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Fulltext Trilateral retinoblastoma

Limn YS, Juraida E, Alagaratnam J, Menon BS

Med J Malaysia, 2011 Jun;66(2):156-7.
PMID: 22106703 MyJurnal

Trilateral retinoblastoma is the association of hereditary bilateral or unilateral retinoblastoma with a pineal neuroblastic tumour. We describe two cases of trilateral retinoblastoma from a total of 141 cases of retinoblastoma seen over an 8.5 year period. Both had a fatal outcome, with survival times of only 4 and 8 months respectively.

Matched MeSH terms: Retinal Neoplasms/diagnosis*; Retinal Neoplasms/etiology; Retinal Neoplasms/therapy*
Fulltext Retinoblastoma Registry report--Hospital Kuala Lumpur experience

Jamalia R, Sunder R, Alagaratnam J, Goh PP

Med J Malaysia, 2010 Jun;65 Suppl A:128-30.
PMID: 21488473

Retinoblastoma is a childhood ocular cancer. The aim of this paper is to describe the clinical and epidemiological characteristics of patients with retinoblastoma in a major paediatric ophthalmology center in the country. Retrospective information was collected through the retinoblastoma registry. Late presentation with advanced staging is a major problem.

Matched MeSH terms: Retinal Neoplasms/diagnosis*; Retinal Neoplasms/epidemiology; Retinal Neoplasms/surgery
Fulltext Presentation of retinoblastoma patients in Malaysia

Subramaniam S, Rahmat J, Rahman NA, Ramasamy S, Bhoo-Pathy N, Pin GP, et al.

Asian Pac J Cancer Prev, 2014;15(18):7863-7.
PMID: 25292078

BACKGROUND: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia.

MATERIALS AND METHODS: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications.

RESULTS: The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012. There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majority of patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%). Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It was found that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis (12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%). More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients.

CONCLUSIONS: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

Matched MeSH terms: Retinal Neoplasms/mortality; Retinal Neoplasms/epidemiology*; Retinal Neoplasms/pathology*; Retinal Neoplasms/therapy
Fulltext Leucocoria in a boy with Kawasaki disease: a diagnostic challenge

Che Mahiran CD, Alagaratnam J, Liza-Sharmini AT

Singapore Med J, 2009 Jul;50(7):e232-4.
PMID: 19644606

Retinoblastoma, the most common primary intraocular malignancy of childhood, usually presents in the first three years of life. Atypical presentation of retinoblastoma can masquerade as virtually any ocular or orbital pathology, which may lead to diagnostic dilemmas especially in the presence of other systemic diseases. We report a 20-month-old boy who was diagnosed with coronary aneurysm as a complication of Kawasaki disease, and presented with sudden left eye redness. His mother noticed the presence of white pupillary reflex three months earlier. Atypical acute ocular presentation secondary to Kawasaki disease was initially suspected, but the presence of multiple calcification and mild proptosis on imaging suggested characteristics of advanced retinoblastoma. Histopathological examination of the enucleated eye, which revealed a classical rosette pattern appearance, confirmed the diagnosis. Atypical presentations of retinoblastoma are usually associated with advanced disease. The presence of other systemic conditions further complicates the diagnosis. Early diagnosis is important to reduce the mortality and morbidity.

Matched MeSH terms: Retinal Neoplasms/complications; Retinal Neoplasms/diagnosis; Retinal Neoplasms/ultrasonography
Extraocular retinoblastoma

Menon BS, Reddy SC, Maziah WM, Ham A, Rosline H

Med. Pediatr. Oncol., 2000 Jul;35(1):75-6.
PMID: 10881012
Matched MeSH terms: Retinal Neoplasms/epidemiology*; Retinal Neoplasms/pathology*
Ocular involvement in leukemia--a study of 288 cases

Reddy SC, Jackson N, Menon BS

Ophthalmologica, 2003 Nov-Dec;217(6):441-5.
PMID: 14573980

Two hundred and eighty-eight newly diagnosed cases of leukemia (164 males and 124 females, 167 adults and 121 children, 245 acute and 43 chronic, 151 myeloid and 137 lymphoid), aged between 6 weeks and 78 years, were examined for eye changes in the oncology wards within 2 days of diagnosis before starting chemotherapy. Ocular lesions were present in 102 patients (35.4%)--retinal vascular changes in 91 (31.6%); infiltration of ocular tissues in 5 (1.7%), and neuro-ophthalmic signs in 6 (2.1%) cases. Some of the patients had more than one ocular lesion in one or both eyes. The eye changes were seen more often in adults (49.1%) than in children (16.5%), and in myeloid leukemia (41.0%) than in lymphoid leukemia (29.2%). Eye symptoms were present in 29 patients (10%) at initial diagnosis. Since ocular lesions were detected in many asymptomatic leukemia patients, eye examination should be included as a part of routine evaluation at initial diagnosis in these patients.

Matched MeSH terms: Retinal Neoplasms/epidemiology; Retinal Neoplasms/pathology*
RB pocket domain B mutation frequency in Malaysia

Ishak SR, Hanafi H, Alagaratnam JV, Zilfalil BA, Tajudin LS

Ophthalmic Genet., 2010 Sep;31(3):159-61.
PMID: 20565234 DOI: 10.3109/13816810.2010.492816
Matched MeSH terms: Retinal Neoplasms/genetics*; Retinal Neoplasms/epidemiology
Retinopathy in acute leukaemia at initial diagnosis: correlation of fundus lesions and haematological parameters

Reddy SC, Jackson N

Acta Ophthalmol Scand, 2004 Feb;82(1):81-5.
PMID: 14738490

PURPOSE: To determine the prevalence of retinal changes in newly diagnosed acute leukaemia patients, and to establish the relationship between retinal lesions and haematological parameters in these patients.
METHODS: A total of 127 patients with acute leukaemia (myeloid and lymphoid), of both genders, aged between 13 and 77 years, were examined by an ophthalmologist for retinal changes using direct/indirect ophthalmoscopy within 2 days of diagnosis before starting chemotherapy.
RESULTS: Retinal lesions were seen in 62 cases (49%), with intraretinal haemorrhages being the most common lesion (42%). A high white blood cell count was significantly associated with intraretinal haemorrhages (p = 0.04) and white-centred haemorrhages (p = 0.001), while a low platelet count was significantly associated with intraretinal haemorrhages (p = 0.03) in acute myeloid leukaemia patients.
CONCLUSIONS: A high white blood cell count may be considered as important as a low platelet count in the pathogenesis of leukaemic retinopathy.

Matched MeSH terms: Retinal Neoplasms/blood; Retinal Neoplasms/diagnosis*; Retinal Neoplasms/drug therapy
Spectrum of germ-line RB1 gene mutations in Malaysian patients with retinoblastoma

Mohd Khalid MK, Yakob Y, Md Yasin R, Wee Teik K, Siew CG, Rahmat J, et al.

Mol Vis, 2015;21:1185-90.
PMID: 26539030

The availability of molecular genetic testing for retinoblastoma (RB) in Malaysia has enabled patients with a heritable predisposition to the disease to be identified, which thus improves the clinical management of these patients and their families. In this paper, we presented our strategy for performing molecular genetic testing of the RB1 gene and the findings from our first 2 years of starting this service.

Matched MeSH terms: Retinal Neoplasms/ethnology; Retinal Neoplasms/genetics*; Retinal Neoplasms/pathology
Chemoreduction for intraocular retinoblastoma in Malaysia

Menon BS, Juraida E, Alagaratnam J, Mohammad M, Ibrahim H, George TM, et al.

J Pediatr Hematol Oncol, 2007 Jan;29(1):2-4.
PMID: 17230058

In the last decade, chemotherapy in combination with focal therapy (chemoreduction) has been increasingly used in intraocular retinoblastoma to avoid enucleation and radiotherapy. The aim of this study was to assess the feasibility and outcome of chemoreduction in Malaysian children with retinoblastoma. This was a prospective study from August 2001 to January 2006. Twenty children (25 eyes) were given 4 cycles of chemoreduction, after which the response was assessed. Fourteen eyes showed a complete response, 10 eyes showed a partial response, and 1 eye had progressive disease. Twelve eyes developed progressive disease later, 9 after an initial complete response and 3 after a partial response. Overall, progressive disease occurred in 52%. There were 2 treatment failures, in Reese-Elsworth groups 3 and 4. Both eyes required enucleation. One eye in group 5 required second line chemotherapy to achieve a complete response. No eyes were irradiated. Five children (25%) defaulted follow-up, one of whom returned with disseminated disease. In conclusion, 4 cycles of chemoreduction achieved a durable complete response in only 12% of eyes. Chemoreduction is feasible in Malaysia but requires good patient compliance and close follow-up.

Matched MeSH terms: Retinal Neoplasms/therapy*
Fulltext Intra-arterial chemotherapy for retinoblastoma: Our first three-and-a-half years' experience in Malaysia

Liu CC, Mohmood A, Hamzah N, Lau JH, Khaliddin N, Rahmat J

PLoS One, 2020;15(5):e0232249.
PMID: 32357178 DOI: 10.1371/journal.pone.0232249

AIM: To report our first three-and-a-half years' experience with intra-arterial chemotherapy (IAC) in managing retinoblastoma (RB).
METHODS: Single institution, retrospective, interventional case series of 14 retinoblastoma patients managed with IAC from December 2014 to June 2018. Demographics were described. Outcomes measures were tumor response, treatment complications and globe salvage.
RESULTS: Subjects' mean age at the first administration of IAC was 31.4 months. 57.1% of the eyes were Group D and E retinoblastoma, while 79% were bilateral disease. 93% of the eyes were as secondary treatment. Of 32 IAC cannulations performed, 23 (71.8%) were successful and received chemotherapy drug melphalan. Each eye received a mean of 1.8 (range 1-4) IAC injections. 53% of the eyes showed regression post treatment. After a mean follow up period of 19 months, globe salvage rate was 38%. Most of the adverse effects experienced were localized and transient.
CONCLUSION: IAC has provided an added recourse in the armamentarium of retinoblastoma treatment in our center. IAC treatment is a viable alternative in the treatment of retinoblastoma to salvage globe, for eyes that would conventionally require enucleation especially in bilateral disease.

Matched MeSH terms: Retinal Neoplasms/drug therapy*
Optic Nerve Infiltration in Systemic Metastatic Retinal Lymphoma (SMRL): Multimodal Imaging and Challenges in Diagnosis

Mohd Fauzi Yap MFB, Mohd Zain A, Tumain NR, Palaniappan S, Nasaruddin RA, Md Din N

Ocul Immunol Inflamm, 2020 Sep 24.
PMID: 32967510 DOI: 10.1080/09273948.2020.1800050

A 45-year-old man was diagnosed with diffuse large B-cell lymphoma stage IV which was confirmed by celiac lymph node biopsy. He subsequently completed six cycles of R-CHOP chemotherapy. Six months later, he presented with panuveitis OU with positive relative afferent pupillary defect OD. OCT revealed hyper-reflective lesions and irregularity of the retinal pigment epithelium OU. Fundus fluorescein angiogram shows hyper-auto fluorescence and granular changes on the retina. A month later, he developed swollen optic disc OD and hemorrhagic retinitis OU and treated as presumed CMV retinitis. Anti-TB was started after a positive Mantoux test. He finally consented for a vitreous biopsy which showed atypical lymphoid cells highly suggestive for vitreoretinal lymphoma and subsequently received intravitreal methotrexate OU.
CONCLUSION: Optic nerve infiltration in systemic metastatic retinal lymphoma may have initial occult signs but with profound visual loss. Ocular infections like CMV retinitis and tuberculosis may mask and delay the diagnosis in immunocompromised patients.

Matched MeSH terms: Retinal Neoplasms