No abstract available.
Radiotherapy* ; Retinoblastoma* ; Rhabdomyosarcoma*

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

The risk of radiotherapy-related secondary cancers in children with constitutional retinoblastoma 1 (RB1) mutations has led to reduced use of external beam radiotherapy (EBRT) for RB. Presently, tumor reduction with chemotherapy with or without focal surgery (chemosurgery) is most commonly undertaken; EBRT is avoided as much as possible and is considered only as the last treatment option prior to enucleation. Nevertheless, approximately 80% of patients are diagnosed at a locally advanced stage, and only 20-25% of early stage RB patients can be cured with a chemosurgery strategy. As a whole, chemotherapy fails in more than two-thirds of eyes with advanced stage disease, requiring EBRT or enucleation. Radiotherapy is still considered necessary for patients with large tumor(s) who are not candidates for chemosurgery but who have visual potential. When radiation therapy is indicated, the lowest possible radiation dose combined with systemic or local chemotherapy and focal surgery may yield the best clinical outcomes in terms of local control and treatment-related toxicity. Proton beam therapy is one EBRT method that can be used for treatment of RB and reduces the radiation dose delivered to the adjacent orbital bone while maintaining an adequate dose to the tumor. To maximize the therapeutic success of treatment of advanced RB, the possibility of integrating radiotherapy at early stages of treatment may need to be discussed by a multidisciplinary team, rather than considering EBRT as only a last treatment option.
Child ; Child, Preschool ; Eye Neoplasms/genetics ; Genes, Retinoblastoma/genetics ; Humans ; Radiotherapy Dosage ; Retinal Neoplasms/*radiotherapy ; Retinoblastoma/genetics/*radiotherapy

The authors reviewed their experiences of external beam radiotherapy (EBR) as an initial treatment in retinoblastoma patients to determine its long-term effect on subsequent tumor control and complications. A total of 32 eyes in 25 patients that underwent EBR for retinoblastoma were reviewed retrospectively. The patients consisted of 21 boys and 4 girls of median age at treatment of 7.1 months. Radiation doses ranged from 35 to 59.4 Gy. The 10-yr ocular and patient survivals were 75.4% and 92.3%, respectively. Nine of the 32 eyes progressed; 7 of these were enucleated and 2 were salvaged by focal treatment. According to the Reese-Ellsworth classification, 4 of 5 eyes of Group II, 13 of 16 Group III eyes, 2 of 4 Group IV eyes, and 5 of 7 Group V eyes were retained, and of the 32 eyes, 13 had visual acuity better than 20/200. Eleven patients experienced a radiation-induced complication. No patient developed a second malignancy during follow-up. Despite the limited number of patients enrolled, EBR may provide a mean of preserving eyeball and vision for some advanced lesions.
Child ; Child, Preschool ; Eye Enucleation ; Female ; Humans ; Infant ; Korea ; Male ; Radiotherapy/*methods ; Radiotherapy Dosage ; Retinal Neoplasms/*radiotherapy ; Retinoblastoma/*radiotherapy ; Retrospective Studies ; Survival Rate ; Treatment Outcome

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*

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

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

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 report the result of combined chemotherapy and radiotherapy for 5 retinoblastoma patients. METHODS: Retrospective study of 5 patients (8 eyes) who were diagnosed with retinoblastoma of group III to V in the Reese-Ellsworth (R-E) classification was done. After 6 cycles of chemotherapy consisting of Vincristine, VP-16 (Etoposide) and Carboplatin, Radiotherapy (4500Gy/5weeks) was done. Neoaedjuvant therapy consisting of Nimustine and Carboplatin was done, and supplementary laser photocoagulation was done in 1 case. Mean follow-up period is 33 months. RESULTS: Significant reduction of tumor size and regression were observed in five eyes of R-E classification group V, and slight reduction of tumor size and regression were observed in three eyes of R-E classification groups III and IV. There was no recurrence and no new mass was detected. CONCLUSIONS: Combined chemotherapy, radiotherapy and supplementary therapy may save eyeballs which were diagnosed with retinoblastoma, even R-E classification group V.
Carboplatin ; Classification ; Drug Therapy* ; Etoposide ; Follow-Up Studies ; Humans ; Light Coagulation ; Nimustine ; Radiotherapy* ; Recurrence ; Retinoblastoma* ; Retrospective Studies ; Vincristine

OBJECTIVETo study clinic therapeutic effect about reconstruction of severe orbital and cul-de-sac deformity after the radiotherapy with transcranial orbitotomy advancement combining cascade free flap both dorsum pedis flap and anterior tibial fascial flap.

METHODSFive cases was subjected to orbital and cut-de-sac severe deformities after both operation and radiotherapy because of retinoblastoma. The technique included transcranial orbital advancement by anterior orbital osteotomy and rigid fixed with titanic plate by coronal incision, and meanwhile incising the cul-de-sac which would be extended circumference around the central incision separation, and then designing extent of cascade flap consisted of dorsum pedis flap and anterior tibial fascial flap according to the size of cul-de-sac defect and extent of temporal depression. Then, the aforementioned two parts of cascade flap were transplanted into cul-de-sac and temple respectively. There is either the superficial temporal artery and vein or facial artery and jugular vein to chose vascular anastomosis.

RESULTSAll flaps survived. After 3 to 6 months following up, the results showed satisfactory orbital contour and temporal depression improved significantly in all cases. After the conjunctival sac were fixiformed with prefabricated eye prosthesis mode about 3 months. 3 cases have good appearance with wearing eye prosthesis and the other 2 cases' appearance is poor. One of the poor appearance cases, with depressed eye socket, have orbital implant underlying conjunctival sac in secondary operation. The other one, with swallowed inferior fornix, is transplanted autogenous hard palatal mucosa into inferior fornix in secondary operation. In addition, delayed healing in donor site of dorsum pedis occurred in one of the 4 cases.

CONCLUSIONSIt is a reliable procedure about reconstruction of severe orbital and cul-de-sac deformity after both the operation and radiotherapy with transcranial orbitotomy advancement combining cascade free flap both dorsum pedis flap and anterior tibial fascial flap. All deformity was corrected by one staged procedure which lessen patient's suffering and shorten patient's hospital stay and spare patient's costs.

Adolescent ; Adult ; Female ; Humans ; Male ; Orbital Diseases ; etiology ; surgery ; Osteotomy ; Radiotherapy ; adverse effects ; Reconstructive Surgical Procedures ; methods ; Retinoblastoma ; radiotherapy ; surgery ; Surgical Flaps ; Young Adult