Philippine retinoblastoma initiative multi-eye center study 2010-2020.

AIM: To provide a comprehensive and more representative national data on the disease, especially on treatment options and outcomes, and to determine access of retinoblastoma patients from Luzon, Visayas and Mindanao to eye care, and determine if access is associated with delay in consultation, staging and outcomes. METHODS: Cohort study of retinoblastoma patients seen in eleven institutions located in the three major areas of the Philippines namely Luzon, Vizayas and Mindanao from 2010-2020. RESULTS: Totally 636 patients, involving 821 eyes, were included. Majority (57%) were from Luzon and were seen in institutions in Luzon (72%). Annually, 58±10 new cases were seen with 71% having unilateral disease. Median delay of consultation remained long at 9 (3, 17)mo, longest in patients with unilateral disease (P<0.02) and those from the Visayas (P<0.003). Based on the International Retinoblastoma Staging System, only 35% of patients had Stage 1 while 47% already had extraocular disease. Enucleation was the most common treatment received by 484 patients while intravenous chemotherapy was received by 469. There were 250 (39%) patients alive, 195 (31%) dead, 85 (13%) abandoned, 17 (3%) refused and 89 (14%) with no data. CONCLUSION: This study presents the largest cohort of retinoblastoma patients in the Philippines in terms of patients' and participating institutions' number and geographical location and type of institution (private and public). It also presents more comprehensive data on the treatments used and outcomes (survival, globe salvage, and vision retention rates). Delay in consultation was still long among patients leading to advanced disease stage and lower survival rate. Despite increasing capacity to diagnose and manage retinoblastoma in the country, the delay of consultation remains long primarily due to accessibility issues to eye care institutions especially in the Visayas and financial concerns. The delay was still significant that overall survival rate remain low.

Recalcitrant Optic Nerve and Retinal Infiltration in a Relapse of Acute Lymphoblastic Leukaemia.

Isolated ocular relapse of leukaemia is rare. We present the case of a 20-year-old male with T-cell acute lymphoblastic leukaemia (ALL) who reported sudden blurring of vision in both eyes 6 months after documentation of ALL remission. Ocular examination showed bilateral infiltrative optic neuropathies and serous retinal detachments. Cerebrospinal fluid and bone marrow samples were negative for blast cells. Systemic work-up did not reveal any other sites of involvement. The ocular infiltration partially responded to reinduction chemotherapy, intraconal steroids, and radiotherapy. This report demonstrates a challenging case of isolated ocular ALL relapse presenting as bilateral optic nerve and retinal infiltration.

Intravitreal melphalan for persistent retinoblastoma vitreous seeds.

A 7-month-old male presented with bilateral retinoblastoma, initially group E OD and group C OS (International Intraocular Retinoblastoma Classification). The patient underwent enucleation with adjuvant chemotherapy and radiotherapy on the right eye for extraocular retinoblastoma. The main tumors on the left eye regressed with combined neoadjuvant chemotherapy with focal therapy, but had persistent sphere vitreous seeds overlying the tumor at the superotemporal mid-periphery and at the inferior periphery. Intravitreal injection of melphalan was performed. Vitreous seeds were clinically undetectable after 2 cycles of injection. Six months from the 2nd intravitreal injection of melphalan, there was no evidence of new tumors nor vitreous seeds in the left eye. No documented complications of intravitreal melphalan injection were experienced. This is the first documented successful treatment of vitreous seeds with intravitreal melphalan at the Ocular Oncology/Retinoblastoma Unit of the University of the Philippines, Philippine General Hospital.

Retinal vasoproliferative tumour secondary to retinitis pigmentosa sine pigmento.

Retinitis pigmentosa can be associated with exudative vasculopathy in rare instances, which can manifest as retinal vasoproliferative tumours. We present the case of a 33-year-old woman previously diagnosed with retinitis pigmentosa sine pigmentosa in both eyes. She was asymptomatic and just came in for a routine follow-up eye examination. Thorough examination of the peripheral retina on the right eye revealed a dome-shaped retinal tumour with a feeder vessel and surrounding exudative changes at the superotemporal periphery, consistent with a secondary retinal vasoproliferative tumour from retinitis pigmentosa. She subsequently underwent focal laser photocoagulation of the tumour which resulted in tumour stabilisation. While exudative vasculopathy is very uncommon in retinitis pigmentosa, ophthalmologists need to be aware of its occurrence in such patients. Vision loss may occur from exudation, haemorrhage, retinal detachment and neovascularisation. A thorough examination of the peripheral retina is warranted in these cases.

Retinoblastoma presenting as haemorrhagic choroidal detachment in a 10-year-old girl.

To report a case of retinoblastoma presenting as haemorrhagic choroidal detachment in a 10-year-old girl. The patient, apparently well, presented with sudden blurring of vision in the setting of blunt trauma. On examination and ocular ultrasound, haemorrhagic choroidal detachment was suspected. She underwent external drainage via sclerostomies. The haemorrhage resolved ultrasonographically, but the vision did not improve. Five months postoperatively, she presented with sudden eye pain, lid swelling and proptosis. The ancillary workups were consistent with panophthalmitis and was treated as such. Six months postoperatively, the pain and lid swelling persisted; hence, the painful near absolute eye was enucleated. The histopathology revealed small round blue cells with necrosis consistent with retinoblastoma. Immunohistochemistry was positive for synaptophysin and negative for S100 confirming retinoblastoma. Retinoblastoma is the most common intraocular malignancy in children. The importance of increased awareness of its various presentations is of utmost importance to prevent life-threatening and vision-impairing complications.