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Cystic Lung Disease
: What Can Cause Holes in the Lung?
Category: Diffuse lung disease
?
Hyun Jung Koo, Mi Young Kim, Jae Woo Song
Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Learning Objectives
To know the pathogenesis of creation of cystic lung lesions
Pathogenesis
To learn the imaging findings of various cystic lung disease
Imaging Findings Cystic
Lung
DiseaseDifferential Diagnosis
To differentiate many cystic lung diseases using clinical and imaging findings
• None
Financial Disclosures
Pathogenesis
Check valve air cyst or ball-valve effect Pulmonary LCH, LAM, Lymphocytic interstitial pneumonia
Drainage of necrotic lung parenchyma Pneumatocele
Vascular occlusion or ischemic necrosis Septic embolism, metastasis
Dilatation of bronchioles Desquamative interstitial pneumonia, Light chain deposition disease
Focal collection of air in interstitial tissue
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Cyst
Pathology
Round circumscribed space surrounded by an epithelial or fibrous wall
CT scans
Round parenchymal lucency or low-attenuating area with a well-defined interface
with normal lung
Usually thin-walled (2 mm) and occur without associated pulmonary emphysema
– Thin-walled airspaces: patients with LAM or LCH
– Thicker-walled honeycomb cysts: patients with end-stage fibrosis
Usually contain air but may contain fluid or solid material
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Bleb
Definition
A small gas-containing space within the visceral pleura or in the subpleural lung,
not larger than 1 cm in diameter
CT scans
A thin-walled cystic air space contiguous with the pleura
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Bulla
Pathology
An airspace measuring more than 1 cm—usually several centimeters—in diameter,
sharply demarcated by a thin wall that is no greater than 1 mm in thickness.
Usually accompanied by emphysematous changes in the adjacent lung
CT scans
A rounded focal lucency or area of decreased attenuation, 1 cm or more in
diameter, bounded by a thin wall
Often associated with other signs of pulmonary emphysema
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Emphysema
Pathology• Permanently enlarged airspaces distal to the terminal bronchiole with destruction
of alveolar walls• Classified in terms of the part of the acinus predominantly affected
– proximal (centriacinar, more commonly termed centrilobular, emphysema)
– distal distal (paraseptal emphysema)
– whole acinus (panacinar or, less commonly, panlobular emphysema)
CT scans• Focal areas or regions of low attenuation, usually without visible walls
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Honeycombing
Pathology• Destroyed and fibrotic lung tissue containing numerous cystic airspaces with thick
fibrous walls• Late stage of various lung diseases, with complete loss of acinar architecture. • Size from a few millimeters to several centimeters in diameter• Variable wall thickness, and lined by metaplastic bronchiolar epithelium
CT scans• Usually 3–10 mm but occasionally as large as 2.5 cm• Usually subpleural and characterized by well-defined walls• A CT feature of established pulmonary fibrosis• Often considered specific for pulmonary fibrosis and is an important criterion in
the diagnosis of usual interstitial pneumonia
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Cavity
Definition• Usually produced by the expulsion or drainage of a necrotic part of the lesion via
the bronchial tree• Sometimes contains a fluid level
CT scans• A gas-filled space, seen as a lucency or low-attenuation area, within pulmonary
consolidation, a mass, or a nodule• Cavitating consolidation: the original consolidation may resolve and leave only a
thin wall.
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Diffuse cystic lung disease
• Pulmonary Langerhans cell histiocytosis (PLCH)
• Lymphocytic interstitial pneumonia (LIP)
• Desquamative interstitial pneumonia (DIP) and respiratory
bronchiolitis–associated interstitial lung disease (RB-ILD)
• Birt-Hogg-Dube syndrome (BHD)
• Metastasis
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Diffuse cystic lung disease
Pulmonary Langerhans cell histiocytosis (PLCH)17/M Chest pain, dyspnea, smoking (+)
Reticulonodular pattern with upper and mid lung zone distributionSparing of costophrenic anglesLung volumes preserved or enlargedPneumothorax
Leader-cath inserted
Case 1
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Pulmonary Langerhans cell histiocytosis (PLCH)18/M Left chest pain (3 month earlier)
Cysts seen approximately 80% and nodules seen 60~80% of patientsEnd-stage disease may be difficult to differentiate from extensive bullous emphysema
Basic pathogenesis: Langerhans’ cell proliferation, centered around the membranous and the respiratory bronchiole
Case 2
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Pulmonary Langerhans cell histiocytosis (PLCH)
35/M
Several thin-walled cystic lesions in both upper lobes.Focal osteolytic lesion with soft tissue involving right scapula (yellow arrow). Right scapula needle biopsy: Langerhans cell histiocytosis
Case 3
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Pulmonary Langerhans cell histiocytosis (PLCH)
Radiology report:Multiple nodules with cavitary nodules in both lungs. Possible metastasis from unknown primary site
62/M
Lung, core needle biopsy: Langerhans cell histiocytosisCase 4
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Bilateral thin-walled cysts scattered throughout the lungs with no zonal predominance More uniform in size, round, and diffuse in distribution of cysts Diffuse throughout the lung including costophrenic angles
30/F Left chest pain
Lymphangioleiomyomatosis (LAM)
Case 5
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lymphangioleiomyomatosis (LAM)
2016-03-312011-10-10
Case 5
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lymphangioleiomyomatosis (LAM)
41/F
Case 6
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lung: Lymphangioleiomyomatosis(LAM)
Calcified tubers, cerebral hemisphere and ventricles Angiomyolipomas
Lymphangioleiomyomatosis (LAM) and tuberous sclerosis (TSC)
26/F, Chest pain, recurrent pneumothorax (x 5)
Basic pathogenesis: idiosyncratic smooth muscle cell proliferation then cyst formation Case 7
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lymphocytic interstitial pneumonia (LIP)
72/F, Dyspnea
Sjogren’s syndrome-Lymphocytic interstitial pneumonia
GGO and areas of consolidation Peribronchovascular thickening Interlobular septal thickening Subpleural nodules Lymphadenopathy
2014-07-21Benign lymphoproliferative disorderCharacterized by pulmonary interstitial infiltration of lymphocytes with varying admixtures of plasma cells, and lymphoid follicles.
2015-01-30
Lymphocytic interstitial pneumonia
Case 8
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lymphocytic interstitial pneumonia (LIP)
Sjogren’s syndrome-Lymphocytic interstitial pneumonia
2016-3-30On steroid therapy
Thin-walled cysts with a basal predominance (1–30 mm) Typically less numerous than in LAM or LCH.
Case 8
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Lymphocytic interstitial pneumonia (LIP)
Sjogren’s syndrome-Lymphocytic interstitial pneumonia, Amyloidosis
10 yrs later
59/F
10 yrs later
Case 9
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Desquamative interstitial pneumonia (DIP)
48/M, 30 PY
A peripheral subpleural and basal predominance of ground-glass opacity Small cystic spaces may develop within the areas of ground-glass opacity Coexistent emphysema Case 10
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Desquamative interstitial pneumonia (DIP)
52/M
These small cysts are shown to represent bronchiolectasis and dilated alveolar ducts About a third of patients
Case 11
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Respiratory Bronchiolitis (RB)-ILD50/M, Current smoker
RB-ILD with emphysema ?Advanced pulmonary LCH ?
Lung, wedge resection: Smoking associated respiratory bronchiolitis
Case 12
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Discrete thin-walled cysts, mostly in the lower lobes Vary in size and shape Large lung cysts are frequently multiseptated. Total extent of lung involvement being less than 30%
Birt-Hogg-Dube Syndrome23/F, Dyspnea, familial history of pneumothorax
Lung wedge resection:- Subpleural bullae.- Single parenchymal cysts with focal smooth muscle proliferation.
Susceptibility to kidney tumor (RCC, oncocytoma, AML), renal and pulmonary cysts, and noncancerous tumors of hair follicles, called fibrofolliculomas
Case 13
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
RUL wedge resection: adenocarcinoma, moderately differentiated, micropapillary predominant type, EGFR/ALK mutation (-)
Metastasis67/M, Dry cough
Case 14
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Metastasis
Case 15
67/M, Dry cough
Sarcoma including angiosarcoma, osteosarcoma, epithelioid hemangioendothelioma
Squamous cell carcinoma, head and neck cancer in men and cervix cancer in women
Adenocarcinoma including colorectal cancer
Skin, (left forehead), punch biopsy:- VASCULAR TUMOR, FAVOR ANGIOSARCOMA.
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Focal or multifocal cystic lung disease
• Pneumatocele• Fungal infection including invasive aspergillosis, mucormycosis• Tuberculosis• Pneumocystis jiroveci pneumonia (PJP) • Paragonimiasis
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Focal or Multifocal cystic lung disease
Pneumatocele
Case 16/17
36/F
2 months later
After pneumonia, thin-walled air cyst lesion is appeared in the right upper lobe.
41/F
Sequelae of previous septic embolism with pneumatocele caused by MRSA
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Invasive pulmonary aspergillosis30/F AML BMT GVHD Bronchiolitis obliterans
2016-3-28 2016-7-11 Case 18
Tx: Voriconazole
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Pulmonary tuberculosis
2007-9-20Case 19
Tx: AntiTBc med.
19/M
2007-7-9
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Pneumocystis jiroveci pneumonia (PJP)
35/M
Paragonimiasis
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
5-15 mm filled with either fluid or gas, within the consolidation
About 60% of patients Subpleural linear opacities communicating
with a cyst, suggestive of worm migration tracks
63/M
Case 20/21
Pneumocystis jiroveci pneumonia
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
47/M
Pulmonary LCH
17/M
Birt-Hogg-Dube Syndrome
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
Paragonimiasis
57/M 63/M
Summary
Disease Clinical feature HRCT feature Association
Pulmonary LCH SmokerYoung adultsPneumothorax
Early centrilobular nodulesSparing of CPA
Pulmonary HT
LAM and tuberous sclerosis-Sporadic-TS complex
PneumothoraxChylous effusion-Perimenopausal women-Seizures, skin lesions
Throughout the lungs No zonal predominance-Tiny nodules, MMPH
Angiomyolipoma-AD inherited
LIP-Follicular Bronchiolitis & LIP-AmyloidosisLight chain deposition Ds.
-Sjögren disease, cardiovascular Ds., AIDS-Renal failure, Sjögren disease
-Peri-vascular cysts, smaller nodules-Cysts and larger nodules
-Lymphoproliferative disorder, lymphoma-Multiple myeloma , Macroglobulinemia
DIP and RB–ILD Smoker, male Subpleural and basal predominance of ground-glass opacity Coexistent emphysema
Birt-Hogg-Dube syndrome Skin lesions, Fibrofolliculomas Basilar subpleural lentiform cysts Renal oncocytoma, RCC, AD inherited
Cystic metastasis History of malignancy, CA-125 (+) Peripheral nodulesGGO, by hemorrhage
CRC, sarcoma, H & N or cervical cancer
Septic embolism Fever, sepsis Peripheral nodulesWedge consolidation
Pulmonary or extra-pulmonary infection
Seaman et al. AJR 2011; 196:1305–1311, Modification
Introduction Terminology Diffuse CLD Multifocal CLD Quiz Summary
References
1. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008 Mar;246(3):697-722.
2. Seaman DM, Meyer CA, Gilman MD, McCormack FX. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol. 2011 Jun;196:1305-11.
3. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest 2008; 133:507–516.4. Jeong YJ, Lee KS, Chung MP, et al. Amyloidosis and lymphoproliferative disease in Sjögren
syndrome: thin section computed tomography findings and histopathologic comparison. J Comput Assist Tomogr 2004; 28:776–781.
5. Chan DP, Griffith JF, Lee TW, Chow LT, Yim AP. Cystic pulmonary metastases from epithelioid cell sarcoma. Ann Thorac Surg 2003; 75:1652–1654
6. Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc2003;78:744–752.
7. Ichikawa Y, Kinoshita M, Koga T, Oizumi K, Fujimoto K, Hayabuchi N. Lung cyst formation in lymphocytic interstitialpneumonia: CT features. J Comput Assist Tomogr 1994;18:745–748.
8. Weisbrod GL, Chamberlain D, Herman SJ. Cystic change (pseudocavitation) associated with bronchioloavleolar carcinoma: a report of four patients. J Thorac Imaging 1995;10:106–111.
Thank you for your attention.
Contact: [email protected]