Lag time for retinoblastoma in the UK revisited: a retrospective analysis

OBJECTIVES: To explore current delays in diagnosis of retinoblastoma (Rb) and effect on outcome with comparison to a study from the 1990s. SETTING: Primary, secondary, tertiary care: majority from South of England. PARTICIPANTS: A retrospective analysis of 93 new referrals of sporadic (non-familial) Rb to a specialist Rb unit in London, UK from January 2006 to February 2014. PRIMARY AND SECONDARY OUTCOMES: International Intraocular Retinoblastoma Classification, lag times including parental delay and healthcare professional delay, patients requiring enucleation and requirement of adjuvant chemotherapy postenucleation (high-risk Rb). RESULTS: During the study period, 29% presented via accident and emergency (A&E). The median referral time from symptom onset to visiting primary care (PC) was 28 days and PC to ophthalmologist 3 days (range 0-181 days). The median time from local ophthalmologist to the Rb Unit was 6 days (0-33). No significant correlation was found between delay and International Classification of Retinoblastoma grade (p>0.05) or between postenucleation adjuvant chemotherapy and enucleation groups (p>0.05). Less enucleations (60%) are being performed compared with the previous study (81%) (p=0.0015). CONCLUSIONS: Parents are attending A&E more compared with the 1990s and this may reflect the effect of public awareness campaigns. More eyes are being salvaged despite a similar number of children requiring adjuvant chemotherapy. High-risk Rb and Group E eyes do not correlate with increased lag time in the UK. Other determinants such as tumour biology may be more relevant

Ocular oncology: advances in retinoblastoma, uveal melanoma and conjunctival melanoma

BACKGROUND: Retinoblastoma, uveal and conjunctival melanomas are important malignancies within the remit of ocular oncology. Outlined are the diagnostic features and management principles, as well as advancements in the field and current challenges. SOURCES OF DATA: Original papers, reviews and guidelines. AREAS OF AGREEMENT: Most eyes with retinoblastoma (International Intraocular Retinoblastoma Classification (IIRC) Group A-D) are salvaged, whereas advanced cases (Group E) remain a challenge. Despite a high rate of local tumour control in uveal melanoma, metastatic spread commonly occurs. Conjunctival melanoma is treated by complete resection, but high rates of local recurrence occur, with the possibility of systemic relapse and death. AREAS OF CONTROVERSY: Use of the IIRC in retinoblastoma, and systemic screening in melanomas. GROWING POINTS: Utilization of novel treatment modalities in retinoblastoma and an increasing understanding of the genetic basis of melanomas. AREAS TIMELY FOR DEVELOPING RESEARCH: Improvements in chemotherapy delivery in retinoblastoma and prognostic tests in melanomas

Targeting tyrosine kinases for treatment of ocular tumors

Uveal melanoma is the most common intraocular primary malignant tumor in adults, and retinoblastoma is the one in children. Current mainstay treatment options include chemotherapy using conventional drugs and enucleation, the total removal of the eyeball. Targeted therapies based on profound understanding of molecular mechanisms of ocular tumors may increase the possibility of preserving the eyeball and the vision. Tyrosine kinases, which modulate signaling pathways regarding various cellular functions including proliferation, differentiation, and attachment, are one of the attractive targets for targeted therapies against uveal melanoma and retinoblastoma. In this review, the roles of both types of tyrosine kinases, receptor tyrosine kinases and non-receptor tyrosine kinases, were summarized in relation with ocular tumors. Although the conventional treatment options for uveal melanoma and retinoblastoma are radiotherapy and chemotherapy, respectively, specific tyrosine kinase inhibitors will enhance our armamentarium against them by controlling cancer-associated signaling pathways related to tyrosine kinases. This review can be a stepping stone for widening treatment options and realizing targeted therapies against uveal melanoma and retinoblastoma.N