Over the past decade, chemotherapy combined with focal therapy is being used increasingly as primary management for patients with RETINOBLASTOMA (Rb). There has been a trend away from EBRT toward conservative treatments.Since EBRT has not been widely used as primary treatment for Rb for the past several years, a proposed new international intraocular RETINOBLASTOMA classification (IIRC) is being presented in different meetings and articles.This article discusses different aspects of intraocular RETINOBLASTOMA based on more recent published data emphasizing on the new group classification of Rb (IIRe).

Systemic chemotherapy has become the cornerstone of therapy for RETINOBLASTOMA. The advent of novel chemotherapeutic and antiangiogenesis agents together with new routes of drug administration including periocular, intravitreal and intra-ophthalmic artery injection will hopefully revolutionize the management of this sight-threatening and potentially fatal infantile ocular neoplasm.

Background: RETINOBLASTOMA is the most common embryonic tumor of retina of children. This tumor include 3-4% of all children malignancies during the age range 0-14 years. In this study, we reported the incidence, therapeutic protocols, and treatment outcome of children with RB in South west Iran.Methods: We retrospectively investigated the medical reports clinical and pathological features of RB cases at referral Shafa Hospital of Ahvaz city located in Southwest Iran, between 1994 and 2014.Results: Our results showed leukocoria, white discoloration in the pupil, as the most common primary symptom in RETINOBLASTOMA patients. Forty-two percent of our patients had bilateral neoplasms. Based on histological investigations, stage IV was most common stage in our patients (79.1%).Conclusion: In conclusion, our study showed that leukocoria, stage IV and Choroid involvement as most common symptom, stage and organ involvement, respectively and inherited cases is rare in our population. The results of our study can be as a base for further researches in future.

Purpose: To evaluate the role of intravitreal injection of carboplatin in the management of intraocular RETINOBLASTOMA in patients referring to Farabi Eye Hospital during 1999-2000. Patients and methods: This is a randomized double-blind matched clinical trial. Definite diagnosis of intraocular RETINOBLASTOMA was made based on clinical examination, ultrasonography or orbital CT-scanning tomography. A comprehensive ophthalmic examination was performed with the patient under general anesthesia. Anterior chamber, iris, lens, vitreous, retina and intraocular tumors were assessed carefully. The greatest basal diameter and the thickness of each tumor were estimated through indirect ophthalmoloscopy according to disc diameter. After ruling out the exteraocular or systemic metastasis, the eyes were divided into 10 blocks according to Reese-Ellswrth classification and then the eyes in each block were divided randomly into two groups; the control group and the case group. The control group only received systemic chemotherapy including vincristine, etoposide, and carboplatin (VEC) at least for 6 sessions. The case group received the same chemotherapy plus intravitreal carboplatin. The intravitreal carboplatin administered as 2 injection of 5 mcg with 2 weeks interval. All patients were reexamined under general anesthesia monthly and the greatest basal diameter and the thickness of each tumor and the vitreous status were reassessed each time and when the volume of tumor reduced, laser therapy was performed. All examinations were done by a single physician. The results were compared and analyzed with Chi-square, Fisher exact test, and T-test. Results: This study was conducted on 45 tumors in 19 eyes of 13 patients. In the control group there were 20 tumors in 8 eyes and in the case group there were 25 tumors in 11 eyes. There was 1.8±l.8 disc diameter or 81% decrease in tumor thickness in the case group (P<0.001)that was two times greater than in control group (P<0.09). There was 56% decrease in the greatest basal diameter of the tumors in the case group. About 91% of the eyes in the case group and 75% of the eyes in the control group saved from enucleation. In the control group 6 eyes had advanced stages of tumor (Reese-Ellsworth group IV, V) which two of them were enucleated and in the case group from 6 eyes which had advanced stages of tumor just one eye was enucleated. Conclusion: Intravitreal carboplatin plus systemic chemotherapy using VEC inhibited the tumor growth more effectively in comparison with systemic chemotherapy alone.

Systemic chemotherapy regimens and focal treatment modalities such as radioactive plaque therapy have dramatically improved the outcomes of treatment for RETINOBLASTOMA such that patient survival with modern monitoring and treatment now exceeds 95%. Although the vast majority of patients respond adequately to current therapeutic strategies, treatment for a subset of patients with advanced RETINOBLASTOMA remains challenging.

Purpose: To report a case of retinocytoma associated with bilateral RETINOBLASTOMA. Case Report: A 3-year-old girl presenting with left eye exotropia was referred to our ophthalmic oncology service for a second opinion. Funduscopy under general anesthesia revealed one prominent retinocytoma associated with five discrete RETINOBLASTOMAs in the left eye and three discrete RETINOBLASTOMAs in the right eye.Conclusion: Although retinocytoma is often a benign tumor, it may be associated with RETINOBLASTOMA. Therefore, periodic examination of the fundus is necessary in these patients.

In this issue of Iranian Journal of Ophthalmology (IRJO) Han Yue et al1 from China have presented a very interesting investigation on "Histopathologic Risk Factors of RETINOBLASTOMA: A Retrospective Study of 104 Enucleated Eyes" which we are very grateful of the authors. Before indicating few points about this article and their pathologic classification it is worthwhile to mention the "Proceedings of the Consensus Meeting from the International RETINOBLASTOMA Staging Working Group on the pathology Guidelines for the Examination of Enucleated Eyes and Evaluation of Prognostic Risk Factors in RETINOBLASTOMA".