[Congenital thoracic deformities and 3D custom-made implants. New classification based on a series of 789 treated cases]. / Malformations thoraciques congénitales et implants sur mesure 3D. Nouvelle classification basée sur une série de 789 cas traités.

The authors present a new study on 789 cases of congenital thoracic malformations including 638 pectus excavatum and 151 Poland syndromes, according to a new classification which completes Chin's one. All these malformations were treated with silicone elastomer implants. The contribution of computer-aided design and manufacturing (CAD/CAM) since 2008 is essential. The one-stage surgical protocol is precisely described. The results are impressive, permanent, for life, and complications are rare. The authors evoke a common vascular etiopathogenesis theory at the embryonic stage and question the heavy techniques of invasive remodeling that are most often unjustified.

[Place of 3D custom-made implants after failure of modeling steno-chondro-plasties]. / Place des implants sur mesure 3D après échec des sterno-chondro-plasties modelantes.

Most common congenital malformation of the thorax, Pectus Excavatum affects about one in 500 people. Several surgical or medical techniques have been proposed. Some are followed by complications or insufficient results even though their constant functional value is highly controversial. Secondary surgery with a deep customized 3D elastomer implant, may be an elegant effective and safe solution compared to others; it allows a good aesthetic result expected by patients in the absence of any respiratory or cardio-vascular functional context.

[Multiple synchronous lung adenocarcinoma: Interest of molecular biology for staging]. / Adénocarcinomes pulmonaires multifocaux synchrones : intérêt de la biologie moléculaire pour la stadification.

INTRODUCTION: The presence of multiple synchronous lung tumors is not a rare event. Distinguishing intra-pulmonary metastases from multiple synchronous lung adenocarcinoma is a challenge for pathologists and physicians. We present observation of a patient with three lung tumors corresponding to three adenocarcinomas for which molecular analysis had a significant impact on tumor staging. OBSERVATION: Three suspect lesions were discovered in a 61-year-old patient, a smoker, in each lobe of the right lung. Right pneumonectomy with lymph node dissection was performed. Pathological examination showed that each tumor was in fact an adenocarcinoma. In order to more precisely indicate tumor staging, molecular analysis was performed with next generation sequencing showing a different point mutation in a driver gene on each tumor. The final diagnosis is that the three tumors are distinct synchronous tumors, which must be staged separately. CONCLUSIONS: In modern-day practice of thoracic oncology and of surgical pathology, molecular biology represents a complement for tumor staging in the event of multiple lung tumors.

[Silica causes difficulties]. / La silice cause des difficultés.

Silicosis and sarcoidosis are two very distinct entities in the literature. All the additional non-invasive examinations, including the chest CT scan, often do not differentiate them. The history, including occupational exposure to identified silica particles, is a discriminating factor. However, due to the pathogenic power of silica, it would be possible to have the simultaneous development of these two pathologies in the same patient. To illustrate this situation, here is the case of a 62-year-old patient, who presented initially with a picture of dyspnea and productive cough. The chest CT showed micronodular peribronchovascular infiltrates and mediastinal lymphadenopathy. The other additional examinations did not find anything specific. In the diagnostic process, the patient had multiple endoscopic samples which did not make it possible to be conclusive on one or the other of these pathologies. He therefore underwent a surgical lung biopsy which revealed histological lesions compatible with the two pathologies. Recent studies suggest that inhaled particles, especially silica, could be responsible for the pattern of sarcoidosis. However, it is difficult to say whether, in this case, silica was responsible for the development of sarcoidosis.

External validation of a risk score in the prediction of the mortality after esophagectomy for cancer.

This study was designed as an external evaluation of the Steyerberg score in the prediction of different categories of postoperative mortality after esophagectomy on a large nationwide database of thoracic surgeons. Data collection was obtained from the Epithor national database encompassing the majority of thoracic procedures performed in France. We retrospectively compared the predicted to the observed postoperative 30-day (30DM), 90-day (90DM) and in-hospital mortality (IHM) rate in each decile of equal patient. Patients included in the study were operated for an esophageal cancer and Gastroesophageal junction (GEJ). Steyerberg score was determined according to its logarithmic formula obtained from a sum score including age, comorbidities, neoadjuvant treatment and hospital volume. Deviation of observed from theoretically expected number of deaths was investigated using the calibration test of Hosmer-Lemeshow. Discrimination of the score was determined using the measure of the area under the receiver operating characteristic curve (AUC) of each category of mortality. Over a 9-year period, 1039 consecutive patients underwent an esophagectomy over 42 centers. Among them, 18 centers were considered as intermediate or high-volume institutions, and 24 were low-volume institutions. There were 841 males (81%) with a mean age of 62.3 ± 10 years. Preoperative treatment was allocated to 420 patients (40%). Numbers of comorbidity was: 1 in 261 patients (25%), 2 in 264 patients (25%), 3 in 383 patients (36%) and 4 in 5 patients (1%). The 30DM, 90DM and IHM rate were, respectively, 5.6%, 9.2% and 9.6%. The main causes of postoperative deaths were related to pulmonary complications (44%), complications of the gastric interposition (28%), cardiologic and thromboembolism events (10%). For 30DM, there were significant differences between predicted/observed mortalities in four deciles, whereas there was no significant difference for 90DM and for IHM. In term of calibration, there was a fair agreement of the Steyerberg score with observed 30DM. Predictions were above 20% for seven deciles. Calibration seemed more adequate for 90DM and for IHM. Predictions were above 20% for only three deciles but deviations were not significant. In terms of discrimination, for the 30DM the Steyerberg score overpredicted, the observed mortality rate and AUC was 0.64 (CI 95%: 0.57-0.71). For the 90DM, AUC indicated 0.63 (CI 95%: 0.57-0.68). For the IHM, AUC indicated 0.63 (CI 95%: 0.58-0.68). Steyerberg scoring system seems to be a moderate risk score of the prediction of the IHM and 90DM. This score appears to have a fair discrimination for the 30DM. Nevertheless, because of its simplicity, we believe that this simple predictive score is relevant and transportable to others institution performing such surgery for benchmarking purposes. A reappraisal of the score adapted to current surgical cohort is required.

Xenograft biologic mesh in parietal and general surgery: Technical assessment and review of clinical effectiveness and safety data.

STUDY AIM: To describe the main technical characteristics of biologic prostheses used for parietal reinforcement and to present the state of the art on their risk/benefit ratio. METHODS: We conducted a technical analysis of manufacturer specifications of the biologic prostheses that are currently available in France accompanied by a literature review by selecting meta-analyses and systematic reviews, randomized controlled trials and publications of health technology rating agencies. RESULTS: Biological implants for parietal reinforcement are mainly intended for use in a contaminated environment where the use of synthetic prostheses is contra-indicated. We identified fourteen systematic reviews and meta-analyses and one randomized controlled trial. Six ongoing clinical trials were identified as well as two clinical trials that had been interrupted. In the current state of knowledge, there are no high-level evidence data on the therapeutic contribution of biologic prostheses that allow prioritization of the various biologic prostheses according to their characteristics or their different manufacturing processes. CONCLUSION: Pending the results of current randomized controlled trials to validate the indications and an eventual specific reimbursement, indications for the use of biologic parietal reinforcement prostheses seems to be limited to rare clinical situations and only after collegial discussion.

[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives]. / Prothèse trachéobronchique dans le syndrome de Mounier-Kuhn : nouvelles perspectives.

INTRODUCTION: Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital condition, the management of which is complex. We report the case of a patient who was treated with interventional endoscopy. OBSERVATION: We describe the case of a 74-year-old man with a diagnosis of tracheobronchomegaly who was admitted in 2003 with a background of deteriorating respiratory status and the occurrence of postural syncope. He initially received a tracheobronchial silicone Y prosthesis, extended with metal prostheses at the tracheal and bronchial level. This arrangement remained stable until 2011. He then began to develop episodes of asphyxia related to posterior dislocation of the tracheobronchial prosthesis, after breakage of the metallic mesh tracheal prosthesis. A new tracheobronchial prosthesis Y was then placed, custom-made from a 3D model of the airways. This was clinically and functionally effective. DISCUSSION: This case describes the management of a patient with Mounier-Kuhn syndrome by interventional bronchoscopy, with the adaptation of prosthetic materials, on an individual basis, to the anatomy of the patient's airway.

[Locoregional treatments of brain metastases for patients with metastatic cutaneous melanoma: French national guidelines]. / Traitements locorégionaux de métastase(s) cérébrale(s) chez des patients atteints d'un mélanome cutané métastatique : recommandations nationales françaises.

INTRODUCTION: The management of metastatic cutaneous melanoma is changing, marked by innovative therapies. However, their respective use and place in the therapeutic strategy continue to be debated by healthcare professionals. OBJECTIVE: The French national cancer institute has led a national clinical practice guideline project since 2008. It has carried out a review of these modalities of treatment and established recommendations. METHODS: The clinical practice guidelines development process is based on systematic literature review and critical appraisal by experts. The recommendations are thus based on the best available evidence and expert agreement. Prior to publication, the guidelines are reviewed by independent practitioners in cancer care delivery. RESULTS: This article presents the results of bibliographic search, the conclusions of the literature and the recommendations concerning locoregional treatments of brain metastases for patients with metastatic cutaneous melanoma.

[Management of patients with metastatic cutaneous melanoma: French national guidelines. French National Cancer Institute]. / Prise en charge thérapeutique des patients atteints d'un mélanome cutané métastatique : recommandations nationales françaises. L'Institut national du cancer.

BACKGROUND: Recent years have seen the emergence of new molecules for the treatment of patients with metastatic cutaneous melanoma, with significant benefits in terms of survival and the opening of new therapeutic perspectives. In addition, many techniques are currently being developed for locoregional treatment of metastatic sites. Management of metastatic melanoma is thus fast-changing and is marked by innovative therapeutic approaches. However, the availability of these new treatments has prompted debate among healthcare professionals concerning their use and their place in therapeutic strategy. AIMS: Since 2008, the French National Cancer Institute (INCa) has been leading a project to define and diffuse national clinical practice guidelines. It has performed a review of these treatment methods, which it aims to circulate, and it is seeking to develop recommendations in order to allow nationwide implementation of innovative approaches while promoting good use thereof. METHODS: The clinical practice guidelines development process is based on systematic literature review and critical appraisal by experts within a multidisciplinary working group, with feedback from specialists in cancer care delivery. The recommendations are thus based on the best available evidence and expert agreement. Prior to publication, the guidelines are reviewed by independent practitioners in cancer care delivery. RESULTS: This article presents the national recommendations for first- and second-line systemic treatment and for locoregional treatment of metastatic sites in patients presenting metastatic cutaneous melanoma.

Treatment of esophageal perforation in octogenarians: a multicenter study.

Esophageal perforation is associated with significant mortality, and this may markedly increase with advanced age. This multicenter study investigates this issue in patients older than 80 years. Data on 33 patients >80 years old who underwent conservative (10 patients), endoclip (one patient), stent grafting (11 patients), or surgical treatment (11 patients) for esophageal perforation were collected from nine centers. Surgical repair consisted of repair on drain in one patient, primary repair in seven patients, and esophagectomy in two patients. Among patients who underwent stent grafting, one required repeat stenting and another stent graft repositioning. One patient was converted to surgical repair after stent grafting. Thirteen patients (39.4%) died during the 30-day and/or in-hospital stay. Their mortality was significantly higher than in a series of patients<80 years old (13.0%, 21/161 patients, P=0.001). Three patients (30.0%) died after conservative treatment, one (100%) after treatment with endoclips, five (45.5%) after stent grafting, and four (36.4%) after surgical repair (P=0.548). Early survival with salvaged esophagus was 42.4% (conservative treatment: 70.0% endoclips 0%, stent grafting: 54.5%, and surgical repair: 54.5%, respectively, P=0.558). Estimated glomerular filtration rate<60 mL/minute/1.73 m2 (70.0% vs. 25.0%, P=0.043) and sepsis (100% vs. 32.1%, P=0.049) at presentation were associated with increased risk of early mortality in univariate analysis. Esophageal perforation in octogenarians is associated with very high early and intermediate high mortality irrespective of the treatment method used.

[Nodular presentation of a cryptogenic organizing pneumonia]. / Présentation nodulaire d'une pneumopathie organisée cryptogénique.

INTRODUCTION: Cryptogenic organizing pneumonia is inflammatory and proliferative pulmonary diseases whose specific radiologic feature are bilateral and migrant opacities. OBSERVATION: An isolated peripheral nodule of the left lower lobe was discovered on chest X-ray of a man who presented with isolated chronic cough. As this nodule has a positive FDG positron emission tomography uptake (PET) but with inconclusive fiberoptic bronchoscopy, the patient was sent to surgeon and a wedge-resection was processed because intraoperative analysis did not show any tumour. Histopathological study was in favour of organizing pneumonia. Search for potential cause remained negative and the diagnosis of cryptogenic organizing pneumonia was retained. CONCLUSION: Cryptogenic organizing pneumonia may mimic lung cancer, presenting as an isolated peripheral nodule with positive PET. Histopathological study remains absolutely necessary to retain the diagnosis because of dramatic differences in prognosis and therapy.

[Comparison of simple aspiration versus standard drainage in the treatment of large primary spontaneous pneumothorax]. / Comparaison de l'efficacité d'une exsufflation simple par rapport au drainage thoracique dans le traitement du pneumothorax spontané complet. Étude EXPRED: EXsufflation d'un PREmier pneumothorax versus Drainage.

BACKGROUND: Chest tube drainage is the standard treatment of a large spontaneous pneumothorax. Aspiration is an alternative technique that is simple and rapid to learn, and the success rate seems identical to chest tube drainage. Its widespread use justifies studies to define its place in the management strategy of spontaneous pneumothorax. METHODS: We propose a multicentre, prospective, randomized, open trial with two parallel groups. The main objective is to compare the therapeutic efficacy of a simple aspiration with chest tube drainage for a first large spontaneous pneumothorax. The hypothesis is that aspiration is not inferior to a chest drain in its immediate effect. The secondary objectives are to compare the therapeutic efficacy at 24h and at one-week, the relapse rate at one year, and the tolerance and complications. A comparison of both the medical and economic aspects will be made. With an α-risk of 0.05 and a ß-risk of 0.10, a proportion of failures of 30% expected in both groups and a target of non-inferiority of δ=0.15, the number of subjects to be included is 200 per group, totalling 400 in all. EXPECTED RESULTS: In the case of equivalence, this study should help to better define the place of aspiration compared to chest tube drainage in the management of spontaneous pneumothorax.

[HELLP syndrome and ruptured subcapsular hepatic haematoma. Case report and therapeutic options]. / HELLP syndrome et hématome sous-capsulaire du foie rompu. Stratégie thérapeutique à partir d'un cas clinique.

We report a case of spontaneous hepatic rupture secondary to HELLP syndrome. A favourable evolution was observed after massive transfusion and surgical management limited to hepatic packing. Subcapsular hepatic haematoma is a rare complication of preeclampsia occurring mainly in the context of HELLP syndrome. A high maternal and foetal mortality is observed. Different therapeutic options are presented including medical, surgical and radiological interventions. A unique strategy cannot be defined. Multidisciplinary approach seems mandatory. Surgery should remain as less aggressive as possible.

[Sentinel node mapping in anorectal melanoma]. / Ganglion sentinelle et mélanome malin anorectal.

Anorectal melanoma is a rare condition and its surgical management is controversial. This article reports the case of a patient with anorectal melanoma who underwent abdominoperineal resection and Sentinel Lymph Node biopsy. Melanoma was classified pT4aN0. Fifty months after initial treatment, the patient is still alive disease free. SLN mapping allows better surgical excision of the presumed sites of the lymphatic dissemination in melanoma. SLN biopsy improve the accuracy of nodal staging. In case of sentinel node metastasis, it allows early therapeutic lymphadenectomy of the sentinel nodes's basin and could therefore reduce the high rate of regional recurrence in anorectal melanoma. Moreover, knowing the exact histological status of the regional nodes means that the relative merits of abdominoperineal resection and wild local excision could be compared in relation to tumor thickness.

Sentinel lymph node detection in primary melanoma with preoperative dynamic lymphoscintigraphy and intraoperative gamma probe guidance.

BACKGROUND: This study assessed the value of the radioisotopic method used alone, and factors influencing relapse rates, for sentinel lymph node (SLN) mapping in primary melanoma. METHODS: One hundred and thirty-three patients with a diagnosis of melanoma (thickness greater than 0.75 mm) underwent gamma probe-directed lymphatic mapping in a prospective single-centre study. RESULTS: Mean Breslow thickness was 3 mm. At least one SLN was identified in 132 patients (mean 1.8 nodes per patient); the success rate was 99.2 per cent. Twenty-two patients (16.7 per cent) had a metastasis within the SLN. The mean tumour thickness in patients with a metastatic SLN was 4.4 mm compared with 2.7 mm for patients with a negative SLN (P < 0.001). The median time to recurrence was 20.4 months in SLN-negative patients compared with 8.5 months in those with SLN metastasis (P < 0.001). Ten (9.1 per cent) of the 110 SLN-negative patients developed recurrence. Three patients relapsed in the previously mapped lymphatic basin after a median follow-up of 27.1 months. CONCLUSION: This study confirmed the reliability and accuracy of SLN mapping using a radioisotope technique, and also the importance of the SLN as a predictive factor for survival. There was a low risk of locoregional recurrence when the SLN was not involved.

[Isolated intrapulmonary plasmacytoma; diagnostic and therapeutic difficulties]. / Plasmocytome intra-pulmonaire isolé: difficultés diagnostiques et thérapeutiques.

Localised intrapulmonary plasmacytoma represents less than 5% of all extramedullary plasmacytomas. Their diagnosis is usually made difficult by a radiological presentation and non specific endoscopic findings as well as non contributory biochemical and cytological data. This often justifies the recourse to more invasive histological investigation aided by immunohistochemical techniques. We report the case of a patient who presented with an isolated plasmacytoma in the form of two parenchymatous pulmonary masses and who was treated by a combination of surgical resection and external radiotherapy. A search for multiple intraosseous myeloma remained negative. Unfortunately a relapse in the form of mediastinal invasion occurred 6 months after the completion of treatment and led to the death of the patient.

Interest of PS100 assay when (99m)Tc sestamibi scintigraphy failed to identify lymph node metastases of melanoma.

The study evaluated the contribution of serum PS100 assay to the detection of lymph node metastases during the follow-up of patients previously treated for a malignant melanoma, in addition to (99m)Tc sestamibi (MIBI) scintigraphy and investigation for gene MDR1, in order to detect chemoresistance phenomena. The study included 37 patients with a clinically questionable lymph node around basin lymphatic areas of the previously surgically-treated malignant melanoma. The sensitivity and specificity of PS100 assay were 91% and 86.5%, respectively. The sensitivity and specificity of MIBI scintigraphy were 95% and 85%, respectively. Overexpression of gene MDR1 was observed in six cases. In the event of negative scintigraphic findings, the concomitant analysis of PS100 levels and the scintigraphic result enabled the metastatic MDR+ patients to be distinguished from the non-metastatic patients. PS100 assay may therefore be proposed for the follow-up of malignant melanoma.

Invasive thymoma: a clinical study of 23 cases.

BACKGROUND: Invasive thymoma is a rare mediastinal tumor. Clinicopathological characteristics that influence survival of patients with this tumor are under debate. Treatment is based on tumor resection. The benefice of therapies, such as radiation therapy (RT) and/or chemotherapy (CT) as adjuvant treatments to surgery, or palliative therapy to unresectable or recurrent thymoma are discussed. OBJECTIVES: The aim of this study was to assess patients with invasive thymoma, with specific emphasis on factors predicting survival. METHODS: We studied retrospectively 23 patients with invasive thymoma. Parameters assessed were age, presenting symptoms, histological features, stage at diagnosis, treatment modalities and survival. All patients received primary therapy: 11 patients (48%) had tumor resection associated with CT and/or RT, while 12 patients had palliative therapy including RT and/or CT. Regimens for CT were based on cisplatin. RESULTS: Patients' mean age was 58 years. Three patients had stage II disease at diagnosis (13%), 8 patients had stage III (35%) and 12 patients had stage IV (52%). Median overall survival was 20 months (range: 4-160) and five-year survival rate was 43.5% (10 patients). Surgical resection had a significant impact on survival (p < 0.0001). Survival was also related to stage of the disease at diagnosis (p = 0.006), but not to histology of the tumor (p = 0.12). Salvage treatment was of clinical importance: 5 out of 15 patients (33.3%) who relapsed during a 5-year follow-up responded to a multimodality therapeutic approach that affected survival (p = 0.019). CONCLUSION: Factors determining the outcome of these tumors are the stage of the disease at diagnosis, and the adequacy of surgical removal. Salvage treatment of recurrent thymoma may give a moderate response rate and improve survival.

[Detection of lymphatic metastasis from malignant melanoma after identification of the sentinel node by preoperative lymphoscintigraphy and intraoperative radioisotopic detection]. / Recherche des métastases ganglionnaires des mélanomes malins après isolement du ganglion sentinelle par lymphoscintigraphie préopératoire et détection radio-isotopique peropératoire. Résultats préliminaires.

OBJECTIVES: The aim of this study was to evaluate the detection of the first lymph node draining the primary tumour site, using a radioisotopic mapping alone and to determine whether a preoperative lymphoscintigraphy using technetium sulfur colloid and a hand-held gamma detecting probe could improve the detection of the sentinel lymph node (SLN) in melanoma. PATIENTS AND METHOD: From January to December 1998, 36 patients with a cutaneous melanoma larger than 0.75 mm, stage I TNM were included in this prospective study. Mean Breslow was 1.85 mm. The distribution of melanoma was head and neck (n = 9), trunk (n = 7), upper extremities (n = 4), lower extremities (n = 16). Preoperative lymphoscintigram and intraoperative detection were used. The first hot lymph node was supposed to be the SLN. RESULTS: In all cases, a lymph node was found and nine patients had more than one SLN (average number of SLN per patient: 1.25). Aberrant drainages were found in seven patients (19.4%): 1 in-transit lymph node, three paradoxical bassins, three bypasses). Four out of 36 patients had lymph node metastases and underwent elective lymph node dissection. CONCLUSION: The radio-isotopic technique used alone for the identification of the SLN is efficient in melanoma with a 100% detection rate in this short series.