RESUMEN
PURPOSE: To report on the frequency of cysts and tumors of the pineal gland in patients with retinoblastoma. DESIGN: Observational retrospective case control study. SETTING: Institutional. study population: Four hundred eight patients treated for retinoblastoma from January 2000 to January 2012 at Wills Eye Institute, Philadelphia, Pennsylvania, USA. OBSERVATION PROCEDURE: Magnetic resonance imaging (MRI) features of the pineal gland were evaluated in all patients with retinoblastoma. Characteristics of patients with pineal cysts and pineoblastoma were reviewed. MAIN OUTCOME MEASURES: Comparison of frequency of pineal gland cyst and pineoblastoma in children managed with systemic chemoreduction vs other methods. RESULTS: Of 408 patients, treatment included systemic chemoreduction in 252 (62%) and nonchemoreduction methods in 156 (38%). Overall, 34 patients (8%) manifested pineal gland cyst and 4 (1%) showed pineoblastoma. Of all 408 patients, comparison (chemoreduction vs nonchemoreduction) revealed pineal cyst (20/252 vs 14/156, P = .7) and pineoblastoma (1/252 vs 3/156, P = .1). The pineal cyst (n = 34) (mean diameter 4 mm) was asymptomatic (n = 34), followed conservatively (n = 34), and with minimal enlargement (n = 2, 9%) but without progression to pineoblastoma. The cyst was found in 22 germline and 12 nongermline patients (P = .15). Among the 4 patients with pineoblastoma, all had germline mutation and 2 had family history of retinoblastoma. Among all patients with family history of retinoblastoma (n = 45), 2 (4%) developed pineoblastoma. The pineoblastoma was asymptomatic in 2 patients and symptomatic with vomiting and headache in 2 patients. The mean interval from date of retinoblastoma detection to pineal cyst was 2 months (median 2, range 0-8 months) and to pineoblastoma was 27 months (median 28, range 7-46 months). Management included aggressive chemotherapy and radiotherapy, with 2 survivors. CONCLUSIONS: Pineal gland cyst was incidentally detected in 8% of retinoblastoma patients, causing no symptoms, and without progression to pineoblastoma. Pineoblastoma was detected in 1% of patients and fewer patients who received systemic chemotherapy developed pineoblastoma, possibly indicating a systemic protective effect.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Quistes del Sistema Nervioso Central/diagnóstico , Glándula Pineal/patología , Pinealoma/diagnóstico , Neoplasias de la Retina/diagnóstico , Retinoblastoma/diagnóstico , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/terapia , Estudios de Casos y Controles , Quistes del Sistema Nervioso Central/mortalidad , Quistes del Sistema Nervioso Central/terapia , Quimioradioterapia , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Pinealoma/mortalidad , Pinealoma/terapia , Neoplasias de la Retina/mortalidad , Neoplasias de la Retina/terapia , Retinoblastoma/mortalidad , Retinoblastoma/terapia , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
PURPOSE: To describe the outcome of intraocular tumor resection by partial lamellar sclerouvectomy (PLSU) in the pediatric age group. DESIGN: Retrospective, interventional case series. PARTICIPANTS: We included 37 patients who underwent PLSU for intraocular tumors under age 21 years. METHODS: Medical records were reviewed for clinical, operative, and histopathologic features. MAIN OUTCOME MEASURES: Globe salvage, visual acuity, surgical side effects, tumor control, and tumor-related metastasis and death. RESULTS: The mean patient age was 9 years (median, 10; range, 0.25-17) and there were 22 (59%) females and 15 (41%) males. The tumor involved mainly the iris (n = 27; 73%), ciliary body (n = 7; 19%), or choroid (n = 3; 8%). The mean tumor basal dimension was 7 mm (median, 6; range, 3-15) and thickness was 3.6 mm (median, 3; range, 0.2-9.6). The histopathologic diagnosis was melanoma (n = 19; 51%), cyst (n = 5; 14%), medulloepithelioma (n = 4; 10%), melanocytoma (n = 3; 8%), lacrimal gland choristoma (n = 2; 5%), gliosis (n = 1; 3%), nevus (n = 1; 3%), hemorrhage (n = 1; 3%), and leiomyoma (n = 1; 3%). Intraoperative adverse effects included vitreous loss in 19 (51%) and hyphema in 4 (11%). Postoperative side effects included early transient hyphema in 9 (24%) and late cataract in 12 (32%). Local tumor recurrence was detected in 5 cases (13%) at mean interval of 28 months, including melanoma (n = 3) and medulloepithelioma (n = 2). Enucleation was necessary in 9 (24%) cases for recurrence in 3 (8%), prophylactic in eyes with high-grade malignancy in 5 (13%), and for blind painful eye in 1 (3%). Of the 28 salvaged eyes, final visual acuity was ≥ 20/40 in 18 (64%), 20/50 to 20/100 in 5 (18%), and <20/200 in 5 (18%). There were no cases of metastasis or death at a mean of 51 months of follow-up. CONCLUSIONS: A surgically challenging procedure, PLSU can achieve control of selected intraocular tumors. Medulloepithelioma responds poorly to local resection and in most cases enucleation is required. If the globe is salvaged, visual acuity is ≥ 20/40 in 64% of children. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.
Asunto(s)
Procedimientos Quirúrgicos Oftalmológicos , Neoplasias de la Úvea/cirugía , Adolescente , Niño , Preescolar , Enucleación del Ojo , Femenino , Humanos , Lactante , Complicaciones Intraoperatorias , Masculino , Complicaciones Posoperatorias , Esclerótica/cirugía , Resultado del Tratamiento , Ultrasonografía , Úvea/cirugía , Neoplasias de la Úvea/diagnóstico por imagen , Neoplasias de la Úvea/patología , Agudeza Visual/fisiologíaRESUMEN
A 3-year-old girl presented with a black pupillary margin iris mass in the right eye diagnosed as an iris pigment epithelial (IPE) cyst, central (pupillary) type. The diagnosis was verified by anterior-segment optical coherence tomography (AS-OCT), which initially showed a circumscribed, round thin-walled cyst with clear lumen attached by a strand to the IPE. On follow-up 3 months later, the IPE cyst had deflated and AS-OCT showed an irregular collapsed surface with no lumen and optical shadowing. The authors report a case in which AS-OCT was used to provide information regarding the diagnosis and behavior of an IPE cyst.