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PURPOSE: To investigate clinical and radiological differences between kidney metastases to the lung (RCCM +) and metachronous lung cancer (LC) detected during follow-up in patients surgically treated for Renal Cell Carcinoma (RCC). METHODS: cM0 surgically-treated RCC who harbored a pulmonary mass during follow-up were retrospectively scrutinized. Univariate logistic regression assessed predictive features for differentiating between LC and RCCM + . Multivariable analyses (MVA) were fitted to predict factors that could influence time between detection and histological diagnosis of the pulmonary mass, and how this interval could impact on survivals. RESULTS: 87% had RCCM + and 13% had LC. LC were more likely to have smoking history (75% vs. 29%, p < 0.001) and less aggressive RCC features (cT1-2: 94% vs. 65%, p = 0.01; pT1-2: 88% vs. 41%, p = 0.02; G1-2: 88% vs. 37%, p < 0.001). The median interval between RCC surgery and lung mass detection was longer between LC (55 months [32.8-107.2] vs. 20 months [9.0-45.0], p = 0.01). RCCM + had a higher likelihood of multiple (3[1-4] vs. 1[1-1], p < 0.001) and bilateral (51% vs. 6%, p = 0.002) pulmonary nodules, whereas LC usually presented with a solitary pulmonary nodule, less than 20 mm. Univariate analyses revealed that smoking history (OR:0.79; 95% CI 0.70-0.89; p < 0.001) and interval between RCC surgery and lung mass detection (OR:0.99; 95% CI 0.97-1.00; p = 0.002) predicted a higher risk of LC. Conversely, size (OR:1.02; 95% CI 1.01-1.04; p = 0.003), clinical stage (OR:1.14; 95% CI 1.06-1.23; p < 0.001), pathological stage (OR:1.14; 95% CI 1.07-1.22; p < 0.001), grade (OR:1.15; 95% CI 1.07-1.23; p < 0.001), presence of necrosis (OR:1.17; 95% CI 1.04-1.32; p = 0.01), and lymphovascular invasion (OR:1.18; 95% CI 1.01-1.37; p = 0.03) of primary RCC predicted a higher risk of RCCM + . Furthermore, number (OR:1.08; 95% CI 1.04-1.12; p < 0.001) and bilaterality (OR:1.23; 95% CI 1.09-1.38; p < 0.001) of pulmonary lesions predicted a higher risk of RCCM + . Survival analysis showed a median second PFS of 10.9 years (95% CI 3.3-not reached) for LC and a 3.8 years (95% CI 3.2-8.4) for RCCM + . The median OS time was 6.5 years (95% CI 4.4-not reached) for LC and 6 years (95% CI 4.3-11.6) for RCCM + . CONCLUSIONS: Smoking history, primary grade and stage of RCC, interval between RCC surgery and lung mass detection, and number of pulmonary lesions appear to be the most valuable predictors for differentiating new primary lung cancer from RCC progression.
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Carcinoma de Células Renales , Progresión de la Enfermedad , Neoplasias Renales , Neoplasias Pulmonares , Neoplasias Primarias Secundarias , Humanos , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/cirugía , Carcinoma de Células Renales/cirugía , Carcinoma de Células Renales/patología , Carcinoma de Células Renales/secundario , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Femenino , Anciano , Neoplasias Primarias Secundarias/patología , Neoplasias Primarias Secundarias/epidemiología , NefrectomíaRESUMEN
The aim of this study was to assess the impact of BMI on perioperative outcomes in patients undergoing VATS lobectomy or segmentectomy. Data from 5088 patients undergoing VATS lobectomy or segmentectomy, included in the VATS Group Italian Registry, were collected. BMI (kg/m2) was categorized according to the WHO classes: underweight, normal, overweight, obese. The effects of BMI on outcomes (complications, 30-days mortality, DFS and OS) were evaluated with a linear regression model, and with a logistic regression model for binary endpoints. In overweight and obese patients, operative time increased with BMI value. Operating room time increased by 5.54 minutes (S.E. = 1.57) in overweight patients, and 33.12 minutes (S.E. = 10.26) in obese patients (P < 0.001). Compared to the other BMI classes, overweight patients were at the lowest risk of pulmonary, acute cardiac, surgical, major, and overall postoperative complications. In the overweight range, a BMI increase from 25 to 29.9 did not significantly affect the length of stay, nor the risk of any complications, except for renal complications (OR: 1.55; 95% CI: 1.07-2.24; P = 0.03), and it reduced the risk of prolonged air leak (OR: 0.8; 95% CI: 0.71-0.90; P < 0.001). 30-days mortality is higher in the underweight group compared to the others. We did not find any significant difference in DFS and OS. According to our results, obesity increases operating room time for VATS major lung resection. Overweight patients are at the lowest risk of pulmonary, acute cardiac, surgical, major, and overall postoperative complications following VATS resections. The risk of most postoperative complications progressively increases as the BMI deviates from the point at the lowest risk, towards both extremes of BMI values. Thirty days mortality is higher in the underweight group, with no differences in DFS and OS.
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Sobrepeso , Delgadez , Humanos , Sobrepeso/complicaciones , Índice de Masa Corporal , Delgadez/complicaciones , Neumonectomía/efectos adversos , Cirugía Torácica Asistida por Video/efectos adversos , Resultado del Tratamiento , Obesidad/complicaciones , Complicaciones Posoperatorias/etiología , Estudios RetrospectivosRESUMEN
X-linked chronic granulomatous disease is a rare disease caused by mutations in the CYBB gene. While more extensive knowledge is available on genetics, pathogenesis, and possible therapeutic options, mitochondrial activity and its implications on patient monitoring are still not well-characterized. We have developed a novel protocol to study mitochondrial activity on whole blood of XCGD patients before and after transplantation, as well as on XCGD carriers. Here we present results of these analyses and of the restoration of mitochondrial activity in hyperinflamed X-linked Chronic Granulomatous Disease after hematopoietic stem cell transplantation. Moreover, we show a strong direct correlation between mitochondrial activity, chimerism, and DHR monitored before and after transplantation and in XCGD carriers. In conclusion, based on these findings, we suggest testing this new ready-to-use marker to better characterize patients before and after treatment and to investigate disease expression in carriers.
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Enfermedad Granulomatosa Crónica , Trasplante de Células Madre Hematopoyéticas , Humanos , Enfermedad Granulomatosa Crónica/diagnóstico , Enfermedad Granulomatosa Crónica/genética , Enfermedad Granulomatosa Crónica/terapia , Quimerismo , Fagocitos , HeterocigotoRESUMEN
The novel coronavirus (Covid-19), as of January 2021, infected more than 85 million people worldwide, causing the death of about 1,840 million. Italy had more than 2 million infected and about 75,000 deaths. Many hospitals reduced their ordinary activity by up to 80%, to leave healthcare staff, wards, and intensive care unit (ICU) beds available for the significant number of Covid-19 patients. All this resulted in a prolonged wait for hospitalization of all other patients, including those with non-small cell lung cancer (NSCLC) eligible for surgery. The majority of thoracic surgery departments changed the clinical-therapeutic path of patients, re-adapting procedures based on the needs dictated by the pandemic while not delaying the necessary treatment. The establishment of Covid-19-free hub centers allowed some elective surgery in NSCLC patients but most of the operations were delayed. The technology has partly facilitated patients' visits through telemedicine when security protocols have prevented face-to-face assessments. Multidisciplinary consultations had to deal also with the priority of the NSCLC cases discussed. Interpretation of radiologic exams had to take into account the differential diagnosis with Covid-19 infection. All the knowledge and experience of the past months reveal that the Covid-19 pandemic has not substantially changed the indications and type of surgical treatment in NSCLC. However, the diagnostic process has become more complex, requiring rigorous planning, thus changing the approach with the patients.
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BACKGROUND: The deeper knowledge of non-small-cell lung cancer (NSCLC) biology and the discovery of driver molecular alterations have opened the era of precision medicine in lung oncology, thus significantly revolutionizing the diagnostic and therapeutic approach to NSCLC. In Italy, however, molecular assessment remains heterogeneous across the country, and numbers of patients accessing personalized treatments remain relatively low. Nationwide programs have demonstrated that the creation of consortia represent a successful strategy to increase the number of patients with a molecular classification. PATIENTS AND METHODS: The Alliance Against Cancer (ACC), a network of 25 Italian Research Institutes, has developed a targeted sequencing panel for the detection of genomic alterations in 182 genes in patients with a diagnosis of NSCLC (ACC lung panel). One thousand metastatic NSCLC patients will be enrolled onto a prospective trial designed to measure the sensitivity and specificity of the ACC lung panel as a tool for molecular screening compared to standard methods. RESULTS AND CONCLUSION: The ongoing trial is part of a nationwide strategy of ACC to develop infrastructures and improve competences to make the Italian research institutes independent for genomic profiling of cancer patients.
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Carcinoma de Pulmón de Células no Pequeñas/diagnóstico , Neoplasias Pulmonares/diagnóstico , Carcinoma de Pulmón de Células no Pequeñas/genética , Detección Precoz del Cáncer , Genómica , Humanos , Italia , Neoplasias Pulmonares/genética , Tamizaje Masivo/métodos , Medicina de Precisión/métodos , Estudios Prospectivos , Sensibilidad y EspecificidadRESUMEN
Background and objectives: The histological classification and staging of thymic tumors remains a matter of debate. The correlation of computed tomography (CT) parameters with tumor histology and stage also still has to be completely assessed. The aim of this study was therefore to analyze the correlation of radiological parameters with histological and staging classifications of thymomas evaluating their prognostic role. Methods: Data of 50 patients with thymoma submitted to a complete surgical treatment between 2005 and 2015 were retrospectively analyzed. Tumors were classified according to the WHO and Suster and Moran (S&M) histological classifications and to the Masaoka-Koga and tumor, node and metastases (TNM) staging systems. The correlation of CT features with histology and stage and the prognostic role of histopathological and radiological features were assessed. Results: Five-year overall (OS) and disease-free survival (DFS) were 90.3% and 81.1%, respectively. A significant correlation of DFS with the Masaoka-Koga (p = 0.001) and TNM staging systems (p = 0.002) and with the S&M (p = 0.02) and WHO histological classifications (p = 0.04) was observed. CT scan features correlated with tumor stage, histology and prognosis. Moderately differentiated tumors (WHO B3) had a significantly higher incidence of irregular shape and contours (p = 0.002 and p = 0.001, respectively) and pericardial contact (p = 0.036). A larger tumor volume (p = 0.03) and a greater length of pleural contact (p = 0.04) adversely influenced DFS. The presence of pleural (p < 0.001) or lung invasion (p = 0.02) and of pleural effusion (p = 0.004) was associated with a significantly worse OS. Conclusions: Pre-operative CT scan parameters correlate with stage and histology, and have a prognostic role in surgically treated thymomas.
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Timoma , Neoplasias del Timo , Humanos , Estadificación de Neoplasias , Pronóstico , Estudios Retrospectivos , Timoma/diagnóstico por imagen , Timoma/patología , Timoma/cirugía , Neoplasias del Timo/diagnóstico por imagen , Neoplasias del Timo/cirugía , Tomografía Computarizada por Rayos XRESUMEN
Background According to the current pathological classification, lung adenocarcinoma includes histological subtypes with significantly different prognoses, which may require specific surgical approaches. The aim of the study was to assess the role of CT and PET parameters in stratifying patients with stage I adenocarcinoma according to prognosis. Patients and methods Fifty-eight patients with pathological stage I lung adenocarcinoma who underwent surgical treatment were retrospectively reviewed. Adenocarcinoma in situ and minimally-invasive adenocarcinoma were grouped as non-invasive adenocarcinoma. Other histotypes were referred as invasive adenocarcinoma. CT scan assessed parameters were: ground glass opacity (GGO) ratio, tumour disappearance rate (TDR) and consolidation diameter. The prognostic role of the following PET parameters was also assessed: standardized uptake value (SUV) max, SUVindex (SUVmax to liver SUVratio), metabolic tumour volume (MTV), total lesion glycolysis (TLG). Results Seven patients had a non-invasive adenocarcinoma and 51 an invasive adenocarcinoma. Five-year disease-free survival (DFS) and cancer-specific survival (CSS) for non-invasive and invasive adenocarcinoma were 100% and 100%, 70% and 91%, respectively. Univariate analysis showed a significant difference in SUVmax, SUVindex, GGO ratio and TDR ratio values between non-invasive and invasive adenocarcinoma groups. Optimal SUVmax, SUVindex, GGO ratio and TDR cut-off ratios to predict invasive tumours were 2.6, 0.9, 40% and 56%, respectively. TLG, SUVmax, SUVindex significantly correlated with cancer specific survival. Conclusions CT and PET scan parameters may differentiate between non-invasive and invasive stage I adenocarcinomas. If these data are confirmed in larger series, surgical strategy may be selected on the basis of preoperative imaging.
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Adenocarcinoma del Pulmón/diagnóstico por imagen , Adenocarcinoma del Pulmón/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Adenocarcinoma del Pulmón/mortalidad , Adenocarcinoma del Pulmón/cirugía , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Estadificación de Neoplasias , Pronóstico , Radiofármacos , Estudios Retrospectivos , Tasa de Supervivencia , Carga TumoralRESUMEN
Primary chondrosarcoma of the trachea is an extremely rare tumor. We report two cases of tracheal chondrosarcoma describing the role of surgical and conservative treatment. Endoscopic treatment with rigid bronchoscopy was performed in both patients to restore airway patency and obtain histological specimens for diagnosis. One of the patients subsequently underwent successful tracheal resection and reconstruction. The other patient, who had a contraindication to surgical treatment due to associated diseases underwent iterative endoscopic LASER treatment and is alive three years after the first diagnosis. Surgical treatment remains the treatment of choice of tracheal chondrosarcoma. When surgery is contraindicated endoscopic treatment may allow relatively longterm survival due to the slow growth of these tumors.
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Condrosarcoma/terapia , Terapia por Láser , Neoplasias de la Tráquea/terapia , Broncoscopía , Condrosarcoma/diagnóstico , Endoscopía , Humanos , Procedimientos de Cirugía Plástica , Neoplasias de la Tráquea/diagnósticoRESUMEN
Endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive procedure used for lung cancer diagnosis and staging. Several aspects, including pathological analysis, may impact on its diagnostic accuracy. Differences in diagnostic accuracy between the different specimen processing techniques have not been demonstrated. Cytological slides are generally adequate for diagnosis, subtyping and genotyping. However, some pathological laboratories may require cell blocks or histological core biopsies for a complete molecular profiling. Rapid on-site evaluation (ROSE) is a technique for immediate evaluation of samples obtained with EBUS-TBNA. The aims of ROSE are to increase sampling adequacy, improving diagnostic yield of EBUS-TBNA and ensuring collection of adequate material for ancillary studies. However, the reported data on the impact of ROSE in the diagnostic yield of EBUS-TBNA and in lung cancer diagnosis and staging are controversial. Some series reported a valuable contribution of ROSE to diagnosis and staging of lung cancer and a high concordance between ROSE and the final diagnosis. However, randomized trials failed in finding differences in diagnostic yield between EBUS-TBNA performed with and without ROSE. The yield of EBUS-TBNA for molecular analyses varies between 72% and 98%, and ROSE may warrant the collection of adequate material for molecular profiling. In lung cancer diagnosis and staging a recommended number of three to four passes during EBUS-TBNA at each target is a minimum requirement to obtain enough material for molecular analysis. The use of ROSE may reduce the number of passes for molecular profiling and the number of additional invasive diagnostic procedures. EBUS-TBNA is a procedure with a high accuracy rate and ROSE may contribute to a further improvement of the results. The possibility to avoid additional invasive procedure is an important advantage leading to an overall improvement of patient care.
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OBJECTIVES: A significant number of patients with thymoma develop a recurrence after surgery. The results of previous studies analyzing therapeutic strategies in the treatment of recurrences have been controversial. Aim of the study was to evaluate the role of surgery in the treatment of thymoma recurrences assessing prognostic factors related with survival. METHODS: Between January 1993 and September 2018, 27 of 161 patients had a recurrence after complete thymoma resection. Twenty-one (13%) underwent surgical treatment of the recurrence. Primary tumor stage was: I in a patient, IIa in five, IIb in nine and III in six. The recurrence was regional in all patients and both regional and distant in four. WHO histological classification was: A in two patients, B1 in two, B2 in seven and B3 in 10 patients. RESULTS: Median disease-free survival from surgical treatment of the primary tumor and recurrence was 44 months (1-124). Thirty-eight operations were performed (range 1-5). Complete resection rate was 66%. Adjuvant radiotherapy and chemotherapy after surgical treatment of recurrences were performed respectively in ten and nine patients, including eight patients with multiple recurrences. Five and 10-year tumor-specific survival from recurrence was, respectively, 79% and 66%. Survival analysis showed a significant correlation between survival and WHO classification, with a significantly lower survival in patients with B3 tumors in comparison with A to B2 tumors (p = 0.026). CONCLUSIONS: Long-term survival was observed following surgical treatment of thymoma recurrences. These results were observed even after multiple recurrences when surgical treatment was performed within a multimodality oncological approach.
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Recurrencia Local de Neoplasia/cirugía , Timoma/cirugía , Neoplasias del Timo/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Supervivencia sin Enfermedad , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Pronóstico , Radioterapia Adyuvante , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
Dendritic cells (DCs) initiate adaptive immune responses after their migration to secondary lymphoid organs. The LXR ligands/oxysterols and the RXR ligand 9-cis Retinoic Acid (9-cis RA) were shown to dampen DC migration to lymphoid organs through the inhibition of CCR7 expression. We performed transcriptomics of DCs undergoing maturation in the presence of the LXR ligand 22R-Hydroxycholesterol (22R-HC). The analysis highlighted more than 1500 genes modulated by 22R-HC treatment, including the triggering receptor expressed on myeloid cells (TREM)-1, which was found markedly up-regulated. We tested the effect of other nuclear receptor ligands (NRL) and we reported the induction of TREM-1 following RXR, RAR and VDR activation. From a functional point of view, triggering of TREM-1 induced by retinoids increased TNFα and IL-1ß release, suggesting an active role of NRL-activated TREM-1+ DCs in inflammation-driven diseases, including cancer. Consistently with this hypothesis we detected DCs expressing TREM-1 in pleural effusions and ascites of cancer patients, an observation validated by the induction of TREM-1, LXR and RAR target genes when monocyte-DCs were activated in the presence of tumor-conditioned fluids. Finally, we observed a better control of LLC tumor growth in Trem-1-/- bone marrow chimera mice as compared to wild type chimera mice. Future studies will be necessary to shed light on the mechanism of TREM-1 induction by distinct NRL, and to characterize the role of TREM-1+ DCs in tumor growth.
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BACKGROUND: The introduction of the new TNM staging system for thymic epithelial malignancies produced a significant increase in the proportion of patients with stage I disease. The identification of new prognostic factors could help to select patients for adjuvant therapies based on their risk of recurrence. Neutrophil-to-lymphocyte ratio (NLR) has recently gained popularity as reliable prognostic biomarker in many different solid tumors. The aim of this study is to assess the utility of NLR evaluation as a prognostic marker in patients with surgically-treated thymoma. METHODS: A retrospective analysis was conducted among patients who underwent resection for thymoma in a single center. Patients were divided in two groups, under (low-NLR-Group = 47 patients, 60%) and above (high-NLR-Group = 32 patients, 40%) a ROC-derived NLR cut-off (2.27). Associations with clinical-pathological variables were analyzed; disease-free survival (DFS) was identified as the primary endpoint. RESULTS: Between 2007 and 2017, 79 patients had surgery for thymoma. Overall 5-year DFS was 80%. Univariate survival analysis demonstrated that NLR was significantly related to DFS when patients were stratified for TNM stage (p = 0.043). Five-year DFS in the low-NLR-Group and in the high-NLR-Group were respectively 100 and 84% in stage I-II, and 66 and 0% in stage III. TNM stage resulted as the only independent prognostic factor at multivariate analysis, with hazard ratio of 3.986 (95% CI 1.644-9.665, p = 0.002). CONCLUSIONS: High preoperative NLR seems to be associated to a shorter DFS in patients submitted to surgery for thymoma and stratified for TNM stage.
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Linfocitos/patología , Neutrófilos/patología , Timoma/cirugía , Neoplasias del Timo/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/sangre , Femenino , Humanos , Estimación de Kaplan-Meier , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estadificación de Neoplasias , Pronóstico , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Timectomía , Timoma/sangre , Timoma/diagnóstico , Timoma/patología , Neoplasias del Timo/sangre , Neoplasias del Timo/diagnóstico , Neoplasias del Timo/patologíaRESUMEN
INTRODUCTION: In the search of new therapeutical strategies against non-small-cell lung cancer (NSCLC), the identification of new prognostic factors is crucial. OBJECTIVES: In this study, we analyzed the prognostic value of the liver X receptor-alpha (LXR-alpha), a nuclear receptor of a family of cholesterol derivatives called oxysterols, in patients with radically resected NSCLC. METHODS: We retrospectively reviewed 140 stage II and III surgically treated NSCLC patients that were grouped by percentage of LXR-alpha-positive cells value above or below its median value. Tumor-related survival was evaluated as primary end point. RESULTS: The 5-year overall and tumor-related survival rates were 40% and 46%, respectively. The median percentage of LXR-alpha-positive cells was 20%. Patients with stage II NSCLC had higher LXR-alpha values than those with stage III (P = .04). Univariate analysis demonstrated that both TNM stage and LXR-alpha were significantly related to tumor-related survival (P = .006 and P = .004, respectively). The 5-year tumor-related survival rates in stage II and III NSCLC were 56% and 34%, respectively. The 5-year tumor-related survival rates in high and low LXR-alpha value were 57% and 32%, respectively. The multivariate analysis showed that both TNM stage and LXR-alpha were independent prognostic factors (P = .01 and P = .007, respectively) with hazard ratio of 1.92 and 0.49, respectively. CONCLUSION: LXR-alpha seems to be an independent prognostic factor indicating a better survival in completely resected stage II and III NSCLC patients.
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Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Receptores X del Hígado/metabolismo , Neoplasias Pulmonares/metabolismo , Estadificación de Neoplasias , Neumonectomía , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/diagnóstico , Carcinoma de Pulmón de Células no Pequeñas/cirugía , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Italia/epidemiología , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/epidemiología , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Factores de TiempoRESUMEN
Liposarcomas are rare mesenchymal tumors that usually develop in lower extremities or retroperitoneum; cervico-mediastinal presentation is quite uncommon. These neoplasms are commonly diagnosed at a late stage because they remain asymptomatic until nearby structures are compressed. This makes radical excision particularly challenging. To date, alternative chemoradiotherapy protocols have not yet been standardized. We report a case of a 55-year-old man with a right laterocervical mass and without substantial symptoms. Fine-needle aspiration cytology results were compatible with a well-differentiated liposarcoma. Contrast-enhanced magnetic resonance imaging revealed the magnitude of the mass, which was expanding into the mediastinum; displacing the trachea, esophagus, cervical neurovascular bundle, and thoracic aorta; and encasing the brachiocephalic artery. Compression of the left brachiocephalic vein resulted in a focal enhancement spot in the fourth liver segment, the expression of superior vena cava compression, which can promote the development of collateral venous pathways, such as the caval-mammary-phrenic-hepatic capsule-portal venous pathway. The mass was successfully excised by a team of surgical subspecialists (otorhinolaryngologists and thoracic, cardiac, and vascular surgeons). Adjuvant tomotherapy was administered to increase local disease control. The patient remained disease-free 38 months postoperatively. This case underlines the importance of accurate preoperative radiologic evaluation in patients presenting with neck masses but without substantial symptoms. Because of the involvement of many critical structures, the cooperation of many surgical subspecialties is mandatory to achieve a satisfying oncologic outcome.
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Neoplasias de Cabeza y Cuello/cirugía , Liposarcoma/cirugía , Neoplasias del Mediastino/cirugía , Grupo de Atención al Paciente , Carga Tumoral , Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Neoplasias de Cabeza y Cuello/patología , Humanos , Liposarcoma/diagnóstico por imagen , Liposarcoma/patología , Imagen por Resonancia Magnética/métodos , Masculino , Neoplasias del Mediastino/diagnóstico por imagen , Persona de Mediana EdadRESUMEN
BACKGROUND: IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by serum IgG4 elevation and tissue infiltration of IgG4-positive plasma cells. Substantial overlap between IgG4-RD and antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) exists in terms of organ involvement and histopathological features. A positive ANCA assay is regarded as a highly specific finding in favor of an AAV, and generally influences away from a diagnosis of IgG4-RD. Recent reports, however, have raised the possibility that some patients with IgG4-RD are ANCA positive, thus suggesting reconsideration of the role of ANCA in the diagnostic workup. In the present work, we describe the first case of concomitant biopsy-proven IgG4-RD and granulomatosis with polyangiitis (GPA), demonstrating antiproteinase 3 (PR3) ANCA of the IgG4 subclass in the patient's serum. We also review the literature in order to provide clinicians with tools for interpreting ANCA positivity in IgG4-RD patients. CASE SUMMARY: A 51-year-old woman was referred for left exopthalmos due to lacrimal gland enlargement and increased serum IgG4 concentration. IgG4-RD was suspected and further imaging studies disclosed multiple pulmonary masses in the right lung. Histological analysis of the left lacrimal gland was diagnostic for IgG4-RD, but lung biopsy showed typical features of GPA. ANCA assay was positive for anti-PR3 antibodies. Further immunofluorescence studies demonstrated anti-PR3 antibodies of IgG1 and IgG4 subclass. Treatment with rituximab induced swift remission of both IgG4-RD and GPA manifestations. We identified 9 other reports of patients with IgG4-RD and positive ANCA in the English literature, 5 cases with biopsy-proven IgG4-RD and 4 cases in whom IgG4-RD was diagnosed presumptively. Four patients had also histological evidence of concomitant AAV. CONCLUSION: The present work demonstrates that ANCA positivity in patients with biopsy-proven IgG4-RD should prompt the exclusion of a concomitant vasculitic process; a positive ANCA does not exclude the diagnosis of IgG4-RD; confirmation through immunoenzymatic assays of the ANCA specificity, clinical-pathological correlation, and histopathological evaluation remain crucial steps for the differential diagnosis between AAV and IgG4-RD.
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Anticuerpos Anticitoplasma de Neutrófilos/sangre , Granulomatosis con Poliangitis/diagnóstico , Inmunoglobulina G/sangre , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Biopsia , Diagnóstico Diferencial , Femenino , Granulomatosis con Poliangitis/sangre , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/inmunología , Humanos , Persona de Mediana Edad , Escleritis/etiologíaRESUMEN
BACKGROUND: Primary spontaneous pneumothorax (PSP) is a relatively rare condition in the pediatric population lacking of specific recommendations regarding the management. Video-assisted thoracoscopic surgery (VATS) has gained widespread consensus during the last 10 years. We retrospectively reviewed our experience of VATS in the treatment of pediatric patients affected by PSP in terms of timing of surgery, operative technique, and postoperative outcome. METHODS: Between 1998 and 2014, 58 pediatric patients were treated for PSP. Treatment consisted in pulmonary apicectomy in all patients and pleurodesis. Patients received either apical pleurectomy and mechanical pleurodesis or mechanical pleurodesis alone. RESULTS: Mean age was 16.6 ± 1.6 years (range 10-18) with a male/female ratio of 5:1. Seventeen patients underwent surgery after the first episode of PSP. Apical pleurectomy and mechanical pleurodesis was performed in 30 patients while others received mechanical pleurodesis alone. Conversion to open surgery was needed in four procedures (6.9%). Postoperative complications occurred in 1.7% of cases. Sixteen patients received more than one procedure for contralateral pneumothorax (15 cases) and postoperative recurrence (1 case). The mean follow-up was 95 ± 63 months. Recurrence rate was 12.1%. Univariate analysis showed that recurrence was significantly correlated with younger age (P = 0.044) and postoperative chest tube (P = 0.027). Both univariate and multivariate analysis showed that apical pleurectomy did not prevent recurrences. CONCLUSIONS: VATS is an effective procedure for PSP in pediatric patients. Apical pleurectomy does not seem to prevent recurrence. Due to the increased risk of recurrence of PSP in younger patients, indication to VATS after the first episode might be considered. Pediatr Pulmonol. 2016;51:713-716. © 2016 Wiley Periodicals, Inc.
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Neumotórax/cirugía , Cirugía Torácica Asistida por Video , Adolescente , Adulto , Tubos Torácicos , Niño , Femenino , Humanos , Masculino , Pleurodesia , Complicaciones Posoperatorias , Recurrencia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Solitary fibrous tumours of the pleura are rare neoplasms. These tumours are generally asymptomatic and incidentally diagnosed. Symptoms, if present, are nonspecific such as cough, dyspnea, and chest pain. This report describes the case of a 38-year-old woman admitted to our department after the onset of a right massive spontaneous haemothorax requiring emergency surgical treatment. Intraoperatively a bleeding pleural mass was found to be the cause of the haemothorax. The tumour was successfully resected and the patient made an uneventful recovery. Histological examination revealed the mass to be a solitary fibrous tumour of the pleura.
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Primary tumours of the brachial plexus are rare entities. They usually present as extrathoracic masses located in the supraclavicular region. This report describes two cases of benign schwannomas arising from the brachial plexus with an intrathoracic growth. In the first case the tumour was completely intrathoracic and it was hardly removed through a standard posterolateral thoracotomy. In the second case the tumour presented as a cervicomediastinal lesion and it was resected through a one-stage combined supraclavicular incision followed by left video-assisted thoracoscopic surgery. A brachial plexus tumour should be suspected not only in patients with a supraclavicular or cervicomediastinal mass but also in those with intrathoracic apical lesions. A preoperative magnetic resonance imaging study of brachial plexus should be performed in such cases in order to plan the correct surgical approach.