Neuroblastoma with symptomatic spinal cord compression at diagnosis: treatment and results with 76 cases.

PURPOSE: To report on the treatment of patients with newly diagnosed neuroblastoma presenting with spinal cord compression (SCC). PATIENTS AND METHODS: Of 1,462 children with neuroblastoma registered between 1979 and 1998, 76 (5.2%) presented with signs/symptoms of SCC, including motor deficit in 75 patients (mild in 43, moderate in 22, severe [ie, paraplegia] in 10), pain in 47, sphincteric deficit in 30, and sensory loss in 11. Treatment of SCC consisted of radiotherapy in 11 patients, laminectomy in 32, and chemotherapy in 33. Laminectomy was more frequently performed in cases with favorable disease stages and in those with severe motor deficit, whereas chemotherapy was preferred in patients with advanced disease. RESULTS: Thirty-three patients achieved full neurologic recovery, 14 improved, 22 remained stable, and eight worsened, including three who become paraplegic. None of the 10 patients with grade 3 motor deficit, eight of whom were treated by laminectomy, recovered or improved. In the other 66 patients, the neurologic response to treatment was comparable for the three therapeutic modalities. All 11 patients treated by radiotherapy and 26 of 32 patients treated by laminectomy, but only two of 33 treated by chemotherapy, received additional therapy for SCC. Fifty-four of 76 patients are alive at time of the analysis, with follow-up of 4 to 209 months (median, 139 months). Twenty-six (44%) of 54 survivors have late sequelae, mainly scoliosis and sphincteric deficit. CONCLUSION: Radiotherapy, laminectomy, and chemotherapy showed comparable ability to relieve or improve SCC. However, patients treated with chemotherapy usually did not require additional therapy, whereas patients treated either with radiotherapy or laminectomy commonly did. No patient presenting with (or developing) severe motor deficit recovered or improved. Sequelae were documented in 44% of surviving patients.

Behavior of a cell line derived from a mouse submaxillary adenocarcinoma during the initial 480 days in vitro.

A cell line was established from a transplantable adenocarcinoma, containing viral particles of the A and B type, drived from a tumor appearing spontaneously in the submaxillary region of a male mouse of the C3H/He strain. This line, after 480 days in vitro, did not change the original epithelial-like morphology, the viral expression, the membrane immunofluorescence and the degree of agglutination by various plant lectins. After 208 days of culture, the presence of up to 3 pairs of metacentric chromosomes appeared in about 55% of the cells. However, this change in the chromosomal pattern was not sufficient, at least within the limits of our observation, to modify significantly the other parameters investigated, with the possible exception of the oncogenicity, which showed a modest decrease after 296 days of culture.

Mycophenolate mofetil in primary glomerulopathies.

Mycophenolate mofetil is an immunosuppressive agent that blocks purine biosynthesis, inhibits T and B-lymphocyte and mesangial proliferation. Mycophenolate mofetil is not nephrotoxic like calcineurin inhibitors and is widely used in solid-organ transplantation. Recently, mycophenolate mofetil has been introduced in the treatment of autoimmune diseases and primary glomerulopathies. This review analyzes the literature currently available on the treatment of primary glomerulopathies with mycophenolate mofetil. Encouraging results have been obtained in minimal change nephropathy where it may help to reduce the use of steroids in these patients who are often very young. The results obtained in medium and high risk patients with focal segmental glomerulonephritis and idiopathic membranous nephropathy were less encouraging. Conflicting results have been reported on IgA nephropathy in controlled trials. None of these studies attained level A evidence, meaning that randomized control trials of sufficient statistical significance are necessary to estimate the real effectiveness of mycophenolate mofetil in primary glomerulopathies.

Prepubertal endocrine follow-up in subjects with Wilms' tumor.

Twenty-three prepubertal subjects treated for Wilms' tumor (10 males and 13 females) were endocrinologically evaluated off therapy from 0.5 to 4.08 years. They were divided into two groups: 11 subjects (6M, 5F) who had received chemotherapy only (group 1) and 12 (4M, 8F) who had in addition received abdominal radiation (1,500-3,000 rads) (group 2). Follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin (PRL), thyroid-stimulating hormone (TSH), free thyroxine (FT4), free tri-iodo thyronine (FT3), testosterone (T), estradiol-17 beta (E2), and cortisol (F) were measured by radioimmunoassay (RIA). Plasma levels of TSH, PRL, FT4, FT3, and F were normal in both groups, as were FSH, LH, T, and E2 in group 1. In group 2, female subjects showed FSH levels significantly higher than controls, while LH and E2 were normal; male subjects showed significantly higher LH levels, while FSH and T levels were normal. These results indicate that in the treatment protocol used by us for Wilms' tumor (WT), chemotherapy does not affect endocrine function, whereas abdominal radiation seems to damage gonadal function directly. The present findings indicate that gonadal damage may be revealed in WT before puberty not only in females, as has been previously reported, but also in male subjects.