112 results on '"Krema H"'
Search Results
2. 831P Outcomes of immune checkpoint inhibitors in patients with metastatic uveal melanoma treated with tebentafusp
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Koch, E.C., primary, Arteaga Ceballos, D.P., additional, Vilbert, M., additional, Lajkosz, K., additional, Pimentel Muniz, T., additional, Hirsch, I., additional, Silva Almeida Ribeiro, M.F., additional, Mantle, L., additional, Anczurowski, M., additional, Hogg, D., additional, Saibil, S., additional, Spreafico, A., additional, Krema, H., additional, and Butler, M.O., additional
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- 2022
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3. Conjunctival melanoma treatment outcomes in 288 patients: a multicentre international data-sharing study
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Jain, P., Finger, P.T., Fili, M., Damato, B., Coupland, S.E., Heimann, H., Kenawy, N., Brouwer, N.J., Marinkovic, M., Duinen, S.G. van, Caujolle, J.P., Maschi, C., Seregard, S., Pelayes, D., Folgar, M., Yousef, Y.A., Krema, H., Gallie, B., Calle-Vasquez, A., and Amer Joint Comm Canc Ophthal
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Adult ,Male ,medicine.medical_specialty ,Epidemiology ,medicine.medical_treatment ,Brachytherapy ,Treatment outcome ,Antineoplastic Agents ,Conjunctival Neoplasms ,Cryotherapy ,Kaplan-Meier Estimate ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Treatment Surgery ,Pathology ,medicine ,Humans ,External beam radiotherapy ,Melanoma ,Aged ,Retrospective Studies ,business.industry ,Cancer ,Middle Aged ,Clinical Science ,medicine.disease ,Combined Modality Therapy ,Sensory Systems ,Surgery ,Ophthalmology ,Treatment Outcome ,Current management ,Chemotherapy, Adjuvant ,030220 oncology & carcinogenesis ,030221 ophthalmology & optometry ,Female ,Treatment Medical ,Neoplasm Recurrence, Local ,Protons ,business ,Conjunctiva ,Conjunctival Melanoma - Abstract
BackgroundTo relate conjunctival melanoma characteristics to local control.MethodsRetrospective, registry-based interventional study with data gathered from 10 ophthalmic oncology centres from 9 countries on 4 continents. Conjunctival melanoma patients diagnosed between January 2001 and December 2013 were enrolled in the study. Primary treatments included local excision, excision with cryotherapy and exenteration. Adjuvant treatments included topical chemotherapy, brachytherapy, proton and external beam radiotherapy (EBRT). Cumulative 5-year and 10-year Kaplan-Meier local recurrence rates were related to clinical and pathological T-categories of the eighth edition of the American Joint Committee on Cancer (AJCC) staging system.Results288 patients had a mean initial age of 59.7±16.8 years. Clinical T-categories (cT) were cT1 (n=218,75.7%), cT2 (n=34, 11.8%), cT3 (n=15, 5.2%), cTx (n=21,7.3%) with no cT4. Primary treatment included local excision (n=161/288, 55.9%) followed by excision biopsy with cryotherapy (n=108/288, 37.5%) and exenteration (n=5/288, 1.7%). Adjuvant therapies included topical mitomycin (n=107/288, 37.1%), plaque-brachytherapy (n=55/288, 19.1%), proton-beam (n=36/288, 13.5%), topical interferon (n=20/288, 6.9%) and EBRT (n=15/288, 5.2%). Secondary exenteration was performed (n=11/283, 3.9%). Local recurrence was noted in 19.1% (median=3.6 years). Cumulative local recurrence was 5.4% (3.2–8.9%), 19.3% (14.4–25.5%) and 36.9% (26.5–49.9%) at 1, 5 and 10 years, respectively. cT3 and cT2 tumors were twice as likely to recur than cT1 tumours, but only cT3 had statistically significantly greater risk of local recurrence than T1 (p=0.013). Factors such as tumour ulceration, plica or caruncle involvement and tumour thickness were not significantly associated with an increased risk of local recurrence.ConclusionThis multicentre international study showed that eighth edition of AJCC tumour staging was related to the risk of local recurrence of conjunctival melanoma after treatment. The 10-year cumulative local recurrence remains high despite current management.
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- 2020
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4. Stereotactic radiotherapy for treatment of juxtapapillary choroidal melanoma: 3-year follow-up
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Krema, H., Somani, S., Sahgal, A., Xu, W., Heydarian, M., Payne, D., McGowan, H., Michaels, H., Simpson, E.R., and Laperriere, N.
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Choroid -- Diseases ,Eye cancer -- Care and treatment ,Eye cancer -- Research ,Radiotherapy -- Patient outcomes ,Radiotherapy -- Research ,Melanoma -- Care and treatment ,Melanoma -- Patient outcomes ,Melanoma -- Research ,Health - Published
- 2009
5. 1131P Management of metastatic uveal melanoma (MUM) patients on tebentafusp in a real-world setting
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Ribeiro, M.F., Hirsch, I., Koch, E.C., Mantle, L., Pimentel Muniz, T., Silveira Vilbert, M., Abdulalem, K., Arteaga Ceballos, D.P., Genta, S., Spiliopoulou, P., Vornicova, O., Fournier, C., Giovannetti, E., Krema, H., Saibil, S., Spreafico, A., and Butler, M.O.
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- 2023
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6. 1144P Clinical predictors of therapeutic benefit from anti-PD1 immune checkpoint inhibitors (ICI) in patients (pts) with metastatic uveal melanoma
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Rose, A.A.N., primary, Kelly, D., additional, Hogg, D., additional, Butler, M.O., additional, Saibil, S., additional, Krema, H., additional, King, I., additional, Kamil, Z. Saeed, additional, Ghazarian, D., additional, Ceballos, D.P. Arteaga, additional, Araujo, D.V., additional, Muniz, T., additional, Waldron, J., additional, Laperriere, N., additional, and Spreafico, A., additional
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- 2020
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7. Unusual Orange-Colored Choroidal Metastases
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Krema, H., Mcgowan, H., Tanzer, H., Simpson, R., and Normand Laperriere
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Eye ,Metastasis ,Color ,Orange ,Neuroendocrine ,Sarcoma ,lcsh:Ophthalmology ,lcsh:RE1-994 ,Case Report ,sense organs ,eye diseases - Abstract
Purpose: To report the clinical and paraclinical features of two patients with orangecolored choroidal metastases in whom the primary cancers have not previously been associated with such lesions. Case Report: Orange-colored choroidal lesions were detected on the fundus examination of one patient with metastatic small cell neuroendocrine tumor of the larynx and oropharynx, and in another subject with metastatic alveolar soft part sarcoma of the leg. Although ultrasonographic characteristics of the choroidal masses were comparable to those of choroidal hemangiomas, fluorescein angiography revealed delayed initial fluorescence along with minimal fluorescence in subsequent phases of the angiogram which were in clear distinction from the earlier appearing and progressively intense fluorescence observed with circumscribed choroidal hemangiomas. Conclusion: Small cell neuroendocrine tumors and alveolar soft part sarcomas should be considered among the differential diagnoses for orange-colored choroidal metastases. Identifying these choroidal lesions could facilitate localizing the occult primary tumor. Fluorescein angiography may differentiate a unifocal orange choroidal metastasis from a circumscribed choroidal hemangioma.
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- 2013
8. The Pediatric Choroidal and Ciliary Body Melanoma Study : A Survey by the European Ophthalmic Oncology Group
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Al-Jamal, R.T., Cassoux, N., Desjardins, L., Damato, B., Konstantinidis, L., Coupland, S.E., Heimann, H., Petrovic, A., Zografos, L., Schalenbourg, A., Velazquez-Martin, J.P., Krema, H., Bogdali, A., Markiewicz, A., Romanowska-Dixon, B., Metz, C.H.D., Biewald, E., Bornfeld, N., Kiratli, H., Bronkhorst, I.H.G., Jager, M.J., Marinkovic, M., Fili, M., Seregard, S., Frenkel, S., Pe'er, J., Salvi, S.M., Rennie, I.G., Rospond-Kubiak, I., Kociecki, J., Kiilgaard, J.F., Heegaard, S., Cohen, V.M.L., Sagoo, M.S., Amiryan, A., Saakyan, S., Md, N.I., Krohn, J., Midena, E., Parrozzani, R., Grange, J.D., Kilic, E., Blasi, M.A., Saornil, M.A., Kivela, T.T., Silmäklinikka, Department of Ophthalmology and Otorhinolaryngology, Clinicum, and Ophthalmology
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Male ,Uveal Neoplasms ,PROGNOSIS ,Adolescent ,Medizin ,CHILDREN ,Ophthalmologic Surgical Procedures ,Medical Oncology ,THERAPY ,Eye Enucleation ,MALIGNANT-MELANOMA ,Young Adult ,AGE ,Child ,Child, Preschool ,Choroid Neoplasms ,Ciliary Body ,Europe ,Female ,Health Surveys ,Humans ,Melanoma ,Neoplasm Recurrence, Local ,Ophthalmology ,Photochemotherapy ,Radiotherapy ,Retrospective Studies ,Survival Rate ,Medicine (all) ,3125 Otorhinolaryngology, ophthalmology ,Preschool ,POSTERIOR UVEAL MELANOMA ,NEUROFIBROMATOSIS ,Settore MED/30 - MALATTIE APPARATO VISIVO ,MELANOCYTOSIS ,ADULTS ,Neoplasm Recurrence ,Local ,YOUNG-PATIENTS - Abstract
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. (C) 2016 by the American Academy of Ophthalmology.
- Published
- 2016
9. Hypofractionated Palliative Radiation Therapy for Choroidal Metastases: Clinical Outcomes and Predictors of Overall Survival
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Hahn, E., primary, Velazquez-Martin, J.P., additional, Somani, S., additional, Payne, D., additional, Waldron, J.N., additional, Krema, H., additional, Simpson, R., additional, Laperriere, N.J., additional, and Chung, C., additional
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- 2016
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10. Bilateral diffuse uveal melanocytic proliferation: a management dilemma
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Alrashidi, S., primary, Aziz, A. A., additional, and Krema, H., additional
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- 2014
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11. Visual loss from scleritis in C-ANCA-positive microscopic polyangiitis
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Alrashidi, S., primary, Yousef, Y. A., additional, and Krema, H., additional
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- 2013
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12. A Non-invasive Eye Fixation and Monitoring System for the Treatment of Choroidal Melanoma (CM) Using Gamma-knife (GK) Radiosurgery
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Heydarian, M., primary, Wilson, G., additional, van Prooijen, M., additional, Young-Bin, C., additional, Krema, H., additional, Tamerou, M., additional, Chung, C., additional, Laperriere, N., additional, and Jaffray, D., additional
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- 2012
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13. EP-1249 TUMOR CONTROL AND COSMETIC OUTCOMES OF ORTHOVOLTAGE RADIOTHERAPY FOR MEDIAL CANTHAL BASAL CELL CARCINOMAS
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Herrmann, E., primary, Krema, H., additional, Payne, D., additional, Simpson, R., additional, Laperriere, N., additional, and Chung, C., additional
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- 2012
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14. Management of iris melanomas with 125 iodine plaque radiotherapy
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FERNANDES, BF, primary, KREMA, H, additional, FULDA, E, additional, PAVLIN, CJ, additional, PAYNE, DG, additional, MCGOWAN, HD, additional, BURNIER, MN, additional, and SIMPSON, ER, additional
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- 2010
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15. SU-GG-T-86: Retrospective Evaluation of Symmetrically Loaded COMS Plaques for Juxtapapillary Choroidal Melanoma Patients Using Plaque Simulator
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Beiki-Ardakani, A, primary, Krema, H, additional, Sahgal, A, additional, Simpson, R, additional, Jezioranski, J, additional, Heydarian, M, additional, Xu, W, additional, Weisbrod, D, additional, Payne, D, additional, and Laperriere, N, additional
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- 2010
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16. 21 SIGNIFICANT LATE TOXICITIES ASSOCIATED WITH STEREOTACTIC RADIOTHERAPY FOR JUXTAPAPILLARY CHOROIDAL MELANOMA
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Sahgal, A., primary, Somani, S., additional, Xu, W., additional, Krema, H., additional, Heydarian, M., additional, Michaels, H., additional, McGowan, H., additional, Payne, D., additional, Simpson, R., additional, and Laperriere, N., additional
- Published
- 2009
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17. Sci-Fri AM(2): Brachy-08: Dosimetric Comparison of Different Radiation Techniques in the Treatment of Juxtapapillary Choroidal Melanoma
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Heydarian, M, primary, van-Prooijen, M, additional, Beiki-Ardakani, A, additional, Ruschin, M, additional, Sahgal, A, additional, Krema, H, additional, Payne, D, additional, and Laperriere, N, additional
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- 2009
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18. Management of iris melanomas with 125Iodine plaque radiotherapy.
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Fernandes BF, Krema H, Fulda E, Pavlin CJ, Payne DG, McGowan HD, Simpson ER, Fernandes, Bruno F, Krema, Hatem, Fulda, Emiliano, Pavlin, Charles J, Payne, David G, McGowan, Hugh D, and Simpson, Ernest Rand
- Abstract
Purpose: To report the experience of the Princess Margaret Hospital/University Health Network with the treatment of iris melanoma (IM) with (125)Iodine plaque brachytherapy.Design: Retrospective noncomparative case series.Methods: All cases of IM submitted to (125)Iodine plaque radiotherapy were included. Patients' demographic, clinical, management, and follow-up data were reviewed. Outcome measures included rates of tumor control, eye preservation, systemic metastases, and brachytherapy-related complications.Results: Fourteen IMs were included in the study. All patients had blue/green irises. Mean largest basal dimension and thickness were 7.1 +/- 2.1 mm (range, 4.0 to 11.5 mm) and 2.2 +/- 0.8 mm (range, 1.0 to 3.5 mm), respectively. Ten patients (71%) had seeding and 2 (14%) had glaucoma at presentation. Median follow-up was 26.6 +/- 19.5 months (range, 6 to 72 months). Tumor control was achieved in 100% of the cases and no eye was enucleated because of radiation-induced complications. At last visit, all patients were alive and free of metastasis. Final visual acuity was the same as or better than before treatment in 9 patients (75%). Cataract was the most common complication (8; 75%), followed by persistent glaucoma (2; 17%) and anterior uveitis (1; 8%). No other significant complication was seen during the follow-up period.Conclusions: Plaque radiotherapy is a safe and effective conservative treatment option for IM, although cataract is a common, yet treatable, complication. This treatment scheme circumvents an intraocular procedure and may avoid the dissemination of malignant cells, and provides a margin of safety in the treatment of clinically undetectable disease. [ABSTRACT FROM AUTHOR]- Published
- 2010
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19. Outcome of patients with periocular sebaceous gland carcinoma with and without conjunctival intraepithelial invasion
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Chao, A. N., Shields, C. L., Krema, H., and Shields, J. A.
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- 2001
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20. Looking through a cataract
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Singh, A., Krema, H., and Singh, A. D.
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- 2001
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21. Metastatic conjunctival melanoma: a multicentre international study.
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Jain P, Finger PT, Filì M, Damato B, Coupland SE, Heimann H, Kenawy N, Brouwer NJ, Marinkovic M, van Duinen S, Caujolle JP, Maschi C, Seregard S, Pelayes DE, Folgar M, Yousef Y, Krema H, Gallie B, and Calle Vasquez CA
- Abstract
Background: To reveal clinical findings related to metastatic conjunctival melanoma., Methods: 10 ophthalmic oncology centres (9 countries and 4 continents) shared data to create a large clinical case series. The main outcome measures were the incidence and cumulative risk of systemic metastasis, study mortality rates and Kaplan-Meier patient mortality after developing conjunctival melanoma metastasis., Results: Of 288 patients, 29 developed metastasis. Five had metastasis at presentation, were American Joint Committee on Cancer (AJCC) cT3-category and exhibited tumour-surface ulceration. Four of five (80%) had melanotic tumours with plical and/or caruncular involvement and died within 1 year. One survived 21 months. In contrast, 24 developed metastases during follow-up (mean 4.6±3.2 years). Their primary tumours were cT1 (n=13/24, 54.1%), cT2 (n=6/24, 25%), cT3 (n=2/24, 8.3%) and 3 cTx (12.5%) at presentation. Death had occurred in 17 patients (n=17/24, 71%) by the end of the study. The cumulative risk of systemic metastasis after treatment was 0.4% (95% CI 0.6% to 2.9%) at 1 year, 8.6% (95% CI 5.1% to 14.3%) at 5 years and 22.3% (95% CI 14.5% to 33.5%) at 10 years. Each increase in AJCC cT category was associated with an 89% higher risk for metastasis (HR=1.89, p<0.001). Among all 29 patients who developed metastasis, those who presented with AJCC cT3 disease were at highest risk (p<0.001). Liver and lung (n=13 each) were the most reported metastatic sites., Conclusion: Metastatic conjunctival melanoma was found in 10% of conjunctival melanoma patients. Tumour-specific characteristics including AJCC cT3-category, conjunctival location and surface ulceration were associated with metastatic risk. Survival durations were shorter for those presenting with metastasis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.)
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- 2024
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22. Iodine-125 plaque brachytherapy for the treatment of iris cavernous hemangioma.
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Abrishami M, Beiki-Ardakani A, and Krema H
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- 2024
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23. Primary conjunctival basal cell carcinoma treated with plaque brachytherapy: a rare case report.
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Nag A, Krema H, Saeed Kamil Z, Akbar BA, and Laperriere N
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Primary basal cell carcinoma (BCC) of the conjunctiva is a rare tumor with only six cases reported in literature. We present the case of an 81-year-old male complaining of a slow-growing lesion in his right eye for the past 3 years. The lesion in the temporal bulbar conjunctiva was gelatinous and firmly adherent to sclera. Ultrasound biomicroscopy revealed apparent scleral thinning without invasion. Our provisional diagnosis was ocular surface squamous neoplasia; however, incisional biopsy for histopathological diagnosis revealed that it was a BCC. Due to scleral adhesion and thinning, the lesion was not amenable for surgical resection. Plaque brachytherapy was used to successfully treat the lesion. To the best of our knowledge, this is the first report of brachytherapy administration for treatment of this rare cancer. Previously reported cases were all nodular or pedunculated and treated with surgical excision. For flat, adherent, or invasive lesions, plaque brachytherapy is an effective primary treatment modality.
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- 2024
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24. Intramuscular Hemangioma of Lateral Rectus Muscle with Rare Presentation as an Epibulbar Mass: A Case Report and Review of Literature.
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Nag A, Krema H, Kamil ZS, and Al-Mbaideen S
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Introduction: Intramuscular hemangiomas of extraocular muscles are extremely rare tumors that usually present as retro-orbital masses causing proptosis. We describe a previously unreported presentation, in the form of an epibulbar mass; this easily accessible location allows direct imaging, complete surgical resection, and histopathological confirmation, providing a unique perspective., Case Presentation: A 69-year-old woman presented with a painless dark red mass in the lateral part of the right eye, which had been slowly enlarging over the last 18 months. Clinical features and imaging were suggestive of a benign vascular tumor of the conjunctiva. During surgical resection, the mass was observed to be enmeshed within the fibers of the lateral rectus muscle. Careful dissection from muscle fibers was needed for complete excision. Histopathology revealed the diagnosis of an intramuscular hemangioma of extraocular muscle., Conclusion: In this report, we describe the atypical anterior epibulbar presentation of intramuscular hemangioma of the lateral rectus muscle. We discuss the differential diagnoses and management of this rare tumor along with a review of existing literature. Careful surgical resection achieved complete resolution in this case without recurrence., Competing Interests: The authors have no conflicts of interest to declare., (© 2024 The Author(s). Published by S. Karger AG, Basel.)
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- 2024
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25. Rhegmatogenous retinal detachment associated with an epibulbar tumour.
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Abrishami M, Smith SM, Slomovic AR, Altomare F, and Krema H
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- Humans, Tomography, Optical Coherence methods, Visual Acuity, Male, Female, Eye Neoplasms diagnosis, Eye Neoplasms complications, Eye Neoplasms surgery, Ciliary Body pathology, Ciliary Body diagnostic imaging, Vitrectomy, Scleral Buckling, Retinal Detachment diagnosis, Retinal Detachment etiology, Retinal Detachment surgery
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- 2024
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26. Optic Nerve Sheath Meningioma Presenting as a Slow-Growing Intraocular Mass.
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Lando L, Munoz DG, Dangboon W, Krema H, and Altomare F
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- Humans, Optic Nerve diagnostic imaging, Meningioma diagnostic imaging, Meningioma surgery, Optic Nerve Neoplasms diagnosis, Optic Nerve Diseases, Meningeal Neoplasms diagnosis, Meningeal Neoplasms surgery
- Abstract
Competing Interests: The authors report no conflicts of interest.
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- 2024
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27. Simple and effective immobilization for radiation treatment of choroidal melanoma.
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van Prooijen M, Chau L, Tsui G, Kelly V, Holwell M, Tadic T, Tsang DS, Krema H, and Laperriere N
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At our institution, patients diagnosed with choroidal melanoma requiring external beam radiation therapy are treated with two 6 MV volumetric-modulated arcs delivering 50 Gy over 5 daily fractions. The patient is immobilized using an Orfit head and neck mask and is directed to look at a light emitting diode (LED) during CT simulation and treatment to minimize eye movement. Patient positioning is checked with cone beam computed tomography (CBCT) daily. Translational and rotational displacements greater than 1 mm or 1° off the planned isocenter position are corrected using a Hexapod couch. The aim of this study is to verify that the mask system provides adequate immobilization and to verify our 2-mm planning target volume (PTV) margins are sufficient. Residual displacements provided by pretreatment verification and post-treatment CBCT data sets were used to assess the impact of patient mobility during treatment on the reconstructed delivered dose to the target and organs at risk. The PTV margin calculated using van Herk's method
1 was used to assess patient motion plus other factors that affect treatment position, such as kV-MV isocenter coincidence. Patient position variations were small and were shown to not cause significant dose variations between the planned and reconstructed dose to the target and organs at risk. The PTV margin analysis showed patient translational motion alone required a PTV margin of 1 mm. Given other factors that affect treatment delivery accuracy, a 2-mm PTV margin was shown to be sufficient for treatment of 95% of our patients with 100% of dose delivered to the GTV. The mask immobilization with LED focus is robust and we showed a 2-mm PTV margin is adequate with this technique., Competing Interests: Conflicts of Interest The authors declare no conflicts of interest., (Copyright © 2023 American Association of Medical Dosimetrists. Published by Elsevier Inc. All rights reserved.)- Published
- 2023
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28. Suprachoroidal and vitreous haemorrhage as a presenting feature of metastatic melanoma.
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Kaur J, Popovic M, Krema H, and Koushan K
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- Male, Humans, Vitreous Hemorrhage etiology, Vitreous Hemorrhage pathology, Vitreous Body pathology, Melanoma pathology, Skin Neoplasms pathology, Eye Neoplasms pathology, Neoplasms, Second Primary pathology
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A man in his 70s presented with right ocular pain, vision loss and temporal headache 4 days after undergoing laser peripheral iridotomy. The patient had lost 10 lbs over the preceding 6 weeks and had a medical history significant for a previously excised melanoma of the left arm. During the course of investigations, the patient was referred to oncology for workup, ultimately leading to a diagnosis of metastatic melanoma. Visceral metastases were identified in the lungs, right anterior fourth rib, left femoral distal diaphysis and medial side of the right globe. The patient decompensated and died shortly after his first radiotherapy treatment.This is the first published report of suprachoroidal haemorrhage secondary to metastasis from suspected cutaneous melanoma. The case highlights the importance of considering malignancy on the differential diagnosis for a suprachoroidal haemorrhage of unknown aetiology and involving oncology early for workup and treatment., Competing Interests: Competing interests: MP received financial support from Fighting Blindness Canada and the PSI Foundation., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2023
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29. Oncologist-led germline genetic testing for uveal melanoma.
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Gillies B, Krema H, Chao A, Lando L, Farncombe KM, Butler M, Altomare F, and Kim RH
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- Humans, Retrospective Studies, Tumor Suppressor Proteins genetics, Genetic Testing, Germ-Line Mutation, Genetic Predisposition to Disease, Ubiquitin Thiolesterase genetics, Uveal Melanoma, Melanoma diagnosis, Melanoma genetics, Melanoma pathology, Uveal Neoplasms diagnosis, Uveal Neoplasms genetics, Uveal Neoplasms pathology
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Purpose: To report the genotype and phenotype of a cohort of unselected uveal melanoma (UM) patients who had germline multi-gene panel genetic testing, including the BAP1 gene, from a large multi-ethnic cancer centre. We describe the central role of the medical genetics clinic in collaboration with oncologists in a mainstreaming model to facilitate genetic testing, counselling and streamlining of patients with hereditary cancer predisposition., Methods: A retrospective chart review of clinical and genetic findings of unselected UM patients who had germline genetic testing between December 2019 and October 2021 was conducted. Extracted DNA from peripheral blood samples were analyzed with a multi-gene panel that included at least six genes associated with hereditary melanoma. The correlation between the genotype and the phenotype of the cohort was evaluated. Statistical analysis comprised descriptive and comparative statistics with significance assigned at p < .05. The genetics clinic streamlined patients among the relevant oncology clinics for cancer screening in germline BAP1 positive individuals., Results: In unselected UM patients, 3.5% (4/114) tested positive for a BAP1 pathogenic variant. Germline BAP1 status was associated with a family history of mesothelioma ( p = .0015) and metastatic disease ( p = .017). There were no other significant associations between the patient- or tumour-related characteristics and germline BAP1 results., Conclusion: A germline BAP1 mutation was detected in 3.5% of unselected UM patients. The oncologist-initiated and genetics-led mainstreaming model is a straightforward process and can be utilized for offering genetic testing to all UM patients.
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- 2023
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30. Systemic sarcoidosis presenting as acute rapidly progressive proptosis.
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Silva MVD, Chavez Y, Nicola MLD, Delabie JMA, Rose K, and Krema H
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- Humans, Orbit, Inflammation pathology, Exophthalmos etiology, Exophthalmos pathology, Sarcoidosis complications, Sarcoidosis drug therapy, Sarcoidosis pathology, Orbital Diseases drug therapy, Orbital Diseases etiology, Orbital Diseases pathology
- Abstract
Sarcoidosis is a generalized systemic chronic inflammation that rarely involves the orbit. As a chronic inflammation, sarcoidosis typically manifests with an insidious onset and slowly progressive course. We report a case of acute-onset proptosis resulting from a rapidly growing diffuse orbital mass that simulated malignant growth, which was biopsy proven to be the first manifestation of systemic sarcoidosis. The patient demonstrated complete resolution of proptosis and systemic involvement with long-term corticosteroid treatment.
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- 2023
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31. Integrated, Longitudinal Analysis of Cell-free DNA in Uveal Melanoma.
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Wong D, Luo P, Znassi N, Arteaga DP, Gray D, Danesh A, Han M, Zhao EY, Pedersen S, Prokopec S, Sundaravadanam Y, Torti D, Marsh K, Keshavarzi S, Xu W, Krema H, Joshua AM, Butler MO, and Pugh TJ
- Subjects
- Humans, Neoplasm Recurrence, Local, Uveal Melanoma, Cell-Free Nucleic Acids genetics, Melanoma diagnosis, Uveal Neoplasms diagnosis
- Abstract
Uveal melanomas are rare tumors arising from melanocytes that reside in the eye. Despite surgical or radiation treatment, approximately 50% of patients with uveal melanoma will progress to metastatic disease, most often to the liver. Cell-free DNA (cfDNA) sequencing is a promising technology due to the minimally invasive sample collection and ability to infer multiple aspects of tumor response. We analyzed 46 serial cfDNA samples from 11 patients with uveal melanoma over a 1-year period following enucleation or brachytherapy ( n = ∼4/patient) using targeted panel, shallow whole genome, and cell-free methylated DNA immunoprecipitation sequencing. We found detection of relapse was highly variable using independent analyses ( P = 0.06-0.46), whereas a logistic regression model integrating all cfDNA profiles significantly improved relapse detection ( P = 0.02), with greatest power derived from fragmentomic profiles. This work provides support for the use of integrated analyses to improve the sensitivity of circulating tumor DNA detection using multi-modal cfDNA sequencing., Significance: Here, we demonstrate integrated, longitudinal cfDNA sequencing using multi-omic approaches is more effective than unimodal analysis. This approach supports the use of frequent blood testing using comprehensive genomic, fragmentomic, and epigenomic techniques., (© 2023 The Authors; Published by the American Association for Cancer Research.)
- Published
- 2023
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32. Cytogenetic Abnormalities for Predicting the Risk of Metastases in Choroidal and Ciliary Body Melanoma.
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Chao AN, Rose K, Racher H, Altomare F, and Krema H
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- Humans, Ciliary Body pathology, Chromosome Deletion, Monosomy, Risk Factors, Chromosomes, Human, Pair 3 genetics, Uveal Melanoma, Uveal Neoplasms genetics, Uveal Neoplasms pathology, Melanoma genetics, Melanoma pathology, Choroid Neoplasms genetics, Choroid Neoplasms pathology
- Abstract
Purpose: Choroidal melanoma (CM) and ciliary body melanoma (CBM) are the two most common subtypes of uveal melanoma. Starting from the observation that CBM tends to have a higher metastatic potential than CM, we hypothesized that specific cytogenetic abnormalities could be associated with tumor location - reflecting distinct genetic signatures that would drive the risk of distant spread., Methods: Chromosomal alterations were investigated by molecular cytogenetic techniques in 217 and 97 patients with CM and CBM, respectively. Cox proportional hazards regression analysis was used to identify the independent predictors of distant metastasis., Results: Patients with CBM had larger tumor sizes (P < 0.001), higher disease stages (P < 0.001), and more frequently showed distant metastasis (P = 0.002) than those with CM. On analyzing the entire study cohort, we found that specific chromosomal alterations - including chromosome 8p loss (P < 0.001), 1p loss (P < 0.001), and monosomy 3 (P < 0.005) - were independent predictors of distant metastasis. Based on a decision-tree learning algorithm, we identified three specific subgroups of patients with uveal melanoma at high risk of distant spread. Monosomy 3 occurred significantly more frequently in patients with T3 CBM tumors., Conclusions: Specific cytogenetic abnormalities - including chromosome 8p loss, 1p loss, and monosomy 3 - are independent risk factors for distant metastasis in uveal melanoma. Larger tumor size at presentation and monosomy 3 contribute to a higher metastatic risk in patients with CBM.
- Published
- 2023
- Full Text
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33. Hypertensive uveitis in a patient with uveal plasmacytoma.
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Lando L, Özkurt ZG, and Krema H
- Subjects
- Humans, Hypertension complications, Plasmacytoma complications, Plasmacytoma diagnosis, Uveal Neoplasms complications, Uveal Neoplasms diagnosis, Uveitis diagnosis, Uveitis etiology
- Published
- 2022
- Full Text
- View/download PDF
34. Radiation therapy for primary orbital and ocular adnexal lymphoma.
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Pereira-Da Silva MV, Di Nicola ML, Altomare F, Xu W, Tsang R, Laperriere N, and Krema H
- Abstract
Objective: To report the efficacy and toxicity of External beam Radiotherapy (EBRT) as a sole treatment for MALT and Follicular Primary Orbital and Ocular adnexal Lymphoma (POOAL)., Methods: Retrospective review of all POOAL patients treated with EBRT utilizing megavoltage photon or electron beam radiotherapy between 2003 and 2015. Patient demographics, tumour extent and pathology, radiotherapy techniques, and treatment outcomes were reviewed. The actuarial rates of tumour control and radiation toxicities were calculated using Kaplan-Meier estimates., Results: This study included 167 tumours, of which MALT lymphoma involved 149 (89 %). The conjunctiva and orbit were equally involved as the predominant site (48 %). Megavoltage photon radiotherapy was used in 60 % of predominantly orbital lymphoma and Electron beam with lens shielding in 77 % of the conjunctival lymphoma. The majority (95 %) were treated with a total dose of 25 Gy in 10 fractions. Local control rate was 98 % (CI: 93-100 %) at 5 years. The long-term RT toxicities included dry eye in 27 eyes (16 %) and cataract in 22 (13 %). None of the patients developed significant structural or functional radiation toxicity., Conclusion: External Beam Radiotherapy, with lens shielding whenever indicated, at a dose of 20-30 Gy delivered over 10-20 fractions is an efficacious and safe primary treatment option for POOAL lymphoma, with excellent local control and low incidence of late manageable ocular toxicities., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Author(s).)
- Published
- 2022
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35. Conjunctival Geographic Hemorrhage from Lymphangioma.
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Lando L and Krema H
- Subjects
- Hemorrhage, Humans, Conjunctival Neoplasms complications, Conjunctival Neoplasms diagnosis, Lymphangioma complications, Lymphangioma diagnosis
- Abstract
Competing Interests: The authors have no conflicts of interest to declare.
- Published
- 2022
- Full Text
- View/download PDF
36. Diagnosis of the origin of an epibulbar melanocytic tumor with molecular genomics.
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Gonzalez-H Leon A, Chavez Y, Kamil ZS, Ghazarian D, and Krema H
- Subjects
- Female, Genomics, Humans, Middle Aged, Melanoma diagnosis, Melanoma genetics, Melanoma pathology, Nevus, Blue genetics, Nevus, Blue pathology, Skin Neoplasms genetics, Uveal Neoplasms diagnosis, Uveal Neoplasms genetics, Uveal Neoplasms pathology
- Abstract
Background: Uveal melanoma (UM) and conjunctival melanoma (CM) are distinct entities with different etiologies and genetic background. We present a case of an atypical subconjunctival melanoma arising from a blue nevus., Patients and Methods: A 61-year-old female presented with a partially melanocytic epibulbar mass with surrounding episcleral pigmented spots. The lesion was detached from the overlying conjunctiva without an intraocular component. Excisional biopsy revealed a predominantly epithelioid melanoma, that was suggested to be metastasic, although there was no evidence of a primary melanoma elsewhere., Results: Molecular analysis identified GNAQ and BAP1 pathogenic variants, which strongly suggested the diagnosis as a primary epibulbar melanoma arising from episcleral blue nevus., Conclusion: This case demonstrates the value of tumor molecular analysis using Next Generation Sequencing (NGS) for differentiating the origin of an unusually located ocular melanoma.
- Published
- 2022
- Full Text
- View/download PDF
37. Unilateral Yellowish Eye in a Woman With an Intraocular Mass.
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Lando L, Altomare F, and Krema H
- Subjects
- Female, Humans, Intraocular Pressure
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- 2022
- Full Text
- View/download PDF
38. Cauda equina syndrome as the initial presentation of uveal melanoma metastasis.
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Hanout M, Kamil ZS, Alrjoub M, Laperriere N, Altomare F, and Krema H
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- Humans, Male, Middle Aged, Uveal Melanoma, Cauda Equina Syndrome, Choroid Neoplasms, Melanoma, Uveal Neoplasms diagnosis, Uveal Neoplasms radiotherapy
- Abstract
Purpose: Uveal melanoma extension to the central nervous system (CNS) is exceedingly rare, and can occur through optic nerve invasion. We report a rare clinical case that presented with cauda equina syndrome as the initial manifestation of metastasis of choroidal melanoma, and showed neurotropic extension by histopathology. Our patient did not demonstrate any evidence of systemic metastasis otherwise., Observations: A 60-year-old male patient with treated choroidal melanoma in his right eye, with presumed clinical control, developed radiation-induced neovascular glaucoma refractory to medical therapy. The eye required enucleation for pain control. One month post-enucleation, he presented to the emergency department with severe abdominal pain, urine retention, constipation, and leg weakness. Magnetic resonance imaging (MRI) of the spine showed extensive leptomeningeal involvement along the entire spinal cord and the cauda equina. On further inquisition, the patient noted prior visual field defect in the contralateral eye. Brain MRI revealed intracranial metastasis with chiasmal involvement. The patient underwent radiotherapy for the brain and spine to improve his symptoms, and was ultimately transferred to palliative care., Conclusion and Importance: Optic nerve invasion in uveal melanoma may lead to neurotropic spread of melanoma cells with risk of intracranial and spinal cord metastasis. Neurological symptoms should raise the suspicion of clinicians regarding this complication, which is associated with increased melanoma-related mortality.
- Published
- 2022
- Full Text
- View/download PDF
39. Pigment-induced glaucoma secondary to bilateral diffuse uveal melanocytic proliferation.
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Da Silva MV, Rose K, and Krema H
- Subjects
- Cell Proliferation, Female, Humans, Melanocytes, Glaucoma, Open-Angle etiology, Glaucoma, Open-Angle surgery, Paraneoplastic Syndromes, Ocular, Retinal Neoplasms
- Abstract
We report the first case of pigment-induced glaucoma in a patient presenting with bilateral diffuse uveal melanocytic proliferation (BDUMP) syndrome. Despite treatment with plasma exchange, the patient developed progression of her lesions and bilateral glaucoma. Surgical treatment with Ahmed valve implant was performed to control her increased intra-ocular pressure. This case demonstrates that BDUMP can be a cause of a refractory pigment-induced glaucoma.
- Published
- 2022
- Full Text
- View/download PDF
40. Complete cure of a large resistant keratoacanthoma of the eyelid with intralesional methotrexate.
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Krema H, Gursel Ozkurt Z, Lando L, and Altomare F
- Subjects
- Eyelids, Humans, Injections, Intralesional, Keratoacanthoma diagnosis, Keratoacanthoma drug therapy, Methotrexate therapeutic use
- Published
- 2021
- Full Text
- View/download PDF
41. Applications of iodine-125 plaque radiotherapy for residual or recurrent retinoblastoma.
- Author
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Soliman SE, Bansal A, De Nicola ML, Bhambhwani V, Laperriere N, Gallie BL, and Krema H
- Subjects
- Eye Enucleation, Humans, Iodine Radioisotopes, Neoplasm Recurrence, Local, Retrospective Studies, Treatment Outcome, Retinal Neoplasms radiotherapy, Retinal Neoplasms surgery, Retinoblastoma radiotherapy
- Abstract
Objective: To determine the role of iodine-125 plaque radiotherapy (IPR) as a secondary treatment for localized (solitary or multiple) residual (partially regressed) or recurrent (regrowth after ≥6 months stability) retinoblastoma in the era of systemic and/or regional chemotherapy., Design: A single-institute retrospective, noncomparative, interventional case series managed between July 2014 and June 2019., Participants: Thirteen consecutive eyes of 12 patients with 14 residual or recurrent retinoblastoma tumors treated with IPR. Patients who had to follow up <1 year post-IPR were excluded except for those who had enucleation., Methods: Data collected included pre-IPR treatments, tumor characteristics at IPR, and post-IPR anatomical outcome (local tumor control and globe salvage) and functional outcome (radiation complications)., Results: Local tumor control was achievable in 12 of 14 tumors. Local recurrences were observed in 2 of 5 tumors that exhibited fish-flesh regression after IPR (p = 0.04). Globe salvage was possible in 11 eyes (12 tumors). Only 2 eyes were legally blind and the remaining 9 eyes had vision >20/125. Radiation-induced complications included radiation retinopathy (4/11), radiation papillopathy (1/11), diffuse vitreous hemorrhage (4/11). Eyes with fish-flesh-regressed tumours tended to show more complications, but were statistically insignificant (p = 0.09, Fisher exact test). There was no association of time to IPR (early <6 months vs late >6 months) with occurrence of tumor recurrence or complications (p > 0.05)., Conclusion: IPR offers satisfactory local tumor control and globe salvage in localized recurrent/residual retinoblastoma. Fish-flesh tumor regression after IPR should be closely monitored for further recurrences., (Copyright © 2021 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
42. Spontaneous Regression of Unilateral Conjunctival Primary Acquired Melanosis.
- Author
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Lando L and Krema H
- Subjects
- Aged, 80 and over, Conjunctival Diseases physiopathology, Female, Humans, Melanocytes pathology, Melanosis physiopathology, Remission, Spontaneous, Conjunctival Diseases diagnosis, Melanosis diagnosis
- Published
- 2021
- Full Text
- View/download PDF
43. Development of a Metastatic Uveal Melanoma Prognostic Score (MUMPS) for Use in Patients Receiving Immune Checkpoint Inhibitors.
- Author
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Kelly D, Rose AAN, Muniz TP, Hogg D, Butler MO, Saibil SD, King I, Kamil ZS, Ghazarian D, Ross K, Iafolla M, Araujo DV, Waldron J, Laperriere N, Krema H, and Spreafico A
- Abstract
Metastatic uveal melanoma (mUM) is a rare disease. There are limited data on prognostic clinical factors for overall survival (OS) in patients with mUM treated with immune checkpoint inhibitors (ICI). Retrospective and non-randomized prospective studies have reported response rates of 0-17% for anti-PD1/L1 ± anti-CTLA4 ICI in mUM, indicating a potential benefit only in a subset of patients. This study evaluates the characteristics associated with ICI benefit in patients with mUM. We performed a single-center retrospective cohort study of patients with mUM who received anti-PD1/L1 ± anti-CTLA4 ICI between 2014-2019. Clinical and genomic characteristics were collected from a chart review. Treatment response and clinical progression were determined by physician assessment. Multivariable Cox regression models and Kaplan-Meier log-rank tests were used to assess differences in clinical progression-free survival (cPFS) and OS between groups and identify clinical variables associated with ICI outcomes. We identified 71 mUM patients who received 75 lines of ICI therapy. Of these, 54 received anti-PD1/L1 alone, and 21 received anti-PD1/L1 + anti-CTLA4. Patient characteristics were: 53% female, 48% were 65 or older, 72% received one or fewer lines of prior therapy. Within our cohort, 53% of patients had developed metastatic disease <2 years after their initial diagnosis. Bone metastases were present in 12% of patients. The median cPFS was 2.7 months, and the median OS was 10.0 months. In multivariable analyses for both cPFS and OS, the following variables were associated with a good prognosis: ≥2 years from the initial diagnosis to metastatic disease ( n = 25), LDH < 1.5 × ULN ( n = 45), and absence of bone metastases ( n = 66). We developed a Metastatic Uveal Melanoma Prognostic Score (MUMPS). Patients were divided into 3 MUMPS groups based on the number of the above-mentioned prognostic variables: Poor prognosis (0-1), Intermediate prognosis (2) and Good prognosis (3). Good prognosis patients experienced longer cPFS (6.0 months) and OS (34.5 months) than patients with intermediate (2.3 months cPFS, 9.4 months OS) and poor prognosis disease (1.8 months cPFS, 3.9 months OS); p < 0.0001. We developed MUMPS-a prognostic score based on retrospective data that is comprised of 3 readily available clinical variables (time to metastatic diagnosis, presence of bone metastases, and LDH). This MUMPS score has a potential prognostic value. Further validation in independent datasets is warranted to determine the role of this MUMPS score in selecting ICI treatment management for mUM.
- Published
- 2021
- Full Text
- View/download PDF
44. Cutaneous signs and risk factors for ocular melanoma.
- Author
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McDonald KA, Krema H, and Chan AW
- Subjects
- Humans, Ophthalmology, Referral and Consultation, Risk Factors, Dermatologists, Melanoma diagnosis, Physician's Role, Skin Neoplasms diagnosis, Uveal Neoplasms diagnosis
- Published
- 2021
- Full Text
- View/download PDF
45. A case of multifocal presumed solitary circumscribed retinal astrocytic proliferation lesions in the same eye.
- Author
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McKay BR, Krema H, Yan P, and Weisbrod D
- Subjects
- Astrocytes, Cell Proliferation, Fluorescein Angiography, Humans, Retinal Diseases
- Published
- 2021
- Full Text
- View/download PDF
46. Simultaneous choroidal and conjunctival metastases from renal cell carcinoma.
- Author
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Chao AN, Perez-Ordonez B, Hanout M, Rose K, and Krema H
- Subjects
- Choroid, Conjunctiva, Humans, Carcinoma, Renal Cell diagnosis, Choroid Neoplasms diagnosis, Kidney Neoplasms diagnosis
- Abstract
Competing Interests: None
- Published
- 2020
- Full Text
- View/download PDF
47. Molecular analysis confirms retinoblastoma diagnosis in a histologically undifferentiated retinal tumor in an adult.
- Author
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Soliman SE, Martínez S, De Nicola ML, Kiehl R, and Krema H
- Subjects
- Adult, Female, Humans, Retinal Neoplasms genetics, Retinoblastoma genetics, Cell Differentiation, Mutation, Retinal Neoplasms diagnosis, Retinoblastoma diagnosis, Retinoblastoma Protein genetics
- Abstract
Retinoblastoma is the most common pediatric intraocular cancer. Rarely, it may develop in adults, with different clinical and imaging characteristics that make the diagnosis a challenge. We present a case of a white retinal tumor in a 42-year-old woman that progressed slowly over 3 years and on enucleation an undifferentiated tumor was found without a conclusive diagnosis. Molecular analysis identified RB1 pathogenic variant that confirmed retinoblastoma diagnosis in this discordant clinicopathologic presentation of the tumor.
- Published
- 2020
- Full Text
- View/download PDF
48. Comprehensive characterization of a Canadian cohort of von Hippel-Lindau disease patients.
- Author
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Salama Y, Albanyan S, Szybowska M, Bullivant G, Gallinger B, Giles RH, Asa S, Badduke C, Chiorean A, Druker H, Ezzat S, Hannah-Shmouni F, Hernandez KG, Inglese C, Jani P, Kaur Y, Krema H, Krimus L, Laperriere N, Lichner Z, Mete O, Sit M, Zadeh G, Jewett MAS, Malkin D, Stockley T, Wasserman JD, Xu W, Schachter NF, and Kim RH
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Canada epidemiology, Central Nervous System metabolism, Central Nervous System pathology, Child, Child, Preschool, Female, Hemangioblastoma epidemiology, Hemangioblastoma pathology, Humans, Male, Middle Aged, Mutation, Missense genetics, Pedigree, Penetrance, Pheochromocytoma epidemiology, Pheochromocytoma pathology, Young Adult, von Hippel-Lindau Disease epidemiology, von Hippel-Lindau Disease pathology, Hemangioblastoma genetics, Pheochromocytoma genetics, Von Hippel-Lindau Tumor Suppressor Protein genetics, von Hippel-Lindau Disease genetics
- Abstract
Von Hippel-Lindau disease (VHL) is a heritable condition caused by pathogenic variants in VHL and is characterized by benign and malignant lesions in the central nervous system (CNS) and abdominal viscera. Due to its variable expressivity, existing efforts to collate VHL patient data do not adequately capture all VHL manifestations. We developed a comprehensive and standardized VHL database in the web-based application, REDCap, that thoroughly captures all VHL manifestation data. As an initial trial, information from 86 VHL patients from the University Health Network/Hospital for Sick Children was populated into the database. Analysis of this cohort showed missense variants occurring with the greatest frequency, with all variants localizing to the α- or β-domains of VHL. The most prevalent manifestations were central nervous system (CNS), renal, and retinal neoplasms, which were associated with frameshift variants and large deletions. We observed greater age-related penetrance for CNS hemangioblastomas with truncating variants compared to missense, while the reverse was true for pheochromocytomas. We demonstrate the utility of a comprehensive VHL database, which supports the standardized collection of clinical and genetic data specific to this patient population. Importantly, we expect that its web-based design will facilitate broader international collaboration and lead to a better understanding of VHL., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2019
- Full Text
- View/download PDF
49. Multicenter, International Assessment of the Eighth Edition of the American Joint Committee on Cancer Cancer Staging Manual for Conjunctival Melanoma.
- Author
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Jain P, Finger PT, Damato B, Coupland SE, Heimann H, Kenawy N, Brouwer NJ, Marinkovic M, Van Duinen SG, Caujolle JP, Maschi C, Seregard S, Pelayes D, Folgar M, Yousef YA, Krema H, Gallie B, and Calle-Vasquez A
- Abstract
Importance: Eye cancer staging systems used for standardizing patient care and research need to be validated., Objective: To evaluate the accuracy of the eighth edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual in estimating metastatis and mortality rates of conjunctival melanoma., Design, Setting, and Participants: This international, multicenter, registry-based case series pooled data from 10 ophthalmic oncology centers from 9 countries on 4 continents. A total of 288 patients diagnosed with conjunctival melanoma from January 1, 2001, to December 31, 2013, were studied. Data analysis was performed from July 7, 2018, to September 11, 2018., Interventions: Treatments included excision biopsy, cryotherapy, topical chemotherapy, radiation therapy, enucleation, and exenteration., Main Outcomes and Measures: Metastasis rates and 5-year and 10-year Kaplan-Meier mortality rates according to the clinical T categories and subcategories of the eighth edition of the AJCC Cancer Staging Manual., Results: A total of 288 eyes from 288 patients (mean [SD] age, 59.7 [16.8] years; 147 [51.0%] male) with conjunctival melanoma were studied. Clinical primary tumors (cT) were staged at presentation as cT1 in 218 patients (75.7%), cT2 in 34 (11.8%), cT3 in 15 (5.2%), and cTx in 21 (7.3%). There were no T4 tumors. Pathological T categories (pT) were pTis in 43 patients (14.9%), pT1 in 169 (58.7%), pT2 in 33 (11.5%), pT3 in 12 (4.2%), and pTx in 31 (10.8%). Metastasis at presentation was seen in 5 patients (1.7%). Metastasis during follow-up developed in 24 patients (8.5%) after a median time of 4.3 years (interquartile range, 2.9-6.0 years). Of the 288 patients, 29 died (melanoma-related mortality, 10.1%) at a median time of 5.3 years (interquartile range, 1.8-7.0 years). The cumulative rates of mortality among patients with cT1 tumors were 0% at 1 year, 2.5% (95% CI, 0.7%-7.7%) at 5 years, and 15.2% (95% CI, 8.1%-27.4%) at 10 years of follow-up; among patients with cT2 tumors, 0% at 1 year, 28.6% (95% CI, 12.9%-58.4%) at 5 years, and 43.6% (95% CI, 19.6%-77.9%) at 10 years of follow-up; and among patients with cT3 tumors, 21.1% (95% CI, 8.1%-52.7%) at 1 year of follow-up and 31.6% (95% CI, 13.5%-64.9%) at 5 years of follow-up. Patients with cT2 and cT3 tumors had a significantly higher cumulative mortality rate compared with those presenting with cT1 tumors (log-rank P < .001). Patients with ulcerated melanomas had significantly higher risk of mortality (hazard ratio, 7.58; 95% CI, 1.02-56.32; P = .04)., Conclusions and Relevance: This multicenter, international, collaborative study yielded evidence that the conjunctival melanoma staging system in the eighth edition of the AJCC Cancer Staging Manual can be used to accurately estimate metastasis and mortality rates. These findings appear to support the use of AJCC staging as a tool for patient care and research.
- Published
- 2019
- Full Text
- View/download PDF
50. Retinal perfusion changes in radiation retinopathy.
- Author
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Rose K, Krema H, Durairaj P, Dangboon W, Chavez Y, Kulasekara SI, and Hudson C
- Subjects
- Choroid Neoplasms radiotherapy, Disease Progression, Female, Fundus Oculi, Humans, Melanoma radiotherapy, Middle Aged, Radiation Injuries diagnosis, Radiation Injuries physiopathology, Retinal Diseases diagnosis, Retinal Diseases etiology, Retinal Vessels diagnostic imaging, Retinal Vessels radiation effects, Fluorescein Angiography methods, Radiation Injuries complications, Regional Blood Flow physiology, Retinal Diseases physiopathology, Retinal Vessels physiopathology, Tomography, Optical Coherence methods
- Abstract
Purpose: To investigate retinal blood flow and oxygen saturation changes in patients diagnosed with retinopathy following plaque radiation treatment to treat choroidal melanoma., Methods: Eight patients (mean age 55.75 years, SD 12.58 years) who have developed unilateral ischaemic radiation-related retinopathy as confirmed by wide-field fluorescein angiography were recruited for the study. The fellow eye with no other ocular or retinal pathology was used as control. Both eyes underwent measurement of total retinal blood flow (TRBF) and retinal blood oxygen saturation using prototype methodologies of Doppler Spectral Domain Optical Coherence Tomography (OCT) and Hyperspectral Retinal Camera, respectively., Results: The average TRBF in the retinopathy eye was significantly lower compared to the fellow eye (33.48 ± 12.73 μl/min versus 50.37 ± 15.26 μl/min; p = 0.013). The arteriolar oxygen saturation (SaO
2 ) and venular oxygen saturation (SvO2 ) were higher in the retinopathy eye compared to the fellow eye (101.11 ± 4.26%, versus 94.45 ± 5.79%; p = 0.008) and (62.96 ± 11.05% versus 51.24 ± 6.88%, p = 0.051), respectively., Conclusion: The ionizing radiation seems to have an impact on the TRBF, SaO2 and SvO2 , clinically presenting similar to a rapidly developing diabetic retinopathy. The results show an altered retinal vascular physiology in patients with radiation-related retinopathy., (© 2018 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)- Published
- 2018
- Full Text
- View/download PDF
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