Intravenous chemoreduction or intra-arterial chemotherapy for cavitary retinoblastoma: long-term results.

OBJECTIVE: To assess the long-term results of chemotherapy for cavitary retinoblastoma. METHODS: Retrospective, nonrandomized, interventional case series of 26 cavitary retinoblastomas in 25 eyes of 24 patients. Retinoblastomas were treated with intravenous chemoreduction and/or intra-arterial chemotherapy. Main outcome measures included tumor control, globe salvage, and metastasis. RESULTS: Of 24 patients with cavitary retinoblastoma, the mean age at diagnosis was 16 months. The mean number of cavitary tumors per eye was 1 (median, 1; range, 1-2), with a mean tumor basal diameter of 13 (median, 13; range, 7-24) mm and mean tumor thickness of 7 (median, 6; range, 3-17) mm. The mean number of cavities per tumor was 2 (median, 2; range, 1-5), with a mean cavity diameter of 3 (median, 2; range, 1-10) mm. Related features included vitreous seeds in 7 tumors (27%), subretinal seeds in 6 (23%), and subretinal fluid in 13 (50%). Intravenous chemoreduction was used in 23 tumors (88%); intra-arterial chemotherapy, in 2 (8%); and both, in 1 (4%). After treatment, the mean reduction in tumor base was 22% and mean reduction in tumor thickness was 29%. Despite minimal reduction, tumor recurrence was noted in only 1 eye (4%), globe salvage was achieved in 22 (88%), and there were no cases of metastasis or death during 49 (range, 6-189) months of follow-up. CONCLUSION: Despite minimal visible tumor response to chemotherapy, cavitary retinoblastoma displays relatively stable long-term results.

Fluorescein angiographic findings after intra-arterial chemotherapy for retinoblastoma.

PURPOSE: To evaluate fluorescein angiography (FA) findings after intra-arterial chemotherapy (IAC) for retinoblastoma. DESIGN: Retrospective case series. PARTICIPANTS: Twenty-four eyes of 24 patients. INTERVENTION: Fifty-five IAC procedures for delivery of melphalan 5 mg and possible carboplatin 30 mg. MAIN OUTCOME MEASURES: Vascular flow of iris, retina, and choroid after IAC. RESULTS: All patients received melphalan 5 mg, whereas the first 6 patients also were treated with additional carboplatin 30 mg. The IAC was performed as primary treatment in 17 eyes and as secondary treatment (after systemic chemotherapy) in 7 eyes. Two patients also received external-beam radiotherapy before IAC. At presentation, FA revealed neovascularization of the iris (NVI) in 8 eyes, and after IAC, complete NVI regression was noted in 5 eyes (63%). After a mean follow-up of 13 months after IAC, FA depicted the main tumor with decreased fluorescence in 22 eyes (92%). After 55 ophthalmic artery catheterizations, retinal vascular abnormalities by FA were detected in 7 eyes (13%) and choroidal vascular abnormalities were detected in 6 eyes (11%). The retinal abnormalities included ophthalmic artery obstruction (n = 1), transient ophthalmic artery spasm (n = 1), central retinal artery obstruction (n = 1), branch retinal artery obstruction (n = 2), and peripheral retinal ischemia (n = 2). Additional retinal neovascularization was found in 1 patient. The choroidal abnormalities included sector (n = 5) or diffuse (n = 1) choroidal nonperfusion. New-onset iris neovascularization was found in 2 patients. Retinal vascular abnormalities were diagnosed after median of 1 month after the first IAC, whereas choroidal vascular abnormalities were found after median of 5 months after the first IAC. CONCLUSIONS: Fluorescein angiography suggests that vascular perfusion to the retina and the choroid can be compromised after IAC for retinoblastoma. The most common vascular abnormality was choroidal sector or diffuse nonperfusion.

Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma.

OBJECTIVE: To describe the features of phacomatosis pigmentovascularis (cesioflammea type). DESIGN: Noninterventional retrospective case series composed of 7 patients. RESULTS: Nevus flammeus combined with ipsilateral ocular melanocytosis or melanosis was seen in all 7 patients. Additional contralateral nevus flammeus was observed in 3 patients. Nevus flammeus (unilateral in 4 patients and bilateral in 3 patients) was distributed in trigeminal nerves V1 (n = 3), V2 (n = 7), and V3 (n = 5). Related findings included diffuse choroidal hemangioma (n = 1) and glaucoma (n = 1), with no patients having brain hemangioma or seizures. Ocular pigmentary abnormalities (unilateral in all 7 patients) included congenital ocular melanocytosis (n = 6) and conjunctival acquired melanosis (n = 1). Pigmentation was sectorial (partial) in 5 patients and complete in 2 patients. Melanocytosis involved the periocular skin in 1 patient, sclera in 2 patients, iris in 2 patients, and choroid in 4 patients. In 3 of 6 patients, melanocytosis was visible in the choroid only on dilated fundus evaluation. Related tumors included choroidal melanoma (n = 3), optic disc melanocytoma (n = 1), and conjunctival melanoma in situ (primary acquired melanosis) (n = 1). Melanoma metastasis developed in 1 patient. CONCLUSIONS: Phacomatosis pigmentovascularis shows features of nevus flammeus and more serious ocular pigmentary abnormalities (uveoscleral melanocytosis and conjunctival melanosis). Melanocytosis may be detected only by dilated ocular fundus examination, as found in 3 of 6 patients. Furthermore, choroidal melanoma can develop from melanocytosis, as noted in 3 of our 6 patients (50%). All patients with nevus flammeus should be examined for phacomatosis pigmentovascularis by an ophthalmologist because ocular melanocytosis and uveal melanoma may remain hidden within the eye.