Treatment of stage IV-S neuroblastoma: a study of 34 cases treated between 1982 and 1987

Between January 1982 and December 1987, of 283 children with neuroblastoma treated in the Pediatric Department of Institut Gustave Roussy, 34 were stage IV-S. Two groups were distinguished according to the initial clinical presentation: 16/34 patients had life-threatening symptoms and needed immediate treatment, and 18/34 had no life-threatening symptoms. Hepatic irradiation was used in 12/16 patients of the first group with respiratory distress. Chemotherapy was used in three patients. Of these 16 patients, three patients died of progressive disease (one patient died of a reason not related to the tumor). Twelve are alive in first complete remission and one in second complete remission. Of the 18 patients of the second group, eight had spontaneous complete remission, and one of them relapsed thereafter and died despite treatment. Ten patients needed treatment, radiotherapy, and/or chemotherapy depending on the site of disease progression. Two of them died of the disease. The overall disease-free survival for these 34 patients is 75% at 90 months postdiagnosis. Using this therapeutic strategy, it was possible to avoid any treatment of metastases in 25% of the patients.     

Growth and endocrine disorders in optic glioma

Hypothalamo-pituitary function in children with optic glioma may be impaired by the tumour itself and by the high cranial radiation doses used in treatment. This study evaluates the effect of optic glioma and its treatment on patient growth and pubertal development. Twenty-one patients (13 boys, 8 girls), treated for optic glioma by cranial irradiation (45–55 Grays) at a mean age of 5.4 years, were evaluated before (n=10) and/or after (n=21) irradiation. Growth hormone (GH) deficiency was present in only 1 patient tested before irradiation and in all patients after irradiation. Precocious puberty occurred in 7/21 cases, before irradiation in 5 patients and after irradiation in 2 patients. The cumulative height loss during the 2 years after irradiation was 0.2±0.2 SD (m±SEM) in 7 patients with precocious puberty and 1.1±0.2 SD in 14 prepubertal patients (P<0.01). The corresponding bone age advance over chronological age, evaluated 1–3 years after irradiation, was 1.1±0.5 and –0.7±0.3 year in the two groups (P<0.01). The mean height loss between time of irradiation and the final height was 2.3±0.6 SD (n=6). Primary amenorrhoea, associated with low oestradiol levels, occurred in two of the three girls of pubertal age. These data indicate that the high dose of cranial radiation used to treat optic glioma invariably results in GH deficiency within 2 years and that hGH therapy is required when GH deficiency is documented. Precocious puberty, resulting in apparently normal growth velocity in spite of GH deficiency, should be treated with luteinizing hormone-releasing hormone analogues because of the risk of accelerated bone maturation and reduced final height.     

Imaging in pediatrics. Strategy and economic implications for the Third World]

The cost of acquiring medical imaging equipment is too high for many Third World nations. Ultrasonography and conventional roentegenography provide adequate data in most pediatric patients in tropical countries, for a low maintenance cost. Special training of practitioners in the use of these investigations is essential, before even performing the necessary choices among available equipment.     

A radio-endoscopic case: Barrett esophagus in children

The authors report a case of Barrett's oesophagus in a child. This observation is characterized by the extent of the lesion which is of three types of metaplasic epithelium usually seen in this disease (fundic, cardial and intestinal tissue); the difficulties in differential diagnosis from uncomplicated hiatal hernia justify radiological and endoscopical examinations with multiple biopsies. The discussion centers on the risk of malignant transformation with its therapeutical consequences and the long term follow-up of such patients.     

2nd malignant tumors in children. Study of 38 cases

Thirty-eight children, followed in the pediatric Department of Institut Gustave-Roussy, developed second malignant neoplasms. Intervals between the two neoplasms ranged from 1 to 26 years. The second neoplasms were defined as having a different histologic diagnosis than the first ones: osteosarcoma, fibrosarcoma, thyroid carcinoma, leukemia were the most frequent second neoplasms. The potential carcinogenic part of chemotherapy and radiotherapy is emphasized. In addition, some genetic susceptibility may enhance the carcinogenic effects of therapy. Nevertheless the incidence of second malignant neoplasms is low. Its estimation is discussed here.     

Radiotherapy followed by high dose busulfan and thiotepa: a prospective assessment of high dose chemotherapy in children with diffuse pontine gliomas

BACKGROUND: The role of high dose chemotherapy (HDC) in patients with pediatric brain tumors currently is ill-defined. The purpose of this pilot study was to assess the feasibility and the benefit of HDC after radiotherapy in a group of children with newly diagnosed diffuse pontine gliomas. METHODS: Patients eligible for study were ages 3-18 years with diffuse intrinsic tumors arising in the pons, who were not treated previously with radiotherapy or chemotherapy. Histologic confirmation was not mandatory, provided clinical findings and magnetic resonance imaging were typical. Patients were given focal radiotherapy followed 2-3 months later by HDC. Busulfan (150 mg/m(2) on Days 8, 7, 6, and 5) and thiotepa (300 mg/m(2) on Days 4, 3, and 2) were administered prior to autologous bone marrow transplantation. Survival was the endpoint, and the statistical procedure was based on sequential subgroup analysis. RESULTS: Thirty-six patients were entered on to the study, 12 of whom underwent stereotactic biopsy or open surgery at the time of diagnosis. One patient eventually was excluded due to inappropriate eligibility criteria. All 35 eligible patients received irradiation. Early progression (9 patients) and parental refusal (2 patients) precluded the use of HDC in 11 patients. Three patients died of HDC-related complications. All 21 patients who survived HDC eventually died of disease progression. The median survival time was 10 months for the study group. The median survival time in the subgroup of patients who received HDC was 10 months (range, 3-26 months). Statistical analysis did not suggest any evidence of survival benefit. CONCLUSIONS: For patients with diffuse pontine gliomas, survival using this aggressive treatment modality does not appear to be any better than that reported for conventional radiotherapy.