National Cancer Institute®
Last Modified: November 21, 2001
UI - 20329341
AU - Singh AD; Shields CL; Shields JA; Sternberg P Jr
TI - Occurrence of retinoblastoma and uveal melanoma in the same patient.
SO - Retina 2000;20(3):305-6
AD - Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. firstname.lastname@example.org
UI - 21279979
AU - Roth DB; Scott IU; Murray TG; Kaiser PK; Feuer WJ; Hughes JR; Rosa RH Jr
TI - Echography of retinoblastoma: histopathologic correlation and serial evaluation after globe-conserving radiotherapy or chemotherapy.
SO - J Pediatr Ophthalmol Strabismus 2001 May-Jun;38(3):136-43
AD - Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Fla 33101, USA.
PURPOSE: To assess the sensitivity of echography in detecting retinoblastoma, compare tumor features observed by echography with histopathology data, and assess the usefulness of echography in serially following retinoblastoma tumors after globe-conserving treatments. METHODS: The medical and echography records of all patients treated for retinoblastoma at the Bascom Palmer Eye Institute between 1991 and 1997 were reviewed. All eyes underwent pretreatment echographic evaluation, and eyes treated with external beam radiotherapy, brachytherapy, or chemotherapy underwent serial follow-up echography. RESULTS: Sixty-nine eyes of 48 patients were identified. Echography demonstrated evidence of retinoblastoma in 69 of 69 (100%) eyes and calcification in 63 (91.3%) eyes. Histopathology was superior to echography in detecting optic nerve invasion, extraocular extension, and presence of calcification. CONCLUSION: Echography is a useful adjunct to indirect ophthalmoscopy in establishing the diagnosis of retinoblastoma. While not as specific as histopathology, echographic evaluation before and after treatment of retinoblastoma permits monitoring of treatment response and may aid in detecting recurrent tumor growth or failure to respond to treatment.
UI - 21279984
AU - Wang AG; Hsu WM; Hsia WW; Liu JH; Yen MY
TI - Clinicopathologic factors related to metastasis in retinoblastoma.
SO - J Pediatr Ophthalmol Strabismus 2001 May-Jun;38(3):166-71
AD - Department of Ophthalmology, Taipei Veterans General Hospital, and the Institute of Clinical Medicine, Taiwan.
PURPOSE: To investigate the orbital invasion and metastatic pattern in retinoblastoma, and to analyze the correlation with other pathologic and prognostic factors. METHODS: Pathologic and admission records of 45 patients with a pathologically confirmed diagnosis of retinoblastoma during the past 20 years were reviewed. Clinical risk factors and pathologic findings were analyzed for association with tumor invasion/metastasis using chi-square, Fisher's exact, and Student's t tests. RESULTS: Clinical risk factors such as exophthalmos, cataract, and pseudohypopyon were significantly correlated with tumor invasion/metastasis. The pathologic finding of choroid involvement was highly predictive of tumor invasion/metastasis. Delayed enucleation (>3 months) also was significantly correlated with tumor invasion/metastasis. CONCLUSION: Tumor invasion/metastasis is strongly associated with various ocular, pathologic, and treatment outcome factors. Identification of these risk factors may help plan treatment regimens.
UI - 21411329
AU - Albert D; Syed N; Cancer Committee, College of American Pathologists
TI - Protocol for the examination of specimens from patients with retinoblastoma: a basis for checklists.
SO - Arch Pathol Lab Med 2001 Sep;125(9):1183-8
AD - Department of Ophthalmology, University of Wisconsin Hospital, Madison, WI, USA.
UI - 21411341
AU - Liang SX; Lakshmanan Y; Woda BA; Jiang Z
TI - A high-grade primary leiomyosarcoma of the bladder in a survivor of retinoblastoma.
SO - Arch Pathol Lab Med 2001 Sep;125(9):1231-4
AD - Department of Pathology, University of Massachusetts Medical School, Worcester, MA 01655, USA.
Second nonocular malignancies develop with increased incidence in patients with hereditary retinoblastoma. Osteosarcoma is by far the most common type with an incidence of up to 50%, followed by soft tissue sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases have been reported in the past 2 decades, one in the liver and another one in the urinary bladder, both of which developed after cyclophosphamide therapy. Here we report a case of vesical leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after the diagnosis of a hereditary retinoblastoma. The patient's retinoblastoma was treated with unilateral enucleation without adjuvant radiation or chemotherapy. We believe that this is the first report of vesical leiomyosarcoma occurring in a patient with retinoblastoma without a prior history of radiation or chemotherapy. This report is significant not only because of the rarity of vesical leiomyosarcoma as a second nonocular tumor in retinoblastoma patients, but also because of the infrequency of vesical leiomyosarcoma in general. We also investigated the potential molecular pathogenesis of the leiomyosarcoma.
UI - 21453165
AU - Stannard C; Sealy R; Hering E; Korrubel J; Hill J; Barron A; Knowles R
TI - Localized whole eye radiotherapy for retinoblastoma using a (125)I applicator, "claws".
SO - Int J Radiat Oncol Biol Phys 2001 Oct 1;51(2):399-409
AD - Department of Radiation Oncology, Groote Schuur Hospital and University of Cape Town, Cape Town, South Africa. email@example.com
PURPOSE: To treat children with retinoblastoma, who require whole eye radiotherapy, with a specially designed (125)I applicator that irradiates the eye while sparing the surrounding tissues. METHODS AND MATERIALS: Under general anesthesia, a pericorneal ring is attached to the 4 extraocular muscles, and 4 appendages, each loaded with (125)I seeds, are inserted beneath the conjunctiva in-between each pair of muscles and attached anteriorly to the ring. Twenty-nine eyes were treated. Eighteen received a median dose of 28 Gy during 91 hours and 11 received 40 Gy during 122 hours, when the relative biologic effectiveness was taken as 1 instead of 1.5. Six had received prior chemotherapy. RESULTS: Twenty-four eyes were followed up for 2-157 months (median 29). Although 22 eyes responded, local control was achieved in 13 patients, 3 of whom required additional treatment for new tumors; a further 3 required additional treatment for tumor recurrence as well as new tumors. One of these eyes was enucleated for neovascular glaucoma. All 6 Group I-III eyes and 6 of 18 Group V eyes were retained for 2-157 months (median 39), with good vision in 10 eyes. Three developed cataracts 7, 8, and 12 years later, 1 of which has been removed. CONCLUSIONS: This is a new way of irradiating the whole eye with a minimal dose to the surrounding tissues. The treatment time is only 5 days. It is effective in Groups I-III, but only 33% of Group V eyes retained vision. No late cosmetic defects occurred.
UI - 21465268
AU - Abramson DH; Melson MR; Dunkel IJ; Frank CM
TI - Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma.
SO - Ophthalmology 2001 Oct;108(10):1868-76
AD - Department of Ophthalmology, New York-Presbyterian Hospital, New York Weill Cornell Medical Center, New York, New York, USA.
OBJECTIVE: This study aimed to investigate the incidence, timing, pattern, and distribution of, as well as survival as a result of, third, fourth, and fifth primary tumors in survivors of retinoblastoma. DESIGN: This study was a retrospective case series of patients diagnosed with retinoblastoma and a second malignant neoplasm. Records were examined for demographic, prior treatment, and second tumor information, as well as any evidence of the development of a third, fourth, or fifth nonocular tumor. When possible, telephone inquiries were conducted for follow-up. PARTICIPANTS: The study included 1506 patients followed in the Ophthalmic Oncology Center at New York-Presbyterian Hospital, New York Weill Cornell Medical Center, 211 of whom developed a second tumor and had sufficient treatment data to be useful for analysis. MAIN OUTCOME MEASURES: The development of third and additional nonocular tumors and survival from these tumors were the primary outcome measures. RESULTS: Of 211 second-tumor patients, 142 died before an additional malignancy developed (median survival time, 1.8 +/- 0.3 years) and in 28, third tumors developed (5-year incidence rate, 11%; 10-year incidence rate, 22%; median time to third tumor development, 5.8 +/- 8.3 years). The 5- and 10-year survival rates for this group were 41% and 30%, respectively (median survival time, 4.1 +/- 1.0 years). Of 28 patients in whom third tumors developed, 27 (96%) had received radiation therapy for their retinoblastoma. The most common sites for third tumors were soft tissues of the head (36% of all third tumors) and skin (36% of all third tumors). In six patients, a fourth tumor developed, and in two patients a fifth tumor developed. All fourth and fifth tumors were found in the soft tissues of the head, the skin, or the bones. CONCLUSIONS: Survivors of retinoblastoma in whom second malignant neoplasms develop are at a higher risk for the development of additional tumors than they were for the development of a second tumor. The locations and expected ages at which additional tumors develop are consistent with the patterns we have seen in second tumors.
UI - 92195558
AU - Weinstein GW; Weinstein BD
TI - To see or to live? Case study and commentaries.
SO - Ophthalmic Surg 1992 Feb;23(2):140-3
AD - Department of Ophthalmology, West Virginia University School of Medicine, Morgantown.
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