Workshop Cyst & Lucency. How to Approach
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1 Workshop Cyst & Lucency How to Approach
2 To Approach Cystic Lung Disease True cysts? Cavitary disease Cystic bronchiectasis Mosaic attenuation Subpleural cysts Bullae Paraseptal emphysema Honeycombing Birt Hogg Dubè syndrome (BHD) Parenchymal cysts Solitary/Multiple If multiple: No associated HRCT findings Associated nodules Associated ground glass nodules/opacity
3 How to Approach Cystic Lung Disease Parenchymal cysts w/o other abnormalities 1. Solitary: incidental, pneumatocele, bronchogenic cyst 2. Focal, multifocal, diffuse: o o o Focal: > 1 cyst in one lobe of the lung. Multifocal : > 1 lobe but not all lobes. Diffuse: involving all 5 lobes.
4 Multiple Parenchymal Cysts No associated findings LAM/TS BHD PLCH Metastasis Infection Papillomatosis Associated nodules PLCH LIP Light-chain deposition disease (LCDD) Amyloid Metastasis Associated GGO Pneumocytis Jivorecii pneumonia DIP Chronic HP
5 Chapter 1
6 Question 1 (Code )
7 Question 1 (Code )
8 Question 1 (Code )
9 Question 1 (Code )
10 Air trapping Pulmonary Oligemia GGO with crazy paving Answers of question 1 (Code )
11 Pattern of abnormality Size of PA Bronchus Associated findings Expiration Air trapping Oligemia GGO Geographic area, equal to lobule Normal/ slightly small Dilated and/or thick wall Centrilobular nodule/tib attenuation <100 HU Geographic area, larger than lobule Small with collateral artery Normal to slightly dilated Infarction/Scar attenuation >100 HU Poorly-defined area Normal Normal
12 Air trapping Air trapping Question 1 (Code ) Geographic area of low density, equal to lobule Normal or slightly small PA in low density area Dilated bronchus and/or thick wall May seen ill-defined centrilobular nodule/tib Expiration: No or slightly increase in attenuation (<100 HU) Not improve in follow-up
13 Pulmonary oligemia Oligemia Geographic area of low density, larger than lobule Small PA in low density area, collateral vessel Normal to slightly dilated bronchus Subpleural scar or infarct Expiration: attenuation >100 HU Not improve in follow-up Question 1 (Code )
14 Code 5.15
15 Ground glass opacity Ground glass opacity Poorly-defined area between high and low density area Normal PA Normal bronchus Expiration: attenuation >100 HU May improve on follow-up Question 1 (Code )
16
17 Question 2 (Code 5.15) Inspiration Expiration
18 Question 2 (Code 5.15) Inspiration Expiration
19 Question 2 (Code 5.15) Inspiration
20 Question 2 (Code 5.15) Expiration
21 Question 2 (Code 5.15) Inspiration
22 Question 2 (Code 5.15) Expiration
23 Pulmonary oligemia Geographic area of low density, larger than lobule Small PA in low density area, collateral vessel Expiration: attenuation >100 HU Question 2 (Code 5.15)
24 Question 2 (Code 5.15)
25 Question 3 (Code 5.16) Inspiration Expiration
26 Question 3 (Code 5.16) Inspiration
27 Question 3 (Code 5.16) Expiration
28 Air trapping Geographic area of low density, equal to lobule Normal or slightly small PA Dilated bronchus and/or thick wall Expiration: no/ slightly attenuation <100 HU Question 3 (Code 5.16)
29 Inspiration Expiration
30 Inspiration Expiration Question 4: Code 5.25
31 Air trapping in constrictive bronchiolitis Geographic area of low density, equal to lobule Normal or slightly small PA Dilated bronchus and/or thick wall Expiration: no/ slightly attenuation <100 HU Question 4: Code 5.25
32 Question 5 (Code 5.13)
33 Question 5 (Code 5.13)
34 Question 5 (Code 5.13)
35 Question 5 (Code 5.13)
36 Question 5 (Code 5.13)
37 Bronchiectasis Lack of bronchial tapering Signet-ring sign Cluster of grapes sign Usually found ill-defined centrilobular nodule and/or air trapping Question 5 (Code 5.13)
38 Chapter 2
39 Subpleural Cysts Parenchymal cysts
40 Subpleural Cysts Bullae Round focal lucency > 1 cm in Ø Thin, almost imperceptible wall. May be formed by coalescence of paraseptal emphysema. Can enlarged over time.
41 Subpleural Cysts Paraseptal emphysema Well-defined lucent area < 1 cm. Subpleural & peribronchovascular area. Cyst walls: not abut each other. bound by pleural surface and interlobular septa. Coalescence of cysts bullae. Preserve lung architecture.
42 Honeycombing Air-filled cystic spaces 3-10 mm in Several layers & Share walls Subpleural lung Associated traction bronchiectasis, architectural distortion, volume loss
43 Question 6 (Code 5.2)
44 Question 6 (Code 5.2)
45 Question 6 (Code 5.2)
46 Question 6 (Code 5.2)
47 Question 6 (Code 5.2)
48 Question 6 (Code 5.2) Airspace enlargement with fibrosis (AEF) Multiple thin-walled (mostly < 1 mm thick) cyst, various size Affected lobes: o upper lobe. o upper & middle part of lower lobes. Distribution: Slightly apart from pleura Clinical important: No risk of acute exacerbation (in contrast to UIP) Watanabe et al, Eur J Radiology2015
49 Paraseptal emphysema Honeycombing Characters Usually one layer Several layers Variable AEF Wall thickness Imperceptible 1-3 mm Mostly < 1 mm Affected lobe Usually upper lobe Usually lower lobe Upper lobe Upper & middle part of lower lobes. Location Subpleural Subpleural Slightly apart from pleura Volume of affected area No volume loss Volume loss No volume loss Watanabe et al, Eur J Radiology2015
50 31
51 Thick-walled cystic lesions (TWCLs) Described in CTD associated CPFE by Cottin et al. Arbitrarily defined as cysts > 2 cm in Ø & delimitated by a wall >1 mm thick in an area of the lung where reticulation was present. Cottin et al, Arthritis & Rheumatism 2011 (Code 5.3.2)
52 Question 7 (Code 5.1)
53 Question 7 (Code 5.1)
54 Question 7 (Code 5.1)
55 Question 7 (Code 5.1)
56 Question 7 (Code 5.1)
57 Question 7 (Code 5.1) Paraseptal emphysema & Bulla
58 Thick-walled cystic lesions (TWCLs) Question 7 (Code 5.1)
59 Question 8 (Code 5.11)
60 Question 8 (Code 5.11)
61 Question 8 (Code 5.11)
62 Question 9 (Code 5.11)
63 Question 8 (Code 5.11)
64 Question 8 (Code 5.11) Cyst location: subpleural e.g. bullae, traction bronchiectasis/bronchiolectasis, honeycomb. Vertical distribution: lower Ancillary findings: esophageal dilatation Dx: Systemic sclerosis related f-nsip.
65 Question 9 (Code 5.24)
66 Question 9 (Code 5.24)
67 Question 9 (Code 5.24)
68 Question 9 (Code 5.24)
69 Question 9 (Code 5.24)
70 Neurofibromatosis I Autosomal dominant Café au lait spot, peripheral nerve tumor Thin walled bullae, and cysts in upper-mid lung zone. Fibrosis in lower lung zone. Chest wall and mediastinal involvement Question 9 (Code 5.24)
71 Chapter 3
72
73 Centrilobular emphysema Location: Upper zone predominance. Centrilobular lucency. No distinct wall. Exhibit a central dot: branch of pulmonary artery in the 2 o pulmonary lobule.
74
75 Panlobular Emphysema Basal predominance Diffuse decrease in lung attenuation Pulmonary vascular pruning
76 Panbular emphysema (Code 5.1)
77 Multiple Parenchymal Cysts No associated findings LAM/TS BHD PLCH Metastasis Infection Papillomatosis Associated nodules PLCH LIP Light-chain deposition disease (LCDD) Amyloid Metastasis Associated GGO Pneumocytis Jivorecii pneumonia DIP Chronic HP
78 To Approach Cystic Lung Disease The important features to approach multiple cystic lesions Distribution of cysts Most important Cyst shape and size. Presence of any ancillary findings.
79 Question 10 (Code 5.20)
80 Question 10 (Code 5.20)
81 Question 10 (Code 5.20)
82 Question 10 (Code 5.20)
83 Question 10 (Code 5.20)
84 Question 10 (Code 5.20) LAM Female Uniform thin wall cyst No preferential distribution Occasional pneumocyte hyperplasia nodule Associated with renal angiomyolipoma
85 Question 11 (Code 5.22)
86 Question 11 (Code 5.22)
87 Question 11 (Code 5.22)
88 Question 11 (Code 5.22)
89 Question 11 (Code 5.22)
90 Question 11 (Code 5.22) LCH Almost exclusively in smokers. Young adult, slightly older in women. No gender predilection. Typically combination of nodules, cavitated nodules and cysts. Bizarre-shaped, thick & irregular walled cysts. Poorly or well defined nodules, regress overtime. Associated emphysema, bullae Upper lobe predominance. Relatively sparing lung base & CPA Extrapulmonary manifestations: Bone, pituitary (DI) and skin involvement
91 Question 12 (Code 5.17)
92 Question 12 (Code 5.17)
93 Question 12 (Code 5.17)
94 Question 12 (Code 5.17)
95 Question 12 (Code 5.17)
96 Question 12 (Code 5.17)
97 Question 12 (Code 5.17) Tuberous sclerosis Uniform, small cyst Adenomatoid pneumocyte hyperplasia nodule Basal predominate Increased lung volume a/w renal AML, LAM, SEGA, cardiac rhabdomyoma
98 Cystic lung diseases LCH LAM Tuberous sclerosis Clinical Young adult smokers No gender predilection Female, pneumothorax, chylothorax No sex predilection Familial predisposition Cysts Bizarre-shaped, thick & irregular wall Round shaped, thin wall Nodule Poorly or well defined, regress overtime Occasional pneumocyte hyperplasia Adenomatoid pneumocyte hyperplasia Distribution Upper lobe, Spare lung base & CPA No preferential distribution Basal predominate Associated abnormality Bone, pituitary (DI) and skin involvement Renal AML Renal & hepatic AML, cardiac rhabdomyoma, CNS (SEGA)
99 Question 13: Code 5.26
100 Question 13: Code 5.26
101 Question 13: Code 5.26
102 Question 13: Code 5.26
103 Question 13: Code 5.26
104 LAM Female Uniform thin wall cyst No preferential distribution Occasional pneumocyte hyperplasia nodule Associated with renal angiomyolipoma Question 13: Code 5.26
105 Question 14 (Code 5.4)
106 Question 14 (Code 5.4)
107 Question 14 (Code 5.4)
108 Question 14 (Code 5.4)
109 Question 14 (Code 5.4)
110 Emphysema & interstitial fibrosis Centrilobular emphysema: well defined lucent area with imperceptible wall. Honeycombing: thick walled small cysts located subpleural and basal in distribution Question 14 (Code 5.4)
111 Question 15 (Code 5.18)
112 Question 15 (Code 5.18)
113 Question 15 (Code 5.18)
114 Question 15 (Code 5.18)
115 Question 15 (Code 5.18)
116 Question 15 (Code 5.18) LCH Bizzare shaped Upper lobe, spare costophrenic angles Preserve or increase lung volume Poorly or well defined, regress overtime nodule Associate with Bone, pituitary and skin involvement
117 Question 15 (Code 5.18) Bizarre-shaped cysts - Cloverleaf
118 Question 16: Code 5.27
119 Question 16: Code 5.27
120 Question 16: Code 5.27
121 Question 16: Code 5.27
122 Question 16: Code 5.27
123 Cystic Metastasis Random distribution Peripheral, basal and subpleural distribution. Metastatic disease: H&N, sarcoma, squamous cell CA Question 16: Code 5.27
124 Question 17: Code 5.28
125 Question 17: Code 5.28
126 Question 17: Code 5.28
127 Question 17: Code 5.28
128 Question 17: Code 5.28
129 Tracheobronchial Papillomatosis Solid nodules. Cystic lesions with thin/thick walls Randomly in central, peripheral and occasionally subpleural Lesions in larynx and trachea. Question 17: Code 5.28
130 A 59-year-old female with left breast cancer Question 18 (Code 5.23)
131 Question 18 (Code 5.23)
132 Question 18 (Code 5.23)
133 Question 18 (Code 5.23)
134 Question 18 (Code 5.23)
135 Question 18 (Code 5.23) Dx:???
136 Amyloidosis 6th decade of life. Commonly in a/w Sjögren s syndrome (with/without LIP), lymphoproliferative disease or mucosa-associated lymphoid tissue (MALT) lymphoma. Clinical manifestation: tracheobronchial form: diffuse or focal tracheal narrowing. nodular form: lung nodules. diffuse form with miliary nodules, reticulonodular opacities, and honeycombing Raoof et al, Chest 2016
137 Amyloidosis Nodules: Lower & subpleural predominance. Slow growth. Sharp, smooth, lobulated contours. Varying in size from 0.5 to 15 cm. Often central cavitation or calcification. Cyst: Large thin walled cysts with internal septae abutting bronchiocentric nodules Peripheral predilection Seaman et al, AJR 2011 Raoof et al, Chest 2016
138 Light-Chain Deposition Disease (LCDD) A rare disorder of middle-aged patients (mean age, 67 years). Characterized by deposition of monoclonal immunoglobulin light chains in various organs, including kidneys, lungs, skin, joints, or blood vessels. 75% occur in association with multiple myeloma or macroglobulinemia. Raoof et al, Chest 2016
139 Light-Chain Deposition Disease Lung involvement: rare. CT: Cysts: o believed to correspond to dilation of the small airways. o thin-walled, round, and up to 2 cm in size. Nodules: irregular, multiple, and can be bilateral or unilateral. Patchy areas of consolidation. Lymphadenopathy. (LCDD) Raoof et al, Chest 2016
140 Birt-Hogg-Dubé Syndrome Rare autosomal-dominant inherited disorder; mutations in the folliculin (FLCN) gene which encodes the protein FLCN. Characterized by hair follicle tumors, M.C fibrofolliculomas. High predisposition to malignant renal tumors: often bilateral and multifocal. Other associated systemic conditions: colonic polyposis and ophthalmologic disorders, such as progressive flecked chorioretinopathy & chorioretinal scars Souza et al, AJR 2005
141 Birt-Hogg-Dubé Syndrome Lung cysts in association with BHD 1st described by Toro et al. in > 80% patients, women = men and likely develop in early to mid adulthood (30 40 years). Multiple, thin-walled cysts. Predominant in peripheral lung zones at lung bases and along the mediastinum. Unique feature: abut or encase the proximal portion of the lower pulmonary veins. Souza et al, AJR 2005 Gupta et al, Fam Cancer 2013
142 Birt-Hogg-Dubé Syndrome Souza et al, AJR 2005
143 Birt-Hogg-Dubé Syndrome Should be suspected in a young patient presenting with spontaneous pneumothorax, with personal/family history of pneumothorax, skin lesions or renal tumors. DDx: Lymphangioleiomyomatosis; o Less in number of cysts in BHD. o AML more typical for LAM Langerhans cell histiocytosis. Lymphocytic interstitial pneumonitis. Pneumocystis carinii pneumonia. Souza et al, AJR 2005 Gupta et al, Fam Cancer 2013
144 Question 19: Code 5.29
145 Question 19: Code 5.29
146 Question 19: Code 5.29
147 Question 19: Code 5.29
148 Question 19: Code 5.29
149 Pulmonary Langerhans Cell Histiocytosis 7 years later Question 19: Code 5.29
150 Question 20: Code 5.30
151 Question 20: Code 5.30
152 Question 20: Code 5.30
153 Question 20: Code 5.30
154 Question 20: Code 5.30
155 Lymphocytic interstitial pneumonia (LIP) associated with SjÖgren Syndrome Question 20: Code 5.30 M.C. a/w CNT disease (SjÖgren syndrome, RA, SLE), HIV, drug exposure, allogenic hematopoietic stem cell transplants. Idiopathic rare. Cysts: thin-walled, random or basilar/perivascular distribution. GGO, centrilobular nodules. Thickened interlobular septa. Enlarged mediastinal nodes.
156 Thank you
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