Workshop Cyst & Lucency. How to Approach

Workshop Cyst & Lucency How to Approach

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

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.

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

Chapter 1

Question 1 (Code 5.14.1-5.14.3)

Air trapping Pulmonary Oligemia GGO with crazy paving Answers of question 1 (Code 5.14.1-5.14.3)

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

Air trapping Air trapping Question 1 (Code 5.14.1-5.14.3) 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

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 5.14.1-5.14.3)

Code 5.15

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 5.14.1-5.14.3)

Question 2 (Code 5.15) Inspiration Expiration

Question 2 (Code 5.15) Inspiration

Question 2 (Code 5.15) Expiration

Question 2 (Code 5.15) Inspiration

Question 2 (Code 5.15) Expiration

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)

Question 2 (Code 5.15)

Question 3 (Code 5.16) Inspiration Expiration

Question 3 (Code 5.16) Inspiration

Question 3 (Code 5.16) Expiration

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)

Inspiration Expiration

Inspiration Expiration Question 4: Code 5.25

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

Question 5 (Code 5.13)

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)

Chapter 2

Subpleural Cysts Parenchymal cysts

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.

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.

Honeycombing Air-filled cystic spaces 3-10 mm in Several layers & Share walls Subpleural lung Associated traction bronchiectasis, architectural distortion, volume loss

Question 6 (Code 5.2)

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

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

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)

Question 7 (Code 5.1)

Question 7 (Code 5.1) Paraseptal emphysema & Bulla

Thick-walled cystic lesions (TWCLs) Question 7 (Code 5.1)

Question 8 (Code 5.11)

Question 9 (Code 5.11)

Question 8 (Code 5.11)

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.

Question 9 (Code 5.24)

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)

Chapter 3

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.

Panlobular Emphysema Basal predominance Diffuse decrease in lung attenuation Pulmonary vascular pruning

Panbular emphysema (Code 5.1)

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.

Question 10 (Code 5.20)

Question 10 (Code 5.20) LAM Female Uniform thin wall cyst No preferential distribution Occasional pneumocyte hyperplasia nodule Associated with renal angiomyolipoma

Question 11 (Code 5.22)

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

Question 12 (Code 5.17)

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

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)

Question 13: Code 5.26

LAM Female Uniform thin wall cyst No preferential distribution Occasional pneumocyte hyperplasia nodule Associated with renal angiomyolipoma Question 13: Code 5.26

Question 14 (Code 5.4)

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)

Question 15 (Code 5.18)

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

Question 15 (Code 5.18) Bizarre-shaped cysts - Cloverleaf

Question 16: Code 5.27

Cystic Metastasis Random distribution Peripheral, basal and subpleural distribution. Metastatic disease: H&N, sarcoma, squamous cell CA Question 16: Code 5.27

Question 17: Code 5.28

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

A 59-year-old female with left breast cancer Question 18 (Code 5.23)

Question 18 (Code 5.23)

Question 18 (Code 5.23) Dx:???

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

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

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

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

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

Birt-Hogg-Dubé Syndrome Lung cysts in association with BHD 1st described by Toro et al. in 1999. > 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

Birt-Hogg-Dubé Syndrome Souza et al, AJR 2005

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

Question 19: Code 5.29

Pulmonary Langerhans Cell Histiocytosis 7 years later Question 19: Code 5.29

Question 20: Code 5.30

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.

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