Oncologic outcomes, prognostic factor analysis and therapeutic algorithm evaluation of head and neck mucosal melanomas in France.

BACKGROUND: Head and neck mucosal melanoma (HNMM) is aggressive and rare, with a poor prognosis because of its high metastatic potential. The two main subtypes are sinonasal (sinonasal mucosal melanoma [SNMM]) and oral cavity (oral cavity mucosal melanoma [OCMM]). Consensual therapeutic guidelines considering the primary tumour site and tumour-node-metastasis (TNM) stage are not well established. MATERIAL & METHODS: Patients with HNMM from the prospective national French Rare Head and Neck Cancer Expert Network database between 2000 and 2017 were included. Clinical characteristics, treatment modalities, outcomes and prognostic factors were analysed. RESULTS: In total, 314 patients were included. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 49.4% and 24.7%, respectively, in the surgery group; no long-term survivors were observed when surgery was not feasible. Moreover, even after surgery, a high recurrence rate was reported with a median PFS of 22 months. In multivariate analysis, Union for International Cancer Control (UICC) stage and tumour site correlated with PFS and OS. Postoperative radiotherapy (PORT) improved the PFS but not OS in patients with small (T3) SNMM and OCMM tumours. Nodal involvement was more frequent in patients with OCMM (p < 10-4), although, as in SNMM, it was not a significant prognostic predictor. CONCLUSION: Even early HNMM was associated with poor oncologic outcomes due to distant metastases despite surgical resection with clear margins. Lymph node metastases had no impact on the prognosis, suggesting treatment de-escalation in cervical node management. PORT might be useful for local control.

[Case report: Vasa previa diagnosis and management. How to prevent Benckiser's hemorrhage?]. / Cas clinique : diagnostic et prise en charge d'un vasa praevia. Comment prévenir l'hémorragie de Benckiser ?

Benckiser's haemorrhage is a serious obstetrical complication, following a vasa previa rupture. Incidence of vasa previa is estimated between 1/1150 and 1/5000 pregnancies. This case report illustrates the consequences of a suspected vasa previa rupture. There is no French recommendation of how to treat vasa previa. Different methods of prevention are described and examined thanks to a literature review.

Asteroseismology. Echography of young stars reveals their evolution.

We demonstrate that a seismic analysis of stars in their earliest evolutionary phases is a powerful method with which to identify young stars and distinguish their evolutionary states. The early star that is born from the gravitational collapse of a molecular cloud reaches at some point sufficient temperature, mass, and luminosity to be detected. Accretion stops, and the pre-main sequence star that emerges is nearly fully convective and chemically homogeneous. It will continue to contract gravitationally until the density and temperature in the core are high enough to start nuclear burning of hydrogen. We show that there is a relationship for a sample of young stars between detected pulsation properties and their evolutionary status, illustrating the potential of asteroseismology for the early evolutionary phases.

[Target volumes in cervical lympadenopathies of unknown primary: toward a selective customized approach? On behalf of REFCOR]. / Volumes cibles en radiothérapie des adénopathies cervicales de primitif inconnu : état des lieux et pistes vers une approche sélective raisonnée sous l'égide du Refcor (Réseau d'expertise français des cancers ORL rares).

The treatment of carcinomas of unknown primary revealed by cervical lymphadenopathy is based on neck dissection and nodal and pan-mucosal irradiation to control the neck and avoid the emergence of a metachronous primary. The aim of this review was to assess diagnostic and therapeutic approaches and criteria that may be used for a customized selective approach to avoid severe toxicities of pan-mucosal irradiation. A literature search was performed with the following keywords: cervical lymphadenopathy, unknown primary, upper aerodigestive tract, cancer, radiotherapy, squamous cell carcinoma, variants. The diagnostic workup includes a head and neck scanner or MRI, ((18)F)-FDG PET CT, a panendoscopy and tonsillectomy. Squamous cell carcinoma represents over two thirds of cases. The number of metastatic cervical nodes, nodal level, and histological variant (associated with HPV/EBV status) may determine the primary site origin and might be weighted for the determination of radiation target volumes on a multidisciplinary basis. A selective customized approach is relevant to decrease radiation toxicity only if neck and mucosal control is not impaired. Although no recommendation can yet be made in the absence of sufficient level of evidence, the relevance of systematic pan-mucosal irradiation appears questionable in a number of clinical situations. Accordingly, a customized selective redefinition of target volumes may be discussed and be prospectively evaluated in relation to the therapeutic index obtained.

[An ovarian mucinous borderline tumour with mixed mural nodules]. / Une tumeur ovarienne mucineuse borderline avec des nodules muraux mixtes.

The occurrence of mural nodules in serous or mucinous ovarian tumours is not frequent. Mural nodule can be developed in benign, borderline or malignant tumours. They can be benign, malignant or mixed type. Thus the prognosis of the ovarian tumour can be dramatically modified by the presence if these nodules. Eighty-two cases of mural nodules were reported in the literature, among which we account four cases of mixed nodules type. We report an additional case of mixed type mural nodules of anaplastic carcinoma and sarcoma-like developed in an ovarian mucinous borderline tumour at a 60-year-old woman.We give details about the classification, the differential diagnosis and prognosis of theses nodules.

[Oncogenic osteomalacia: increased production of fibroblast growth factor 23 is not the unique actor]. / Ostéomalacie oncogénique : le FGF 23 ne fait pas tout.

The importance of fibroblast growth factor 23 (FGF 23) has been highlighted in the mechanism of urinary leakage of phosphate in the oncogenic osteomalacia (OO). It is now a component of diagnosis of this disease. We report a 58-year-old man who presented with osteomalacia and hypophosphatemia secondary to urinary leakage of phosphorus. Although serum FGF 23 was normal, the diagnosis of OO was obtained after another cause of acquired prolonged hypophosphatemia has been excluded (hyperparathyroidism and Fanconi syndrome in particular). The search for a deep tumor was performed, allowing the detection of a 12 mm hemangiopericytoma in the upper thigh. Its removal allowed the rapid resolution of clinical symptoms and laboratory abnormalities. The importance of functional sequelae in OO depends on prompt diagnosis. Tumorectomy remains the optimal treatment. Thus, the search for a secreting tumor is essential even in the absence of elevated serum FGF 23.

Mandibular osteosclerotic lesion of a parotid salivary duct carcinoma: demonstration of the neural tropism of these tumors.

INTRODUCTION: Salivary duct carcinoma (SDC) is an uncommon entity of salivary gland cancers with a poor prognosis due to local aggressiveness or distant recurrences involving lymph nodes, lung, and long bones, in which secondary lesions are usually osteolytic. The authors report the first case of mandibular SDC, atypical due to its osteosclerotic presentation and its site, attributed to aggressive neural spread of the tumor along the trigeminal nerve. CASE STUDY: This asymptomatic osteosclerotic bone involvement was diagnosed based on pathological enhancement of the trigeminal nerve demonstrated on MRI and was accompanied by facial nerve involvement up to its third intracranial portion. Radical surgery ensured disease control with continued good quality of life at the 4-year follow-up visit. CONCLUSION: Nerve enhancement on MRI and determination of specific tumor markers (HER-2/neu and p53) should be taken into account to evaluate the prognosis of SDC and to propose appropriate surgical treatment.

A transiting giant planet with a temperature between 250 K and 430 K.

Of the over 400 known exoplanets, there are about 70 planets that transit their central star, a situation that permits the derivation of their basic parameters and facilitates investigations of their atmospheres. Some short-period planets, including the first terrestrial exoplanet (CoRoT-7b), have been discovered using a space mission designed to find smaller and more distant planets than can be seen from the ground. Here we report transit observations of CoRoT-9b, which orbits with a period of 95.274 days on a low eccentricity of 0.11 +/- 0.04 around a solar-like star. Its periastron distance of 0.36 astronomical units is by far the largest of all transiting planets, yielding a 'temperate' photospheric temperature estimated to be between 250 and 430 K. Unlike previously known transiting planets, the present size of CoRoT-9b should not have been affected by tidal heat dissipation processes. Indeed, the planet is found to be well described by standard evolution models with an inferred interior composition consistent with that of Jupiter and Saturn.

[Management of cytomegalovirus infections in patients treated with immunosuppressive drugs for chronic inflammatory diseases]. / Prise en charge de l'infection à cytomégalovirus symptomatique chez les patients traités par immunosuppresseurs pour une maladie inflammatoire chronique.

INTRODUCTION: For patients with chronic inflammatory disease treated by immunosuppressive agents (for example: rheumatoid arthritis or systemic lupus erythematosus), there are no available guidelines in medical literature on the use of antiviral agents for the management of symptomatic cytomegalovirus (CMV) infection. EXEGESIS: A patient treated by methotrexate for a spondylarthritis presented a CMV infection manifested with persistent fever and pneumonia. CMV pp65 antigenemia was of 120 positive nuclei for 100,000 cells. Treatment with valganciclovir allowed a prompt recovery, while treatment by methotrexate was maintained. CONCLUSION: Symptomatic CMV infection evolution is unpredictable and potentially severe in patients with chronic inflammatory diseases receiving immunosuppressive agents. Although there is no data issued from clinical trials, the observation reported in this article and the publications of similar cases in the medical literature indicate that treatment with valganciclovir seems worth to be used in this context. Stopping immunosuppressive therapy does not seem mandatory.

[Hemoglobin targets in anemic patients with chronic kidney disease treated with erythropoietin]. / Erythropoïétine et insuffisance rénale: jusqu'où ne pas aller?

PURPOSE: Erythropoietin is the treatment of the anaemia in chronic kidney disease. A target rate of haemoglobin higher than 11 g/dl was usually proposed, but recent recommendations stated that higher limit of haemoglobin was to be reached, with the aim to improve the quality of life of the patients and to reduce their risks of cardiovascular diseases. These objectives are to be revised, according to the results of recently published clinical trials. CURRENT KNOWLEDGE AND KEY POINTS: Patients treated to reach a high rate of haemoglobin (between 13 and 14,5 g/dl) have an improved quality of life, but a 30% higher mortality rate, compared to patients treated with a lower objective of haemoglobin rate (10-12 g/dl). Hypertension and vascular access thromboses were also more frequent in the patients with the highest haemoglobin rate. Two to three times more erythropoietin was necessary to reach the higher rate of haemoglobin. These results favour a target rate of haemoglobin not higher than 12 g/dl. A polemic followed the results of these clinical trials, mostly in the United States, questioning the way in which the higher limit had been fixed whereas precise data were unavailable. The role of pharmaceutical industry and of for profit dialysis centres was underlined. FUTURE PROSPECTS AND PROJECTS: The next step is now to explain if the excess in cardiovascular morbimortality is related to the haemoglobin rate or to a direct effect of the erythropoietin. Such an understanding is important, the more so as new erythropoiesis-stimulating agents are being developed.

[Hypokalaemia and paralysis: think about the thyroid]. / Hypokaliémie et paralysie: penser à la thyroïde.

Periodic hypokalemic paralysis can be of genetic origin or secondary to other causes of hypokalaemia. The thyreotoxic hypokalemic periodic paralysis (THPP) usually occurs among asian subjects. It is a diagnostic and therapeutic emergency which may lead to life-threatening complications due to hypokalaemia and muscle weakness. The potassium supplementation is followed by a complete recovery after a few hours. We underlined the interest of thyroid assays in patients having an acute muscular paralysis associated with hypokalaemia.

[Systemic corticosteroid treatment and risk of infectious diseases]. / Corticothérapie systémique et risque infectieux.

PURPOSE: It was shown that corticosteroids alter the inflammatory and immune responses. Many publications report on serious infections occurring in patients receiving corticosteroids or presenting with Cushing's syndrome. This information is synthesized in this article. CURRENT KNOWLEDGE AND KEY POINTS: The demonstration of the infectious risk associated with corticosteroids relies on observational data and on biological plausibility. However, this risk remains difficult to quantify, because of many confusing factors such as the patients' associated conditions and immunosuppressive treatments, and the highly variable dose and duration of the corticosteroid treatment. Taking into account the published data, the screening for a chronic infection seems licit among patients receiving a systemic corticosteroid treatment, in particular for those who will receive more than 10 mg of prednisone per day. FUTURE PROSPECTS: Although no clinical trials of prevention of infections in corticosteroid treated patients has been published, a strategy aiming at minimizing the infectious risk of corticosteroid treated patients is proposed, based on the analysis of the literature presented in this article.

[Is there an evidence-based management of idiopathic retroperitoneal fibrosis?]. / Peut-on proposer une prise en charge de la fibrose rétropéritonéale idiopathique validée par les données de la littérature?

BACKGROUND: Nowadays it is quite easy to diagnose idiopathic retroperitoneal fibrosis (IRF), particularly with the help of medical imaging. However there is no guideline about the treatment. PURPOSE: Looking for data about an evidence-based management. METHODS: Screening of the database Medline. Titles and abstracts of articles published between 01/01/1985 and 31/12/2004 have been read to identify clinical trials and series about more than ten patients. RESULTS: No record of any therapeutic trials has been found. Eight series in total, which included 177 patients, were identified. Two of the patients have been treated by an ureteral desobstruction only (endoscopy or nephrostomy), 45 by surgery (ureterolysis), 65 by corticotherapy and 64 both by surgery and steroids. For 38 patients, immunosuppressive drugs were combined with corticotherapy (azathioprine, cyclophosphamide or D-penicillamine). According to the authors, doses and duration of corticotherapy varied. Median follow-up lasted 56 months. The outcome is satisfactory in 73% for surgery alone, 86% for medical treatment alone and 73% for both. The association between steroids therapy and immunosuppressive drugs is efficient in 97% of the cases. No clear data about side effects was mentioned. DISCUSSION: Treatment of the IRF is still empirical, based on surgery and corticotherapy. There is no guideline about the treatment strategy. Although tamoxifen has been proposed, efficacy evidence is lacking. Prospective multicenter studies will help us to progress in the management of the IRF.

[Use of nitrates in hospitalised elderly patients aged 65 and over]. / Utilisation des dérivés nitrés chez les patients âgés de 65 ans et plus hospitalisés en médecine interne.

BACKGROUND: Nitrates are frequently prescribed drugs, although their indications are limited. We studied nitrates' prescription in elderly patients hospitalised in the internal medicine ward of a French teaching hospital. METHODS: Hospitalised patients aged 65 years and more and receiving nitrates in their usual treatment were identified prospectively. A standardised questionnaire was used during a structured medical interview conducted by the same physician for all patients. Informations regarding nitrates' prescription were studied according to the actual recommendations for their use: angina in patients with contraindication to betablockers, acute myocardial infarction and acute pulmonary oedema. RESULTS: Among 256 hospitalised elderly patients, 49 (19% [IC95%: 15-25]) were under nitrates therapy, because of either angina pectoris, heart failure or unknown reason. Cardiologists prescribed nitrates in accordance with guidelines significantly more frequently than non-cardiologists. Transdermal treatment was used in 69% of patients. DISCUSSION: In hospitalised patients aged 65 years and more who are prescribed nitrates in their usual treatment, at least one quarter have no recommended indication for its use. As almost one fifth of patients are receiving nitrates in their usual treatment, the medicoeconomic impact of these useless prescriptions could be significant. Nitrates prescribing can be optimized by following guidelines for their use, and restraining from prescribing the transdermal treatment which is more costly and without evidence-based clinical benefit compared to the oral route.

Clinical significance of tolerant strains of streptococci in adults with infective endocarditis.

OBJECTIVES: To compare the characteristics of patients with endocarditis due to tolerant and non-tolerant Streptococcus strains. PATIENTS AND METHODS: A retrospective nine-year study was conducted in a single tertiary-care hospital. The study included 24 cases of streptococcal endocarditis with known beta-lactam minimal inhibitory and bactericidal concentrations. RESULTS: Ten of the 24 patients concerned were infected with tolerant streptococcal strains, and 14 with non-tolerant strains. Bacterial tolerance was not associated with higher mortality or increased frequency of surgery. Fewer patients infected with tolerant than non-tolerant strains had serum bactericidal titers reaching success-predictive levels, and more of these experienced failure of initial antibiotic treatment and needed longer treatment. CONCLUSIONS: The results of this study strongly suggest that penicillin tolerance of the streptococci responsible for endocarditis has a clinical impact. Consequently, pending a larger prospective study addressing the problem of tolerance, it is clinically relevant to determine the minimal inhibitory and bactericidal penicillin concentrations for all streptococcal isolates causing endocarditis.

[Low cost telepathology]. / Télépathologie à moindre coût.

Low cost, high-quality consumer-type digital cameras are now available on the market to be used for taking macrophotographs and microphotographs by simply fixing the camera over the eyepiece of a conventional light microscope using an adaptator. The quality of the images obtained is as good as obtained with more expensive materials using Tri CCD cameras. Using the JPEG format for compression, the image file size is approximately 180 Ko. We present a low cost approach we have tested for one year in our pathology department.