Uveal melanoma: relatively rare but deadly cancer

Abstract

Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. Tumors are located either in iris (4%), ciliary body (6%), or choroid (90%). The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. Currently, the most widely used first-line treatment options for this malignancy are resection, radiation therapy, and enucleation. There are two main types of radiation therapy: plaque brachytherapy (iodine-125, ruthenium-106, or palladium-103, or cobalt-60) and teletherapy (proton beam, helium ion, or stereotactic radiosurgery using cyber knife, gamma knife, or linear accelerator). The alternative to radiation is enucleation. Although these therapies achieve satisfactory local disease control, long-term survival rate for patients with uveal melanoma remains guarded, with risk for liver metastasis. There have been advances in early diagnosis over the past few years, and with the hope survival rates could improve as smaller tumors are treated. As in many other cancer indications, both early detection and early treatment could be critical for a positive long-term survival outcome in uveal melanoma. These observations call attention to an unmet medical need for the early treatment of small melanocytic lesions or small melanomas in the eye to achieve local disease control and vision preservation with the possibility to prevent metastases and improve overall patient survival.

Amelanotic Ciliochoroidal Melanoma in a Patient with Oculocutaneous Albinism

“…Even less common is choroidal melanoma, with only 5 reported cases to date, including the case of ciliochoroidal melanoma reported here [4-7]. Casswell et al [6] discussed that the theoretical combined incidence of choroidal melanoma in a tyrosinase-positive albino is 1–3 per 100 million, compared to the overall incidence of uveal melanoma in the United States of 5 per million [8]. A summary of clinical features of choroidal melanoma in the 5 reported albino patients include race Caucasian (4/5) or African American (1/5); subtype tyrosinase-positive OCA (1/5), albinoidism (1/5), or undefined (3/5); tumor color amelanotic (5/5), and treatment with iodine-125 radiotherapy (3/5) or enucleation (2/6) [4-7].…”

Successful carbon-ion radiotherapy for choroidal melanoma adjacent to the optic disc: A case report

“…Local recurrence, distant metastasis, and NVG can occur during the late follow-up period [1,6]. Therefore, continuous monitoring was required for this patient.…”

“…Eye-preserving therapeutic modalities are available for choroidal melanoma [1]. However, since damage to the optic nerve cannot be avoided after eye-preserving therapy in patients with choroidal melanoma adjacent to the optic nerve, enucleation is commonly selected for such patients [2].…”

“…Choroidal melanoma is a rare but life-threatening intraocular malignant tumor [1]. Local control of tumors without ocular complications and prevention of metastasis are the principal modalities for the management of choroidal melanoma.…”

Ring-Shaped Leiomyoma of the Ciliary Body

“…Despite its rarity, it has significant clinical ramifications due to both clinical and imaging characteristics which closely resemble other intraocular tumors such as amelanotic melanoma, schwannoma, neurofibroma, lymphoma, and metastatic tumors [1,2]. In contrast to leiomyoma, malignant uveal melanoma is the most common primary intraocular tumor found in adults [4]. Both can manifest as a solid vascular mass with a dome shape and smooth surface.…”