pleura

胸膜
  • 文章类型: Case Reports
    孤立性纤维性肿瘤是罕见的间充质肿瘤,通常发生在胸膜。子宫颈孤立性纤维瘤并不常见。我们报告了第一例患者,该患者因阴道壁腺癌接受了全子宫切除术,并被发现在子宫颈旁子宫并发孤立性纤维瘤。
    一名51岁女性因接触性出血入院。妇科检查发现阴道壁上有3.0×1.0厘米的结节,阴道镜活检显示阴道壁腺癌。在推荐的分期检查之后,患者接受了全子宫切除术,双附件切除术,盆腔淋巴结清扫术,阴道壁切除术.手术期间,在左侧宫颈旁区的肿块中间发现约2×2cm的结节。随后的术后组织病理学检查证实宫颈孤立性纤维瘤伴阴道壁腺癌。患者住院后随访46个月,未观察到复发或远处转移。
    在极少数情况下,孤立性纤维瘤可在宫颈或阴道壁形成大肿块。在手术前和手术中,它们很容易被误诊为良性或恶性宫颈肿瘤。术后病理及免疫组化有助于诊断。大多数孤立性纤维性宫颈肿瘤是良性的,偶尔有低恶性潜能,手术治疗是可行和有效的。
    UNASSIGNED: Solitary fibrous tumors are rare mesenchymal tumors that typically occur in the pleura. Solitary fibrous tumors of the uterine cervix are uncommon. We report the first case of a patient who underwent total hysterectomy for vaginal wall adenocarcinoma and was found to have a concurrent solitary fibrous tumor in the paracervical-uterus.
    UNASSIGNED: A 51-year-old woman was admitted to our hospital due to contact bleeding. A gynecological examination revealed nodules of 3.0 × 1.0 cm on the vaginal wall, and a colposcopy with biopsy revealed adenocarcinoma of the vaginal wall. After the recommended staging examinations, the patient underwent total hysterectomy, double adnexectomy, pelvic lymph node dissection, and vaginal wall resection. During surgery, a nodule measuring approximately 2 × 2 cm was found in the middle of the mass in the left paracervical region. Subsequent postoperative histopathological examination confirmed an solitary fibrous tumor of the uterine cervix with adenocarcinoma of the vaginal wall. The patient was followed up for 46 months after hospitalization, and no recurrence or distant metastases were observed.
    UNASSIGNED: In rare cases, solitary fibrous tumors may form large masses in the cervical or vaginal wall. They can easily be misdiagnosed as benign or malignant cervical tumors before and during surgery. Postoperative pathology and immunohistochemistry are helpful for diagnosis. Most solitary fibrous cervical tumors are benign, occasionally with low malignant potential, and surgical treatment is feasible and effective.
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  • 文章类型: Journal Article
    胸膜腔内的出血可能导致持续的血块形成,称为保留血胸(RH)。RH容易组织,这损害了有效的排水,导致肺限制和呼吸困难。胸膜内纤溶疗法用于清除持续性组织凝块代替手术,但是纤溶素的选择,交付策略,和剂量尚未确定。我们使用最近建立的RH兔模型来测试胸膜腔内递送单链尿激酶(scuPA)是否可以最有效地清除RH。scuPA,或单链组织纤溶酶原激活剂(sctPA),在第7天以间隔8小时的一个或两个剂量通过胸廓造口管递送。使用经胸超声检查评估胸膜凝块溶解,胸部计算机断层扫描,二维和凝块位移测量,和总体分析。两种剂量的scuPA(1mg/kg)在解决RH和促进胸腔积液(PF)的引流方面比2mg/kg的推注剂量更有效。scuPAPF中的红细胞计数,或sctPA处理的兔子是相当的,未观察到明显的胸膜内出血。两种纤维蛋白溶素在清除血凝块和促进胸膜引流方面同样有效。炎症和组织的生物标志物在来自两组的PF中同样是相当的。研究结果表明,单药治疗可能有效清除RH;然而,胸膜腔内scuPA的临床优势仍有待未来的临床试验确定。
    Bleeding within the pleural space may result in persistent clot formation called retained hemothorax (RH). RH is prone to organization, which compromises effective drainage, leading to lung restriction and dyspnea. Intrapleural fibrinolytic therapy is used to clear the persistent organizing clot in lieu of surgery, but fibrinolysin selection, delivery strategies, and dosing have yet to be identified. We used a recently established rabbit model of RH to test whether intrapleural delivery of single-chain urokinase (scuPA) can most effectively clear RH. scuPA, or single-chain tissue plasminogen activator (sctPA), was delivered via thoracostomy tube on day 7 as either one or two doses 8 h apart. Pleural clot dissolution was assessed using transthoracic ultrasonography, chest computed tomography, two-dimensional and clot displacement measurements, and gross analysis. Two doses of scuPA (1 mg/kg) were more effective than a bolus dose of 2 mg/kg in resolving RH and facilitating drainage of pleural fluids (PF). Red blood cell counts in the PF of scuPA, or sctPA-treated rabbits were comparable, and no gross intrapleural hemorrhage was observed. Both fibrinolysins were equally effective in clearing clots and promoting pleural drainage. Biomarkers of inflammation and organization were likewise comparable in PF from both groups. The findings suggest that single-agent therapy may be effective in clearing RH; however, the clinical advantage of intrapleural scuPA remains to be established by future clinical trials.
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  • 文章类型: Case Reports
    食管胸膜瘘(EPF)是一种罕见的并发症,通常与潜在的食管恶性肿瘤有关。我们介绍了一名64岁的男性,他患有左侧脓性气胸,并被诊断为食管癌继发的EPF。影像学检查显示液气胸,食管-肺瘘与胸膜腔连通。通过口服对比剂的计算机断层扫描(CT)扫描证实了诊断。强调这种模式在诊断EPF中的实用性。患者进一步转诊至外科肿瘤学团队进行食管切除和瘘管闭合。
    Esophagopleural fistula (EPF) is a rare complication often associated with underlying esophageal malignancies. We present the case of a 64-year-old male who presented with left-sided pyopneumothorax and was diagnosed with EPF secondary to esophageal carcinoma. Imaging studies revealed a hydropneumothorax with an esophageal-pulmonary fistula communicating with the pleural cavity. The diagnosis was confirmed through computed tomography (CT) scan with oral contrast administration, highlighting the utility of this modality in diagnosing EPF. The patient was further referred to the surgical oncology team for esophageal resection and fistula closure.
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  • 文章类型: Journal Article
    背景:内科胸腔镜检查是一种微创且安全的手术,主要用于无法解释的渗出性胸腔积液,但可考虑用于气胸(PNX)。
    方法:这项回顾性研究包括受PNX影响的参与者,他们从2008年到2021年在一家学术医院接受了带滑石粉袋的医用胸腔镜检查。主要终点是在内科胸腔镜检查后7天内观察到完全的影像学肺再扩张和胸腔引流管没有空气供应。次要终点是在出院后24个月未实现同侧PNX的复发。
    结果:共有95例受原发性自发性PNX(PSP)影响的患者,次级自发PNX(SSP),医源性,并纳入创伤性PNX。17.89%的患者需要额外的手术,只有一名SSP患者需要后续手术。9.47%的患者在出院后24个月内发生同侧PNX复发,中位复发时间为13.5个月。PSP组明显更有可能达到主要终点。胸膜形态与到达主要终点显著相关,在住院期间接受大于或等于4g的累积剂量的滑石粉与较低的满足风险相关。在所有情况下,接受大于或等于4g的累积剂量的滑石导致达到次要终点。医源性和创伤性PNX患者在短期和长期评估中均具有良好的预后。
    结论:内科胸腔镜检查是一种有效的治疗PNX的方法,在急性情况下,在某些病例中,同时防止长期复发。需要大量的前瞻性临床研究来支持和更好地定义医用胸腔镜在当前临床实践中的作用。
    BACKGROUND: Medical thoracoscopy is a minimally invasive and safe procedure mostly performed for unexplained exudative pleural effusions but may be considered for pneumothorax (PNX).
    METHODS: This retrospective study included participants affected by PNX who underwent medical thoracoscopy with talc poudrage at a single academic hospital from 2008 to 2021. The primary endpoint was the observation of complete radiographical lung re-expansion and absence of air supply from the chest drain within 7 days of medical thoracoscopy. The secondary endpoint was achieving no recurrence of ipsilateral PNX at 24 months post-discharge.
    RESULTS: A total of 95 patients affected by primary spontaneous PNX (PSP), secondary spontaneous PNX (SSP), iatrogenic, and traumatic PNX were enrolled. An additional procedure was required by 17.89% of patients, and only one patient with SSP required subsequent surgery. Recurrence of PNX occurred on the same side within 24 months after discharge in 9.47% of patients, with a median time to recurrence of 13.5 months. The PSP group was significantly more likely to achieve the primary endpoint. Pleural morphology was significantly associated with reaching the primary endpoint, while receiving a cumulative dose of talc greater than or equal to 4 g during hospitalization was associated with a lower risk of meeting it. Receiving a cumulative dose of talc greater than or equal to 4 g led in all cases to the achievement of the secondary endpoint. Patients with iatrogenic and traumatic PNX had an excellent prognosis in both the short- and long-term evaluation.
    CONCLUSIONS: Medical thoracoscopy is an effective procedure for treating PNX in the acute setting in selected cases while preventing long-term relapses. Large prospective clinical studies are needed to support and better define the role of medical thoracoscopy in current clinical practice.
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  • 文章类型: Journal Article
    介绍了四种原发性胸膜骨外骨肉瘤。患者为男性,年龄在63至78岁之间(平均:70.5岁),有胸痛症状,咳嗽,呼吸急促.诊断成像显示各种钙化,胸膜基肿块和/或弥漫性胸膜增厚,临床模仿间皮瘤。获得胸腔镜手术材料用于组织病理学诊断。组织学发现显示存在由梭形细胞组成的肿瘤梭形细胞增殖,细胞质很少,细长的核和不明显的核仁。有丝分裂活性很容易鉴定。此外,所有肿瘤均以生成类骨质基质的形式表现出广泛的骨分化;一个肿瘤显示出额外的软骨肉瘤成分,另一个肿瘤显示局灶性粘液样改变。免疫组织化学分析显示,肿瘤细胞对多种抗体均呈阴性,包括角蛋白AE1/AE3,角蛋白5/6,D2-40,EMA,calretinin,WT-1、BerEP4和HEG1;BAP1在所有病例中均保留。此外,CDKN2A(p16)的荧光原位杂交在所有肿瘤中均为纯合缺失阴性。临床随访显示,一名患者在诊断后8个月死亡,一名患者在短暂的诊断后随访中仍存活。本文描述的肿瘤突出了具有异源元件的间皮瘤与胸膜起源的真正骨肉瘤的分离中的挑战性问题。我们建议针对恶性骨和/或软骨分化的肿瘤诊断胸膜骨外骨肉瘤,其中全面的免疫组织化学研究和FISH分析未能为诊断具有异源成分的间皮瘤提供支持。
    Four primary extraskeletal osteosarcomas of the pleura are presented. The patients were men between the ages of 63 and 78 years (average: 70.5 years) who presented with symptoms of chest pain, cough, and shortness of breath. Diagnostic imaging showed variably calcified, pleural-based masses and/or diffuse pleural thickening, clinically mimicking mesothelioma. Thoracoscopic surgical material was obtained for histopathological diagnosis. Histological findings showed the presence of a neoplastic spindle cell proliferation composed of fusiform cells with scant cytoplasm, elongated nuclei and inconspicuous nucleoli. Mitotic activity was easily identified. Additionally, all tumors showed extensive osseous differentiation in the form of osteoid matrix production; one tumor demonstrated additional chondrosarcomatous elements and another showed focal myxoid changes. Immunohistochemical analysis revealed that the tumor cells were uniformly negative for a wide variety of antibodies, including keratin AE1/AE3, keratin 5/6, D2-40, EMA, calretinin, WT-1, BerEP4, and HEG1; BAP1 was retained in all cases. In addition, fluorescence in situ hybridization for CDKN2A (p16) was negative for homozygous deletion in all tumors. Clinical follow-up showed that one patient had died 8 months after diagnosis and one had remained alive with short post-diagnostic follow-up. The tumors herein described highlight a challenging issue in the separation of mesothelioma with heterologous elements from true osteosarcomas of pleural origin. We propose that a diagnosis of extraskeletal osteosarcoma of the pleura is rendered for tumors with malignant osseous and/or cartilaginous differentiation in which comprehensive immunohistochemical studies and FISH analysis have failed to provide support for a diagnosis of mesothelioma with heterologous elements.
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  • 文章类型: Case Reports
    背景:胸膜孤立性纤维性肿瘤(SFTP)是一种罕见的原发性肿瘤,起源于位于间皮衬里胸膜下方的间充质细胞。一个特殊的实体是巨大的SFTP,它在手术管理中提出了独特的挑战。
    方法:介绍了2例中年患者的巨大SFTP。两名患者最近均出现呼吸困难恶化,影像学检查结果与占据整个胸膜腔的巨大肿块一致,同侧肺完全塌陷,心脏和纵隔对侧严重偏离。CT引导下经胸穿刺活检病理结果均为SFTP。进行了手术切除,他们都需要两次开胸手术才能完全切除肿瘤。成功完成了肿瘤的整体切除,术后完全恢复。第一次切除的肿瘤为30×20cm,重3500g,第二个大小为33*x25cm,重4000g。两名患者在手术后呼吸状况均得到改善,随访中无复发。
    结论:使用各种方法去除SFTP:开胸手术,胸骨切开术与半壳延伸的可能性,电视胸腔镜手术(VATS),和机器人辅助手术。手术巨大SFTP(>15cm)时的挑战是双重的:对肺组织和周围纵隔结构的粘连和压迫以及肿瘤的血管过多。对于大型SFTP,最好采用开放式方法。由于复发风险高,切除应以阴性切缘完成。
    结论:这些案例强调了个性化手术策略对管理巨大SFTP的重要性,为处理类似病例的临床医生提供有价值的见解。
    BACKGROUND: Solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor originating from mesenchymal cells located beneath the mesothelial-lined pleura. A special entity is the giant SFTP which presents unique challenges in surgical management.
    METHODS: Two cases of giant SFTPs in middle-aged patients are presented. Both patients presented with a recent worsening dyspnea and had imaging findings consistent with giant masses occupying the entire pleural cavity with a complete collapse of the homolateral lung and substantial contralateral deviation of heart and mediastinum. The pathological results of CT-guided transthoracic core needle biopsy was SFTP in both cases. Surgical resection was performed, and they both required two thoracotomies for complete tumor removal. Successful complete en bloc resection of the tumors was achieved with a total postoperative recovery. The first resected tumor was 30 × 20 cm and weighed 3500 g, the second was 33*x25 cm in size and weighed 4000 g. Both patients respiratory condition improved after the operation and no recurrence were noted in the follow-up.
    CONCLUSIONS: SFTPs are removed using various approaches: thoracotomy, sternotomy with the possibility of hemiclamshell extension, video-assisted thoracoscopic surgery (VATS), and robotic-assisted surgery. The challenge when operating giant SFTP (> 15 cm) is double: adhesions and compression to the lung tissue and surrounding mediastinal structures and the hyper vascular nature of the tumor. For giant SFTPs an open approach is preferable. Resection should be complete with negative margins due to the high risk of recurrence.
    CONCLUSIONS: These cases emphasize the significance of personalized surgical strategies for managing giant SFTPs, providing valuable insights for clinicians addressing similar cases.
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  • 文章类型: Journal Article
    背景:由胸膜引起的钙化性纤维瘤(CFT)是青年和中年人中相对罕见的良性病变。我们报告了一名31岁的胸膜CFT女性,她成功完成了胸腔镜摘除术。
    方法:一名无症状的女性患者出现右下肺野肿块,在例行体检时在胸部X线片上偶然发现。胸部计算机断层扫描显示下纵隔有明确的肿块,最大直径约5.5厘米。食管胃十二指肠镜检查未发现食道异常。内窥镜超声检查(EUS)显示肿瘤明确,没有内部血流。EUS细针抽吸未能确定诊断。因此,胸腔镜下肿瘤摘除术用于诊断和治疗.根据切除标本的组织病理学发现,一名年轻女性身上存在高纤维成分的肿瘤,以及肉芽肿钙化的鉴定,建立了CFT的诊断。
    结论:一名年轻成年女性胸膜引起的CFT成功进行了完全胸腔镜下肿瘤摘除术。
    BACKGROUND: Calcifying fibrous tumor (CFT) arising from the pleura is a relatively rare benign lesion in young and middle-aged adults. We report a 31-year-old woman with pleural CFT who underwent successful complete thoracoscopic enucleation.
    METHODS: An asymptomatic woman presented with a mass in the right lower lung field that was incidentally detected on a chest X-ray during a routine medical checkup. Chest computed tomography showed a well-defined mass in the lower mediastinum, with a maximum diameter of approximately 5.5 cm. Esophagogastroduodenoscopy showed no abnormal findings in the esophagus. An endoscopic ultrasonography (EUS) revealed a well-defined tumor with no internal blood flow. EUS-fine needle aspiration failed to establish a definitive diagnosis. Therefore, thoracoscopic tumor enucleation was performed for diagnostic and therapeutic purposes. Based on the histopathological findings of the resected specimen, the presence of a tumor with a high fibrous component in a young woman, and the identification of granulomatous calcifications, a diagnosis of CFT was established.
    CONCLUSIONS: Complete thoracoscopic tumor enucleation was successfully performed for CFT arising from the pleura in a young adult woman.
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  • 文章类型: Journal Article
    虽然与标准胸骨切开术相比,微创主动脉瓣手术的益处已被广泛描述,在微创手术中保留胸膜完整性(PPI)的影响仍被广泛讨论.本研究旨在确定PPI对微创主动脉瓣置换术(MIAVR)术后和长期结局的作用。
    在1997年至2022年间接受MIAVR(小切口或右前小切口)的所有2,430例连续患者被纳入研究。患者分为2组:有和没有PPI的患者。PPI被认为是维持胸膜闭合,而无需在外科手术结束时插入胸管。使用倾向匹配分析比较PPI组和非PPI组。
    倾向匹配后,每组848例患者(PPI和非PPI)。平均年龄为70.21岁和71.42岁,平均胸外科医师协会预测的死亡风险为0.31%,而非PPI与PPI的死亡率为0.30%,分别。平均随访时间为147.4个月。术后,非PPI与PPI患者的重症监护病房住院时间更长(9.7对17.3小时,P<0.001)和住院时间(5.2天vs8.9天,P<0.001)。呼吸系统并发症的发生率,包括气胸或皮下气肿的发生率,肺不张,非PPI患者的胸腔积液事件明显高于PPI。非PPI的30天全因死亡率高于PPI(0.029vs0.010,P=0.003)。围手术期,短期,非PPI组的长期全因死亡率显著高于对照组.
    MIAVR术后PPI与术后并发症发生率降低相关,缩短停留时间,与非PPI相比,总生存率提高。因此,MIAVR为患者量身定制的维持胸膜完整性的手术方法对短期和长期结局产生积极影响。
    UNASSIGNED: While the benefits of minimally invasive aortic valve surgery compared with standard sternotomy have been widely described, the impact of preservation of pleural integrity (PPI) in minimally invasive surgery is still widely discussed. This study aims to define the role of PPI on postoperative and long-term outcomes after minimally invasive aortic valve replacement (MIAVR).
    UNASSIGNED: All 2,430 consecutive patients undergoing MIAVR (ministernotomy or right anterior minithoracotomy) between 1997 and 2022 were included in the study. Patients were divided into 2 groups: patients with and without PPI. PPI was considered the maintenance of the pleura closed without the need for a chest tube insertion at the end of the surgical procedure. A propensity-matched analysis was used to compare the PPI and not-PPI groups.
    UNASSIGNED: After propensity matching, 848 patients were included in each group (PPI and not-PPI). The mean age was 70.21 versus 71.42 years, and the mean Society of Thoracic Surgeons predicted risk of mortality was 0.31% versus 0.30% in not-PPI versus PPI, respectively. The mean follow-up time was 147.4 months. Postoperatively, not-PPI versus PPI patients had a longer intensive care unit stay (9.7 vs 17.3 h, P < 0.001) and hospital length of stay (5.2 vs 8.9 days, P < 0.001). The rate of respiratory complications including the incidence of pneumothorax or subcutaneous emphysema, pulmonary atelectasis, and pleural effusion events requiring thoracentesis/drainage was significantly higher in not-PPI versus PPI. The 30-day all-cause mortality was higher in not-PPI versus PPI (0.029 vs 0.010, P = 0.003). Perioperative, short-term, and long-term all-cause mortality was significantly higher in the not-PPI group.
    UNASSIGNED: PPI after MIAVR is associated with reduced incidence of postoperative complications, reduced lengths of stay, and improved overall survival compared with not-PPI. Therefore, a MIAVR tailored patient-procedure approach to maintaining the pleura integrity positively impacts short-term and long-term outcomes.
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  • 文章类型: Journal Article
    目的:本研究旨在评估透视引导下经皮经胸胸膜钳活检(PTPFB)对渗出性胸腔积液的诊断表现和手术特点。
    方法:在2014年5月1日至2023年2月28日期间接受PTPFB的渗出性胸腔积液患者被纳入这项回顾性研究。经皮导管引流(PCD)与PTPFB之间的间隔,活检的数量,程序时间,并对手术相关并发症进行评估。敏感性,特异性,使用PCD引流进行胸膜细胞学检查,并计算诊断恶性肿瘤的准确性,PTPFB,并结合PTPFB和胸膜细胞学检查。
    结果:71名患者,包括50名男性和21名女性(平均年龄,69.5±15.3年),包括在这项研究中。最终诊断为良性病变48例(67.6%),恶性病变23例(32.4%)。PCD和活检之间的总间隔为2.4±3.7天。在接受延迟PTPFB的组中,PCD和活检之间的间隔为5.2±3.9天。活检的平均数量为4.5±1.3。平均手术时间为4.4±2.1分钟。1例患者报告轻微出血并发症(1.4%)。敏感性,特异性,胸膜细胞学的准确性,PTPFB,PTPFB和胸膜细胞学联合占47.8%(11/23),100%(48/48),和83.1%(59/71),分别为65.2%(15/23),100%(48/48),和88.7%(63/71),分别为78.3%(18/23),100%(48/48),和93.0%(66/71),分别。细胞学联合PTPFB的敏感性和准确性明显高于单独的细胞学检查(P分别为0.008和0.001)。
    结论:透视引导下的PTPFB对渗出性胸腔积液患者是一种准确安全的诊断技术,具有可接受的诊断性能,并发症发生率低,合理的程序时间。
    OBJECTIVE: This study aimed to evaluate the diagnostic performance and procedural characteristics of fluoroscopy-guided percutaneous transthoracic pleural forceps biopsy (PTPFB) in patients with exudative pleural effusion.
    METHODS: Patients with exudative pleural effusion who underwent PTPFB between May 1, 2014, and February 28, 2023, were included in this retrospective study. The interval between percutaneous catheter drainage (PCD) and PTPFB, number of biopsies, procedural time, and procedure-related complications were evaluated. The sensitivity, specificity, and accuracy of diagnosing malignancy were computed for pleural cytology using PCD drainage, PTPFB, and combined PTPFB and pleural cytology.
    RESULTS: Seventy-one patients, comprising 50 male and 21 female (mean age, 69.5 ± 15.3 years), were included in this study. The final diagnoses were benign lesions in 48 patients (67.6%) and malignant in 23 patients (32.4%). The overall interval between PCD and biopsy was 2.4 ± 3.7 days. The interval between PCD and biopsy in the group that underwent delayed PTPFB was 5.2 ± 3.9 days. The mean number of biopsies was 4.5 ± 1.3. The mean procedural time was 4.4 ± 2.1 minutes. Minor bleeding complications were reported in one patient (1.4%). The sensitivity, specificity, and accuracy for pleural cytology, PTPFB, and combined PTPFB and pleural cytology were 47.8% (11/23), 100% (48/48), and 83.1% (59/71), respectively; 65.2% (15/23), 100% (48/48), and 88.7% (63/71), respectively; and 78.3% (18/23), 100% (48/48), and 93.0% (66/71), respectively. The sensitivity and accuracy of cytology combined with PTPFB were significantly higher than those of cytological testing alone (P = 0.008 and 0.001, respectively).
    CONCLUSIONS: Fluoroscopy-guided PTPFB is an accurate and safe diagnostic technique for patients with exudative pleural effusion, with acceptable diagnostic performance, low complication rates, and reasonable procedural times.
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  • 文章类型: Case Reports
    背景:特发性胸膜实质纤维弹性增生症(IPPFE)是一种非常罕见且缓慢明显进展的慢性肺部疾病。通常累及肺的上叶。这种不寻常的疾病,2013年首次被认为是罕见的特发性间质性肺炎,其特征是内脏胸膜和下肺实质的致密纤维化,并伴有主要在胸膜下肺泡壁的弹性变性。为了提高我们对这种罕见疾病的认识,我们报告一例由病理结果确定的IPPFE。病例报告:一名73岁的男性患者,吸烟者,有慢性阻塞性肺疾病病史,自2022年1月以来,劳累时呼吸困难逐渐恶化,并伴有体重减轻。胸部X线检查发现胸部扩张。胸部高分辨率计算机断层扫描显示双尖胸膜下实质凝结,右上叶有牵张性支气管扩张和胸膜回缩以及弥漫性双侧中央小叶气肿。扫描引导的跨顶叶肺活检显示肺实质纹身有炭疽病沉积,大部分被纤维组织重塑,在偏振光中与许多波浪状和屈光性畸形结构混合在一起。orcein染色证实了这些病变中存在过量的弹性纤维。所有病因调查均为阴性。他的肺功能研究显示可逆性阻塞性通气障碍。经过多学科的讨论,IPPFE的诊断是根据胸部计算机断层扫描在上肺的分布并结合病理类型来确定的。结论:该病例强调了IPPFE的非典型误导性放射学表现以及病理结果在建立诊断中的关键作用。因此,需要进一步的研究来提高我们对这种罕见疾病的认识,并建立明确的IPPFE诊断和治疗指南.
    Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a very rare and a slowly conspicuous progressing chronic lung disease, which usually involves the upper lobes of the lung. This unusual disease, first recognized as a rare idiopathic interstitial pneumonia in 2013, is characterized by dense fibrosis of the visceral pleura and the subjacent lung parenchyma accompanied by elastosis predominating in the subpleural alveolar walls. In the interest of improving our understanding of this uncommon disease, we report a case of IPPFE established by pathology results. Case report: A 73-year-old male patient, smoker, with a medical history of chronic obstructive pulmonary disease, presented since January 2022 with a gradual worsening of dyspnea on exertion and productive cough with weight loss. The chest X-ray detected a thoracic distention. The chest high resolution computed tomography revealed biapical subpleural parenchymatous condensations with tractive bronchiectasis and pleural retraction in the right upper lobe and diffuse bilateral cento-lobular emphysema. A scan-guided trans-parietal lung biopsy showed lung parenchyma tattooed with anthracosic deposits, largely remodeled by fibrous tissue, intermingled with numerous wavy and refractive dyselastotic structures in polarized light. The orcein staining confirmed the presence of excess elastosic fibers within these lesions. All etiological investigations were negative. His lung function studies revealed a reversible obstructive ventilatory disorder. Following a multidisciplinary discussion, the diagnosis of IPPFE was confirmed on the basis of the distribution in the upper lungs on chest computed tomography combined with pathology pattern. Conclusions: This case emphasizes the atypical misleading radiological presentation of IPPFE and the key role of pathological results in establishing the diagnosis. Hence, further studies are needed to improve our understanding of this uncommon disease and to establish clear-cut guidelines for IPPFE diagnosis and management.
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