Pleural Diseases PDF

Pleural diseases Prof Dr Sergülen Dervişoğlu Learning objectives By the end of this course, students should be able to understand the terms of pleural diseases Defines pleural effusion and lists its subtypes. Explains the pathogenetic mechanism of effusion types Describes pneumotho...

Pleural diseases Prof Dr Sergülen Dervişoğlu Learning objectives By the end of this course, students should be able to understand the terms of pleural diseases Defines pleural effusion and lists its subtypes. Explains the pathogenetic mechanism of effusion types Describes pneumothorax, hydrothorax, chylothorax, hemothorax and understand the causes of them Understands the difference between non-inflammatory and inflammatory effusions, and understands the healing methods according to effusion types. Understands pleuritis and gross/microscopic changes of pleuritis acording to its’ subtypes Defines empyema Lists pleural tumors Understands the basic microscopic features of solitary fibrous tumor. Describes the macroscopic features of mesothelioma and lists its microscopic subtypes Lists mediastinal tumors by location Plevral effüzyon Pleural effusion is seen due to primary or secondary lesions of pleura Normally pleural fluid is 15 ml serous and acellular Increased fluid in pleura inflammatory and noninflammatory  Inflammation ( most common pyogenous bacteria) Causes of Bronchogenic carcinomas Pulmonary infarct pleural effusions Viral pleuritis Noninflammatory pleural effusions Hydrothorax: Transudate plenty of fluid accumulation in pleural space Hemothorax: Blood in pleural space (Aort aneurysm rupture, travma) Chilothorax: Lymphatic fluid in pleural space ( obstruction of lymphatics by tumor) Pneumothorax: air in pleural space Hydrotorax Etiopathogenesis; Hydrostatic P Þ (Congestive heart failure) Vascular permeability Þ (pneumonia) Oncositic P à (Nephrotic synd.) Pleural negative P Þ (atelectasis) Lymphatic drainage à (mediastinal carcinomatosis) Hydrothorax Usually bilateral Plenty of fluid accumulated Starts from basal area Usually heals without scar Rarely adhesions between two pleural layer Hemothorax Blood in Pleural space Different fromSero-hemorhagic fluid Frequent cause--- rupture of intrathorasic aortic aneurysm If not managed early inevitably fatal Rarely heals with organization Multiloculated adhesions Chylotorax Milk like lymphatic fluid accumulation Contains fatty material Travma to thoracic ductus Intrathoracic malignant tm, lymphatic dissemination (eg. Lymphoma) Its cause is usually leakage from the thoracic duct or one of the main lymphatic vessels that drain to it. The most common causes are lymphoma and trauma caused by thoracic surgery. If the patient is on a normal diet, the effusion can be identified by its white and milky appearance as it contains high levels of triglycerides. The condition is rare but serious and appears in all mammals. In animals, chylothorax usually results from diseases that cause obstruction to the thoracic duct preventing lymph from draining normally into the venous system. Examples include tumors, heartworm disease, right sided cardiac failure, or idiopathic lymphangiectasia Pneumothorax Air in the pleural space Spontan, traumatic or therapeutic Lung abscess, cavern, bullous emphysema, interstitial emphysema Spontan idiopathic pneumothorax Few ---- resorption Large amount----theraphy (drainage under water) A collapsed lung. A pneumothorax occurs when air leaks into the space between your lung and chest wall. This air pushes on the outside of your lung and makes it collapse. A pneumothorax can be a complete lung collapse or a collapse of only a portion of the lung. Inflammatory pleural effusion Density 1020 Þ fluid= Exudate Serous, serofibrinous and fibrinous pleuritis 4 major mechanism: 1-Blood borne or direct dissemination to pleura 2-Malignant tumor invasion 3-Pulmonary infarct 4-Viral pleuritis Inflammatory pleural effusion Above 40 Û patients ***if fever –PPD – think of malignancy! A large amount and usually hemorrhagic (must be distinguished from hemothorax) Transudate and serous exudate heals without trace  Serous-fibrinous and fibrinious pleuritis Fibrinous pleuritis causes: Collagen diseases; rheumatoid arthritis, SLE Tuberculosis Uremia Lung abscess Lung infarct Radiotherapy Serofibrinous pleuritis and a small amount of fibrinous exudate may resorb or if it is heavy in amount healing can be achieved by organization Fibrinious pleuritis Fibrinous pleuritis Inflammation of the pleura; it may be caused by infection, injury or tumor. It may be a complication of lung diseases, particularly of pneumonia or lung abscess. Typical signs of acute pleurisy include painful respiratory movements causing grunting and rapid shallow breathing. Chronic pleurisy includes empyema with collapse of lung and dyspnea with toxemia, or interference with respiratory movements by adhesions. Fibrinious pleuritis Purulent pleuritis A large amount of pus collection = emphyema Bacterial or mycotic lung inflammation Secondary or primary Lymphogenous or hematogenous Subdiaphragmatic dissemination â subphrenic or liver abscess, peritonitis Heals with organization  Liver abscess Plevra tümörleri Chronic tuberculous empyema in 66- year-old man diagnosed with tuberculous pleuritis 23 years earlier. CT scan obtained at level of lower thorax shows large loculated pleural fluid collection in right lower lateral hemithorax. Note surrounding pleural thickening and calcifications. Adenocarcinoma associated with chronic tuberculous empyema of 30 years' duration in 69-year-old man. CT scans of right lower hemithorax show soft-tissue mass lesion (arrows, A), which extends to posterior chest wall with adjacent rib destruction and is enhanced heterogeneously (B). Note adjacent extensive pleural thickening and calcifications. Pleural tumors Solitary fibrous tumor (SFT) Benign cystic mesothelioma Mesothelioma Malignant mesothelioma Benign Pleural Tumors Solitary fibrous tumor Benign cystic mesothelioma ( rare at pleura more common at peritoneum) No relationship with asbestosis Solitary Fibrous Tumor Fibroblastic tumor characterized by haphazardly arranged spindled to ovoid cells, prominent staghorn vasculature and NAB2-STAT6 gene rearrangement Solitary fibrous tumor Clinical features Symptoms associated with anatomic location Somatic soft tissue tumors present as slow growing, painless mass Abdominopelvic tumors produce symptoms due to organ impingement Pleural tumors often discovered incidentally but could grow exophytically into lungs Paraneoplastic syndrome (Doege-Potter syndrome) extremely rare; due to IGF2 production by tumor SFT/Radiological findings CT: well defined, sometimes lobulated mass that is isodense to skeletal muscle with heterogeneous contrast enhancement MRI: T1 intermediate and T2 hypointense (cellular / fibrous areas) to hyperintense (myxoid areas) signals Gross Pathology: Solitary fibrous tumors (SFT) in deep soft tissues present as large, well-circumscribed but usually unencapsulated tumors. When arising from serosal surfaces such as pleura, they can grow as an exophytic mass. The size generally ranges from 5 to 10 cm. The cut surface is grayish-white or red- brown and may show hemorrhagic or cystic areas. SFT Haphazard arrangement of spindled to ovoid cells arranged around branching and dilated vasculature within variably collagenous stroma Occurs at any anatomical site with a wide range of histological patterns CD34 usually strong and diffuse but nonspecific STAT6 immunohistochemistry highly sensitive and specific surrogate for NAB2-STAT6 gene fusion Risk stratification preferred over anatomic staging Soliter fibröz tümör A 74-year-old man with malignant solitary fibrous tumor of the pleura with malignant characteristics, who presented a gradually enlarged pleural mass and subsequent brain metastasis. A and B, Enhanced chest CT transverse and coronal images of the mediastinal window at the right and left atrium levels, respectively, showing a bulky, lobulated, heterogeneously enhancing mass involving the right hemi- diaphragm, displacing the heart and right lower lobe, and mimicking sarcoma. Solitary Fibrous Tumor /Prognosis 10 - 30% (local or distant) recurrence rate 5 and 10 year disease specific survival rates 89 and 73%, respectively Adverse prognostic factors: mitotic count > 4 per 10 high power fields, tumor size > 10 cm, necrosis, hypercellularity, cellular atypia, dedifferentiation, location in chest, abdomen or pelvis, TERT promoter mutations Numerous risk stratification models exist, the most widely used one classifies tumors into groups of low, intermediate and high risk for metastasis using mitotic count, age, tumor size and necrosis  Malignant mesothelioma VİSCERAL OR PARİETAL ASBEST EXPOSURE + RİSK 7-10% PLEURA DERİVED SMOKİNG RİSK Þ  Malign mezotelyoma Asbest probably makes DNA damage Two tumor supressor p16/CDKN2A (9p21 Kr and oncogenic genes shows ) mutations on mutations : mesothelial cells ? In mesothelioma +, Neurofibromatosis 2 SV40 (simian virus 40 ) carcinogen virus ; NF2 (22 q12 Kr) 60-80% inactiavtes tumor supressor genes Malignant mesothelioma clinical presentation and prognosis Chest pain, Theraphy is 50% of patients is dyspnea, repeated difficult; early not alive in 1 year pleural effusion in pleura-pulmonary after dx short term resection Distant met. * +radiotherapy Malignant but lymphatic +chemotherapy locally aggressive dissemination are rare  Malign mezotelyoma Malign mezotelyoma serozal yüzeylerdeki mezotel hücrelerinden ve çoğunlukla (%90) plevradan kaynaklanan oldukça agresif bir neoplazmdır. Periton, perikard ve nadiren testisin tunika vaginalisi hastalığın geliştiği diğer alanlar olarak izlenmektedir. Etiyolojisinde asbest ve erionit liflerine maruziyet önemli rol oynar. Asbest maruziyeti ile tümör gelişimi arasındaki süre 25-45 yıldır Hastalık en sık 50-70 yaş aralığında görülür. Başlıca semptomlar tümörün göğüs duvarına invazyonu ve plevral sıvı birikimi sonucu ortaya çıkan nefes darlığı ve göğüs ağrısıdır.  Peritoneal mesotheliomas appear as disseminated nodules or thickened plaques scattered over the peritoneal surfaces. The serosal surface of the abdominal viscera may be studded with small and large firm nodules. Intraperitoneal adhesions and ascites are frequently present. This gross specimen photograph shows malignant mesothelioma of the peritoneum in a 69 y/o male. The abdominal surface of the diaphragm is covered with variously sized tumor nodules Malignant mesothelioma Epitheloid type malignant mesothelioma Sarcomatoid type mesothelioma Biphasic mesothelioma (epitheloid+ mesenchymal cells) Malignant mesothelioma Malignant mesothelial cells falling into the effusion are often of epithelioid morphology, and these cells may fall in the form of clusters, cell balls or papillae and may contain psammoma bodies. Although the cells show a wide spectrum ranging from very quiet appearance to highly pleomorphic features at the cytological level, they do not show cytological atypia as pronounced as the atypia of carcinoma metastases.  Malignant mesothelioma Epithelial and sarcomatous component Epithelial cells make gland or papillary arrangements Sarcomatous component spindle shaped sarcoma cells Fibrosarcoma like Epithelial komponent cytokeratin and Epithelial Membrane Antigen (EMA) positive, Calretinin positive, CEA negative Sarcomatous component vimentin positive  Malignant mesothelioma The most common histological The cells lining the tubules and patterns are solid, tubulopapillary papillae vary from low cuboidal, and trabecular. Less common relatively quiescent-looking to patterns are micropapillary, more atypical and larger-diameter adenomatoid (microcystic), clear cells. Psammoma bodies may be cell, transitional, desuduoid and seen small cell.  SARCOMATOIID, DESMOPLASTIC AND BIFASIC MESOTHELIOMA Sarcomatoid malignant mesothelioma; It consists of malignant mesothelial cells with a storiform distribution within dense collagenized tissue, provided that they constitute at least 50% of the tumor.  Desmoplastic MM It consists of atypical spindle- shaped cells distributed within a dense hyalinized stroma in at least 50% of the tumor. The most important criterion to distinguish desmoplastic MM from organized pleuritis is the presence of fatty tissue invasion.  Biphasic MM It is characterized by the presence of features of sarcomatoid/desmoplastic mesothelioma or epithelioid MM in at least 10% of the tumor. Malign mezotelyoma  Genetic features and prognosis Malignant mesothelioma Tumor supressor genes NF2, CDKN2A(P16INK4a),CDKN2B (P15INK4b) and BAP1 mutations Long survival rate is low. Young patient, epithelioid type and early TNM stage are important in long median survival and response to treatment.  LOCALİZED MALİGNANT MESOTHELIOMA They are rare tumors and are macroscopically localized nodular lesions. Although it shows the ultrastructural, immunohistochemical and microscopic features of diffuse MM, it shows neither the microscopic nor macroscopic features of diffuse pleural spread. The average age is 60-65 and it is slightly more common in men. The role of asbestos exposure in its etiology is unclear. While they may present clinically with chest pain, dyspnea, fever, night sweats and weight loss, they may also be diagnosed incidentally. LOCALİZED MALİGNANT MESOTHELIOMA Mediastinal tumors-1 Upper mediastinum ° Lymphoma ° Thymoma ° Thyroid lesions ° Metastatic carcinoma ° Parathyroid tumors Anterior mediasten ° Thymoma °Teratoma °Lymphoma ° Thyroid lesions ° Parathyroid tumors Mediastinal tumors-2 Posterior mediastinum ° Neurogenic tumors(schwannoma,neurofibroma) ° Lymphoma ° Gastroenteric hernia Middle mediastinum ° Bronchogenic cyst ° Pericardial cyst ° Lymphoma