, vitreous hemorrhage occurred in patients who were treated with lower doses (less than 5mg), then retreated with higher doses secondary to a lack of response at the lower dose. Systemic side effects were mild, with mild cytopenias not requiring hospitalization. 75% of patients avoided enucleation or radiation therapy, with patients treated at lower doses (3 or 5mg) requiring enucleation 36% of the time versus 0% for those treated at higher doses (7.5mg). Conclusions. Superselective intraarterial chemotherapy offers an exciting globe-salvaging technique for the primary and salvage treatment of retinoblastoma. Standard dosing regimens have not been determined and vary between institutions. The current study presents dosing strategies for intraarterial melphalan in the treatment of retinoblastoma at a single specialized center. Financial disclosure. None 15 RB30 THE GENERAL HEALTH AND PSYCHOSOCIAL FUNC- TIONING OF ADULT SURVIVORS OF RETINOBLAS- TOMA: PRELIMINARY RESULTS OF THE RETINOBLAS- TOMA SURVIVOR STUDY Ira J Dunkel1, Jennifer S Ford1, Charles A Sklar1, Kevin C Oeffinger1, Joanne F Chou1, Yuelin Li1, Danielle Novetsky Friedman1, Mary McCabe1, Nancy Kline1, Leslie L Robison 2, Ruth A Kleinerman3, Brian P Marr1, David H Abramson1 (dunkeli@mskcc.org) 1. Memorial Sloan-Kettering Cancer Center, New York, NY, United States, 2. St. Jude Children’s Research Hospital, Memphis, TN, United States, 3. National Cancer Institute, Bethesda, MD, United States Purpose. To describe the health status, quality of life, and psychosocial functioning of adult retinoblastoma survivors. Methods. A self-report descriptive study (modified CCSS questionnaire with added VFQ-25 vision questions) was conducted via mail and/or telephone interview with adult retinoblastoma survivors who had been treated in New York. Results. 470 (48%) of 987 potential subjects participated. Mean age at study entry was 43.4 years (SD 11); 52.1% are female, 90.4% white/non- Hispanic, and 53.6% had bilateral retinoblastoma. Most were currently employed full-time (72.2%), married (54.4%), and had at least some college education (81.0%). The majority endorsed having good overall health (94.4%), having good eyesight (61.4%), and having no physical disabilities (61.8%), but 15.4% reported difficulty obtaining health insurance. A history of psychological dysfunction was reported by 30.7%, and 35.4% reported having anxiety as a result of their retinoblastoma. Many worried about passing retinoblastoma to their children (52.3%), had concerns about their future health (69.9%) or developing a cancer RETINOBLASTOMA Abstracts 45 (70.5%), and were dissatisfied with their facial appearance (78.2%). Participants who reported any disability, poor health, anxiety due to retinoblastoma, worry about passing retinoblastoma to their children, concerns about future health or developing a cancer, were significantly more likely to have been diagnosed with bilateral than unilateral retinoblastoma. Conclusions. Most adult retinoblastoma survivors are high functioning and healthy, but many expressed concerns about the long-term implications of their retinoblastoma. Future analyses will be conducted to compare these results to a control (CCSS sibling) population. Financial disclosure. None 2323 RB31 LATE MEDICAL OUTCOMES IN SURVIVORS OF EXTRA- OCULAR RETINOBLASTOMA: THE MEMORIAL SLOAN- KETTERING CANCER CENTER (MSKCC) EXPERIENCE D. Novetsky-Friedman1, C.A. Sklar1, K.C. Oeffinger1, N.A. Kernan1, Y. Khakoo1, B.P. Marr2, S.L. Wolden3, D.H. Abramson2, I.J. Dunkel1 (friedmad@mskcc.org) 1. Department of Pediatrics, 2. Ophthalmic Oncology Service; 3. Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York, USA Purpose. To provide the first report characterizing chronic health conditions among survivors of extra-ocular retinoblastoma. Methods. Retrospective analysis of late medical outcomes in 20 survivors of extra-ocular retinoblastoma diagnosed between 1990 and 2009. Late effects were graded using the NCI’s CTCAE v4.0 scale. All patients received intensive multimodality therapy, which included conventional chemotherapy (n=20, 100%), radiation therapy (n=14, 70%), and/or high-dose chemotherapy with autologous stem cell transplant (n=18, 90%). Results. The median follow-up was 10.9 years from initial diagnosis of intra-ocular RB (range 1.8-18.8 years) and 7 years from diagnosis of extra-ocular RB (range 0.9-18.4 years). After excluding visual defects, which were present in 100% of survivors, the most common long-term complications were hearing loss (n=14, 70%), short stature (n=8, 40%), and neurocognitive delay (n=7, 35%). Eighty-percent of survivors exhibited ≥2 non-visual long-term effects of any grade. Except short stature, which was not graded for severity, grade 3-4 toxicities were limited to: ototoxicity (n=8; n=4 require hearing aids), secondary malignancies (n=5), ovarian dysfunction (n=1), and unequal limb length (n=1). Grade 4 secondary malignant neoplasms (SMNs) developed at a median of 11.6 years after initial diagnosis; two of the five patients died of their SMN. Cardiac, pulmonary, hepatobiliary or renal toxicities were not identified in any survivors. Conclusions. Late effects are commonly seen in survivors of extra-ocular retinoblastoma but the majority are mild-moderate in their severity. Longer comprehensive follow-up is needed to fully assess treatmentrelated chronic health conditions in this population. Financial disclosure. None 1755 RB32 VARIATION OF SECOND CANCER RISK BY FAMILY HISTORY OF RETINOBLASTOMA AMONG LONG-TERM SURVIVORS
Page 1 and 2: XV th NH City Hotel and Tower Boliv
Page 3 and 4: List of Congress Meetings of the In
Page 5: Table of Contents Welcome address K
Page 8 and 9: Keynote Lectures Lymphoma of the oc
Page 10 and 11: The venue for the Biannual Meeting
Page 12 and 13: Buenos Aires Downtown MAP 12 Venue
Page 15 and 16: MORNING 8.30-10.10 Papers (Moderato
Page 17 and 18: 50 RES 26 USING THE GLYCOLYTIC INHI
Page 19 and 20: 2226 RES 4 NOTCH SIGNALING PROMOTES
Page 21 and 22: a reduction in the number of living
Page 23 and 24: array further detected copy number
Page 25 and 26: 615 RES 21 TRB2 AND SKP2 IN THE RET
Page 27: following treatment with 2-fluorode
Page 30 and 31: 1909 Rb14 RESULTS OF PATIENTS WITH
Page 32 and 33: Posters Retinoblastoma 2006 RBp100
Page 34 and 35: 120 RBp135 MANAGEMENT OF AN ECTOPIC
Page 36 and 37: After treatment, 10 patients lived
Page 38 and 39: Methods. Six cycles of carboplatin
Page 40 and 41: 1940 RB15 PROTON THERAPY FOR RECURR
Page 42 and 43: 4. Fluoroscopy for cannula placemen
Page 46 and 47: Ruth A. Kleinerman1, Chu-ling Yu1,
Page 48 and 49: 2006 RBp100 RETINOBLASTOMA ASSESSME
Page 50 and 51: months. Mean delay between beginnin
Page 52 and 53: not associated with severity of RB.
Page 54 and 55: Conclusions. During a ten-year peri
Page 56 and 57: 30 RBp127 TREATMENT MODULATION IN R
Page 58 and 59: (follow-up 5 years). Two years afte
Page 61 and 62: 8.30-9.00 Poster presentations ECOp
Page 63 and 64: Uvea (Moderators: B. Damato, M. Sag
Page 65 and 66: Morning 8.30-9.00 Poster presentati
Page 67 and 68: 1621 EC1 EVALUATION OF THE “HEDGE
Page 69 and 70: Purpose. Ocular surface squamous ne
Page 71 and 72: (cargusale@yahoo.com) Oncology Serv
Page 73 and 74: of the tissues most commonly affect
Page 75 and 76: 2128 RF1 RETINOBLASTOMA David H. Ab
Page 77 and 78: Ocular Oncology Service, St Barthol
Page 79 and 80: Arturo Irarrazaval, Pablo Cazon, Os
Page 81 and 82: A CASE OF TEARING AND SWELLING OF T
Page 83 and 84: 1849 ECp101 FIVE CASES OF CARCINOMA
Page 85 and 86: patients. The isolated involvement
Page 87 and 88: exudative fluid from the hemangioma
Page 89: maximum of 7 rituximab injections.
Page 92 and 93: 153 UM14 PHENO-GENOTYPIC IDENTIFICA
Page 94 and 95:
8.30-9.00 Poster presentations UMp1
Page 96 and 97:
2253 UM1 PRELIMINARY STUDY OF VASCU
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Purpose. To determine the presence
Page 100 and 101:
gene expression profile of melanocy
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the rarity of UM, timely completion
Page 104 and 105:
C. Metz, T. Gkika, W. Sauerwein, N.
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Patients in the triamcinolone group
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1350 Ump101 INDUCED EXPRESSION OF P
Page 110 and 111:
Purpose. To report the Results of R
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1547 Ump113 LONG-TERM OBSERVATIONS
Page 114 and 115:
2038 Ump119 VALUE OF DOPPLER ANALYS
Page 116:
Methods. A total of 4070 patients w
Page 119:
First authors Naseripour, Masood No
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