Visual fields in retinoblastoma survivors.

OBJECTIVE: To describe the visual field defects in retinoblastoma survivors and relate those defects to characteristics such as tumor size, tumor location, and treatment modality. METHODS: Thirty-one patients treated for retinoblastoma were included in this study. Humphrey visual fields were determined in 33 eyes. RESULTS: Twenty-seven patients (29 eyes, 68 tumors) had sufficient diagnosis and treatment data available for further analysis. Twenty-six of the 27 patients had both absolute and relative visual field defects. Four types of visual field defects were observed and correlated with location of the tumor and therapy to the individual tumors: (1) no residual defect, (2) absolute scotoma, (3) arcuate and sector scotoma, and (4) "pseudo"-visual field defects caused by relative enophthalmos resulting from radiation. CONCLUSIONS: Patients with retinoblastoma demonstrate a variety of long-term visual field defects after treatment for their intraocular disease. Characteristics that determine the size and type of defects are tumor size, tumor location, and treatment method.

Retinoblastoma. A survivor 12 years after treatment for metastatic disease.

A 21-year-old man currently is well 12 years after his last treatment for metastatic retinoblastoma.

[Apropos of a case of renal oncocytoma with hepatic localization]. / A propos d'un cas d'oncocytome rénal avec localisations hépatiques.

A renal oncocytoma was treated by radical nephrectomy. Although it was considered benign, hepatic localizations were discovered seven years later. The authors discuss the problems of whether these were secondary metastatic lesions or a new development in the evolution of the disease.

[Treatment of retinoblastoma by radioactive cobalt and xenon photocoagulation]. / Traitement du rétinoblastome par cobalt radioactif et photocoagulation au xénon.

During the years 1961-1975, 50 cases of retinoblastoma in 31 boys and 19 girls from all parts of Poland were under treatment in the Ophthalmological Clinic in Cracow. The ages of the children at the time of diagnosis ranged from a few months to 10 years. The largest group included children between 1 and 3 years. In the majority of patients, neoplasm affected both eyes. The eye with the most advanced changes was enucleated, and the other subjected to treatment if the size of the tumor gave a prognosis of at least partial preservation of vision. In the case of unilateral tumors, treatment was undertaken if the changes were not too advanced. In the patients under treatment, 60Co according to themethod of Stallard and xenon light coagulation was applied. The observation period varied between 0.5 and 15 years. In our work, a correlation was found between the results and the size of the tumors, their localization, and postoperative complications. Our treatments proved successful in 70% (50.0-90.9%) of the cases.

Mesothelioma following Wilms' tumor in childhood.

A high percentage of children with Wilms' tumor are cured with multimodal treatment. A small percentage of these children will develop second tumors, perhaps related to a genetic predisposition to neoplasia or possibly secondary to the treatment utilized for Wilms' tumor. Malignant mesothelioma has been associated with contact with asbestos but has also been reported after radiation exposure. Two patients are reported who developed malignant mesothelioma of the pleura after treatment for Wilms' tumor in childhood. Both received orthovoltage radiation; one patient also received triethylenemelamine (TEM), an alkylating agent closely related to nitrogen mustard, for 5 years. Factors in the development of second tumors are discussed.

Nonocular second primary tumors after retinoblastoma: retrospective study of 111 patients treated by electron beam radiotherapy with or without TEM.

The objective of this study was to assess the role of various clinical and treatment factors involved in the long-term incidence of nonocular second primary tumors following retinoblastoma. The study was based on 111 patients treated between 1963 and 1977 according to the same radiotherapy protocol (electron beam radiotherapy) alone or in combination with triethylene melamine (TEM). Various statistical methods were used to obtain the actuarial survival curve, the cumulative incidence of second primary tumors, and comparisons of patient groups and subgroups. The 5-, 10-, 20-, and 30-year survival rates were 75%, 70%, 63%, and 55% with a follow-up of 23 to 35 years. The study reports the various parameters concerning 111 children and 17 second primary tumors: sex, age at treatment, histology of the retinoblastoma and second primary tumors, site of second tumors (anatomic and compared with irradiation field), radiation dose, time to onset, and chemotherapy with or without TEM. The results are discussed and compared with the data reported in the literature. Electron beam radiotherapy at a dose of 45 Gy does not eliminate the risk of nonocular second primary tumors. TEM also does not modify survival or the overall incidence of second primary tumors, but significantly increases the risk of second primary tumors outside the irradiation field.

Retinoblastoma.

Newer concepts in the diagnosis and treatment of retinoblastoma are briefly discussed. The role of ultrasonography, EMI scanning and LDH assays in the anterior chamber are evaluated. The greatest problems in differential diagnosis in recent years have been larval granulomatosis caused by Toxicara canis, Coat's disease and unclassifiable retinal dysplasias. The Ellsworth-Reese classification is discussed. The "cure rate" has been widely misinterpreted as survival and has led to many misapprehensions in the treatment of unilateral disease. The cases in group IV now do better for a variety of reasons and the results in orbital cases have been more extensively studied. The genetics of retinoblastoma have been intensively studied in our laboratory along with Dr. Kitchin and some newer concepts are briefly discussed. The relative roles of orthovoltage, external beam 60Co, linear accelerators and betatron are presented. Dosage level is distinctly related to radiation complications, especially retinal vascular necrosis and fatal radiation-induced neoplasms. The relationship of the latter to the pattern of inheritance is to be stressed. Chemotherapy has been used both as an adjuvant to radiation and for the destruction of micrometastases and a new protocol is described. The results of treatment are presented to update the 1966 report at the Gonin Club Meeting in Munich.

[Results of treatment in cases with bilateral retinoblastoma (author's transl)]. / Behandlungsergebnisse bei beiderseitigem Retinoblastom.

Immediate and late results of treatment in 132 cases with bilateral retinoblastoma (the children were aged between two months and six years) were analyzed. Combined treatment comprised enucleation of the more affected eye, further chemotherapy, X-ray treatment and photocoagulation. Cytostatic compounds used were "TET" (analogous to "TEM") and "Prospidin". Of the treated children, 44.8% are alive; the period of observation ranged from seven to 16 years.

Chemotherapy of childhood medulloblastoma.

A number of chemotherapeutic agents have been shown to have anti-tumor activity against childhood medulloblastoma. Ten-year survival with optimal surgery and radiation therapy ranges from 5% to 25%. Carefully controlled clinical trials utilizing a combination of surgery, radiation therapy, and chemotherapy should be employed in the early stages of the disease in an attempt to define which drugs are most effective, and to improve the survival rate.

Non-Hodgkin's malignant lymphomata in adults: chemo-radiotherapy in stages III and IV.

Patients presenting with Stage III or IV non-Hodgkin's malignant lymphoma were given chemotherapy; about 20% complete remission was obtained for both stages. The addition of radiotherapy increased the incidence to 70% in Stage III patients. The duration of first complete remission was longer for Stage III (25% of the patients are still in first remission at 7 years) than for Stage IV (0%). The survival was longer for nodular lymphosarcoma patients (25% are alive at 7 years for Stages III and IV) than for diffuse lymphosarcomata and reticulosarcomata (10%). Among the new drugs, VM 26 is able to produce a good frequency of remission in patients in relapse.