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JCI Insight May 2021Pleural fibrosis is defined as an excessive deposition of extracellular matrix that results in destruction of the normal pleural tissue architecture and compromised...
Pleural fibrosis is defined as an excessive deposition of extracellular matrix that results in destruction of the normal pleural tissue architecture and compromised function. Tuberculous pleurisy, asbestos injury, and rheumatoid pleurisy are main causes of pleural fibrosis. Pleural mesothelial cells (PMCs) play a key role in pleural fibrosis. However, detailed mechanisms are poorly understood. Serine/arginine-rich protein SRSF6 belongs to a family of highly conserved RNA-binding splicing-factor proteins. Based on its known functions, SRSF6 should be expected to play a role in fibrotic diseases. However, the role of SRSF6 in pleural fibrosis remains unknown. In this study, SRSF6 protein was found to be increased in cells of tuberculous pleural effusions (TBPE) from patients, and decellularized TBPE, bleomycin, and TGF-β1 were confirmed to increase SRSF6 levels in PMCs. In vitro, SRSF6 mediated PMC proliferation and synthesis of the main fibrotic protein COL1A2. In vivo, SRSF6 inhibition prevented mouse experimental pleural fibrosis. Finally, activated SMAD2/3, increased SOX4, and depressed miRNA-506-3p were associated with SRSF6 upregulation in PMCs. These observations support a model in which SRSF6 induces pleural fibrosis through a cluster pathway, including SRSF6/WNT5A and SRSF6/SMAD1/5/9 signaling. In conclusion, we propose inhibition of the splicing factor SRSF6 as a strategy for treatment of pleural fibrosis.
Topics: Animals; Fibrosis; Humans; Male; Mice; Mice, Inbred C57BL; Phosphoproteins; Pleura; Pleural Diseases; Serine-Arginine Splicing Factors; Signal Transduction
PubMed: 33905374
DOI: 10.1172/jci.insight.146197 -
Surgical Case Reports Oct 2020Pulmonary metastasis of scalp angiosarcoma (SA) is a rare, but life-threatening disease, challenging to diagnose and manage. We report two cases of pneumothorax and...
BACKGROUND
Pulmonary metastasis of scalp angiosarcoma (SA) is a rare, but life-threatening disease, challenging to diagnose and manage. We report two cases of pneumothorax and hemothorax with pathologically proven metastasis of SA in the parietal pleura, which was not predictable from images and difficult to manage.
PATIENT A
A 73-year-old man with SA underwent chemoradiotherapy and surgical resection for primary skin lesion, was sent to our department to treat right empyema, which was developed during chest tube drainage for pneumothorax. Computed tomography (CT) showed multiple bullous lesions. We performed repetitive video-assisted thoracoscopic surgery (VATS) for the debridement and hemostasis; however, hemothorax was uncontrollable. The repeated cytology of pleural effusion showed no malignancy. We eventually performed fenestration and metastatic SA was pathologically diagnosed by the biopsy of parietal pleura. The patient developed respiratory failure and uncontrolled anemia, which were fatal.
PATIENT B
A 71-year-old man with SA previously treated with chemoradiotherapy was referred to our department for left pneumothorax. CT showed multiple bullous lesions at apex without any changes at parietal pleura. VATS was performed and the apex bullous lesion with air leakage was resected. The parietal pleura showed several dark-red spots and the biopsy was undertaken. The pathological diagnosis was a metastasis of SA along with visceral pleura and parietal pleura. The patient then developed right pneumothorax and left hemopneumothorax. Bilateral pleurodesis was ineffective and the patient died due to deteriorating general condition.
CONCLUSIONS
In patients with a history of SA who develop pneumothorax and hemothorax, metastatic SA to visceral and parietal pleura should be always considered. Surgical biopsy, not cytology, is needed for pathological diagnosis. Lesions in the parietal pleura prior to hemothorax were thoracoscopically observed in one case. Surgeons must recognize that conventional surgical intervention or pleurodesis will have unsatisfactory results.
PubMed: 33090280
DOI: 10.1186/s40792-020-01001-w -
Medicina (Kaunas, Lithuania) Jul 2022Background and Objectives: Pneumothorax implies the presence of air in the pleural space between the visceral and parietal pleura. The aim of this study was to...
Background and Objectives: Pneumothorax implies the presence of air in the pleural space between the visceral and parietal pleura. The aim of this study was to investigate the incidence, clinical characteristics, risk factors, therapy and perinatal outcome in neonates with pneumothorax in a tertiary care center. Materials and Methods: A retrospective study based on a five-year data sample of neonates with pneumothorax was conducted in a Maternity Hospital with a tertiary NICU from 2015 to 2020. We included all neonates with pneumothorax born in our hospital and compared demographic characteristics, perinatal risk factors, anthropometric parameters, comorbidities, clinical course and method of chest drainage between term (≥37 GW) and preterm (<37 GW) neonates. Results: The study included 74 newborns with pneumothorax, of which 67.6% were male and 32.5% were female. The majority of women (59.5%) had no complications during pregnancy. Delivery was mainly performed via CS (68.9%). Delivery occurred on average in 34.62 ± 4.03 GW. Significantly more (p = 0.001) children with pneumothorax were born prematurely (n = 53; 71.6%) than at term (n = 21; 28.4%). Most of the neonates had to be treated with ATD (63.5%) and nCPAP (39.2%), but less often they were treated with surfactant (40.5%) and corticosteroids (35.1%). O2 therapy lasted an average of 8.89 ± 4.57 days. Significantly more (p = 0.001) neonates with pneumothorax had additional complications, pneumonia, sepsis, convulsions and intraventricular hemorrhage (68.9%). However, most children had a good outcome (83.8%) and were discharged from the clinic. Fatal outcomes occurred in six cases, while another six neonates had to be transferred to referral neonatal centers for further treatment and care. Conclusion: Significantly more children with pneumothorax were born prematurely than at term. With adequate therapy, even premature newborns can successfully recover from pneumothorax.
Topics: Child; Female; Humans; Incidence; Infant, Newborn; Male; Pneumothorax; Pregnancy; Pulmonary Surfactants; Retrospective Studies; Risk Factors
PubMed: 35888683
DOI: 10.3390/medicina58070965 -
Cancers Aug 2023Solitary fibrous tumors of the pleura (pSFT) are a relatively rare neoplasms that can arise from either visceral or parietal pleura and may have different aggressive... (Review)
Review
Solitary fibrous tumors of the pleura (pSFT) are a relatively rare neoplasms that can arise from either visceral or parietal pleura and may have different aggressive biological behaviors. Surgery is well known to be the cornerstone of the treatment for pSFT. We reviewed the existing literature, focusing on the role of surgery in the management and treatment of pSFT. All English-written literature has been reviewed, focusing on those reporting on the perioperative management and postoperative outcomes. Surgery for pSFT is feasible and safe in all experiences reported in the literature, but surgical approaches and techniques may vary according to the tumor dimensions, localization, and surgeons' skills. Long-term outcomes are good, with a 10-year overall survival rate of more than 70% in most of the reported experiences; on the other hand, recurrence may happen in up to 17% of cases, which occurs mainly in the first two years after surgery, but case reports suggest the need for a longer follow-up to assess the risk of late recurrence. Malignant histology and dimensions are the most recognized risk factors for recurrence. Recurrence might be operated on in select patients. Surgery is the treatment of choice in pSFT, but a radical resection and a careful postoperative follow-up should be carried out.
PubMed: 37627194
DOI: 10.3390/cancers15164166 -
Indian Journal of Surgical Oncology Jun 2021This study aimed at reporting the surgical management of locally advanced thymoma (Masaoka stages III and IVA) and evaluating the factors predicting the survival. This...
This study aimed at reporting the surgical management of locally advanced thymoma (Masaoka stages III and IVA) and evaluating the factors predicting the survival. This is a retrospective analysis of patients operated for locally advanced thymoma from March 2012 to December 2019 in a thoracic surgery center in India. An analysis of all perioperative variables including complications was carried out. The influence of various predictors on survival was assessed by log-rank test. Out of total 54 patients, 42 (77.8%) had stage III and 12 (22.2%) had stage IVA. Upfront surgery was done in 34 (63%) patients, and induction chemotherapy was given in 20 (37%) patients. Pericardium was the commonest structure resected (79.6%) followed by the lung (51.8%), phrenic nerve (48.1%), major vascular structures (40.7%), parietal pleura (22.2%), diaphragm (9.2%), and right atrial appendage (1.8%). Forty-seven (87%) cases had complete (R0) resection, and the remaining 7 (12.9%) cases had incomplete (R1/R2) resection. There were no perioperative deaths (< 90 days). The median follow-up was 58 months. Overall survival (OS) and disease-free survival (DFS) at 5 years were 77.8% and 75.9%. Higher age (> 60 years), incomplete surgical resection, type B histology, and "> 3" structures resected with tumor were the poor prognostic factors for survival. An aggressive surgical approach, by an experienced team of cardiac and thoracic surgeons, aimed at complete resection is vitally important and can achieve excellent surgical and oncological outcomes even in locally advanced thymomas.
PubMed: 34295079
DOI: 10.1007/s13193-020-01215-2 -
Current Problems in Cancer Dec 2023Pleural mesothelioma (PM) is a cancer of the pleural surface, which is aggressive and may be rapidly fatal. PM is a rare cancer worldwide, but is a relatively common... (Review)
Review
Pleural mesothelioma (PM) is a cancer of the pleural surface, which is aggressive and may be rapidly fatal. PM is a rare cancer worldwide, but is a relatively common disease in Turkey. Asbestos exposure is the main risk factor and the most common underlying cause of the disease. There have been significant improvements in diagnoses and treatments of many malignancies; however, there are still therapeutic challenges in PM. In this review, we aimed to increase the awareness of health care professionals, oncologists, and pulmonologists by underlining the unmet needs of patients with PM and by emphasizing the need for a multidisciplinary treatment and management of PM. After reviewing the general information about PM, we further discuss the treatment options for patients with PM using immunotherapy and offer evidence for improvements in the clinical outcomes of these patients because of these newer treatment modalities.
Topics: Humans; Immunotherapy; Mesothelioma; Pleura; Pleural Neoplasms; Turkey
PubMed: 37845104
DOI: 10.1016/j.currproblcancer.2023.101017 -
Journal of Cardiothoracic Surgery Feb 2023Solitary fibrous tumors (SFTs) are rare mesenchymal pleural neoplasms with an overall good prognosis and low recurrence rate if completely resected and if degree of... (Review)
Review
Solitary fibrous tumors (SFTs) are rare mesenchymal pleural neoplasms with an overall good prognosis and low recurrence rate if completely resected and if degree of differentiation is favorable. Within the last decade, advances in research have led to more reliable methods of differentiating SFTs from other soft tissue tumors. Historically, several markers were used to distinguish SFTs from similar tumors, but these markers had poor specificity. Recent evidence showed NAB2-STAT6 fusion gene to be a distinct feature of SFTs with 100% specificity and sensitivity. Surgical resection, with an emphasis on obtaining negative margins, is the mainstay of treatment for SFTs. Preoperative planning with detailed imaging is imperative to delineate the extent of disease and vascular supply. One important radiologic distinction to aid delineation of a pleural-based tumor compared to a pulmonary parenchymal-based tumor is the angle that the tumor forms with the chest wall, which is obtuse for a pleural-based tumor, and acute for tumors of the lung parenchyma. Often, preoperative tissue diagnosis is not available, and surgery is both diagnostic and curative. Intraoperatively, emphasis should be on complete resection with negative margins. SFTs are resected via several approaches: thoracotomy, sternotomy with the option of hemi-clamshell extension, video-assisted thoracoscopic surgery, and robotic approach, which is increasingly being used and is our preference. We recommend a minimally invasive approach for most lesions, and have resected SFTs of the pleura that are up to 12 cm with the robotic approach. However, the current literature often cites 5 cm as the cut off for an open thoracotomy. Nevertheless, even with larger tumors, a minimally invasive robotic approach is our preference and practice. For giant SFTs (> 20 cm), an open approach may be preferable. Multiple thoracotomies and rib resection may be required to gain adequate exposure and ensure complete resection in these tumors. However, it is noteworthy that most of these tumors have a soft consistency and thus, once bagged, can easily be removed minimally invasively, and thus minimally invasive approach should not be completely ruled out. Recurrence in SFTs usually results from incomplete resection and redo surgery may portend a favorable prognosis.
Topics: Humans; Pleura; Severe Fever with Thrombocytopenia Syndrome; Solitary Fibrous Tumors; Pleural Neoplasms; Prognosis
PubMed: 36823638
DOI: 10.1186/s13019-023-02168-7 -
Multimedia Manual of Cardiothoracic... Aug 2023This video tutorial is a step-by-step demonstration of the indwelling pleural catheter insertion technique in a patient who was diagnosed with malignant pleural...
This video tutorial is a step-by-step demonstration of the indwelling pleural catheter insertion technique in a patient who was diagnosed with malignant pleural effusions due to multiple metastases. Placing an indwelling pleural catheter is a novel method to treat chronic pleural effusions, especially secondary to malignancies. This method is particularly useful in patients with a trapped lung or with reduced life expectancy in whom more-invasive procedures are contraindicated. Indwelling pleural catheters are well tolerated.
Topics: Humans; Pleura; Pleural Effusion, Malignant; Catheters
PubMed: 37615545
DOI: 10.1510/mmcts.2023.045 -
Frontiers in Oncology 2022Primary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on...
BACKGROUND
Primary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on extra-neural metastases from primary IEs.
METHODS
PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of extra-neural metastases from primary IEs. Clinical features, management strategies, and survival were analyzed.
RESULTS
We collected 48 patients from 43 studies. Median age was 13 years (range, 2-65). Primary IEs were frequently located in the parietal (22.9%) and frontal (16.7%) lobes, and mostly treated with resection (95.8%) and/or radiotherapy (62.5%). Most IEs were of grade-III (79.1%), and few of grade-I (6.3%) or grade-II (14.6%). 45 patients experienced intracranial recurrences, mostly treated with resection (86.7%), radiotherapy (60%), and/or chemotherapy (24.4%). Median time-interval from primary IEs was 28 months (range, 0-140). Most extra-neural metastases were diagnosed at imaging (37.5%) or autopsy (35.4%). Extra-neural metastases were multifocal in 38 patients (79.1%), mostly involving cervical or hilar lymph-nodes (66.7%), lung/pleura (47.9%), and/or scalp (29.1%). Surgical resection (31.3%), chemotherapy (31.3%) and locoregional radiotherapy (18.8%) were the most common treatments for extra-neural metastases, but 28 (58.3%) patients were not treated. At last follow-up, 37 patients died with median overall-survivals from primary IEs of 36 months (range, 1-239), and from extra-neural metastases of 3 months (range, 0.1-36). Overall-survival was significantly longer in patients with grade-I and II IEs (P=0.040).
CONCLUSION
Extra-neural metastases from primary IEs are rare, but mostly occur at later disease stages. Multidisciplinary management strategies should be intended mostly for palliation.
PubMed: 35574408
DOI: 10.3389/fonc.2022.831016 -
The Clinical Respiratory Journal Nov 2022
Topics: Humans; Therapeutic Irrigation; Pleural Diseases; Pleura; Pleural Effusion; Empyema, Pleural; Fibrinolytic Agents
PubMed: 36173249
DOI: 10.1111/crj.13548