Long-term follow-up of children with retinoblastoma.

Twenty-one pediatric retinoblastoma (RB) patients treated between 1976 and 1994 were evaluated for late treatment-related complications. Median age at diagnosis was 24 months; median age at follow-up was 12 years; median follow-up time was 12 years. Of the 21 patients, 14 had unilateral RB and 7 had bilateral RB. Thirteen patients had received external radiotherapy and 8 children were treated by chemotherapy. Twenty-one patients had undergone enucleation. Radiation-induced cataracts were found in 3 patients, radiation retinopathy in 1, enucleation and postradiotherapy contracted socket in 1, very low visual acuity postradiotherapy in 3, severe hypotelorism in 2, growth hormone deficiency in 2, neurocognitive disorders in 6, and orbital deformation due to radiation bone atrophy was moderate-severe in 12 patients. Azoospermia was found in 1 patient treated by cyclophosphamide and vincristine. The most frequent sequela in this group of RB-cured children were postradiotherapy orbital deformation due to bone atrophy and neurocognitive disabilities. Late radiation effects must be avoided by using modern, innovative, and more sophisticated radiotherapeutic techniques. Late treatment-related complications justify the long-term follow-up of childhood RB survivors.

The role of chemotherapy in childhood soft tissue sarcomas other than rhabdomyosarcomas: experience of the Northern Israel Oncology Center.

Forty-three children with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS) were treated at the Northern Israel Oncology Center in Haifa, Israel, from 1971 to 1996. The male:female ratio was 1.5:1 and the median age of patient was 10 years (range, 3 months-18 years). The most common histopathologic diagnoses were fibrosarcoma (32.5%) and synovial sarcoma (16%). The sites of primary tumor were lower limb (35%), trunk (18%), upper limb (16%), head and neck (16%), and retroperitoneum (11%). By Intergroup Rhabdomyosarcoma Study classifications, 13 patients presented as group I, 15 patients as group II, 10 patients as group III, and 5 patients as group IV. Median follow-up time was 63 months (range, 6 months-18 years). The estimated survival after a 5-year period is 72% (SE +/- 17) for patients in group I, 75 +/- 15% in group II, 90 +/- 9% for patients in group III, and 40 +/- 21% for patients in group IV. Eleven patients relapsed; 4/6 who developed local relapse were cured and are alive with disease, while 4/5 who developed distant metastases are dead. For the 28 patients who underwent complete resection at diagnosis, the estimated survival after a 5-year period is 87 +/- 5% vs. 60 +/- 17% for the 15 patients who underwent partial excision or biopsy. Local radiotherapy was delivered after surgery to group III patients. Preoperative and postoperative chemotherapy was delivered to the patients of groups III and IV, and postoperative chemotherapy only to group II patients. Chemotherapy produced demonstrable gain in survival for group II and III patients but not for patients with metastases. The authors conclude that an aggressive surgical approach is needed in patients with NRSTS. Chemotherapy may help as a preoperative treatment in bulky disease or as a postoperative treatment for microscopic residual disease.

Prognostic significance of DNA ploidy in childhood astrocytomas.

The ability to divide subsets of children with astrocytoma into prognostic groups is limited because only a few clinical and pathologic variables are available. This study evaluated DNA ploidy as a potential prognostic factor in 30 children with diagnosed gliomas and examined the correlation of flow cytometric analysis to other parameters such as sex, age at diagnosis, histologic grading, localization of tumor, and completeness of surgical resection. Seventeen children with low-grade glioma and 13 with high-grade glioma were retrospectively reviewed; mean age of the patients was 8.2 years, and mean follow-up of the population was 7.6 years. The tumor was localized to the cerebrum in 19 patients, the cerebellum in 7 patients, the brain stem in 3 patients, and the spine in 1 patient. Fourteen patients underwent complete excision and 16 patients underwent partial excision. DNA diploidy was demonstrated in 21 patients and aneuploidy in 9 patients. Twenty children had no evidence of disease and 10 died of disease. Of the patients with diploid tumors, 81% survived, compared to only 33% survival among patients with aneuploid tumors (p < .011). By Cox regression analysis with age, gender, type of excision, grade, location of tumor, and ploidy as independent variables, ploidy was a statistically significant predictor of survival (p = .043). This investigation provides further evidence that flow cytometry may have prognostic value in children with gliomas. Thus, a larger number of tumors can be studied to extend and validate these observations.

Wilms' tumor: the Northern Israel Oncology Institute experience.

Between 1971 and 1992, 42 children (median age = 2 years) with histologically proven Wilms' tumor were evaluated at the Northern Israel Oncology Institute. Most patients were staged according to National Wilms' Tumor Study (NWTS) criteria by which 18 were clinical stage I-II and 24 were III-IV. Combined therapy (surgery, chemotherapy, and radiotherapy) was given with a change to lower dose radiotherapy (< 2,000 cG) after 1981. The pre-1981 group (13 patients) suffered six relapses, most of which were pulmonary, whereas the 1981-1992 group (29 patients) had three relapses. The actuarial 5-year survival rate using Kaplan-Meier tables was 79% for the entire group but 100% for the 1981-1992 group and 38% for the 1971-1980 group. One case of immediate hepatic toxicity and one sudden death, unknown cause, of a patient in remission occurred in the group. Late deleterious effects were seen in five other patients; four had scoliosis and one developed osteoid osteoma of a rib. Despite these encouraging results, the necessity of ongoing monitoring for further long-term toxicities is apparent.