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Approximately 5% of all melanoma tumors arise in ocular tissue. Most ocular melanoma (85%) is uveal (iris, ciliary body or choroid) in origin and it is the most common intraocular malignant tumor. Tumors arise from the pigment cells (melanocytes).

Retinoblastoma is an embryonal intraocular tumor that is nearly always diagnosed in the first 5 years of childhood. The overall survival of patients with retinoblastoma is high, but long-term sequelae are a major concern for many survivors. The most common late effects of retinoblastoma and its treatment are compromised vision or loss of the eye, ototoxicity after chemotherapy, cataract or cosmetic defects after radiotherapy, and psychological challenges.

Retinoblastoma represents the most common intraocular malignant tumor in children and worldwide is the most common intraocular malignancy. Uveal melanomas are the most common malignant intraocular tumors in adults. This review chapter includes key clinical features and important histopathologic findings for diagnosis and prognosis of both entities.

This section will discuss the role of enucleation and exenteration in the setting of the two most common primary intraocular tumors – retinoblastoma and uveal melanoma. The treatment of these tumors is complex and usually requires a multidisciplinary approach, which is beyond the scope of this chapter. Eye removal (enucleation or exenteration) is only one option of many for these patients and is the main focus of this chapter. Evisceration should never be performed if an eye has or is suspected of having an intraocular tumor.

Retinoblastoma is the most common intraocular tumor in childhood. Topotecan has been widely used as an antineoplastic agent for retinoblastoma treatment. Topotecan penetration into living tumorspheres is quantified using confocal microscopy. This fluorescent drug dyes the living tissue and it can be recorded in a sequence of images over a period of time. The effective penetration of the drug depends on culture characteristics and requires a very specific timing. This penetration time is calculated empirically by an expert. The purpose of this work is to offer a statistical model to automatically estimate the penetration time of topotecan in the cell, based on the information obtained from a sequence of tumorsphere images.

In the United States and other developed nations, more patients with retinoblastoma will die from non-ocular second malignant neoplasms than from their primary intraocular tumor. There is an increased lifetime risk of the development of second malignant neoplasms in survivors of heritable retinoblastoma compared with their nonheritable counterparts. Radiation increases the risk of second malignant neoplasms and should be avoided when possible. All patients at risk must be educated to remain vigilant for future signs or symptoms of malignancies and counseled to avoid exposure to other mutagens. The prognosis for patients with second malignant neoplasms is guarded and underscores the importance of counseling patients with the heritable form of the disease.

The following chapter describes step-by-step the surgical management of an intraocular tumor biopsy. The chapter contains the surgical management of a transretinal tumor biopsy, a transscleral tumor biopsy, an anterior chamber tumor biopsy, a vitreous biopsy, a chorioretinal biopsy, and last but least an intraocular biopsy in suspected lymphoproliferative disease.

Choroidal nevus is the most common type of intraocular tumor with an estimated prevalence of 6.5% and 1.4% in Caucasian and Asian populations, respectively [1]. Due to pigmentation, melanotic choroidal nevi are relatively opaque to internal examination under white light. Operating at infrared wavelengths, swept source optical coherence tomography penetrates deeper into pigmented tissue and in the case of melanotic choroidal nevi can demonstrate internal features of nevi, such as blood vessels and granularity, as well as adjacent choriocapillaris abnormalities.

The relentless work ethic of Marie Sklodowska-Curie realized the discovery of radium. This led to its medical application and advent for the mainstay treatment of the most common primary intraocular tumor, uveal melanoma. It is clear that intellectual, physical and emotional obstacles challenged Marie’s accomplishments. Accompanied by her husband, Marie’s systemic approach using quantitative methodology allowed her to make numerous pioneering landmarks in history.