Retinoblastoma is the most common ocular malignancy in childhood. The association of bilateral retinoblastoma with ectopic midline intracranial tumor, trilateral retinoblastoma syndrome, is rare, but the prognosis is very poor with even with various combination treatments of surgery, chemotherapy, and radiotherapy including neuraxis. The cause of treatment failure was tumor dissemination along the neuraxis in approximately 50% of the cases. We experienced three cases of trilateral retinoblastoma, and two of them are alive so far with multi-modal treatment.
Drug Therapy ; Prognosis ; Radiotherapy ; Retinoblastoma* ; Treatment Failure

Radiotherapy result was analyzed in 23 children with retinoblastoma treated in Seoul National University Hospital from 1980 to 1987. Three (17%) had bilateral tumor at diagnosis. Among 20 children with unilateral retinoblastoma 13 children got radiotherapy after enucleation, 2 were treated with radiotherapy alone, and 5 were delivered with radiotherapy after relapse. Of 15 non-recurrent unilateral tumors, there were 5 stage II children, 8 stage III, and 2 stage IV by staging system proposed by St. Jude Children's Research Hospital. Chemotherapy was combined when resection margin of the optic nerve was positive or when malignant cell was found in SCF. Of 12 children who completed radiotherapy, local or distant failure was not found but 2 cases of relapse at the contralateral retina were observed. Their 5 year survival rate was 82.2%. Another case of contralateral relapse was detected in children who was treated with radiotherapy alone. Thus overall frequency of the bilateral disease was 33%. Prognosis of recurrent tumors were so poor that no cases of CR was obtained and that 3 year survival rate was 20%. Two of 3 bilateral cases at diagnosis were in NED status. Complication were sunken orbit only. Result of radiotherapy was so good in early stage or small bulk tumor that treatment delay after diagnosis must not be allowed.
Child ; Diagnosis ; Drug Therapy ; Humans ; Optic Nerve ; Orbit ; Prognosis ; Radiotherapy* ; Recurrence ; Retina ; Retinoblastoma* ; Seoul ; Survival Rate

Retinoblastom is the most common intraocular malignancy in childhood and the survival rate has recently been raised by an adequate method of examination and treatment. We report a case that a child having bilateral retinoblastoma treated with enucleation, external beam irradiation, and chemotherapy is diagnosed as an osteosarcoma of the distal radius 9 years after the initial treatment, and died. This case shows the regular examinations for long bone of the extremities are necessary in a child survived after the treatment of bilateral retinoblastoma.
Child ; Drug Therapy ; Extremities ; Humans ; Osteosarcoma* ; Radiotherapy ; Radius ; Retinoblastoma* ; Survival Rate ; Survivors*

The clinicopathologic characteristics, treatments and prognosis of retinoblastoma, the most common intraocular tumor in children, were studied retrospectively in 101 cases(128eyes) who visited our clinic from 1987 to 1996. All patients were followed for 2 years or longer. Twenty seven cases were bilateral and seventy four cases were unilateral,. Eihgty four eyes were enucleated. Twenty one eyes were successfully treated by conservative modality. The rest refused any type of treatment. Orbital recurrence or distant metastasis were observed in 5 cases after enucleation without systemic treatment, and one died in spite of irradiation and aggressive chemotherapy. It is widely recognized that the prognosis for survival of retinoblastoma patients, especially unilateral, is excellent, however the authors emphasize the importance of careful postoperative histopathologic review and long term periodic follow-up to improve long-term prognosis.
Child ; Drug Therapy ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Orbit ; Prognosis* ; Recurrence ; Retinoblastoma* ; Retrospective Studies

We analyzed retrospectively 58 patinets of retinoblastoma for the first, ocular symptoms, gender, family history, funduscopic findings, treatment modalities and results from 1987 to 1993. Fourteen patients were bilateral, forth-four were unilateral, and seventy-two eyes were affected in sum. The average of age of the patients was 25.6 months. Their first ocular symptoms were leukocoria, squint and etc. Three patients were suspected as familial type of retinoblastoma. We claasified the patients according to the affected degree of the retina;over three-fourths of the retina was affected in 34 eyes. We treated them with enucleation, radiation, chemotherapy, episcleral plaque radiotherapy, laser photocoagulation and cryotherapy. After these treatments, orbital recurrence was observed in one case and among the ten eyes treated with eye-conserving treatments, nine eyes are maintained with periodic examination under general anesthesia and the treatment of laser photocoagulation and cryotherapy until now.
Anesthesia, General ; Cryotherapy ; Drug Therapy ; Humans ; Light Coagulation ; Orbit ; Radiotherapy ; Recurrence ; Retina ; Retinoblastoma* ; Retrospective Studies ; Strabismus

PURPOSE: To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients. METHODS: Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation. RESULTS: Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation. CONSLUSION: Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.
Child ; Cryotherapy ; Diagnosis ; Drug Therapy ; Female ; Humans ; Infant ; Light Coagulation ; Medical Records ; Protons ; Retinoblastoma*

PURPOSE: To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients. METHODS: Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation. RESULTS: Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation. CONSLUSION: Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.
Child ; Cryotherapy ; Diagnosis ; Drug Therapy ; Female ; Humans ; Infant ; Light Coagulation ; Medical Records ; Protons ; Retinoblastoma*

BACKGROUND: Chemotherapy with 5-fluorouracil (5-FU) has been one of the mainstay in breast cancer treatment. The effects of high dose 5-FU on cell cycle regulation were studied in breast caner cells. METHODS: A breast cancer cell line MCF-7 was used. Protein expressions of G1/S cyclins, p21(Waf1/Cip1), cdk2, E2F1 and retinoblastoma were tested by western blot analysis. Immunoprecipitation and immune complex kinase assay were done for the assessment of E2F1/RB interacton and the activity of cdk2 respectively. RESULTS: p21(Waf1/Cip1) expression was barely detectable in control cells. With addition of 5-FU level of p21(Waf1/Cip1) were induced and cyclin D3 level was decreased as cell growth decreases. In accordance with increased expression of p21(Waf1/Cip1), cyclin E-associated cdk2 kinase activity was reduced. Retinoblastoma protein (RB) became dephosphorylated and E2F-1 binding activity with RB was increased. CONCLUSION: In this situation of high concentration of 5-FU breast cancer cells tend to be G1/S cell cycle arrested. Overexpression of p21(Waf1/Cip1) and dephosphorylation of RB may mediate the effectss of 5-FU by inhibiting E2F-1 activity, which contributes to G1/S cell cycle arrest. These results could be an indicating landmark for further study of high dose chemotherapy with 5-FU.
Antigen-Antibody Complex ; Blotting, Western ; Breast Neoplasms* ; Breast* ; Cell Cycle Checkpoints ; Cell Cycle* ; Cell Line* ; Cyclin D3 ; Cyclins ; Drug Therapy ; Fluorouracil* ; Immunoprecipitation ; Phosphotransferases ; Retinoblastoma ; Retinoblastoma Protein

Retinoblastoma is the most common intraocular malignancy in childhood. Diagnosis is currently made by ophthalmologists under general anesthesia as it is the gold standard for intraocular assessment. However, evaluations for extraocular disease are also necessary. Treatment strategies vary according to the disease status. If a single eye is involved, the treatment goal is oriented to the removal of the tumor and prevention of relapse. In bilateral retinoblastoma, the main treatment goal is to save monocular vision and save life. This article will explore the available treatment options for retinoblastoma including enucleation, radiotherapy, local therapy, intravenous chemotherapy, intra-arterial injection and intra-vitreal injections. There were recent advances in our understanding on the genetic pathophysiology of the retinoblastoma protein gene in tumorigenesis, which may help developing future treatment. Early detection of retinoblastoma is important for prolonging survival and improving quality of life.
Anesthesia, General ; Carcinogenesis ; Diagnosis ; Drug Therapy ; Injections, Intra-Arterial ; Quality of Life ; Radiotherapy ; Recurrence ; Retinoblastoma Protein ; Retinoblastoma ; Stem Cell Transplantation ; Vision, Monocular

PURPOSE: To evaluate the clinical results of proton beam radiation therapy (PBRT) for treatment of retinoblastoma. METHODS: Children with retinoblastoma who were treated with chemotherapy and focal treatment such as brachytherapy and thermotherapy but showed no response or developed recurrences later received PBRT. The PBRT strategy was designed to concentrate the radiation energy to the retinoblastoma and spare the surrounding healthy tissue or organs. RESULTS: There were three patients who received PBRT. The first patient received PBRT because of an initial lack of tumor regression with chemotherapy and brachytherapy. This patient showed regression after PBRT. The second patient who developed recurrence of retinoblastoma as diffuse infiltrating subretinal seeding was taken PBRT. After complete regression, there was recurrence of tumor and the eye was enucleated. The third patient had unilateral extensively advanced retinoblastoma. Initial chemotherapy failed and tumor recurred. The tumor responded to PBRT and regressed significantly. However, the eye developed sudden multiple recurrences, so we had to perform enucleation. CONCLUSIONS: PBRT for retinoblastoma was effective in cases of showing no response to other treatment modalities. However, it should be carefully applied when there was recurrence of diffuse infiltrating subretinal seeding or extensively advanced retinoblastoma initially.
Child, Preschool ; Combined Modality Therapy ; Drug Resistance ; Female ; Humans ; Infant ; Male ; Retinal Neoplasms/drug therapy/*radiotherapy/surgery ; Retinoblastoma/drug therapy/*radiotherapy/surgery ; Retrospective Studies ; Treatment Outcome