Cystic Lung Disease : What Can Cause Holes in the...

Cystic Lung Disease

: What Can Cause Holes in the Lung?

Category: Diffuse lung disease

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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


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


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


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


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


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


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.


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


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


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


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


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


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



2016-03-312011-10-10

Case 5



41/F

Case 6


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


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



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



Sjogren’s syndrome-Lymphocytic interstitial pneumonia, Amyloidosis

10 yrs later

59/F

10 yrs later

Case 9


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


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


Respiratory Bronchiolitis (RB)-ILD50/M, Current smoker

RB-ILD with emphysema ?Advanced pulmonary LCH ?

Lung, wedge resection: Smoking associated respiratory bronchiolitis

Case 12


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


RUL wedge resection: adenocarcinoma, moderately differentiated, micropapillary predominant type, EGFR/ALK mutation (-)

Metastasis67/M, Dry cough

Case 14


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.


Focal or multifocal cystic lung disease

• Pneumatocele• Fungal infection including invasive aspergillosis, mucormycosis• Tuberculosis• Pneumocystis jiroveci pneumonia (PJP) • Paragonimiasis


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


Invasive pulmonary aspergillosis30/F AML BMT GVHD Bronchiolitis obliterans

2016-3-28 2016-7-11 Case 18

Tx: Voriconazole


Pulmonary tuberculosis

2007-9-20Case 19

Tx: AntiTBc med.

19/M

2007-7-9


Pneumocystis jiroveci pneumonia (PJP)

35/M

Paragonimiasis


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


47/M

Pulmonary LCH

17/M

Birt-Hogg-Dube Syndrome


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


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.

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