Complete and Repeated Response of a Metastatic ALK-rearranged Inflammatory Myofibroblastic Tumor to Crizotinib in a Teenage Girl.

Inflammatory myofibroblastic tumors (IMT) are rare tumors in children and young adults, considered by the World Health Organization to be intermediate malignancies and rarely metastasizing, with the presence of an anaplastic lymphoma kinase rearrangement in about 50% of the cases. We report the case of a teenager who presented with a metastatic aggressive IMT that was life-threatening despite multiple treatments, and which responded repeatedly to anaplastic lymphoma kinase-targeted crizotinib therapy. Crizotinib induced drastic primary tumor regression, which was sufficient to allow surgical resection and to control distant disease. This case shows that crizotinib is a promising therapy in IMT, even in adolescents and young adults.

Technique and results of femoral bifurcation endarterectomy by eversion.

OBJECTIVE: This study evaluated, in a contemporary prospective series, the safety and efficacy of femoral endarterectomy using the eversion technique and compared our results with results obtained in the literature for the standard endarterectomy with patch closure. METHODS: Between 2010 and 2012, 121 patients (76% male; mean age, 68.7 years; diabetes, 28%; renal insufficiency, 20%) underwent 147 consecutive femoral bifurcation endarterectomies using the eversion technique, associating or not inflow or outflow concomitant revascularization. The indications were claudication in 89 procedures (60%) and critical limb ischemia in 58 (40%). Primary, primary assisted, and secondary patency of the femoral bifurcation, clinical improvement, limb salvage, and survival were assessed using Kaplan-Meier life-table analysis. Factors associated with those primary end-points were evaluated with univariate analysis. RESULTS: The technical success of eversion was of 93.2%. The 30-day mortality was 0%, and the complication rate was 8.2%; of which, half were local and benign. Median follow-up was 16 months (range, 1.6-31.2 months). Primary, primary assisted, and secondary patencies were, respectively, 93.2%, 97.2%, and 98.6% at 2 years. Primary, primary assisted, and secondary maintenance of clinical improvement were, respectively, 79.9%, 94.6%, and 98.6% at 2 years. The predictive factors for clinical degradation were clinical stage (Rutherford category 5 or 6, P = .024), platelet aggregation inhibitor treatment other than clopidogrel (P = .005), malnutrition (P = .025), and bad tibial runoff (P = .0016). A reintervention was necessary in 18.3% of limbs at 2 years: 2% involving femoral bifurcation, 6.1% inflow improvement, and 9.5% outflow improvement. The risk factors of reintervention were platelet aggregation inhibitor (other than clopidogrel, P = .049) and cancer (P = .011). Limb preservation at 2 years was 100% in the claudicant population. Limb salvage was 88.6% in the critical limb ischemia population, with a statistically higher rate for patients with malnutrition (P = .029), preoperative platelet count >450 ×10(9)/L (P = .0071), platelet aggregation inhibitor treatment other than clopidogrel (P = .022), preoperative deep femoral artery occlusion or stenosis >75% (P = .0064), and poor tibial runoff (P = .00042). CONCLUSIONS: Eversion femoral bifurcation endarterectomy is a safe, efficient, and reproducible technique for the treatment of atherosclerotic femoral lesions. Advantages are notable, especially the lack of need for prosthetic angioplasty, eliminating the risk of patch infection or pseudoaneurysms and permitting direct puncture if endovascular procedures are needed for assisted patency.

Midterm results of type B intramural hematoma endovascular treatment.

BACKGROUND: Recently, intramural hematoma (IMH) has been characterized as a rare variant of aortic dissection, falling within the acute aortic syndromes (AAS). Although aortic endovascular treatment seems to provide good results, no consensus has been established regarding the optimal management of IMH affecting the descending thoracic aorta (IMH B). The aim of this study was to assess long-term clinical and morphological results of IMH B treated with a stent graft. METHODS: Between 2002 and 2013, 15 of the 423 (3.5%) consecutive patients with AAS were found to have IMH B. During follow-up, complications related to IMH B were observed in 10 patients, including 5 during the acute period (<14 days). Surgical indications were ulcerations, refractory pain, aneurysm evolution, aortic rupture, and pleural effusion. An endovascular approach was taken for all forms with suitable anatomy. RESULTS: Technical success rate was 100%, with complete exclusion of lesions of the descending thoracic aorta in all cases treated with a stent graft. No neurological complications or deaths occurred within the first 30 postoperative days, nor at the end of the mean follow-up of 46 months (1-137 months). After the aortic stent graft, partial or complete regression of the hematoma was observed in all cases. CONCLUSIONS: IMH B is most often a progressive disease requiring long-term monitoring. In case of complications, stent-graft treatment of the descending thoracic aorta can be performed successfully in most cases. This procedure appears to be a safe technique that provides very good long-term results, with favorable remodeling of the aorta.

Real-world outcomes of lobectomy, segmentectomy and wedge resection for the treatment of stage c-IA lung carcinoma.

OBJECTIVES: To determine safety and survival outcomes associated with lobectomy, segmentectomy and wedge resection for early-stage lung cancer by quiring the French population-based registry EPIdemiology in THORacic surgery (EPITHOR). METHODS: Retrospective analysis of 19 452 patients with stage c IA lung carcinoma who underwent lobectomy, segmentectomy or wedge resection between 2016 and 2022 with curative-intent. Main outcome measures were 90-day mortality and 5-year overall survival estimates. Proportional hazards regression and propensity score matching were used to adjust outcomes for key patient, tumour and practice environment factors. RESULTS: The treatment distribution was 72.2% for lobectomy, 21.5% for segmentectomy and 6.3% for wedge. Unadjusted 90-day mortality rates were 1.6%, 1.2% and 1.1%, respectively (P = 0.10). Unadjusted 5-year overall survival estimates were 80%, 78% and 70%, with significant inter-group survival curves differences (P < 0.0001). Multivariable proportional hazards regression showed that wedge was associated with worse overall survival [adjusted hazard ratio (AHR), 1.23 (95% confidence interval 1.03-1.47); P = 0.021] compared with lobectomy, while no significant difference was disclosed when comparing segmentectomy to lobectomy (1.08 [0.97-1.20]; P = 0.162). The three-way propensity score analyses confirmed similar 90-day mortality rate for wedge resection and segmentectomy compared with lobectomy (hazard ratio: 0.43; 95% confidence interval 0.16-1.11; P = 0.081 and 0.99; 0.48-2.10; P = 0.998, respectively), but poorer overall survival (1.45; 1.13-1.86; P = 0.003 and 1.31; 1-1.71; P = 0.048, respectively). CONCLUSIONS: Wedge resection was associated with comparable 90-day mortality but lower overall survival when compared to lobectomy. Overall, all types of sublobar resections may not offer equivalent oncologic effectiveness in real-world settings.

Non-traumatic diaphragmatic rupture with liver herniation due to endometriosis: A rare evolution of the disease requiring multidisciplinary management.

A 35 year old woman with chronic pelvic endometriosis suffered from right scapular pain. MRI imaging showed a right diaphragmatic rupture with liver herniation. Surgical procedure was performed by thoracotomy. The liver was put back into the abdomen, endometriosis was resected from the diaphragm, interrupted non absorbable suture of the diaphragm was performed and an absorbable mesh was placed. Endometriosis was confirmed on histological analysis of the resected diaphragm. To study this pathology, we performed a systematic review of the literature and found 12 similar cases of diaphragmatic rupture due to endometriosis. Right diaphragm is often involved and rupture is always located on the tendinous portion. Symptoms are mainly cyclic right scapular pain and cathamenial pneumothorax. MRI should be performed in case of suggestive symptoms and a systematic exploration of the diaphragm should be performed at laparoscopy for an early treatment of the lesions to prevent progression to rupture.

Postpneumonectomy-like syndrome after lobectomy: An exceptional situation.

Postpneumonectomy syndrome is a rare complication occurring after pneumonectomy. It is defined as extrinsic compression of a bronchus associated with a significant shift of the mediastinum. A man aged 45 years with history of C7 tetraparesis, presented with a postpneumonectomy-like syndrome 2 months after a right lower lobectomy. We performed a right pneumonectomy combined with implantation of 2 intrathoracic inflatable mammary implants. The patient remained asymptomatic after 6 months of follow-up. We believe this is the first reported case of a postpneumonectomy-like syndrome occurring after lobectomy.

Do atmospheric conditions influence the first episode of primary spontaneous pneumothorax?

OBJECTIVES: Several studies suggest that changes in airway pressure may influence the onset of primary spontaneous pneumothorax (PSP). The aim of this study was to investigate the influence of atmospheric changes on the onset of the first episode of PSP. METHODS: We retrospectively analysed cases of pneumothorax admitted to our department between 1 January 2009 and 31 October 2013. Patients with recurrent pneumothorax, traumatic pneumothorax, older than 35 years or presenting history of underlying pulmonary disease were excluded. Meteorological data were collected from the Météo-France archives. Variation (Δ) of mean atmospheric pressure, and relative humidity, were calculated for each day between the day at which symptoms began (D-day), the day before first symptoms (D-1), 2 days before the first symptoms (D-2) and 3 days before the first symptoms (D-3). RESULTS: Six hundred and thirty-eight cases of pneumothorax were observed during the period of this study; 106 of them (16.6%) were a first episode of PSP. We did not observe any significant differences between days with or without PSP admission for any of the weather parameters that we tested. We could not find any thresholds in the variation of atmospheric pressure that could be used to determine the probability of PSP occurrence. CONCLUSIONS: Variation of atmospheric pressure, relative humidity, rainfall, wind speed and temperature were not significantly related to the onset of the first episode of PSP in healthy patients. These results suggest that the scientific community should focus on other possible aetiological factors than airway pressure modifications.

A misleading cause of pseudo-thymic tumor in ectopic Cushing's syndrome.

A 27-year-old man was referred with typical features of severe Cushing's syndrome. A bilateral adrenalectomy was performed. Three months later, a triangular nodular mediastinal enlargement, evocative of a right anterior thymic tumor, was discovered. Thymectomy was undertaken. Histological examination revealed diffuse thymic hyperplasia with negative immunostaining for adrenocorticotropic hormone. Five years later, a right endobronchial tumor corresponding to a carcinoid tumor was removed.

Migration of a Kirschner pin from the shoulder to the lung, requiring surgery.

A 62-year-old woman underwent a reduction of a proximal reduced humeral fracture, which was fixed by 3 Kirschner pins. One year later, the orthopedic surgeon failed to remove one of the wires. The patient was lost to follow-up, and 4 years later, she presented with hemoptysis, revealing migration of the pin to the lung. The pin was removed through a thoracotomy. Migration to the lung is often revealed by hemoptysis or pneumothorax. Close follow-up and early removal of the pins are mandatory.

Morbidity, mortality and survival after 110 consecutive bilobectomies over 12 years.

OBJECTIVES: To analyse statistical aspects of mortality, morbidity and survival after bilobectomy (BT), an operation rarely studied in the literature. METHODS: One hundred and ten cases were studied, comprising 58 upper-middle bilobectomies and 52 lower-middle bilobectomies performed between 1999 and 2010. Indications were of 9 benign diseases, 12 carcinoid tumours, 5 metastases and 84 non-small cell lung cancers (2 stage 0; 34 stage I; 22 stage II; 25 stage III and 1 stage IV). RESULTS: Mortality was nil. Twenty-six percent of patients experienced significant morbidity, influenced in multivariate analysis by the presence of three or more comorbidities (P = 0.03) and by a forced expiratory volume in 1 s of <60% (P = 0.01). Lower-middle BT was associated with more postoperative complications than upper-middle BT (P = 0.012). The 5-year survival rate of patients with non-small cell lung carcinoma was 82% in stage I, 59% in stage II and 20% in stage IIIA. Survival was significantly influenced by stage (P = 0.0018) and tobacco weaning (P = 0.0012). CONCLUSIONS: BT can be achieved with low mortality, and survival results that are comparable with those unregistered after standard lobectomy. However, almost one quarter of patients experienced significant postoperative complications. Surgical techniques aiming to reduce residual pleural space should be especially considered after lower-middle BT, due to the highest morbidity being associated with this procedure.

Does bilobectomy offer satisfactory long-term survival outcome for non-small cell lung cancer?

BACKGROUND: Results of bilobectomy for non-small cell lung cancer have rarely been studied. METHODS: Retrospective analysis was conducted on patients with non-small cell lung cancer having undergone bilobectomy from January 1999 to June 2012 at our institution. Analysis aimed at determining perioperative mortality and morbidity, and at studying prognostic factors for long-term survival using the 7th TNM classification. RESULTS: A total of 103 patients (85 males; mean age 62 years) underwent upper-middle bilobectomy (n = 54) or lower-middle bilobectomy (n = 49). Histologic examination revealed 51 adenocarcinomas, 43 squamous cell carcinomas and 9 other cell carcinomas. Perioperative mortality was 0.97%. The overall morbidity rate was 71%, whereas the rate of life-threatening complications was 9.6%. Complications were more frequent in men (p = 0.032), in patients with chronic pulmonary obstructive diseases (p = 0.030) and after lower-middle bilobectomy (p = 0.0016). The overall 5-year Kaplan-Meier survival rate was 57.8%. In univariate analysis, factors associated with increased survival were the following: pathologic stage (stage I 74.9%, stage II 64.1%, stage III 28.8%, p = 0.0018); nodal status (N0 vs N1, p = 0.011; N0 vs N2, p = 0.0015; N0 vs N+, p = 0.0008); R status (R0 vs R1, p = 0.0032), and smoking status (past smoker or nonsmoker vs active smoker, p = 0.00054). Multivariate analysis revealed that active smokers (RR = 3.87, CI 95% [1.83 to 8.21]; p = 0.00042) and increasing stage (stage 0: RR=1; stage I: RR = 1.98, CI 95% [1.38 to 2.83]; stage II: RR = 3.90, CI 95% [1.90 to 8.02]; stage III: RR=7.72, CI 95% [2.62 to 22.73]; stage IV: RR = 15.25, CI 95% [3.61 to 64.40]; p = 0.0042) were significantly associated with poorer survival. CONCLUSIONS: Bilobectomy can be performed with low mortality, acceptable morbidity and long term survival in accordance with TNM staging.

Pulmonary botryomycosis on a lung cavity: a rare pulmonary infection mimicking cancer.

Lung botryomycosis is a rare disease. We report what is to our knowledge the first case occurring on a lung cavity. In a 42-year-old man suffering asthenia and cough, a chest radiograph revealed a right upper lobe opacity. Computed tomography scan showed a necrotic mass which was also spiculated. Repeated research for Mycobacterium tuberculosis was negative. The patient underwent a lobectomy. Histological and bacteriological examinations made the diagnosis of botryomycosis, because the cavity presented numerous colonies of pyogenic Fusobacterium nucleatum bacteria. Botryomycosis is a difficult diagnosis that clinically mimics actinomycosis, tuberculosis or cancer. In most cases, surgery is necessary to assess diagnosis and treatment.