G. Kantor

A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases

BACKGROUND The management of desmoid tumours, previously based on strategies employed for sarcomas, should be reassessed, given the morbidity of interventions used in their treatment. METHODS Long-term follow-up (median 123 months) of a series of 106 treated patients with 69 primary and 37 recurrent desmoids, in order to study natural history and outcome. RESULTS Desmoids typically evolved actively over a median period of 3 years, and stabilised thereafter. Recurrences or progression most commonly occurred between 14 and 17 months. Risk factors for recurrence were presentation (primary vs. recurrent), gender, tumour location and resection margins. However, survival was independent from these factors, with equivalent survival whether resection had been performed or not. Tumour control and functional outcome depended on location and presentation. Functional impairment was proportional to number of operations and whether patients had received radiotherapy. Recurrences were observed in 12/23 patients after radiotherapy. CONCLUSION Desmoids are relatively indolent tumours needing different approaches than sarcomas. Direct surgery is advisable only in primary lower trunk wall/girdle locations. Wait-and-see and medical treatment is preferable in other types of presentations.

Surgical resection and radiotherapy for primary retroperitoneal soft tissue sarcoma

METHODS AND MATERIALS Forty-five patients were consecutively treated for primary retroperitoneal soft tissue sarcoma with surgery in combination with radiation therapy in the same institution. The median follow-up time was 53 months (7-108). RESULTS Seventeen (38%) patients had clear microscopic margins (R0 resection), 26 patients (58%) had gross complete surgical excision (R1 resection) and two patients (4%) had a macroscopic residual disease (R2 resection). External radiotherapy doses ranged from 40.8 to 59.4 Gy (mean and median: 49 Gy). Seventeen patients underwent intraoperative radiation therapy (IORT). Moreover, 11 patients received chemotherapy. The overall 1-, 2-, and 5-year survival for all 45 patients were 93, 85 and 60%, respectively. The 1-, 2-, and 5-year locoregional relapse-free rate for the whole group was 91, 70 and 40%, respectively. In univariate analysis, quality of surgery was the only variable to show a significant effect for overall survival (P=0.0386) and for local control (P=0.0059). Tumor size and tumor grade had no statistically significant effect. For the patients receiving IORT+external beam radiation therapy, no difference was observed for survival or locoregional control. The most frequent acute side effects treatment complications were radiation-induced nausea or vomiting (42%) and moderate enteritis (30%). Significant late morbidity was observed for two patients. CONCLUSIONS This study confirms the feasibility of external postoperative radiotherapy with an acceptable level of toxicity. However, the high rate of local relapses (especially in field of radiation) does not demonstrate the usefulness of radiotherapy at the level of dose used and further preferably randomized studies should be planned.

Tomothérapie hélicoïdale : aspects techniques et applications cliniques

Helical tomotherapy is an innovative device combining with the same linac on board-imaging and IMRT facilities. The first national French evaluations, supported by National Institut of Cancer (INCa) are presented. Dosimetric characteristics as quality of homogeneity, cut-off outside target volumes allow IMRT treatments for large and complex volumes and a good organ at risk sparing. First comparative dosimetric studies are discussed.

Role de la radiotherapie dans les affections benignes

Radiation therapy of benign diseases represent a wide panel of indications. Some indications are clearly identified as treatment of arteriovenous malformations (AVM), hyperthyroid ophtalmopathy, postoperative heterotopic bone formations or keloid scars. Some indications are under evaluation as complications induced by neo-vessels of age-related macular degeneration or coronary restenosis after angioplasty. Some indications remain controversial with poor evidence of efficiency as treatment of bursitis, tendinitis or Dupuytrens disease. Some indications are now obsolete such as warts, or contra-indicated as treatment of infant and children.

Dose de tolérance à l’irradiation des tissus sains : l’os chez l’adulte

Radiation tolerance for bone tissue has been mostly evaluated with regard to bone fracture. Main circumstances are mandibula osteoradionecrosis, hip and costal fracture, and patent or radiologic fractures in the treated volume. After radiation therapy of bone metastasis, the analysis of related radiation fracture is difficult to individualize from a pathologic fracture. Frequency of clinical fracture is less than 5% in the large series or cohorts and is probably underevaluated for the asymptomatic lesions. Women older than 50 years and with osteoporosis are probably the main population at risk. Dose-effect relations are difficult to qualify in older series. Recent models evaluating radiations toxicity on diaphysa suggest an important risk after 60 Gy, for high dose-fraction and for a large volume.

Expérience de l’institut Bergonié à propos de 14 cas de tomothérapie hélicoïdale de tumeurs axiales et para-axiales

PURPOSE To evaluate the feasibility of helical tomotherapy intensity-modulated radiotherapy for the treatment of axial and paraspinal tumours. PATIENTS AND METHODS Fourteen consecutive patients with axial and paraspinal tumours at the Bergonié Institute between June 2007 and June 2009 were analysed. Various localisations were observed: four paravertebral tumours, five primitive vertebral tumours and six iliosacral or sacral tumours. RESULTS The dose prescribed to the median of the planning target volume (PTV) varied from 45 to 68Gy depending on histology. The maximum dose delivered to the PTV (Dnear max or D2%) was 60.6Gy (range 46.3-69.1). In the five cases where the tumour was located close to the spinal cord, the median value of Dnear max was 40.1Gy (range 37.8-45.4). In the seven where the tumour was situated close to the cauda equina, the median value of Dnear max was 46.5Gy (range 39.2-60). For two cases in whom the sciatic nerve was the nervous structure at risk, the Dnear max was 64.4 and 67Gy. Six patients were in complete remission, six patients suffered a relapse at a median follow-up of 14.5 months (4-24), two died following local tumour progression and metastasis, tumoral stabilisation had been obtained in two patients. Treatment was well tolerated and was never interrupted in all patients. CONCLUSIONS Results from this preliminary series are encouraging, particularly concerning the good tolerance of this new intensity-modulated radiotherapy presently more available than proton therapy.

Prise en charge des sarcomes des tissus mous des membres par radiothérapie externe

Soft tissue sarcomas are rare tumours. Conservative surgery followed by postoperative radiation therapy represents the gold standard in the majority of cases. Postoperative radiotherapy improves local control without affecting survival. Besides the quality of surgical excision, which remains the major prognostic factor, the importance of the irradiation volume and particularly margins used in external beam radiotherapy were also found to influence local control of the disease. In this study, we propose to conduct a literature review on the present state of our knowledge on this subject in the form of an articulated controversy: in favour or opposed to large margins in external radiotherapy.

Histiocytose langerhansienne osseuse multifocale: place de la radiothérapie

Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100,000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology.

Chirurgie des sarcomes des tissus mous des membres et de la paroi du tronc@@@Surgery for soft tissue sarcoma in the extremities and trunk wall

RésuméEn 20 ans, le taux de contrôle local des sarcomes des tissus mous est passé d’environ 70 à 80 %, grâce entre autres aux progrès de la chirurgie. En dehors des centres spécialisés, la prise en charge des sarcomes reste encore insuffisante, en particulier au moment initial. Ce papier détaille les pièges à éviter au moment du diagnostic, comment procéder à la biopsie. La chirurgie d’exérèse est une chirurgie réglée, dont la place doit être discutée dans un cadre de prise en charge pluridisciplinaire. Une exérèse intempestive peut compromettre les chances de guérison. Elle est évaluée selon le système de la maladie résiduelle (R), qui aide à mieux adapter le traitement complémentaire.AbstractSurgical advances over the last twenty years have increased the rate of local control of soft tissue sarcoma from 70 to 80 %. However, the management of soft tissue sarcoma, especially initial treatment, remains inadequate outside specialized treatment centres. This paper discusses how to minimize diagnostic errors and perform biopsies. Soft tissue sarcoma surgery requires careful planning and must be integrated into a multidisciplinary therapeutic approach. A “whoops” excision can compromise a patient’s prognosis. The quality of surgery is best evaluated by the UICC residual disease (R) system, which helps to adapt complementary treatments.

Contours des volumes cibles en radiothérapie. Généralités

Resume Le contour des volumes cibles est une etape medicale dans la preparation des radiotherapies de conformation. Dans cet article de generalites sont discutees les difficultes de l’evaluation du volume tumoral macroscopique (GTV) et du volume cible anatomoclinique (CTV), la representation des differents volumes selon les recommandations du rapport ICRU 62, les principales discordances inter-observateurs lors de la definition des volumes.