Evaluation of the French medical practices in endometrial cancer management by using quality indicators.

STUDY OBJECTIVE: To evaluate the French quality of care of endometrial cancer using published relevant quality indicators and researche explanatory factors influencing its management. STUDY DESIGN: We used databases from the "Echantillon Généraliste de Bénéficiaires", sample of the French population, to identify cases operated on between 2005-2014. Quality indicators evaluated were: three years survival rate, time between surgery and adjuvant treatment, use of IRMT and 3DCRT for radiotherapy, rate of minimally invasive surgery. Multilevel analysis was performed to identify explanatory factors. RESULTS: 405 women were included. 323 had a follow-up of more three years, and 250 were alive at three years (77.0%). 70 (17.3%) underwent minimally invasive surgery. 73 women of the 153 who received adjuvant treatment (47.8%) started it within 60 days after surgery. Among the 60 patients who received adjuvant radiotherapy, 50 (83.3%) underwent the IRMT or 3DCRT technique. In multilevel analysis, diabetes (OR = 1.24: 95% CI [1.08; 1.41]), and age under 65 (OR = 1.15: 95% CI [1.01; 1.35]) were associated with an increase of the three years survival rate. Lymphadenectomy (OR = 1.12: 95% CI [1.03; 1.22]), and management in an university institution (OR = 1.13: 95% CI [1.01; 1.25]) were associated with adequate technique for the adjuvant radiotherapy. Laparoscopic surgery increased after 2010 (OR = 1.18: 95% CI [1,10; 1,25]). CONCLUSION: Improvements should be made to increase the rate of laparoscopy and reduce the delay before adjuvant treatment for the management of endometrial cancer in France, specifically in non-university centers.

[Gated radiotherapy in breast cancer]. / Asservissement respiratoire en radiothérapie mammaire.

Postoperative radiotherapy is a cornerstone of the local treatment in breast cancer. It has been proved with high level of evidence that it decreases local relapse and improves survival of patients. However, radiotherapy comes with healthy tissue toxicity, heart and lung in particular. With constant improvement of radiation techniques, several methods have been developed to decrease the dose to the heart and the lungs. Sometimes, respiratory maneuvers can help, due to patient's anatomy: the radiotherapy is gated with patient's breath. The Deep Inspiration Breath Hold technique is the most popular and there are several ways to perform it. This note will describe the different systems with published data in order to help the radiation oncologist in the daily practice.

[Intensity modulated radiotherapy as adjuvant post-operative treatment for retroperitoneal sarcoma: acute toxicity]. / Radiothérapie avec modulation d'intensité dans le traitement postopératoire des sarcomes rétropéritonéaux: profil de toxicité aiguë.

PURPOSE: To assess the acute toxicity of intensity modulated radiotherapy as post-operative adjuvant treatment for retroperitoneal sarcoma. PATIENTS AND METHODS: Patients who received adjuvant intensity modulated radiotherapy from January 2009 to September 2010 were retrospectively reviewed. RESULTS: Fourteen patients entered the study (seven primary tumours and seven relapses). All tumours were liposarcoma and had macroscopically complete resection, epiploplasty was systematically realized. Median tumour size was 21 cm (range: 15-45), median planning target volume was 580 cm(3) (range: 329-1172) and median prescribed dose was 50.4 Gy (range: 45-54). Median follow-up was 11.5 months (range: 2-21.4). Acute toxicity was mild: acute digestive toxicity grade 1-2 occurred in 12/14 patients (86%). However, there was no weight loss of more than 5% during radiotherapy and no treatment interruption was required. Two months after completion of radiotherapy, digestive toxicity grade 1 remained present in 1/14 patients (7%). One case of grade 3 toxicity occurred during follow-up (transient abdominal pain). Three relapses occurred: two were outside treaded volume and one was both in and outside treated volume. CONCLUSIONS: Intensity modulated radiotherapy in the postoperative setting of retroperitoneal sarcoma provides low acute toxicity. Longer follow-up is needed to assess late toxicity, especially for bowel, kidney and radio-induced malignancies.

[What is new in the local approach of limb sarcomas and desmoid tumours?]. / Actualisation de la stratégie thérapeutique locorégionale dans les sarcomes des tissus mous et les tumeurs desmoïdes des membres.

The treatment of soft tissue sarcomas of limbs should be discussed within an experienced multimodality team. Surgical resection remains the cornerstone of therapy for localized disease and achieves a five years overall survival around 75% and a local recurrence rate as low as 10% in the best series. In complex cases, neo-adjuvant treatments may be used such as systemic chemotherapy, isolated limb perfusion, or radiotherapy to achieve an optimal conservative approach. Molecular genetics of sarcomas and quality of margins are essential to guide diagnosis and therapeutic selection. In case of marginal or incomplete resection, a new enlarged surgical resection should always be discussed before administration of any adjuvant treatments. Many retrospective studies and two randomized studies (one of adjuvant brachytherapy and one of external beam radiotherapy) have shown that adjuvant radiotherapy after complete surgery significantly reduces the risk of local recurrence in extremity soft tissue sarcomas. A randomized study has compared pre- to postoperative radiotherapy. The results in terms of local control are similar in both arms. The risk of surgical complications is higher in the preoperative arm and the risk of late sequelae is higher in the postoperative arm. A randomized study within the French sarcoma group is ongoing evaluating the omission of postoperative radiotherapy in favourable cases. Presently, the role of systematic first-line invasive treatment (including surgery and/or radiotherapy) of desmoids is debated. It is becoming evident that up to 50% of patients with desmoids benefit from a front-line non-aggressive policy, because growth arrest is a common feature of this disease. Additional study of the molecular determinants of desmoid behaviour is needed to guide treatment.

[The role of radiotherapy in the management of patients with small cell lung cancer]. / Place de la radiothérapie dans la prise en charge des carcinomes bronchiques à petites cellules localisés.

Small cell lung carcinomas are aggressive on account of their high and early risk of dissemination. They represent less than 20% of all lung cancers and only a third of these present with limited stage disease at diagnosis. Currently, treatment is based on synchronous thoracic irradiation and chemotherapy combining platinum salts and etoposide with or without other drugs. Because of the high risk of brain metastases, prophylactic cranial irradiation (PCI) is indicated in patients with a complete response and should be part of the standard management of these patients on the basis of a meta-analysis showing a 5% increase in survival at three years. In limited stage disease 5 year survival rates can reach 25% but the majority of patients will relapse. This progress is the consequence of a better combination of thoracic and cerebral irradiation and polychemotherapy. Even in extensive disease PCI reduces the risk of brain metastases and significantly improves overall survival. Many issues are subject to further clinical research concerning modalities of combination radio-chemotherapy, radiotherapy target volumes, optimum dosage, and the use of drugs in association with irradiation.

[Lung cancer: is there a place for elective nodal irradiation?]. / Cancers bronchiques : la radiothérapie prophylactique des aires ganglionnaires a-t-elle encore une place ?

The use of conformal radiotherapy in lung cancer has considerably evolved with the advent of improved staging technologies and methods of radiation delivery. Patients with limited disease, inoperable for medical reasons, may be treated with conformal radiotherapy alone; patients with more advanced disease are treated with combined chemo-radiotherapy. If local control may be improved by radiotherapy dose escalation according to several studies, toxicity and more particularly pulmonary toxicity seems to be related to radiation volume. Thus the use of elective nodal irradiation is being questioned. Data for early stage (stage I) non-small-cell lung cancer treated with conformal radiotherapy or stereotactic hypofractionated radiotherapy strongly supports the use of smaller fields that do not incorporate elective nodal regions; local control and survival rates approach those of surgical series. In locally advanced non-small cell lung cancer, eliminating elective nodal irradiation allows to maximize tumor dose and minimize normal tissue toxicity in combined modality treatments; results are encouraging. The use of staging modalities such as positron emission tomography and eventually oesophageal ultrasonography is increasing, allowing to encompass the tumor volume with more accuracy. Several studies have confirmed that involved-field irradiation results into a regional nodal rate of less than 10%. Further larger-scale studies would be needed to definitely establish "no elective nodal irradiation" as a standard in non-small cell lung cancer. There are very few data concerning small cell lung cancer.

[Small cell lung cancer (CPC). Management of patients presenting with limited stage small cell carcinoma of the lung]. / Les cancers à petites cellules. Modalités actuelles de la prise en charge des patients présentant un carcinome bronchique à petites cellules localisé.

Small cell carcinomas represent less than 20% of all lung cancer. Only a third of these patients present with limited stage disease. Treatment is based on a combination of chemotherapy and radiotherapy including a platinum salt with or without another drug. Conformational thoracic radiotherapy is administered either classically fractionated or in an accelerated form. Prophylactic cranial irradiation is indicated in patients with a good response. An improvement in outcomes has been obtained with this regimen. Even in limited stage disease 5 year survival remains about 25% in the best series. The majority of patients will relapse and the risk of cerebral metastases is particularly high, reaching nearly 50% at 2 years even in patients with a complete response. Prophylactic cranial irradiation should be part of the standard management of patients with a complete response on the basis of a meta analysis showing a 5% increase in survival at three years. Even though combination treatments have improved survival, a number of questions remain that should stimulate further clinical trials to establish the optimum regimes of chemotherapy and radiotherapy and the optimum strategies for combining the two. In addition the potential role of targeted therapy in selected patients must be examined.

[Thymic tumors]. / Tumeurs épithéliales thymiques.

Thymomas and thymic carcinomas are rare and slow-growing tumors, which develop within the anterior mediastinum. Thymomas are often associated with autoimmune disorders and most particularly myasthenia gravis. The treatment of choice remains a complete surgical resection. Postoperative radiotherapy is often combined in case of invasive thymoma invading into adjacent organs. Postoperative radiotherapy in stage II with invasion into capsule has been more controversial lately. In inoperable locally advanced, or metastatic thymic tumors, neoadjuvant cisplatin-based followed by surgery and radiotherapy has given interesting results in the past years.

Síndrome nefrótico congénito y síndrome de down: reporte de un caso y revisión de la literatura / Congenital nephrotic syndrome and down syndrome: report of case and literature revision

El síndrome nefrótico congénito durante el primer año de vida es poco frecuente en nuestro medio, al igual que en otros países de latinoamérica; lo contrario ocurre en otros países europeos donde la prevalencia es mayor: Se ha encontrado asociaciones de esta síndrome con alteraciones embrionarias como el nefroblastoma, anormalidades genitales, seudohermafroditismo, síndrome uñarótula y displasia congénita de la cadera. Se describe el caso clínico de un lactante menor femenina de 6 meses de edad con síndrome nefrótico congénito y síndrome de down; esta asociación no ha sido descrita previamente en la literatura nacional. El estudio histopatológico mostró disminución en el número de glomerulos y la lesión observada en los gromérulos fue una esclerosis mesangial difusa retráctil

[Familial juvenile nephronopthisis]. / Nefronoptisis juvenil familiar.

For the last 20 years, 11 patients with clinicopathologic diagnosis of nephronopththisis or medullary cystic disease, have been studied at the Nephrology Department of the Hospital Infantil de México. The initial manifestations took place at ages from 3 to 12 years and consisted of polyuria and polydipsia with reduction of vasopressin resistant urinary concentration capacity. Most cases showed anemia and pondostatural delay even at periods previous to renal insufficiency initiation. All cases showed normal urinary sediment with persistently negative urine cultures and progressed to chronic renal insufficiency after 1 to 10 years of evolution. Eight cases showed familial character. Histopathologic findings were similar and consisted of tubular disorders with alternation of atrophic tubuli having thickened basal and dilated tubuli having normal or flattened epithelium; renal medullary cysts were shown in some cases.