Phase II study of rapid-scheduled etoposide in paediatric soft tissue sarcomas.

Twenty three patients with paediatric soft tissue sarcomas who had relapsed or refractory disease were treated with a rapid schedule of intravenous etoposide (100 mg/m2 daily on three consecutive days, weekly over 3 weeks). The regimen was well tolerated with predictable myelotoxicity. In 19 patients with rhabdomyosarcoma, there was a response rate of 42%. This appears to be better than previously reported with conventional three weekly schedules. These data indicate that for rhabdomyosarcoma, as for some other tumours, a divided dose regimen may be the optimal schedule and is worthy of further evaluation.

[Diagnosis of peripheral joint involvement in children]. / Diagnostic d'une atteinte articulaire périphérique chez l'enfant.

The diagnosis is assessed by a meticulous anamnesis, the research of associated clinical signs and the duration of the follow-up. If the clinical onset is recent (< 15 days), bacterial osteo-arthritis is to be treated in emergency; traumatic causes are easily advocated; reactive arthritis is infrequent. If the articular disease has been persistent or recurrent for 15 days to 3 months, and because of the urgency of the treatment, it should be unacceptable not to recognize a leukaemia or a metastatic neuroblastoma; the same is true for bacterial origins and some of the vascular alterations of growth cartilage. Reactive arthritis, frequently due to a digestive infection, has also to be considered. The localised articular lesions of traumatic or malformative origin, may be diagnosed by magnetic resonance imaging and arthroscopy. When the duration of the articular disease exceeds 3 months, the diagnosis of juvenile chronic arthritis, spondylarthropathy and connectivitis (disseminated lupus, panarteritis, dermatomyositis) or of some rare systemic diseases (Behçet, sarcoidosis, periodic disease) has to be considered. The non somatic and/or psychogenic causes must be accepted only if any organic disease has been excluded. The diagnosis of the so-called "growth pains" remains too often a medical error.

[Rehabilitation of children with chronic inflammatory arthritis]. / Rééducation de l'enfant atteint d'arthrite inflammatoire chronique.

All children with chronic arthritis require rehabilitation. Indications of the various techniques are mainly dependent upon the stage of the disease. The choice of the most appropriate technique (preservation of articular mobility, preservation of the muscular strength, orthoses) are based on an accurate assessment of each joint and on imaging. Specific measures are indicated for each localisation. The handicaps resulting from the disease and from its treatments (corticosteroids) should be reduced by adequate information of the family, adaptation of the living environment, and technical helps.

[Bone complications from chronic etretinate intoxication in children]. / Complications osseuses de l'intoxication chronique par l'étrétinate chez l'enfant.

Clinical, roentgenographic and biologic features of etretinate bone toxicity in a 13-year-old girl with pachyonychia congenita syndrome are reported. Etretinate is a synthetic derivative of vitamin A that infrequently induces bone and joint abnormalities in children. The following manifestations can be observed: cortical hyperostosis, pain, calcification of tendons, thinning of long bones, demineralization, premature closure of epiphyses, or abnormal remodelling. Onset of these anomalies is often delayed since etretinate has a long half-life. Mechanisms are unknown. We advocate use of the minimum effective dosage and regular monitoring of patients.

[Registries of cancer in children in France]. / Les registres des cancers de l'enfant en France.

The specificity of childhood cancers and the lack of available incidence data in Europe (with the exception of the Manchester and Turin registries) led to the creation of two French regional childhood cancer registries (CCR), in Nancy (1983) and Marseille (1984); both are population based CCR, respectively covering 535,236 and 809,196 children (0 to 14 years). All malignant neoplasms, brain tumours (whatever the grading) and borderline malignancies are included. Data have been collected from medical and administrative sources. Registration is active and each source is recontacted annually. The registries contact all physicians who might include children among their patients (private and hospital practice) and pathology-cytology laboratories. The University Hospital Centers and Anti-Cancer Centers in adjacent regions and in Paris are contacted. Data collected are the following: name, age, sex, address, time and method of diagnosis, histology, anatomical site, stage, treatment and sources of information. We combined our own data with those of a general cancer registry, set up in Strasbourg in 1975 and covering 205,889 children. The reliability of the methodology is attested by the similarity of results to those obtained in other European, US and Australian CCR. This type of registry is needed for organizing studies on the descriptive, analytical and experimental epidemiology in pediatric oncology.

Juvenile hyaline fibromatosis: a report of two severe cases.

Two unrelated children with a severe form of juvenile hyaline fibromatosis are described. In addition to painful flexion contractures of all of the large joints, oral and skin lesions, and typical radiologic appearance of osteolytic defects, both girls had marked growth retardation and recurrent infections. Both children died in early infancy of overwhelming infection.

Phase II study of ifosfamide in childhood brain tumors: a report by the French Society of Pediatric Oncology (SFOP).

Forty-two evaluable pediatric patients with a variety of recurrent primary brain tumors participated in a phase II ifosfamide trial. Their mean age was 10 years. All patients were treated with ifosfamide, 3 g/m2/day for 2 days every 2 weeks. Response was assessed on clinical and radiological criteria after at least 2 courses of therapy. The overall response rate was 12% (5/42). One complete and 2 partial responses were documented in 21 patients with medulloblastoma. A partial response was demonstrated in 1 patient with primitive neurectodermal tumor (PNET) and in 1 patient with ependymoma. No activity was observed in astrocytic tumors. Toxicity was primarily neurologic (16 out of 54 patients, 30%). Hematological toxicity, without severe morbidity, was encountered in 9% of courses (16/179). Ifosfamide, administered at this dose regimen has modest efficacy in the treatment of recurrent childhood medulloblastoma and ependymoma and appears inactive for gliomas. Further trials with other dose schedules are necessary to assess the activity of this drug. However, according to the neurotoxicity observed in our trial, we would not recommend building a protocol using ifosfamide for highly progressive brain tumors.