Animal models in retinoblastoma research

Advances in animal models of retinoblastoma have accelerated research in this field, aiding in understanding tumor progression and assessing therapeutic modalities. The distinct pattern of mutations and specific location of this unique intraocular tumor have paved the way for two types of models- those based on genetic mutations, and xenograft models. Retinoblastoma gene knockouts with an additional loss of p107, p130, p53 and using promoters of Nestin, Chx10, and Pax6 genes show histological phenotypic changes close to the human form of retinoblastoma. Conditional knockout in specific layers of the developing retina has thrown light on the origin of this tumor. The use of xenograft models has overcome the obstacle of time delay in the presentation of symptoms, which remains a crucial drawback of genetic models. With the advances in molecular and imaging technologies, the current research aims to develop models that mimic all the features of retinoblastoma inclusive of its initiation, progression and metastasis. The combination of genetic and xenograft models in retinoblastoma research has and will help to pave way for better understanding of retinoblastoma tumor biology and also in designing and testing effective diagnostic and treatment modalities

Uveal melanoma in children and teenagers

To review the features and prognosis of uveal melanoma in children. Methods: Retrospective case series. Of 122 children with uveal melanoma, there were 53 [43%] male and 69 [57%] female patients. In this group, the mean age at presentation was 15 years [median 16 years, range 3-20 years]. Age at presentation was 0 to 5 years in 4 [3%], 5.1 to 10 years in 14 [11%], 10.1 to 15 years in 43 [35%], and 15.1 to

Multicavitary ciliary body melanoma presenting as a cyst

Cyst-like cavities in uveal melanoma occur rarely and can simulate a benign intraocular cystic lesion resulting in delayed diagnosis and inappropriate management. Herein, we describe a 66-year-old Caucasian female who presented with a "cystic" ciliary body mass in the right eye oculus dexter [OD]. Slit lamp examination OD showed anterior bulging of the iris temporally from an underlying pigmented ciliary body mass and transillumination disclosed slight shadow from the tumor. Ultrasound biomicroscopy [UBM] revealed multiple cyst-like cavities within a tumor, lined by "thick walls" of at least 200 microm and occupying 80% of the tumor volume. A clinical diagnosis of multi-cavitary ciliary body melanoma was suspected and partial lamellar sclero iridocyclectomy was performed. Histopathology confirmed the diagnosis of low-grade spindle melanoma of the ciliary body with multiple empty and fluid filled cyst-like cavities without epithelial lining. UBM is an important diagnostic tool in the differentiation of "thick walled" cavitary melanoma from "thin walled" benign pigment epithelial cyst

Cystoid macular edema as the initial manifestation of choroidal melanoma

Uveal melanomas are a common clinical entity that initially present in a variety of ways. Cystoid macular edema is caused by many conditions, but it is rarely associated with uveal melanoma. We report two cases of patients that presented with visually significant cystoid macular edema that was later found to be secondary to choroidal melanoma. We describe the patients' course and treatment and provide a mechanism for the potential cause of edema in patients with uveal melanoma