RESUMEN
BACKGROUND: Leiomyosarcoma of the vena cava (LMS-VC) is a rare entity with poor oncological outcomes and a lack of histological staging prognostic factors. METHODS: Outcomes of consecutive patients operated on LMS-VC between March 2003 and May 2022, in two specialized sarcoma centers were reported. RESULT: Forty-one patients were identified. Median size of LMS-VC was 9 cm with 68% of complete obstruction. After surgery, severe complication rate was 30%. No postoperative mortality was reported. Microscopic complete excision was obtained for 71% of patients, R1 for 27% and one patient presented an R2 resection. Grade 3 was found in 24%. After a median follow-up of 70 months, 3 years disease-free survival (DFS) and 5 years DFS were 34% and 17%, and 3 years overall survival (OS) and 5 years OS were 74% and 50%. Distant metastasis concerned 54% of recurrences, local 7% and local and distant 5%. Multivariate analysis showed that FNCLCC grade (p < 0.001) and perioperative chemotherapy (p = 0.026) were significant factors for DFS. In multivariate analysis, FNCLCC grade was a significant factor for OS (p = 0.004). DISCUSSION: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor.
RESUMEN
OBJECTIVES: Lower bilobectomy (LBL) leaves a residual pleural space potentially associated with adverse postoperative outcomes. In selected patients, right sleeve lower lobectomy (RSLL) with anastomosis between the middle lobe bronchus and intermediate bronchus is feasible. The outcomes of RSLL and LBL have not been compared. The aim of this study was to compare post-operative and long-term outcomes of RSLL and LBL in patients with lung cancer. METHODS: We retrospectively included patients managed by RSLL or LBL at our referral chest-surgery institution between 2001 and 2019. Post-operative complications and mortality were compared. Kaplan-Meier curves were plotted to compare overall and disease-free survival rates. RESULTS: We identified 23 patients with RSLL and 96 with LBL. Postoperative mortality was 9 % after RSLL and 5 % after LBL (p = 0.41). Bronchial fistula developed in 3 (13 %) RSLL patients and 6 (6 %) LBL patients (p = 0.23). Pleural space complications were significantly less common after RSLL (4/23 [17 %] vs. 45/96 [47 %], p = 0.03). Long-term vital capacity was significantly higher in the RSLL group (91 % vs. 64 %, p < 0.01). Five-year survival did not differ significantly between groups (84 % vs. 72 %, p = 0.09). CONCLUSIONS: RSLL was associated with similar postoperative mortality and long-term survival compared to LBL. However, pleural space complications were less common and lung function was better after RSLL than after LBL. When feasible, RSLL may deserve preference over LBL in patients with lung cancer managed at highly experienced centres.
RESUMEN
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed. The proportion of females was higher in each subgroup. No significant difference in the anti-AChR titers was observed. Interestingly, the proportion of AChR+ MG patients was notably lower among adult MG patients aged between 30 and 40 years, at 69.7%, compared to over 82.4% in the other subgroups. Thymic histological data were examined in patients who underwent thymectomy during the year of MG onset. Notably, in pre-JMG, the percentage of thymectomized patients was significantly lower (32.9% compared to more than 42.5% in other subgroups), and the delay to thymectomy was twice as long. We found a positive correlation between anti-AChR antibodies and germinal center grade across patient categories. Additionally, only females, particularly post-JMG patients, exhibited the highest rates of lymphofollicular hyperplasia (95% of cases) and germinal center grade. These findings reveal distinct patterns in JMG patients, particularly regarding thymic follicular hyperplasia, which appears to be exacerbated in females after puberty.
Asunto(s)
Autoanticuerpos , Miastenia Gravis , Receptores Colinérgicos , Timectomía , Timo , Humanos , Miastenia Gravis/patología , Miastenia Gravis/epidemiología , Femenino , Masculino , Adulto , Francia/epidemiología , Timo/patología , Timo/cirugía , Adolescente , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Receptores Colinérgicos/inmunología , Adulto Joven , Niño , Estudios de Cohortes , Centro Germinal/patología , Centro Germinal/inmunologíaRESUMEN
INTRODUCTION: Lung graft allocation can be based on a score (Lung Allocation Score) as in the USA or sequential proposals combined with a discrete priority model as in France. We aimed to analyse the impact of allocation policy on the outcome of urgent lung transplantation (LT). METHODS: US United Network for Organ Sharing (UNOS) and French Cristal databases were retrospectively reviewed to analyse LT performed between 2007 and 2017. We analysed the mortality risk of urgent LT by fitting Cox models and adjusted Restricted Mean Survival Time. We then compared the outcome after urgent LT in the UNOS and Cristal groups using a propensity score matching. RESULTS: After exclusion of patients with chronic obstructive pulmonary disease/emphysema and redo LT, 3775 and 12 561 patients underwent urgent LT and non-urgent LT in the USA while 600 and 2071 patients underwent urgent LT and non-urgent LT in France. In univariate analysis, urgent LT was associated with an HR for death of 1.24 (95% CI 1.05 to 1.48) in the Cristal group and 1.12 (95% CI 1.05 to 1.19) in the UNOS group. In multivariate analysis, the effect of urgent LT was attenuated and no longer statistically significant in the Cristal database (HR 1.1 (95% CI 0.91 to 1.33)) while it remained constant and statistically significant in the UNOS database (HR 1.12 (95% CI 1.05 to 1.2)). Survival comparison of urgent LT patients between the two countries was significantly different in favour of the UNOS group (1-year survival rates 84.1% (80.9%-87.3%) vs 75.4% (71.8%-79.1%) and 3-year survival rates 66.3% (61.9%-71.1%) vs 62.7% (58.5%-67.1%), respectively). CONCLUSION: Urgent LT is associated with adverse outcome in the USA and in France with a better prognosis in the US score-based system taking post-transplant survival into account. This difference between two healthcare systems is multifactorial.
Asunto(s)
Trasplante de Pulmón , Humanos , Trasplante de Pulmón/mortalidad , Trasplante de Pulmón/estadística & datos numéricos , Francia/epidemiología , Estados Unidos/epidemiología , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de Supervivencia , Obtención de Tejidos y Órganos/estadística & datos numéricos , Adulto , Puntaje de Propensión , AncianoRESUMEN
Bordetella hinzii (B. hinzii), a Gram-negative bacillus commonly associated with respiratory infections in animals, has garnered attention for its sporadic cases in humans, particularly in immunocompromised individuals. Despite its opportunistic nature, there remains limited understanding regarding its pathogenicity, diagnostic challenges, and optimal treatment strategies, especially in the context of immunosuppression. Herein, we present the first documented case of acute bronchitis caused by B. hinzii in an immunocompromised patient following double-lung transplantation. The patient, a former smoker with sarcoidosis stage IV, underwent transplant surgery and subsequently developed a febrile episode, leading to the identification of B. hinzii in broncho-alveolar lavage samples. Antimicrobial susceptibility testing revealed resistance to multiple antibiotics, necessitating tailored treatment adjustments. Our case underscores the importance of heightened awareness among clinicians regarding B. hinzii infections and the imperative for further research to elucidate its epidemiology and optimal management strategies, particularly in immunocompromised populations.
Asunto(s)
Infecciones por Bordetella , Bordetella , Huésped Inmunocomprometido , Trasplante de Pulmón , Trasplante de Pulmón/efectos adversos , Humanos , Bordetella/aislamiento & purificación , Infecciones por Bordetella/microbiología , Infecciones por Bordetella/diagnóstico , Masculino , Persona de Mediana Edad , Antibacterianos/uso terapéutico , Antibacterianos/farmacología , Receptores de TrasplantesRESUMEN
BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.
Asunto(s)
Neoplasias Renales , Nefrectomía , Venas Renales , Tumor de Wilms , Humanos , Tumor de Wilms/cirugía , Tumor de Wilms/patología , Femenino , Masculino , Estudios Retrospectivos , Neoplasias Renales/cirugía , Neoplasias Renales/patología , Preescolar , Niño , Lactante , Estudios de Seguimiento , Tasa de Supervivencia , Pronóstico , Venas Renales/cirugía , Venas Renales/patología , Atrios Cardíacos/cirugía , Atrios Cardíacos/patología , Terapia Neoadyuvante , Vena Cava Inferior/patología , Vena Cava Inferior/cirugíaRESUMEN
PURPOSE: To assess the prognostic factors and patterns of failure of patients consecutively treated with surgery and postoperative radiation therapy (PORT) for thymic epithelial tumours (TET). PATIENTS AND METHODS: Data from 192 TET patients who were operated and received PORT at a single centre from 1990 to 2019 was retrospectively analysed. RESULTS: Most patients had thymoma (77 %, B247%), were classified Masaoka-Koga stage III (35 %) or IV (32 %) and had a R0 (75 %) resection. Radiotherapy was delivered at a median dose of 50.4 Gy (range, 42-66 Gy; ≥ 60 Gy in 17 %), 63 (33 %) patients were treated by intensity-modulated radiation therapy and elective nodal radiotherapy was used for 37 %. At a median follow-up of 10.9 years, the 10-year overall survival (OS) and progression-free survival (PFS) rates were 62 % (95 % CI: 54-70 %) and 47 % (95 % CI: 39-55 %), respectively. Locoregional recurrence (LRR) occurred in 72/192 (38 %) patients, distributed as 6 local, 45 regional and 21 both local and regional. LRR were mainly located to the pleura: 66/72 (92 %) and 16/72 (22 %; 16/192 in total, 8 %) were in-field. Distant relapse (DR) were observed in 30 patients (16 %), resulting in 10-year locoregional (LRC) and distant control rates of 58 % (95 % CI: 50-66 %) and 82 % (95 % CI: 77-88 %), respectively. In the multivariate analysis, Masaoka-Koga stage (HR [hazard ratio]: 1.9; p = 0.001), thymic carcinomas/neuroendocrine tumours (TC) (HR: 1.6; p = 0.045) and ECOG PS > 1 (HR: 1.9; p = 0.02) correlated with poorer OS. Higher Masaoka-Koga stage (HR: 2.6; p < 0.001) associated with a decreased LRC but not R1 status (HR: 1.2; p = 0.5) or WHO histology classification. TC (HR: 3.4; p < 0.001) and a younger age (HR: 2.5; p = 0.02) correlated with DR. CONCLUSION: Approximately one-third of the TET in our study experienced a LRR, mainly to the pleura, and 8% in total were in-field. The place of radiotherapy should be better defined in higher risk thymoma patients within prospective randomized studies.
Asunto(s)
Neoplasias Glandulares y Epiteliales , Neoplasias del Timo , Humanos , Neoplasias del Timo/radioterapia , Neoplasias del Timo/patología , Neoplasias del Timo/mortalidad , Neoplasias del Timo/cirugía , Masculino , Femenino , Persona de Mediana Edad , Anciano , Adulto , Estudios Retrospectivos , Estudios de Seguimiento , Neoplasias Glandulares y Epiteliales/radioterapia , Neoplasias Glandulares y Epiteliales/patología , Neoplasias Glandulares y Epiteliales/mortalidad , Neoplasias Glandulares y Epiteliales/cirugía , Anciano de 80 o más Años , Adulto Joven , Recurrencia Local de Neoplasia , Radioterapia Adyuvante , Radioterapia de Intensidad Modulada/métodos , Adolescente , Timoma/radioterapia , Timoma/patología , Timoma/mortalidad , Pronóstico , Tasa de SupervivenciaRESUMEN
Objective: To evaluate outcomes of surgical repair of postesophagectomy neoesophagus-airway fistulas (NEAFs). Methods: We retrospectively included consecutive patients with NEAF managed by various techniques at our center between August 2009 and July 2021. Result: Of the 11 patients (median age, 60 years; interquartile range, 58, 62), 4 had received induction chemoradiotherapy and 4 others induction chemotherapy. NEAF was mainly a complication of anastomotic leakage (n = 6) or attempted stenosis treatment (n = 3). The airway mainly involved was the trachea (n = 8). Airway defects were repaired by resection-anastomosis (n = 5), perforator flaps (n = 4), pedicled pericardium (n = 1), and/or direct suturing (n = 2). Gastric conduit defects were repaired by perforator flaps (n = 6), direct suturing (n = 2), or pedicled pericardium (n = 1). Of the 7 perforator flaps, 4 were internal mammary-artery, two dorsal intercostal-artery, and one supraclavicular-artery flaps. After a median follow-up of 100 months, 2 patients died on early postoperative course from NEAF repair failure and 3 from late NEAF recurrence at 4, 11, and 33 months. Among the remaining 6 patients, 1 died from local tumoral recurrence at 13 months, 1 was last on follow-up at 27 months, alive and eating normally. The other 4 were free from NEAF recurrence and dysphagia or swallowing disorder at 50 months' follow-up. These 4 results were obtained thanks to perforator flap interposition and airway resection anastomosis. Conclusions: Surgical NEAF repair using perforator flap interposition may provide satisfactory long-term function after strong prehabilitation.
RESUMEN
Background: Lung or heart-lung transplantation (LT/HLT) for severe pulmonary hypertension (PH) as the primary disease indication carries a high risk of waiting list mortality and post-transplant complications. France and the UK both have coordinated PH patient services but with different referral pathways for accessing LT services. Methods: We conducted a comparative analysis of adult PH patients listed for LT/HLT in the UK and France. Results: We included 211 PH patients in France (2006-2018) and 170 in the UK (2010-2019). Cumulative incidence of transplant, delisting and waiting list death within 3â years were 81%, 4% and 11% in France versus 58%, 10% and 15% in the UK (p<0.001 for transplant and delisting; p=0.1 for death). Median non-priority waiting time was 45â days in France versus 165â days in the UK (p<0.001). High-priority listing occurred in 54% and 51% of transplanted patients respectively in France and the UK (p=0.8). Factors associated with achieving transplantation related to recipients' height, male sex, clinical severity and priority listing status. 1-year post-transplant survival was 78% in France and 72% in the UK (p= 0.04). Conclusion: Access to transplantation for PH patients is better in France than in the UK where more patients were delisted due to clinical deterioration because of longer waiting time. High rates of priority listing occurred in both countries. Survival for those achieving transplantation was slightly better in France. Ensuring optimal outcomes after transplant listing for PH patients is challenging and may involve early listing of higher risk patients, increasing donor lung utilisation and improving allocation rules for these specific patients.
RESUMEN
PURPOSE: To describe the technique and review the oncological and surgical results of the En Bloc resection assisted by retroperitoneal laparoscopy in a single prone position for tumors in the thoracolumbar region. METHODS: Monocentric retrospective case study. Procedure was performed in a single prone position by a dual team of spine and thoracovascular surgeons. An endoscopic balloon was inflated in the right retroperitoneal cavity. A plan was developed between the anterior spine and vena cava as well as abdominal aorta with segmental vessels ligation. Structures at risk were safely protected under endoscopy during horizontal or sagittal osteotomies. RESULTS: From 2021, seven patients aged a median 52 years old (range, 34-67) were included. Involved spinal segments went from T11 to L3. Surgery was aborted in one case due to massive bleeding and ventilating difficulties. There were two partial and four total vertebral resections. Median operating duration and estimated blood loss were 405 min (range, 360-540) and 2.1 L (range, 1.2-19), respectively. Postoperative complications consisted of 1 urinary infection; 1 transient urinary retention; 1 posterior wound infection; 1 pneumothorax; 1 persistent partial motor deficit; 1 transient confusion; 1 pulmonary embolism; 1 CSF leak; 1 subdural hematoma; 1 retroperitoneal lymphocele. All margins were uncontaminated. All patients were alive and ambulatory at last follow-up. CONCLUSION: Early results suggest En Bloc resection assisted by retroperitoneal videoscopy in tumors from T11 to L3/4 disk space is feasible, less invasive and safe. Careful surgical planning and experience in endoscopic vascular surgery are mandatory.