Intraocular reticulum cell sarcoma: its dramatic response to systemic chemotherapy and its angiogenic potential

A 77-year-old woman with reticulum cell sarcoma in apparent remission presented with neovascular glaucoma. Ultrasonography demonstrated a 360 degree ring tumor of the ciliary body which totally regressed in 3 weeks after reinsitution of systemic chemotherapy. This appears to be a ring reticulum cell sarcoma of the ciliary body and is apparently the first reported case of an intraocular reticulum cell sarcoma which has undergone such a dramatic response to combined chemotherapy. A review of the English literature of all published cases of ocular reticulum cell sarcoma (16 cases) discloses no similar case, and emphasizes for the first time the angiogenic potential of this tumor. Secondary glaucoma was found to be associated with intraocular reticulum cell sarcoma in approximately two-thirds of the reported cases, and neovascular glaucoma in about one-quarter of these cases.