Chantal Rodary

Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84

The second International Society of Paediatric Oncology (SIOP) study for rhabdomyosarcoma (MMT84) had several goals. The two principal aims were: (1) to improve the survival of children with rhabdomyosarcoma; and (2) to reduce the late effects from therapy by restricting the indications for surgery and/or radiotherapy after good response to initial chemotherapy. A further aim was to investigate the role of high-dose chemotherapy in young patients with parameningeal primary tumours. 186 previously untreated eligible patients entered the study. Patients with completely resected primary tumour received three courses of IVA (ifosfamide, vincristine and actinomycin D). Patients with incompletely resected tumour received six to 10 courses of IVA according to stage. Patients achieving complete remission with chemotherapy alone did not usually receive radiotherapy or undergo extensive surgery, but patients remaining in partial remission received local therapy with surgery and/or radiotherapy. Only patients over 5 years of age with parameningeal disease and patients over 12 years with tumours at any site were given systematic irradiation. Complete remission was achieved in 91% (170/186) of all patients. With a median follow-up of 8 years, the 5-year overall survival was 68% (+/- 3% standard error of the mean (SEM) and the 5-year event-free survival 53% (+/- 4% SEM). These results show an improvement over previous SIOP study (RMS75) in which survival was 52% and event-free survival was 47%. Among the 54 patients who exhibited isolated local relapse, 35% (19/54) survived in further remission longer than 2 years after retreatment, including local therapy (surgery +/- radiotherapy). Analysis of the overall burden of therapy received by all surviving children (including primary treatment and treatment for relapse if required) showed that 24% (28/116) were treated by limited surgery followed by three courses of IVA, 29% (34/116) were treated by chemotherapy alone (after initial biopsy) and 13% (15/116) received chemotherapy plus conservative local treatment (limited surgery or radiotherapy for residual disease). Only 34% (39/116) received intensive local therapy defined as radical wide field radiotherapy or radical surgery or both. Compared with the results obtained in the previous SIOP study, treatment in MMT84 was based on response to initial chemotherapy and, despite an overall reduction of the use of local therapy, significantly improved survival for patients with non-metastatic disease. This trial, also for the first time, provides evidence that retreatment after local relapse can achieve long-term second remissions.

The development of the DEGRR: A scale to assess pain in young children with cancer

The Gustave Roussy Child Pain Scale (Douleur Enfant Gustave Roussy, DEGRRScale) is a scale for grading prolonged pain in children aged 2–6 years with cancer. The scale comprised six behaviours specific to pain items, five psychomotor inertia items, and four anxiety items, with a total score ranging from 0 to 60. This work was designed to confirm the scale structure and to study its construct validity and convergent validity.

Primary chemotherapy in the treatment of rhabdomyosarcoma in children: trial of the International Society of Pediatric Oncology (SIOP) preliminary results.

This therapeutic trial deals with 81 patients with stage III rhabdomyosarcoma (RMS), from different centers of the International Society of Pediatric Oncology. These patients were included between October 1975 and March 1983. The aim of this work is to minimize the sequelae of treatment without jeopardizing the survival rate. After a pretrial course of VAC, two groups of patients are compared: the first treated with systematic extensive surgery or radiotherapy on the initial tumor volume, and the second treated with combined chemotherapy using vincristine, dactinomycin, adriamycin, cyclophosphamide until maximum tumor reduction, followed by radiotherapy or surgery on the residual mass only. Eighty one patients were included in the trail; 15 patients were not randomized due to the failure of the pretrial course of chemotherapy, 3 were excluded after randomization (2 were found not to have RMS, and in one case the protocol was not observed). The preliminary results for the 63 patients show a survival rate of 40% at 3 years. The sequential scheme for patients at 3 years follow-up does not show a superiority in either arm.

Quality of life of patients treated during childhood for a bone tumor: Assessment by the Child Health Questionnaire

To determine which factors impact on quality of life of patients with bone tumor, we used the Children Health Questionnaire French version. Thirty‐seven patients (25 males, 19 osteosarcoma, 18 Ewing sarcoma) were studied. At assessment, median age was 15 years, median follow‐up was 4 years. Mean scores were 60, 81, 76, 74, 70, 87 for general health, physical functioning, pain, mental health, self‐esteem, and family activity, respectively. Lower results were observed for mental health in girls, for physical functioning, and self‐esteem in patients with endoprosthesis, and for family activity and pain in patients who had relapsed.

La douleur chez l’enfant de 2 à 6 ans: mise au point d’une échelle d’évaluation utilisant l’observation du comportement

RésuméUne nouvelle échelle d’évaluation de la douleur de l’enfant de 2 à 6 ans, utilisant l’observation, a été élaborée dans une unité d’oncologie pédiatrique, à partir du recueil rétrospectif de descriptions d’enfants algiques. Des items d’atonie psychomotrice et d’anxiété ont été inclus dans l’échelle. L’échelle a été testée sur 80 enfants atteints de cancer, chaque enfant ayant été évalué par 2 infirmières et 2 auxiliaires de puériculture.La sensibilité et la fiabilité se sont révélées satisfaisantes. L’Analyse Factorielle des Correspondances a montré que le 1er facteur (51% de la variance) était déterminé à la fois par des items de douleur et d’atonie psychomotrice. Un second facteur (13% de la variance) oppose des items de douleur aux items d’anxiété.Ces résultats suggèrent que chez l’enfant de moins de 6 ans la douleur entraîne une atonie psychomotrice qui pourrait être proportionnelle à son intensité.SummaryThis study was designed to test a new rating observational scale for the diagnosis and grading of pain evoked by cancer in children of 2 to 6 years. We began by collecting retrospective clinical findings consisting of descriptions of children in pain. From these descriptions, an item scale was built up comprising overall patterns of behavior specific to pain. Because psychomotor atonia and anxiety items occured very frequently in the descriptions, they were included in the rating scale. The present preliminary report gives the results of the scale, which was tested in 80 children, chosen because they were liable to be in pain. Each child was evaluated by 2 nurses and 2 auxiliaries. Sensitivity and reproducibility were satisfactory. Factorial Correspondence Analysis showed that both psychomotor atonia and pain items contributed to the first axis, which accounted for 51% of the variance. A second axis was found, which contrasted anxiety and pain items, but it only accounted for 13% of the variance. These results suggest that in young children, pain evoked by severe disease leads to a depression-like reaction that correlates with that pain’s intensity.