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BAB 2

TINJAUAN PUSTAKA

2.1 Anatomi Pleura

Pleura merupakan membran serosa yang tersusun dari lapisan sel yang
embriogenik berasal dari jaringan selom intraembrional dan bersifat memungkinkan
organ yang diliputinya mampu berkembang, mengalami retraksi atau deformasi sesuai
dengan proses perkembangan anatomis dan fisiologis suatu organisme (Rasad,2015).
Pleura viseral membatasi permukaan luar parenkim paru termasuk fi sura interlobaris,
sementara pleura parietal membatasi dinding dada yang tersusun dari otot dada dan
tulang iga, serta diafragma, mediastinum dan struktur servikal (Moore, 2010). Pleura
viseral dan parietal memiliki perbedaan inervasi dan vaskularisasi. Pleura viseral
diinervasi saraf-saraf otonom dan mendapat aliran darah dari sirkulasi pulmoner,
sementara pleura parietal diinervasi sarafsaraf interkostalis dan nervus frenikus serta
mendapat aliran darah sistemik (Light, 2010). Pleura viseral dan pleura parietal
terpisah oleh rongga pleura yang mengandung sejumlah tertentu cairan pleura.

Gambar 1 Anatomi Sistem Respirasi

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Gambar 2 Pleura viseral dan parietal serta struktur sekitar pleura

2.1.1 Struktur Mikroskopis Pleura

Pleura terbagi menjadi lima lapisan, yaitu lapisan selapis mesotel, lamina
basalis, lapisan elastik superfi sial, lapisan jaringan ikat longgar dan lapisan jaringan
fibroelastik dalam (Wang, 2010). Kolagen tipe I dan III yang diproduksi oleh lapisan
jaringan ikat merupakan komponen utama penyusun matriks ekstraseluler pleura dan
merupakan 80% berat kering struktur ini (Wang, 2010). Lapisan jaringan fibroelastik
dalam menempel erat pada iga, otot-otot dinding dada, diafragma, mediastinum dan
paru (Lee, 2015). Lapisan jaringan ikat longgar tersusun atas jaringan lemak,
fibroblas, monosit, pembuluh darah, saraf dan limfatik (Wang, 2010). Pengamatan
pada hewan domba mengungkapkan bahwa ketebalan pleura dari permukaan rongga
pleura dengan lapisan jaringan ikat yang menaungi pembuluh kapiler dan pembuluh
limfatik adalah 25 – 83 μm pada pleura viseral dan 10 – 25 μm pada pleura parietal
(Light, 2010). Proses inflamasi mengakibatkan migrasi sel-sel inflamasi harus
melewati lapisan jaringan ikat longgar menuju lamina basalis kemudian menuju
rongga pleura setelah melewati mesotel (Wang, 2010). Mesotel berdasarkan
pengamatan mikroskop elektron berbentuk gepeng, berbenjolbenjol dan berukuran
sekitar 4 μm (Wang, 2010). Mesotel memiliki retikulum endoplasma kasar dan halus,
mitokondria dan beberapa jenis vesikel mikropinositotik terikat membran sehingga
memiliki fungsi fagositik dan eritrofagositik saat terlepas dari tautan antarsel (Wang,
2010). Mesotel saling terhubung oleh desmosom di tautan antarsel bagian basal
(Light, 2010). Bentuk komunikasi antar mesotel adalah tautan antar sel bagian apikal
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dan tautan tipe ZO-1 (Wang, 2010). Mesotel memiliki mikrovili berdiameter sekitar
0,1 μm dan panjang sekitar 1 – 3 μm dengan kepadatan 2 – 3 sel/μm2 yang
meningkatkan luas permukaan sel sehingga meningkatkan fungsi-fungsi terkait fi
siologi membran dan sekresi asam hialuronat. Mikrovili terutama ditemukan pada
mesotel pleura parietal sementara kepustakaan lain menyebutkan lebih banyak
ditemukan di pleura viseral (Lee, 2015). Senyawa vascular endothelial growth factor
(VEGF) disekresikan mesotel sebagai respons terhadap pajanan lipopolisakarida,
trombin dan bakteri menyebabkan peningkatan permeabilitas endotel pleura terutama
terhadap protein (Wang, 2010). Senyawa growth factor-β1 dan fibroblast growth
factor menyebabkan pleura mengalami transisi menjadi fibroblas (Light, 2010).
Senyawa intercellular adhesion molecule (ICAM)-1 yang diekspresikan mesotel
sebagai respons terhadap tumor necrosis factor (TNF)-α dan interferon (IF)-γ
menyebabkan migrasi netrofi l atau monosit melalui integrin cluster of diff erentiation
(CD)-11 atau CD-18 sehingga terjadi perlekatan pleura menyebabkan keadaan
pleuritis (Wang, 2010). Mikrovili mesotel mensekresi asam hialuronat untuk
mengurangi friksi antara paru, dinding dada dan diafragma saat proses respirasi (Lee,
2015). Senyawa ini juga berfungsi sebagai sawar selektif pertukaran ion-ion dan
molekul kecil antara alveolus, jaringan interstitial paru dan rongga pleura, pengaturan
respons inflamasi, penyembuhan pleura, fagositosis bakteri dan partikel mineral
seperti serat asbestos, lateks dan quartz (Wang, 2010). Reseptor kaderin merupakan
reseptor dependen kalsium yang diekspresikan pleura untuk mempertahankan
morfologi dan permeabilitas pleura (Wang, 2010). Mesotel memiliki proteinase-
activated receptor-2 (PAR2) yang berperan dalam proses inflamasi dengan melepas
kemokin dan menarik netrofi l ke dalam rongga pleura sehingga terjadi fibrosis pleura,
adhesi pleura atau fibrotoraks (Lai, 2014). Senyawa-senyawa lain yang diekspresikan
mesotel seperti keratin epitelial, fibronektin, vimentin, kolagen, elastin, laminin dan
proteoglikan merupakan senyawasenyawa spesies oksidan kerja panjang yang
berhubungan dengan integritas sel dan sesuai dengan sifat sel-sel epitel dan fibroblas
(Wang, 2010). Sitokin lain yang dapat ditemukan di pleura antara lain interleukin (IL)-
1 yang berhubungan dengan proses fibrogenesis pleura, IL-2 dan IL-5 yang
menginduksi produksi limfosit, IL-3 yang menginduksi proliferasi dan ketahanan
eosinofi l, IL-4 yang menahan infiltrasi sel menuju rongga pleura, IL-6 yang
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menginduksi maturasi limfosit B, IL-8 yang merupakan kemotaksin netrofi l, IL-10


yang merupakan sitokin antiinfl amasi, IL-12 yang akan memperkuat respons
imunologis selular dan respons sitotoksik terhadap patogen intraselular dan tumor, IL-
13 yang merupakan kemoatraktan eosinofi l, IL-16 yang akan menginduksi sitokin
proinfl amasi, monocyte chemotactic peptide (MCP-1), transforming growth factor
(TGF)-β, basic fibroblast growth factor (b-FGF), endostatin sebagai inhibitor
angiogenesis dan platelet-activating factor (PAF) yang bersifat meningkatkan
permeabilitas vaskular (Wang, 2010).

2.1.2 Cairan Pleura

Cairan pleura mengandung 1.500 – 4.500 sel/ mL, terdiri dari makrofag (75%),
limfosit (23%), sel darah merah dan mesotel bebas.2,12,14,15 Cairan pleura normal
mengandung protein 1 – 2 g/100 mL.9 Elektroforesis protein cairan pleura
menunjukkan bahwa kadar protein cairan pleura setara dengan kadar protein serum,
namun kadar protein berat molekul rendah seperti albumin, lebih tinggi dalam cairan
pleura.3 Kadar molekul bikarbonat cairan pleura 20 – 25% lebih tinggi dibandingkan
kadar bikarbonat plasma, sedangkan kadar ion natrium lebih rendah 3 – 5% dan kadar
ion klorida lebih rendah 6 – 9% sehingga pH cairan pleura lebih tinggi dibandingkan
pH plasma.3 Keseimbangan ionik ini diatur melalui transpor aktif mesotel.16 Kadar
glukosa dan ion kalium cairan pleura setara dengan plasma.3

2.1.3 Struktur Makroskopis Pleura

Pleura normal memiliki permukaan licin, mengkilap dan semitransparan. Luas


permukaan pleura viseral sekitar 4.000 cm2 pada laki-laki dewasa dengan berat badan
70 kg. Pleura parietal terbagi dalam beberapa bagian, yaitu pleura kostalis yang
berbatasan dengan iga dan otot-otot interkostal, pleura diafragmatik, pleura servikal
atau kupula sepanjang 2-3 cm menyusur sepertiga medial klavikula di belakang otot-
otot sternokleidomastoid dan pleura mediastinal yang membungkus organ-organ
mediastinum. Bagian inferior pleura parietal dorsal dan ventral mediastinum tertarik
menuju rongga toraks seiring perkembangan organ paru dan bertahan hingga dewasa
sebagai jaringan ligamentum pulmoner, menyusur vertikal dari hilus menuju
diafragma membagi rongga pleura menjadi rongga anterior dan posterior.
Ligamentum pulmoner memiliki pembuluh limfatik besar yang merupakan potensi
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penyebab efusi pada kasus traumatik. Pleura kostalis mendapat sirkulasi darah dari
arteri mammaria interkostalis dan internalis. Pleura mediastinal mendapat sirkulasi
darah dari arteri bronkialis, diafragmatik superior, mammaria interna dan
mediastinum. Pleura servikalis mendapat sirkulasi darah dari arteri subklavia. Pleura
diafragmatik mendapat sirkulasi darah dari cabang-cabang arteri mammaria interna
serta aorta toraksika dan abdominis. Vena pleura parietal mengikut jalur arteri dan
kembali menuju vena kava superior melalui vena azigos. Pleura viseral mendapat
sirkulasi darah dari arteri bronkialis menuju vena pulmonaris. Ujung saraf sensorik
berada di pleura parietal kostalis dan diafragmatika. Pleura kostalis diinervasi oleh
saraf interkostalis, bagian tengah pleura diafragmatika oleh saraf frenikus. Stimulasi
oleh infl amasi dan iritasi pleura parietal menimbulkan sensasi nyeri dinding dada dan
nyeri tumpul pada bahu ipsilateral. Tidak ada jaras nyeri pada pleura viseral walaupun
secara luas diinervasi oleh nervus vagus dan trunkus simpatikus. Eliminasi akumulasi
cairan pleura terutama diatur oleh sistem limfatik sistemik di pleura parietal. Cairan
masuk ke dalam rongga pleura melalui arteriol interkostalis pleura parietal melewati
mesotel dan kembali ke sirkulasi melalui stoma pada pleura parietal yang terbuka
langsung menuju sistem limfatik. Pleksus limfatikus superfi sialis terletak pada
jaringan ikat di lapisan subpleura viseral dan bermuara di pembuluh limfe septa
lobularis dan lobaris. Jaringan limfatikus ini dari pleura kostalis menyusur ventral
menuju nodus limfatik sepanjang arteri mammaria interna atau dorsal menuju ujung
sendi kostosternal, dari pleura mediastinal menuju nodus limfatikus trakeobronkial
dan mediastinum, dan dari pleura diafragmatik menuju nodus parasternal, frenikus
medialis dan mediastinum superior. Cairan pleura tidak masuk ke dalam pleksus
limfatikus di pleura viseral karena pleura viseral lebih tebal dibandingkan pleura
parietal sehingga tidak terjadi pergerakan cairan dari rongga pleura ke pleura viseral.
Gangguan duktus torasikus karena limfoma maupun trauma menyebabkan akumulasi
cairan limfe di rongga pleura menyebabkan chylothorax.

2.2 Fisiologi Pleura

Pleura berperan dalam sistem pernapasan melalui tekanan pleura yang ditimbulkan
oleh rongga pleura. Tekanan pleura bersama tekanan jalan napas akan menimbulkan
tekanan transpulmoner yang selanjutnya akan memengaruhi pengembangan paru
dalam proses respirasi (Sureka, 2015). Pengembangan paru terjadi bila kerja otot dan
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tekanan transpulmoner berhasil mengatasi rekoil elastik (elastic recoil) paru dan
dinding dada sehingga terjadi proses respirasi (Knipe, 2015). Jumlah cairan rongga
pleura diatur keseimbangan Starling yang ditimbulkan oleh tekanan pleura dan
kapiler, kemampuan sistem penyaliran limfatik pleura serta keseimbangan elektrolit
(Boyland, 2010). Ketidakseimbangan komponen-komponen gaya ini menyebabkan
penumpukan cairan sehingga terjadi efusi pleura (Wang, 2010).

2.3.1 Fisiologi tekanan pleura

Tekanan pleura secara fisiologis memiliki dua pengertian yaitu tekanan cairan pleura
dan tekanan permukaan pleura (Miserocchi, 2009). Tekanan cairan pleura
mencerminkan dinamik aliran cairan melewati membran dan bernilai sekitar -10
cmH2 O.

Gambar 3. Perubahan volume paru, tekanan alveolar, tekanan pleura dan tekanan
transpulmoner selama respirasi biasa2

Tekanan permukaan pleura mencerminkan keseimbangan elastik rekoil dinding dada


ke arah luar dengan elastik rekoil paru ke arah dalam. Nilai tekanan pleura tidak serupa
di seluruh permukaan rongga pleura; lebih negatif di apeks paru dan lebih positif di
basal paru. Perbedaan bentuk dinding dada dengan paru dan faktor gravitasi
menyebabkan perbedaan tekanan pleura secara vertikal; perbedaan tekanan pleura
antara bagian basal paru dengan apeks paru dapat mencapai 8 cmH2 O. Tekanan
alveolus relatif rata di seluruh jaringan paru normal sehingga gradien tekanan resultan
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di rongga pleura berbeda pada berbagai permukaan pleura. Gradien tekanan di apeks
lebih besar dibandingkan basal sehingga formasi bleb pleura terutama terjadi di apeks
paru dan merupakan penyebab pneumotoraks spontan (Miserocchi, 2009). Gradien ini
juga menyebabkan variasi distribusi ventilasi.4 Pleura viseral dan parietal saling
tertolak oleh gaya potensial molekul fosfolipid yang diabsorpsi permukaan masing-
masing pleura oleh mikrovili mesotel sehingga terbentuk lubrikasi untuk mengurangi
friksi saat respirasi (Miserocchi, 2009). Proses tersebut bersama tekanan permukaan
pleura, keseimbangan tekanan oleh gaya Starling dan tekanan elastik rekoil paru
mencegah kontak antara pleura viseral dan parietal walaupun jarak antarpleura hanya
10 μm.2,5 Proses respirasi melibatkan tekanan pleura dan tekanan jalan napas. Udara
mengalir melalui jalan napas dipengaruhi tekanan pengembangan jalan napas yang
mempertahankan saluran napas tetap terbuka serta tekanan luar jaringan paru (tekanan
pleura) yang melingkupi dan menekan saluran napas. Perbedaan antara kedua tekanan
(tekanan jalan napas dikurangi tekanan pleura) disebut tekanan transpulmoner.
Tekanan transpulmoner memengaruhi pengembangan paru sehingga memengaruhi
jumlah udara paru saat respirasi. Hubungan perubahan tekanan pleura, tekanan
alveolus, tekanan transpulmoner dan volume paru ditunjukkan pada gambar 3 (Light,
2010).

2.2.2 Fisiologi cairan pleura

Rongga pleura terisi cairan dari pembuluh kapiler pleura, ruang interstitial paru,
saluran limfatik intratoraks, pembuluh kapiler intratoraks dan rongga peritoneum
(Miserocchi, 2009). Neergard mengemukakan hipotesis bahwa aliran cairan pleura
sepenuhnya bergantung perbedaan tekanan hidrostatik dan osmotik kapiler sistemik
dengan kapiler pulmoner. Perpindahan cairan ini mengikuti hukum Starling berikut:

5 Jv = Kf × ([P kapiler – P pleura] - σ [π kapiler – π pleura])

Jv : aliran cairan transpleura,


Kf : koefisien filtrasi yang merupakan perkalian konduktivitas hidrolik membran dengan luas
permukaan membran,
P : tekanan hidrostatik,
σ : koefi sien kemampuan restriksi membran terhadap migrasi molekul besar,
π : tekanan onkotik.
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Gambar 4. Skema tekanan dan pergerakan cairan pada rongga pleura manusia

Perkiraan besar perbedaan tekanan yang memengaruhi pergerakan cairan dari kapiler
menuju rongga pleura ditunjukkan pada Gambar 4. Tekanan hidrostatik pleura parietal
sebesar 30 cm H2 O dan tekanan rongga pleura sebesar -5 cm H2 O sehingga tekanan
hidrostatik resultan adalah 30 – (-5) = 35 cmH2 O. Tekanan onkotik plasma 34 cmH2
O dan tekanan onkotik pleura 5 cmH2 O sehingga tekanan onkotik resultan 34 – 5 =
29 cmH2 O. Gradien tekanan yang ditimbulkan adalah 35 – 29 = 6 cmH2 O sehingga
terjadi pergerakan cairan dari kapiler pleura parietal menuju rongga pleura. Pleura
viseral lebih tebal dibandingkan pleura parietal sehingga koefisien filtrasi pleura
viseral lebih kecil dibandingkan pleura parietal. Koefisien filtrasi kecil pleura viseral
menyebabkan resultan gradien tekanan terhadap pleura viseral secara skematis
bernilai 0 walaupun tekanan kapiler pleura viseral identik dengan tekanan vena
pulmoner yaitu 24 cmH2 O. Perpindahan cairan dari jaringan interstitial paru ke
rongga pleura dapat terjadi seperti akibat peningkatan tekanan baji jaringan paru pada
edema paru maupun gagal jantung kongestif (Miserocchi, 2009).

2.3 Tumor Pleura

Tumor pleura adalah pertumbuhan abnormal sel yang ditemukan di pleural


space. Tumor pleura hampir selalu berupa metastatik (keganasan) dan sulit untuk di
evakuasi. Prognosis dari tumor pleura biasanya buruk kecuali Localized Fibrous
Tumor (LFTP), hanya satu dari delapan jenis LFTP merupakan keganasan, presentase
sembuh setelah operasi pengangkatan sangat tinggi meskipun ukurannya besar.
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Kanker pleura paling sering terjadi akibat kanker metastasis (kanker sekunder), yang
dipicu oleh sel kanker yang menyebar ke pleural space (paling sering dari paru).
Pasien yang memiliki riwayat kanker memiliki resiko lebih tinggi terkena kanker
pleura, terlebih lagi jika terapi yang sudah dilakukan tidak dapat mengontrol
pertumbuhan sel kanker. Meskipun begitu, angka kejadian kanker pleura sangatlah
kecil, terjadi pada 1 dari 2000 pasien kanker.

Sel kanker dapat berpindah ke pleural space melalui sistem hematogen maupun
sistem limfatik. Bisa juga terjadi karena kontak langsung dimana jaringan kanker
menekan pleura dari paru-paru. Metastatis tumor plura bisasanya menyebabkan efusi
pleura. Biasanya cairan berupa darah, sehingga dapat menunjang penegakan
diagnosis. Pengambilan sampel cairan pleura dapat membantu mengetahui kondisi
pasien terkini.

LFTP mungkin tidak menimbulkan gejala apapun. Biasanya ditemukan pada


saat pasien melakukan foto x-ray untuk tujuan yang lain. Tetapi metastasis pleura
tumor menimbulkan gejela yang mirip dengan kanker paru-paru seperti:

 Sesak napas
 Nyeri dada
 Rasa tidak nyaman
 Batuk
 Penurunan berat badan

Salah satu penyebab metastasis tumor pleura adalah komplikasi dari


mesothelioma, yaitu kanker paru yang berhubungan dengan paparan asbestos. Namun
kanker yang lain dapat juga menimbulkan metastatik tumor pleura. Sangat sedikit
yang diketahui tentang penyebab LFPT, kebanyakan tumor ini bersifat benign.

Radiological assessment of pleural tumors requires complete knowledge of


pleural anatomy. Various benign, malignant and tumor-like conditions can involve the
pleura. Malignant pleural neoplasms are more common. The most common tumor-
like condition involving the pleura is pleural thickening. Radiological features of
pleural disease can have varied spectrum including pleural effusion, pleural plaques,
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and nodular pleural thickening. Differentiation of pleural neoplasms from pulmonary


and extrapleural neoplasms is crucial in making appropriate diagnosis.

2.3.1 Differentiating pleural, pulmonary, and extrapleural neoplasms

Pulmonary neoplasms usually have acute angles with the chest wall, are centered in
the lung, and engulf the pulmonary vasculature. A pleural neoplasm shows obtuse
angles with the lateral chest wall with tapered margins [Figures [Figures33 and
and4],4], displaces the pulmonary vasculature, changes its location on respiration, and
may show incomplete border sign on chest radiograph – i.e., only a portion of the
margin of mass is depicted on chest radiograph. Next step lies in differentiation of
pleural from extrapleural origin of the mass. Extrapleural neoplasms may arise from
extrapleural fat, ribs, intercostal muscles, and neurovascular bundle; typical pleural
neoplasms do not cause erosion of ribs and displace the extrapleural fat outward, while
extrapleural neoplasms displace the extrapleural fat inward

Loculated empyema: (A) Chest radiograph showing pleural-based opacity (arrow) with
tapering obtuse margins in left hemithorax; (B) axial contrast-enhanced CT scan showing
loculated collection (arrowhead) with peripherally enhancing thick walls
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Calcified empyema: (A) Chest radiograph showing volume loss right hemithorax with veil-
like calcified (arrow) pleural opacity; (B) axial contrast-enhanced CT scan showing evidence
of calcified chronic empyema (arrow) with proliferation of extrapleural fat and crowding of
ribs suggestive of volume loss in right hemithorax

Differentiating pleural, pulmonary, and extrapleural neoplasms


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2.3.2 Tumors and tumor-like conditions involving the pleura

Various benign, malignant, and tumor-like conditions can involve the pleura [Table
2]. Malignant neoplasms are more common than benign neoplasms. Pleural tumors
can have a varied imaging spectrum – may be unilateral or bilateral, calcified, or
noncalcified, and focal or diffuse.

Benign and malignant pathologies of pleura

2.3.3 Pleural thickening

Pleural thickening may be focal or diffuse. Diffuse pleural thickening is defined as


thickening of pleura (more than 5 mm) with combined area of involvement more than
25% of chest wall if bilateral and 50% involvement if unilateral.[1] Apical pleural
thickening is a normal aging process, but if the thickening is more than 2 cm, it
requires further work-up [Figure 5]. On Computed Tomography (CT) scan, malignant
pleural thickening is nodular (>1 cm), shows circumferential involvement, and
involves the mediastinal pleura. On imaging, benign pleural thickening appears as a
diffuse involvement of pleura. Pleural thickening greater than 5 cm in width, 8 cm in
craniocaudal extent, and 3 mm in thickness usually suggests a benign etiology [Table
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3].[2] Causes of diffuse pleural thickening are empyema, asbestosis, hemothorax,


pulmonary fibrosis, irradiation, previous surgery, trauma, and drugs. In developing
countries, tuberculosis is an important cause of pleural thickening. Pleural
involvement in tuberculosis is either due to rupture of subpleural caseous focus within
the lung, hematogenous dissemination, or involvement from an adjacent lymph node.
Tubercular pleural involvement may be in the form of pleural effusion, pleural
thickening, empyema, bronchopleural or pleurocutaneous fistula, or calcifications. On
imaging, volume loss, calcifications, and proliferation of extrapleural fat are
suggestive of diffuse benign pleural thickening. Fluorine-18 fluorodeoxyglucose
positron emission Computed Tomography (18F-FDG PET CT) cannot reliably
differentiate benign and malignant pleural thickening. However, a standardized uptake
value (SUVmax) greater than 2 requires further evaluation with clinical correlation or
image-guided biopsy.[3,4] Pleural plaques are deposits of hyalinized collagen fibers
in the parietal pleura. Pleural plaques may be calcified or noncalcified. On imaging,
pleural plaques are seen as focal pleural thickening.
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Apical pleural thickening: Chest radiograph showing apical pleural thickening (arrowhead) in
left apical region

Pleural thickening
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2.3.4 Solitary fibrous tumor

Solitary fibrous tumor of pleura (SFTP) is also known as localized fibrous tumor or
localized pleural mesothelioma.[5] It is usually seen in the age group of 45-60 years.
Most of these tumors are benign, but in 20% cases, they can be malignant. The tumor
usually arises from the visceral pleura in 80% of cases. On imaging, SFTP appears as
a soft tissue pleural-based neoplasm with areas of necrosis, hemorrhage, and cystic
changes [Figures [Figures66 and and7].7]. Calcification may be seen in up to 26% of
cases. Heterogeneous enhancement is seen post-contrast. On magnetic resonance
imaging (MRI), hypointense solid mass is seen on T1- and T2-weighted images.
Necrosis and cystic degeneration changes show high T2 signal intensity.
Differentiation of benign and malignant fibrous tumors is difficult on imaging.
Features suggestive of malignant fibrous tumors are presence of calcification,
effusion, atelectasis, mediastinal shift, and chest wall invasion [Figures [Figures88
and and99].[6,7] Presence of stalk also suggests benign nature. On CT, the stalk is
identified as a linear soft tissue extending into the pleura/interlobar fissure/hilum.
Presence of stalk is also confirmed by change in its location on respiration.
Associations of SFTP are clubbing, hypertrophic osteoarthropathy (Pierre–Marie–
Bamberger syndrome), and hypoglycemia (Doege–Potter syndrome). Hypoglycemia
occurs as a result of the production of insulin-like growth factor II (IGF-II) by these
tumors.[8] Hypertrophic osteoarthropathy occurs as a result of production of ectopic
growth hormone-like substance and is more common with tumors greater than 7 cm.
Histologically, morphology is similar to that of a low-grade spindle cell neoplasm.
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Benign solitary fibrous tumor: (A) Chest radiograph showing pleural-based opacity (arrow)
in right hemithorax with peripheral obtuse margins; (B) axial contrast-enhanced CT scan
showing heterogeneously enhancing pleural-based mass (arrowhead) proved to be benign
fibrous pleural tumor

Pleural fibroma: (A) Chest radiograph showing lobulated pleural-based opacity (arrow) in
right apical region; (B) axial contrast-enhanced CT scan showing heterogeneously enhancing
peripheral mass lesion (arrow) in a biopsy-proven case of benign pleural fibroma
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Malignant solitary fibrous tumor of pleura: Plain axial CT scan showing pleural-based soft
tissue lesion with peripheral as well as internal calcification (arrow) abutting the liver
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Malignant fibrous tumor of pleura: Axial contrast-enhanced CT scan showing


heterogeneously enhancing mass lesion left hemithorax (arrowhead) causing mediastinal
displacement to the right

2.3.5 Malignant mesothelioma

Mesothelioma is a highly malignant and locally aggressive tumor seen in the sixth or
seventh decade of life. It is associated with asbestos exposure, with an average latency
of 35-40 years for its development. Hypertrophic osteoarthropathy and intermittent
hypoglycemia are less common than SFTP. Most carcinogenic form of asbestos is
crocidolite. Insulation workers, shipyard workers, construction workers, workers in
heating trades, and asbestos miners are at greatest risk. Other factors which predispose
to development of mesothelioma are radiation therapy, tuberculosis, and chronic
empyema. On imaging, diffuse nodular pleural thickening, pleural plaques, and
pleural effusion are usually seen [Figures [Figures1010 and and11].11]. The latent
period for pleural plaque formation is usually 20 years and presence of pleural plaques
is a strong indicator of asbestos exposure. Typically, pleural plaque is seen adjacent
to ribs, involving sixth to ninth ribs. Pleurae along the intercostal spaces, costophrenic
angles, and lung apices are less frequently involved. Large pleural effusion without
mediastinal shift may also be seen [Figures [Figures1212 and and13].13].
Calcifications are seen involving the diaphragmatic parietal pleura [Figure 14].[9,10]
On MRI, the lesions show low to intermediate signal intensity on T1-W images and
high signal intensity on T2-W images with post-contrast enhancement. Differentiation
from metastatic carcinoma is difficult; however, unilateral involvement and volume
loss of affected hemithorax favors mesothelioma. Imaging criteria for unresectability
includes encasement of diaphragm and involvement of extrapleural fat, ribs, or other
mediastinal structures
21

Malignant mesothelioma: Axial contrast-enhanced CT scan showing enhancing nodular


pleural thickening (arrows) involving the costal and mediastinal pleura, extending into the
major fissure (arrowhead) with crowding of ribs suggestive of volume loss changes in left
hemithorax
22

Malignant mesothelioma: Axial contrast-enhanced CT scan showing homogeneously


enhancing nodular pleural thickening (arrows) involving the mediastinal and costal pleura
with volume loss changes in left hemithorax
23

Mesothelioma presenting as pleural collections: Axial contrast-enhanced CT scan showing


nodular thickening of pleura involving right hemithorax with small pleural collections
(arrows)
24

Mesothelioma presenting as a pleural effusion: Axial contrastenhanced CT scan showing


moderate left pleural effusion as loculated collection with thickening of pleura (arrows) in a
case of mesothelioma
25

Mesothelioma and pleural plaques: (A) Axial plain CT scan showing calcified (arrows) and
noncalcified (arrowhead) pleural plaques; (B) axial plain CT scan image showing calcified
plaque (black arrow) classically involving the diaphragmatic parietal pleura in a construction
worker

2.3.6 Lymphoma

Both Hodgkin's and non-Hodgkin's lymphoma can involve the pleura. On imaging,
effusion, pleural nodules, focal or diffuse pleural thickening may be seen, which show
homogeneous contrast enhancement. Associated mediastinal and hilar
lymphadenopathy is also seen [Figures [Figures1515 and and16].16]. Cystic/necrotic
changes and calcification are seen post-chemotherapy. Circumferential pleural
involvement is less common in lymphoma.
26

Pleural lymphoma: Axial contrast-enhanced CT scan showing heterogeneously enhancing


lobulated mass lesion involving the diaphragmatic pleura (arrow) and invading the chest wall
in a case of high-grade lymphoma

Pleural lymphoma: Axial contrast-enhanced CT scan showing homogeneously enhancing


nodular pleural thickening (arrows) involving the costal pleura with mediastinal
lymphadenopathy (asterisk)

2.3.7 Calcifying fibrous pseudotumor

The term calcifying fibrous pseudotumor was coined by Fetsch et al. in 1993.[12]
Previously, these tumors were termed as “childhood fibrous tumor with psammoma
bodies.” These neoplasms occur in children and young adults. History of previous
inflammation is a prerequisite for the diagnosis. On imaging, extensive solitary or
multifocal masses with calcifications are seen [Figures [Figures1717 and
and1818].[13,14]
27

Calcifying fibrous pseudotumor: (A) Chest radiograph showing pleural-based calcified


opacity (arrowhead) left hemithorax with incomplete border sign; (B) plain axial CT scan
image showing pleural-based calcified lesion (arrow) with no destruction of underlying ribs
28

Calcifying fibrous pseudotumor: Plain axial CT scan showing calcified pleural-based opacity
in right hemithorax (arrowhead)

2.3.8 Pleural metastases

Adenocarcinomas are known to cause pleural metastasis more frequently than other
histological types of cancers. Common primary sites are from lung, breast, lymphoma,
and ovary [Figures [Figures1919–21]. Invasive thymoma can also involve the pleura
[Figure 22]. On imaging, pleural effusion is the most common finding. Diffuse or
focal nodular pleural thickening may be seen. Increased 18F-FDG uptake on PET-CT
is seen in malignant pleural thickening and effusion.[2]

Pleural metastases: Axial contrast-enhanced CT scan showing heterogeneously enhancing


pleural-based soft tissue (white arrow) with rib destruction (black arrow) in a case of pleural
metastases from renal cell carcinoma
29

Pleural metastases: Axial contrast-enhanced CT scan showing nodular pleural thickening


(arrows) involving the costal and mediastinal pleura with malignant pleural effusion in a case
of metastatic ovarian adenocarcinoma
30

Pleural drop metastases in invasive thymoma: Axial contrast-enhanced CT image showing


heterogeneously enhancing anterior mediastinal mass (arrowhead) with mild left pleural
effusion and ipsilateral pleural implants (arrows)
31

Pleural metastases: Axial contrast-enhanced CT scan showing heterogeneously enhancing


pleural-based mass lesion (arrow) in left hemithorax with extrathoracic extension in a case
of metastatic adenocarcinoma

2.3.9 Askin tumor

Askin tumor is an aggressive malignant tumor of primitive neuroectodermal origin


belonging to the Ewing tumor family. Most of these tumors arise from the soft tissues
of the chest wall or lung periphery. It is usually seen in children and adolescents. On
histopathology, malignant, small round cells with Homer–Wright rosettes are seen.
Balanced reciprocal chromosomal translocation between chromosomes 11 and 22 is
diagnostic. On imaging, unilateral involvement is generally seen in the form of
nodular pleural thickening. Infiltration into the chest wall, mediastinum, and
sympathetic chain is pathognomonic. Pleural effusion and rib destruction may or may
not be seen [Figure 23].
32

Askin tumor: (A) Chest radiograph showing inhomogeneous opacity (arrow) right hemithorax
obscuring right hemidiaphragm without mediastinal shift; (B) axial contrast-enhanced CT
scan showing heterogeneously enhancing nodular pleural-based lesions (arrows) involving
the costal and mediastinal pleura with characteristic involvement of the sympathetic chain
(arrowhead) in right paraspinal region

2.3.10 Rare pathologies of pleura

2.3.10.1 Pleural lipoma

Pleural lipoma is often an incidental finding. It is one of the most common benign
tumors of pleura. On CT, lipoma shows fat density and no contrast enhancement.
Presence of enhancing septa within the mass suggests liposarcoma.

2.3.10.2 Pleural splenosis

Pleural splenosis results from displaced splenic tissue into the thorax following trauma
on the left side. On imaging, multiple soft tissue lesions of variable sizes are seen
implanted on pleura, with enhancement similar to splenic tissue. Gold standard for
diagnosis is scintigraphy with 99mTc heat-damaged tagged erythrocytes.

2.3.10.3 Other

Other rare pathologies of pleura are mesothelial cysts, epithelioid


hemangioendothelioma, Castleman disease, sarcomas [Figure 24], malignant fibrous
33

histiocytoma, leukemic infiltration, Erdheim–Chester disease, and extraskeletal


osteosarcoma. Extraskeletal osteosarcoma is a rare malignant neoplasm and
constitutes 1.2% of all soft tissue sarcomas. It should be considered in the differential
diagnosis for a rapidly growing calcified pleural mass in an elderly. Other causes of
malignant pleural calcification are metastasis from osteosarcoma, chondrosarcoma,
parosteal osteosarcoma, and mesothelioma.[15]

Spindle cell sarcoma of pleura: (A) Chest radiograph showing complete opacification of right
hemithorax (arrowhead) with mediastinal shift to the left; (B) axial contrast-enhanced CT
scan showing heterogeneously enhancing nodular pleural-based lesions with pleural
effusion displacing the heart to the left

Pleural pseudotumor[16] is fluid collection within a lung fissure. Most common site
for pseudotumor is minor fissure. Common causes of pleural pseudotumor are
congestive heart failure, cirrhosis, and renal insufficiency. On chest radiographs,
classical lenticular or biconvex opacity is seen in the fissure. It usually resolves after
therapy with diuretic agents.

An approach to correct diagnosis of pleural tumors depends on the pattern of


involvement – focal or diffuse, unilateral or bilateral, and calcified or noncalcified
[Table 4]. The role if imaging is to identify pleural thickening, differentiate benign
and malignant pleural thickening, and identify the cause if possible. An appropriate
34

clinical history, imaging findings and, if required, image-guided biopsy may be used
to clinch the diagnosis.

Approach to diagnosis of pleural pathologies