Cyst and Cystlike Lung Lesions

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Cystic and cystlike lung lesions: what radiologists shouldknow

Poster No.: C-0050Congress: ECR 2013Type: Educational ExhibitAuthors: A. Martinez Calvo1, M. J. Martnez-Sapia Llanas2, V. Pazos

Silva3; 1Vilagarca De Arousa/ES, 2 A Corua/ES, 3A Corua/ESKeywords: Lung, Respiratory system, Thorax, Conventional radiography, CT-

High Resolution, CTDOI: 10.1594/ecr2013/C-0050

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Learning objectives

- To define the characteristics of a pulmonary cyst and to establish the differences withother injuries that can simulate it.

- To describe the semiology of different diseases that manifest with cystic andpseudocystic pulmonary lesions.

- To know the main clinical data that will help us to reach the correct diagnosis.

Background

Lung cysts are not uncommon and can be found in a wide variety of conditions.

A cyst is defined as an abnormal pulmonary parenchymal space, typically rounded,circumscribed, that is surrounded by an epithelial or fibrous wall of variable thickness(usually < 2 mm) and which contains air, although some may contain fluid. Fig. 1

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Fig. 1: Lung cyst on CT. Parenchymal lesion rounded, circumscribed, thin-walled andcontaining air inside (arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

On chest radiographs, findings of a cyst are often subtle, consisting of lucencies andsometimes linear or curvilinear opacities that make a reticular pattern. Fig. 2

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Fig. 2: Lung cysts in conventional radiography. Chest X-ray of a patient withpulmonary lymphangioleiomyomatosis. There is a diffuse reticular pattern with somesmall radiolucent areas corresponding to cysts (arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

An air-fluid level can improve the visibility of a cyst and may suggest infection.

We review and illustrate the clinical and radiological findings of the most common causesof lung cysts.

Cystic lung diseases:

1. Cystic adenomatoid malformation2. Pulmonary sequestration3. Pneumatoceles4. Tracheobronchial papillomatosis5. Metastases6. Langerhans cell histiocytosis7. Lymphangioleiomyomatosis/Tuberous Sclerosis Complex8. Lymphocytic interstitial pneumonia9. Neurofibromatosis type 110. Birt-Hogg-Dub syndrome11. Light-chain deposition disease12. Bullae

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13. Honeycombing

We also describe the features of some entities such as emphysema and bronchiectasiswhich may mimic true cystic diseases.

Cavitary processes, unlike cystic lesions, have thick and irregular walls, representingair-filled spaces within an area of greater attenuation (consolidation, mass or nodule).Cavitary lesions are not objective of our review.

Images for this section:

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Fig. 1: Lung cyst on CT. Parenchymal lesion rounded, circumscribed, thin-walled andcontaining air inside (arrow).

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Fig. 2: Lung cysts in conventional radiography. Chest X-ray of a patient withpulmonary lymphangioleiomyomatosis. There is a diffuse reticular pattern with somesmall radiolucent areas corresponding to cysts (arrow).

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Imaging findings OR Procedure details

CYSTIC LUNG DISEASES

1. Cystic adenomatoid malformation

It represents a group of anomalies characterized by the presence of a mass ofdisorganized lung tissue.

Usually they are diagnosed in childhood by repeated infections, but can be discoveredin adulthood. Risk of malignant transformation exists, so the recommended treatment issurgical resection.

Usually the disease appears as complex cystic masses up to 12 cm. The cysts have athin wall and may contain air, fluid or both. It may occur in the upper lobes but are mostcommon in the lower. In 15% of cases are bilateral.

Unlike pulmonary sequestration, the arterial supply is pulmonary, not systemic.

There are three types:

Type 1: the most frequent (50%). It is usually diagnosed in adulthood and it appears asmultiple cysts of 2 cm or greater. Fig. 3

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Fig. 3: Cystic adenomatoid malformation type 1. Adult patient with recurrentrespiratory infections. In MDCT, a multicystic mass is identified with a dominant cystover 3 cm (arrow) and blood supply through pulmonary arteries.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

Type 2: Multiple cysts smaller than 2 cm

Type 3: Numerous microscopic cysts that often have a more solid appearance onimaging. It is the least common and worse prognosis.

2. Pulmonary sequestration.

It is one of the most common pulmonary malformations. Clinically it manifests withrecurrent pneumonias or hemoptysis.

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It can be intralobar or extralobar.

- Intralobar sequestrations are the most frequent. Usually, they are located in thelower lobes and share the visceral pleural covering of an otherwise normal pulmonarylobe. They lack a normal communication to the tracheobronchial tree and they presentsystemic arterial supply. The venous drainage is usually through normal pulmonary veins.

They usually appear as solid or complex masses with one or more cysts that may containair and/or liquid. Adjacent emphysematous changes are common. Fig. 4

Fig. 4: Intralobar pulmonary sequestration. A. Multicystic mass with multiple fluidlevels (black arrow) in left lower lobe. B. Arterial supply through an anomalous branchoriginates in the descending thoracic aorta (white arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

- Extralobar sequestrations are almost always found during the patients' first days toweeks of life and, less frequently, in late infancy or early childhood. An extralobarsequestration consists of a discrete, accessory lobe of nonaerated lung tissue that isinvested in its own pleural envelope. If the anomalous tissue contains air, one can inferthe persistence of a patent connection to the gastrointestinal tract. They are typicallysupplied by an anomalous artery that arises directly from the thoracic or abdominal aortaand the venous drainage is usually systemic through the azygos and hemiazygos systemor superior vena cava into the right atrium.

3. Pneumatoceles

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They are air cysts most frequently caused by pneumonia or trauma. In the acute phasethey can be thick-walled. They tend to become thinner in the chronic phase.

When due to infection, Pneumococcus species, Escherichia coli, Klebsiella species, andStaphylococcus species are the most common offending bacterial organisms. They areusually transient and resolve spontaneously. Fig. 5

Fig. 5: Pneumatocele developed as a complication of pneumonia. Large thin-walledcystic lesion in the right lung of a pediatric patient.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

Among nonbacterial organisms, Pneumocystis jiroveci pneumonia has become animportant cause of pneumatoceles in recent years. It is a fungal infection. It is almostexclusively seen in immunosuppressed patients, especially in those with AIDS who havea CD4 count below 200 cells/mm3. It may progress to respiratory failure and even death.

It often appears as a pattern of bilateral, multifocal, mainly symmetric ground-glassopacities, with perihilar and bibasilar predominance. More focal areas of consolidation

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are also common. Cysts tend to occur mostly after multiple infections and can have anapical predominance, subpleural and size of between 1 and 5 cm. Fig. 6

Fig. 6: Pneumocystis jiroveci pneumonia. HIV patient with weight loss, dyspneaand fever. There is a right perihilar ground glass opacity (asterisk), small patchyconsolidations and peripheral thin-walled cyst (arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

The development of posttraumatic pneumatoceles is often preceded by lacerations andcontusions of the lung and healing occurs within weeks to months.

4. Tracheobronchial papillomatosis

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It is the result of infection of the upper airway by the human papilloma virus.

The disease has a bimodal age distribution, occurring most often early in life or in thefourth decade.

Infection often occurs at the time of the childbirth when the newborn passes through aninfected channel.

Most cases are limited to the larynx and subglottic region but the disease can spread tothe trachea, bronchi or alveoli, probably by airborne dissemination of laryngeal disease.Lung lesions are seen in less than 1% of affected patients and may occur after 10 yearsof laryngeal involvement.

Clinically, tracheobronchial papillomatosis can manifest as hoarseness, dyspnea andhemoptysis.

The imaging findings are nodules with intraluminal growth in the larynx, trachea andbronchi. Lung lesions are well-defined nodules, single or multiple. These nodules maycavitate resulting cysts with thick or thin walls. They usually have posterior predominance.

Lung lesions do not resolve spontaneously. There is progression to squamous cellcarcinoma in 2% of patients with lung involvement. The prognosis for these patients ispoor. The progression of the disease often leads to respiratory failure and death. Fig.7 and 8

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Fig. 7: Tracheobronchial papillomatosis with lung involvement. There are severalnodules in the wall of the trachea (white arrows), multiple solid pulmonary nodules(asterisks) and cysts (black arrows) in both lower lobes.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

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Fig. 8: Tracheobronchial papillomatosis with lung involvement. Same patient severalyears later with a mass in the left upper lobe which was a lung carcinoma (asterisk).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

5. Cystic metastases

Thin-walled cystic metastases are infrequent but they can be developed in many tumors,including sarcoma, squamous cell cancer, transitional cell carcinoma of the bladder,melanoma... They tend to have different sizes and a basilar predominance. Fig. 9

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Fig. 9: Cystic metastases. Carcinoma of sigmoid colon (white arrow in D), with multipleliver metastases (black arrows in C) as well as solid (black arrow in B) and cystic (whitearrow in A) lung metastases.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

The appropriate history of primary malignancy is critical to suggest this diagnosis.

Cystic transformation of solid lung metastases after chemotherapy is not uncommon.

6. Lymphangioleiomyomatosis

It is a disorder characterized pathologically by abnormal proliferation of atypical smoothmuscle cells in the lungs and in thoracic and retroperitoneal lymphatics.

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It affects women of childbearing age. However, it also can present after menopause inwomen undergoing estrogen hormonal treatment.

Dyspnea and hemoptysis are common presenting symptoms. Chylothorax andpneumothorax are also frequently seen, either at presentation or during the course ofthe disease. Fig. 12

Pulmonary cysts are invariably present. Cysts are well-defined, smooth and regular,generally less than 25-30 mm, interspersed with normal lung. The distribution is diffuseand, in contrast to Langerhans cell histiocytosis, does not spare the costophrenic angles.Air trapping is uncommon. Fig. 10

Fig. 10: Pulmonary lymphangioleiomyomatosis. Young woman having multiple lungcysts with diffuse distribution that does not spare the costophrenic angles.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

The most common abdominal findings include renal angiomyolipomas, enlarged lymphnodes and lymphangioleiomyomas. Fig. 11 and 12

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Fig. 11: Pulmonary lymphangioleiomyomatosis and renal angiomyolipoma. A andB. Middle-aged woman having multiple thin-walled cysts with diffuse distribution thatdoes not spare the costophrenic angles. C. Large right renal mass with heterogeneousattenuation predominantly fatty (asterisk).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

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Fig. 12: Pulmonary lymphangioleiomyomatosis with pneumothorax and retroperitoneallymphangioleiomyoma. A. Young woman with pulmonary lymphangioleiomyomatosis inadvanced stage, with multiple thin-walled cysts and right apical pneumothorax (arrow).B. Hypodense mass with septa localized in the retroperitoneal perivascular space(asterisk).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

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Surgical oophorectomy and hormone therapy can improve the prognosis in somepatients. Lung transplantation is the definitive treatment of this progressive disease, butrecurrence can occur in the transplanted lung.

Tuberous Sclerosis Complex

It is an inheritable autosomal dominant disorder characterized by multifocal systemichamartomas that may affect the central nervous system, skin, heart, kidneys and otherorgans. The Vogt triad of epileptic seizures, mental retardation and facial angiofibromas(formerly misnamed as adenoma sebaceum) is exhibited by only approximately 29% ofaffected patients.

Pulmonary involvement in tuberous sclerosis is indistinguishable fromlymphangioleiomyomatosis, but tuberous sclerosis can also occur in men. For manyyears, it has been debated whether lymphangioleiomyomatosis represents a formefrustrated of tuberous sclerosis complex.

7. Langerhans cell histiocytosis

Pulmonary Langerhans cell histiocytosis is a rare pulmonary disorder that typically affectsyoung adults between the ages of 20 and 40 years and is associated with cigarettesmoking (90%). There is no sex predilection.

Most patients are symptomatic and the most frequent presenting complaints includenonproductive cough and dyspnea.

Pneumothorax is the initial manifestation in 15% of patients and is almost always presentduring the course of the disease.

Peribronchiolar infiltration of Langerhans and inflammatory cells results inbronchiolocentric stellate nodules. The nodular lesions frequently cavitate and form thick-and thin-walled cysts, which are thought to represent enlarged airway lumina.

Radiographically, it appears as multiple nodules that range from 1 to 15 mm. The nodulesare predominantly distributed in the upper and middle lung zones, usually bilateral andsymmetric, with sparing of the lung bases near the costophrenic sulci. Fig. 13

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Fig. 13: Langerhans cell histiocytosis. Young male smoker having in the upper lobessmall multiple thin-walled cystic lesions (black arrow) and micronodules (white arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

The presence of cysts usually occurs during the later stages of the disease. The cystshave variable size and shape. In early stages measured around 10 mm but eventuallymay coalesce, acquire bizarre shapes and measure up to 80 mm. Fig. 14

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Fig. 14: Langerhans cell histiocytosis in advanced stage. We see multiple cystspredominantly in upper and middle areas with bizarre shapes and some coalescing(white arrow in B).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

Lung volumes are normal or increased in most patients.

In most patients, the main imaging feature is a combination of nodular and cysticlesions. Demonstration of cysts and centrilobular nodules with sparing of the costophrenicangles, in a young adult cigarette smoker, allows a confident prospective diagnosis ofLangerhans cell histiocytosis. Fig. 15

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Fig. 15: Langerhans cell histiocytosis. Male smoker with a combination of small cysts(black arrows) and centrilobular micronodules (white arrows) in upper and middlefields, with preservation of the inferior.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

This disease may resolve or stabilize with cessation of smoking and corticosteroids ormay progress to fibrosis.

8. Lymphocytic interstitial pneumonia

It is a benign lymphoproliferative disorder characterized by a diffuse interstitial infiltrationof mononuclear cells, predominantly lymphocytes and plasma cells.

Symptoms are nonspecific, the most frequent cough and dyspnea.

Lymphocytic intersticial pneumonia can be idiopathic or associated with collagen-vascular diseases, especially Sjgren syndrome and AIDS. Other less frequentlyreported disorders associated include autoimmune thyroid disease, Castleman disease,systemic lupus erythematosus, myasthenia gravis, pernicious anemia and chronic

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active hepatitis. It is currently controversial whether lymphocytic interstitial pneumoniarepresents a form of early lymphoma.

During the acute phase, diffuse or multifocal ground-glass opacities and poorly definedcentrilobular nodules are seen. However, these opacities may regress so that cysts arethe only residual finding in more chronic cases.

Cysts are usually less numerous than in Langerhans cell histiocytosis orlymphangioleiomyomatosis. Cysts range between 1 and 30 mm, usually with a basalpredominance. Fig. 16

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Fig. 16: Lymphocytic interstitial pneumonia. Patient with Sjgren syndrome presentingextensive ground-glass opacities, small consolidations (arrow in A) and several thin-walled cysts (arrow in B).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

Less frequently, lymphocytic intersticial pneumonia presents septal thickening, patchyconsolidations and lymphadenopathy.

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The usual treatment is steroid therapy, after which ground-glass opacities can resolve.The cysts, however, persist even after therapy.

9. Neurofibromatosis type 1

Neurofibromatosis type 1 or von Recklinghausen disease is the most commonneurocutaneous syndrome (1:2000 - 1:3000), fundamentally characterized by caf-au-lait spots and subcutaneous neurofibromas. The nervous system is usually affected bythe presence of a wide variety of tumors, the most frequent optic nerve glioma.

Rarely, it can be associated with multifocal lung cysts, which most commonly involveboth upper lobes. Other chest manifestations include subcutaneous and intercostalneurofibromas, ribbonlike ribs, meningoceles, mediastinal masses and pulmonaryfibrosis.

10. Birt-Hogg-Dub syndrome

Birt-Hogg-Dub syndrome is a rare autosomal-dominant disorder characterized by:

- Facial fibrofolliculomas distributed over the face, neck and upper trunk.

- Renal tumors ranging from benign oncocytomas to renal cell carcinomas (often bilateraland multifocal).- Thin-walled pulmonary cysts.

- Spontaneous pneumothorax.

Cysts are often elongated and subpleural in distribution, between 10 and 50 mm and withbasal predominance.

11. Light-chain deposition disease

Light-chain deposition disease occurs in middle-aged patients and commonly involvesthe kidneys. Seventy-five percent of cases occur in association with multiple myelomaor macroglobulinemia. Although lung involvement is rare, can result in respiratory failureand require lung transplantation.

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The radiographic findings include nodules, lymphadenopathy and cysts usually between1 and 30 mm with a diffuse distribution.

12. Bullae

Bullae are very thin-walled cysts measuring more than 1 cm, but could occupy an entirelobe. They are commonly subpleural and are mostly seen in both upper lobes in patientswith coexisting centrilobular and paraseptal emphysema. Fig. 17

Fig. 17: Bullae. Multiple large thin-walled subpleural cysts in both lung apex.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

13. Honeycombing

It is one of the manifestations of fibrotic lung disease and represents the end stageof multiple entities, especially usual interstitial pneumonia but also in conditions suchas nonspecific interstitial pneumonia, sarcoidosis, chronic hypersensitivity pneumonitis,asbestosis and postradiation fibrosis.

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It is produced by the disruption of normal lung structure with airspaces coalescence dueto breakage of the alveolar walls and the formation of cystic lesions.

On CT can see a reticular pattern with multiple cysts of similar size, usuallybetween 3 and 10 mm, sharing walls and arranged in two or more adjacent layersconferring a honeycomb appearance. They typically have a subpleural location, patchy,predominantly in basilar regions and bilateral. Fig. 18

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Fig. 18: Honeycombing. Patient with usual interstitial pneumonia, having multiplesmall subpleural cysts that are arranged in two or more adjacent layers, predominantlyperipheral and in basilar regions (arrows).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

After forced expiration cysts become smaller, indicating connection to the airway.

Honeycombing is usually accompanied by other signs of fibrosis such as tractionbronchiectasis or bronchiolectasis, reticulation and ground-glass opacities.

Summary of cystic lung diseases

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Table 1: Summary of cystic lung diseases.References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

PSEUDOCYSTIC LUNG DISEASES

1. Emphysema

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It is characterized by permanent overdistention airspace distal to the terminal bronchiole,with destruction of alveolar walls and without obvious fibrosis. This results in loss of lungelasticity and produces airflow obstruction, hyperinflation and air trapping.

Conventional radiography shows emphysema relatively advanced stage, when there aresigns of air trapping and hyperinflation. CT is much more sensitive and identifies directlythe destruction of lung tissue.

Emphysema is classified according to the affected area of the secondary lobule:

- Centrilobular

It is the most common and is usually associated with smoking. It affects the central portionof the secondary pulmonary lobule and predominantly in the upper lobes.

On CT we can see multiple lucent rounded areas of a few millimeters, without visiblewalls, which differentiates it from true cystic lesions.

Lobular arteriole can be identified in the hyperlucency center. In emphysema, the vesselscan traverse the lucent spaces, while true cysts are surrounded or are independent ofthe vessels. Fig. 19

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Fig. 19: Centrilobular emphysema. There are multiple radiolucent lesions withoutdefined wall. In some of them we can see inside the lobular arteriole (arrow).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

- Panlobular

It is much less common, often associated with Alpha-1 antitrypsin deficiency. Panlobularemphysema is characterized by the uniform destruction of the secondary pulmonarylobule, from its centre to the periphery with predominance in lower lobes. Low attenuation

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areas are not as evident as in centrolobular emphysema since there is no contrast withnormal adjacent parenchyma, and diffuse vascular distortion is another common finding.

- Paraseptal

Paraseptal emphysema destroys the periphery of the secondary pulmonary lobule.It affects the lung adjacent to the visceral pleura and interlobular septa. Unlikehoneycombing, subpleural cystic spaces are arranged in one single layer. Cysts largerthan 1 cm are called bullae. Fig. 20

Fig. 20: Paraseptal emphysema. Multiple subpleural thin-walled cysts arranged in onesingle layer.

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References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

2. Bronchiectasis

Bronchiectasis represents an irreversible bronchial dilatation. The pathogenesis ofbronchiectasis is complex, with a large number of etiologies and associations recognized:

- Post-infective

- Congenital (Cystic fibrosis, ciliary dyskinesia...)- Obstruction (neoplasia, broncholith)- Immunodeficiencies

- Allergies (allergic bronchopulmonary aspergillosis...)- Aspiration or inhalation of toxic

- Rheumatic diseases

- Pulmonary fibrosis

According to their morphology they can be divided into cylindrical, varicose or cystic.Cystic bronchiectasis are saccular dilatation of the bronchi and can be mistaken for cystsat CT if thin-section images are not reviewed. Multiplanar reformations can be helpfulto distinguish cystic bronchiectasis in which there is continuity of a bronchus with thin-walled lucencies, and true cysts in which such communication does not exist. Fig. 21

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Fig. 21: Cylindrical bronchiectasis. Multiple rounded radiolucent lesions with thin wallin the right lower lobe (arrow in A and B). MinIP coronal reconstruction shows thecontinuity of the lesions with normal bronchial tree (arrow in C).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

Other characteristic signs of bronchiectasis are:

- The signet ring sign

The ring of soft-tissue attenuation represents the wall of the dilated bronchus seen on across-sectional CT scan, whereas the low-attenuating circle of air represents air withinthe dilated bronchus. The circle of soft-tissue attenuation abutting the ring represents across-sectional image of the pulmonary artery that lies adjacent to the dilated bronchus.Fig. 22

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Fig. 22: Cylindrical bronchiectasis in a patient with cystic fibrosis. Signet ring sign(black arrows).References: Radiologa, Hospital do Salns - Vilagarca De Arousa/ES

- The tram-track sign

In a longitudinal section of the bronchus, the tram-track sign refers to parallel thickeningof bronchial walls that do not show normal tapering toward the lung periphery.

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Images for this section:

Fig. 3: Cystic adenomatoid malformation type 1. Adult patient with recurrent respiratoryinfections. In MDCT, a multicystic mass is identified with a dominant cyst over 3 cm(arrow) and blood supply through pulmonary arteries.

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Fig. 4: Intralobar pulmonary sequestration. A. Multicystic mass with multiple fluid levels(black arrow) in left lower lobe. B. Arterial supply through an anomalous branch originatesin the descending thoracic aorta (white arrow).

Fig. 5: Pneumatocele developed as a complication of pneumonia. Large thin-walledcystic lesion in the right lung of a pediatric patient.

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Fig. 6: Pneumocystis jiroveci pneumonia. HIV patient with weight loss, dyspneaand fever. There is a right perihilar ground glass opacity (asterisk), small patchyconsolidations and peripheral thin-walled cyst (arrow).

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Fig. 7: Tracheobronchial papillomatosis with lung involvement. There are several nodulesin the wall of the trachea (white arrows), multiple solid pulmonary nodules (asterisks) andcysts (black arrows) in both lower lobes.

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Fig. 8: Tracheobronchial papillomatosis with lung involvement. Same patient severalyears later with a mass in the left upper lobe which was a lung carcinoma (asterisk).

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Fig. 9: Cystic metastases. Carcinoma of sigmoid colon (white arrow in D), with multipleliver metastases (black arrows in C) as well as solid (black arrow in B) and cystic (whitearrow in A) lung metastases.

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Fig. 10: Pulmonary lymphangioleiomyomatosis. Young woman having multiple lung cystswith diffuse distribution that does not spare the costophrenic angles.

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Fig. 11: Pulmonary lymphangioleiomyomatosis and renal angiomyolipoma. A and B.Middle-aged woman having multiple thin-walled cysts with diffuse distribution that doesnot spare the costophrenic angles. C. Large right renal mass with heterogeneousattenuation predominantly fatty (asterisk).

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Fig. 12: Pulmonary lymphangioleiomyomatosis with pneumothorax and retroperitoneallymphangioleiomyoma. A. Young woman with pulmonary lymphangioleiomyomatosis inadvanced stage, with multiple thin-walled cysts and right apical pneumothorax (arrow). B.Hypodense mass with septa localized in the retroperitoneal perivascular space (asterisk).

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Fig. 13: Langerhans cell histiocytosis. Young male smoker having in the upper lobessmall multiple thin-walled cystic lesions (black arrow) and micronodules (white arrow).

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Fig. 14: Langerhans cell histiocytosis in advanced stage. We see multiple cystspredominantly in upper and middle areas with bizarre shapes and some coalescing (whitearrow in B).

Fig. 15: Langerhans cell histiocytosis. Male smoker with a combination of small cysts(black arrows) and centrilobular micronodules (white arrows) in upper and middle fields,with preservation of the inferior.

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Fig. 16: Lymphocytic interstitial pneumonia. Patient with Sjgren syndrome presentingextensive ground-glass opacities, small consolidations (arrow in A) and several thin-walled cysts (arrow in B).

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Fig. 17: Bullae. Multiple large thin-walled subpleural cysts in both lung apex.

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Fig. 18: Honeycombing. Patient with usual interstitial pneumonia, having multiple smallsubpleural cysts that are arranged in two or more adjacent layers, predominantlyperipheral and in basilar regions (arrows).

Fig. 19: Centrilobular emphysema. There are multiple radiolucent lesions without definedwall. In some of them we can see inside the lobular arteriole (arrow).

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Fig. 20: Paraseptal emphysema. Multiple subpleural thin-walled cysts arranged in onesingle layer.

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Fig. 21: Cylindrical bronchiectasis. Multiple rounded radiolucent lesions with thin wall inthe right lower lobe (arrow in A and B). MinIP coronal reconstruction shows the continuityof the lesions with normal bronchial tree (arrow in C).

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Fig. 22: Cylindrical bronchiectasis in a patient with cystic fibrosis. Signet ring sign (blackarrows).

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Table 1: Summary of cystic lung diseases.

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Conclusion

HRCT and especially MDCT are substantially more sensitive techniques thanconventional radiography for the detection of structural changes and assessing thepattern of affectation of the different cystic lung diseases.

Although CT plays an essential role in the evaluation of cystic lung diseases, we mustalways consider the clinical, functional and analytical aspects to establish the finaldiagnosis.

References

1. Cantin L, Bankier AA, Eisenberg RL. Multiple cystlike lung lesions in the adult. AJRAm J Roentgenol. 2010 Jan;194(1):W1-W11.

2. Seaman DM, Meyer CA, Gilman MD, McCormack FX. Diffuse cystic lung diseaseat high-resolution CT. AJR Am J Roentgenol. 2011 Jun;196(6):1305-11.3. Abbott GF, Rosado-de-Christenson ML, Frazier AA, Franks TJ, Pugatch RD, GalvinJR. From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 2005 May-Jun;25(3):803-28.4. Pallisa E, Sanz P, Roman A, Maj J, Andreu J, Cceres J.Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologiccorrelation. Radiographics. 2002 Oct;22 Spec No:S185-98.

5. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From thearchives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics.2004 May-Jun;24(3):821-41.6. Attili AK, Kazerooni EA, Gross BH, Flaherty KR, Myers JL, Martinez FJ. Smoking-related interstitial lung disease: radiologic-clinical-pathologic correlation.Radiographics. 2008 Sep-Oct;28(5):1383-96; discussion 1396-8.

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