Multiple Cyst Lung Study

Residents Section Pat tern of the Month
Cantin et al.
Multiple Cystlike Lung Lesions in the Adult
Residents Section
Pattern of the Month
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Residents
Multiple Cystlike Lung Lesions

inRadiology in the Adult
Luce Cantin1 Multiple lung cysts in the adult population TABLE 1: Causes of Multiple Cystlike
Alexander A. Bankier can be encountered in a wide variety of condi- Lung Lesions in Adults
Ronald L. Eisenberg tions. A cyst is defined as a round circumscribed
Blebs and bullae
space that is surrounded by an epithelial or fi-
Cantin L, Bankier AA, Eisenberg RL brous wall of variable thickness. A cyst usually Congenital pulmonary airway malformation
has a thin and regular wall (< 2 mm) and con- Honeycombing
tains air, although some may contain fluid. The Langerhans cell histiocytosis
thin wall of cystic lesions differentiates them
Lymphangioleiomyomatosis
from cavitary processes, which have thicker
and irregular walls and will not be discussed in Lymphocytic interstitial pneumonia
this article. On chest radiographs, findings of a Metastases
cyst are often subtle, consisting of lucencies and
Neurofibromatosis type 1
sometimes linear opacities. An airfluid level
can improve the visibility of a cyst and may Pneumatoceles
suggest infection. For the investigation of cysts, Pneumocystis jiroveci pneumonia
CT is substantially more sensitive than chest Tracheobronchial papillomatosis
radiography, showing a cyst as a ringlike lu-
cency surrounded by a thin wall (Fig. 1).
Table 1 summarizes the most common causes
of multiple lung cysts. Pitfalls in the detection of
lung cysts on CT include cystic bronchiectasis
and emphysema accompanied by bullae.
Keywords: cysts, lung, pattern
DOI:10.2214/AJR.09.3540
Received August 27, 2009; accepted after revision

September 15, 2009.
1
All authors: Department of Radiology, Beth Israel
Deaconess Medical Center, Harvard Medical School, 330
Brookline Ave., Boston, MA 02215. Address correspond
ence to R. L. Eisenberg (rleisenb@bidmc.harvard.edu).
WEB
This is a Web exclusive article.
AJR 2010; 194:W1W11 A B

0361803X/10/1941W1 Fig. 1Multiple cysts.
A, Multiple infected lung cysts with airfluid levels (arrows) on chest radiograph.
American Roentgen Ray Society B, In another patient, multiple ringlike lucencies (arrows) in lymphangioleiomyomatosis.
AJR:194, January 2010 W1

Cantin et al.
Fig. 2Multiple upper

lobe predominant
bullae (arrow) in
emphysematous patient.
Blebs and Bullae

Blebs and bullae are sharply defined, air-containing spaces that are bounded by curvilin-
ear, hairline shadows. According to the Fleischner Society Glossary of Terms for Thoracic
Imaging, a bleb is a cystic space l cm or less in diameter; anything larger than this is defined
as a bulla. Bullae can reach substantial size and occupy an entire lobe. Blebs and bullae are
commonly subpleural and are mostly seen in both upper lobes in patients with coexisting
centrilobular and paraseptal emphysema (Fig. 2). In severe cases, or in patients with recurrent
pneumothoraces, bullectomy can improve pulmonary function or even be curative.
Congenital Pulmonary Airway Malformation

Congenital pulmonary airway malformation, formerly known as congenital cystic ade-
nomatoid malformation, is a rare multicystic, intralobar mass of disorganized lung tissue,
most often seen in the lower lobes. It is usually diagnosed in children but can be left unrecog-
nized until adulthood. Congenital pulmonary airway malformation can be categorized into
three subtypes on the basis of its appearance. Type 1 is most frequent, especially in the adult
population, and consists of multiple cysts 2 cm or greater in diameter. On CT, the disease ap-
pears as a large, air-filled multicystic lesion (Fig. 3). Type 2 is defined as multiple cysts
smaller than 2 cm. In type 3, the least frequent type, there are numerous microscopic cysts
that often have a more solid appearance on imaging. Type 3 has the worst prognosis, which
presumably is the reason it is uncommon in adults. Complications of congenital pulmonary
airway malformation include infection and malignant transformation. Therefore, surgical re-
section is recommended for definitive treatment.
Honeycombing
Honeycombing is one of the manifestations of fibrotic lung disease. The most common
cause of honeycombing is usual interstitial pneumonia (UIP), but this appearance can also be
seen in conditions such as nonspecific interstitial pneumonia, sarcoidosis, chronic hypersen-
sitivity pneumonitis, asbestosis, and postradiation fibrosis.
On CT, honeycombing consists of multiple cysts of variable thickness and size, with more
than one layer of cysts, usually distributed bilaterally and mostly in the subpleural and basilar
regions (Fig. 4). It is usually accompanied by other signs of fibrosis, such as traction bron-
chiectasis or bronchiolectasis, reticulation, and ground-glass opacities.
W2 AJR:194, January 2010

A B
Fig. 3Congenital pulmonary airway malformation

type 1 in adulthood.
AC, Chest radiograph (A) and coronal (B) and
transverse (C) CT reformations show right lower lobe
multicystic lesion (long arrows). Double short arrows
in C point to fluid-filled cyst.
C
Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a destructive disorder that occurs almost exclu-
sively in smokers and has no sex predominance. LCH is characterized pathologically by the
formation of granulomas containing Langerhans cells; clinical findings include cough, dysp-
nea, and fatigue. Pneumothorax can also occur, either at the time of presentation or later
during the course of the disease. As with other smoking-related diseases, such as emphysema,
respiratory bronchiolitis, and desquamative interstitial pneumonia, LCH typically has upper
lobe predominance with relative sparring of both bases. However, in some cases, LCH shows
a more diffuse distribution with peribronchovascular predominance.

Cantin et al.
Fig. 4Honeycombing. Multiple predominantly

subpleural and basilar cysts (arrows) in patient with
usual interstitial pneumonia.
Fig. 5Langerhans cell

histiocytosis.
A and B, Transverse
CT images show
bizarre-shaped
cysts (black arrows),
cavitary nodules
(white arrows), and
noncavitary nodules
(curved arrows) in
upper lobe predominant
distribution.
B

Radiographically, LCH appears as multiple nodules that range from 1 to 10 mm. The pres-
ence of cysts usually occurs only during the later stages of the disease. On CT, bizarre-shaped
cysts are associated with noncavitary nodules that progressively cavitate to become thick-
walled cavitary nodules and then thin-walled cysts (Fig. 5). The CT findings of LCH are often
characteristic and reflect the temporal heterogeneity of the disease. Treatment consists of
smoking cessation, which can result in improvement of the abnormalities.
Lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is a disorder of smooth muscle proliferation that pri-
marily affects women of childbearing age. However, it also can present after menopause in
women undergoing estrogen hormonal treatment. This condition is indistinguishable from
pulmonary involvement in tuberous sclerosis, which can also occur in men. Dyspnea and
hemoptysis are common presenting symptoms. Chylothorax and pneumothorax are also fre-
quently seen, either at presentation or during the course of the disease.
On chest radiographs, there are increased lung volumes and often a suspicion of linear or
ringlike lucencies. CT shows well-defined, smooth, and regular cysts interspersed with nor-
mal intervening lung (Fig. 6). In contrast to LCH, the distribution is diffuse and does not
spare the costophrenic angles. Lung nodules are typically absent in LAM, whereas they are
Fig. 6Lymphangioleio
myomatosis.
A, Chest radiograph
shows increased lung
volumes and fine linear
opacities.
B, Transverse CT image
shows multiple, well-
defined, regular cysts
interspersed with normal
intervening lung.
B

Cantin et al.
an essential part of the appearance in LCH. In the later stages of LAM, the cysts become
larger and tend to become more numerous and coalescent. There may be associated lymph-
adenopathy and renal angiomyolipomas. Lung transplantation is the definitive treatment of
this progressive disease, but recurrence can occur in the transplanted lung.
Lymphocytic Interstitial Pneumonia

Lymphocytic interstitial pneumonia (LIP) is typically associated with collagenvascular

diseases, especially Sjgren syndrome, and AIDS. Other less frequently reported disorders
associated with LIP include autoimmune thyroid disease, Castleman disease, systemic lupus
erythematosus, myasthenia gravis, pernicious anemia, and chronic active hepatitis. It is cur-
rently controversial whether LIP represents a benign lymphoproliferative disorder or a form
of early lymphoma.
Fig. 7Lymphocytic interstitial

pneumonia (LIP).
A and B, Transverse (A) and
coronal (B) CT reformations show
scattered cysts (arrows) in patient
with Sjgren syndrome and LIP.
C, In another patient with AIDS
and LIP, transverse CT image
shows numerous lung cysts
(arrows).
C

LIP may produce a fine linear or reticulonodular pattern on chest radiographs. On CT, the
lung cysts are usually less numerous than in LCH or LAM. During the acute phase, diffuse
or multifocal ground-glass opacities are seen. However, these opacities may regress so that
cysts are the only residual finding in more chronic cases (Fig. 7). Less frequently, LIP pres-
ents with a reticular or reticulonodular pattern, lung nodules, or consolidation. Lymph node
enlargement can also occur. The usual treatment is steroid therapy, after which ground-glass
opacities can resolve. The cysts, however, persist even after therapy.
Metastases
Thin-walled cystic metastases develop primarily in sarcoma, squamous cell cancer, transi-
tional cell carcinoma of the bladder, and melanoma. Less frequently, this pattern may be seen
with lymphoma. As with other metastatic lesions, cystic metastases tend to have different
sizes and a basilar predominance (Fig. 8). A potential complication of cystic metastases is
pneumothorax, which may require placement of a chest tube.
Fig. 8Multiple cystic colon metastases (arrows).
Neurofibromatosis Type 1
Neurofibromatosis type 1, or von Recklinghausen disease, is clinically characterized by
caf-au-lait spots, neurofibromas, axillary or inguinal freckling, optic nerve glioma, Lisch
nodules, and skeletal lesions. Rarely, it can be associated with multifocal lung cysts, which
most commonly involve both upper lobes (Fig. 9). Other chest manifestations include subcu-
taneous and intercostal neurofibromas, ribbonlike ribs, meningoceles, mediastinal masses,
and pulmonary fibrosis.
Pneumatoceles
Pneumatoceles are thin-walled, gas-filled spaces in the lung that are most frequently caused
by pneumonia, trauma, or inhalation of hydrocarbon fluid. In the acute phase, pneumatoceles
can be thick-walled. They tend to become thinner in the chronic phase. When due to infec-
tion, Pneumococcus species, Escherichia coli, Klebsiella species, and Staphylococcus spe-
cies are the most common offending bacterial organisms, and pneumatoceles tend to appear
in the healing phase. Among nonbacterial organisms, Pneumocystis jiroveci pneumonia has

Cantin et al.
become an important cause of pneumatoceles in recent years (see next section). Infectious
pneumatoceles are usually transient, and spontaneous resolution is generally the rule. The
development of posttraumatic pneumatoceles is often preceded by lacerations and contusions
of the lung, and healing occurs within weeks to months. On CT, pneumatoceles manifest as
scattered thin-walled cysts interspersed with normal lung in areas previously affected by
pneumonia or trauma (Fig. 9).
A B
Fig. 9Pneumatoceles.
A and B, Transverse CT images show pneumatocele
(arrows) before (A) and after (B) treatment in fire
eater.
C, Multiple pneumatoceles (arrows) after recurrent
infections in patient with AIDS.
Pneumocystis Jiroveci Pneumonia

Pneumocystis jiroveci pneumonia (PJP), formerly known as Pneumocystis carinii pneu-
monia, was classified as a protozoan but is now known to be a fungal infection. It is almost
exclusively seen in immunosuppressed patients, especially in those with AIDS who have a
CD4 count below 200 cells/mm3. The symptoms can be insidious but may progress to respira-
tory failure and even death, often following a rapid, fulminant course if left untreated. Once
a common opportunistic infection, PJP is now less frequently seen because patients with
AIDS receive standard prophylaxis with trimethoprim sulfamethoxazole. The diagnosis can
be made by sputum culture, bronchoscopy with lavage, or biopsy.
The radiographic manifestations of PJP vary widely, ranging from a totally normal chest
radiograph to widespread, bilateral, symmetric opacities that are often of ground-glass den-

Fig. 10Pneumocystis
jiroveci pneumonia.
A, Chest radiograph
shows bilateral,
symmetric, mostly
perihilar and bibasilar
ground-glass opacity.
B, Transverse CT image
confirms bilateral,
widespread ground-
glass opacity and shows
scattered lung cysts
(arrow).
sity. The distribution often has perihilar and bibasilar predominance, but it can primarily af-
fect the upper lobes in patients receiving prophylactic therapy for this infection. There may be
a reticular pattern and even progression to consolidation. Pleural effusions are almost always
absent, and their presence should raise the possibility of an alternate diagnosis. On CT, PJP
often appears as a pattern of bilateral, multifocal, mainly symmetric ground-glass opacities
(Fig. 10). More focal areas of consolidation are also common. PJP-related cysts tend to occur
mostly after multiple infections, can have an apical predominance, and may lead to pneu-
mothoraces, which portend a poor outcome. Other less frequent manifestations include a re-
ticular or reticulonodular pattern and pulmonary nodules.
Tracheobronchial Papillomatosis
The rare lung parenchymal involvement by tracheobronchial papillomatosis is usually ac-
companied by very slowly progressive papillomas of the larynx, trachea, and mainstem bron-
chi. The disease has a bimodal age distribution, occurring most often early in life or in the
fourth decade. In adults, it is more prevalent in men. Clinically, tracheobronchial papilloma-
tosis can manifest as hoarseness, dyspnea, and hemoptysis.
The radiographic findings are nonspecific and include cystic lucencies and nodules. On CT,
papillomas of the airway can easily be visualized. The associated cysts are less numerous
than in LAM or LCH and typically have a posterior predominance (Fig. 11).

Cantin et al.
Fig. 11Tracheobronchial
papillomatosis.
A and B, Multiple, bilateral lung
cysts on transverse (A) and
coronal (B) CT reformations.
Arrows point to larger cysts.
Fig. 12Emphysema. Transverse CT image shows Fig. 13Cystic bronchiectasis. Transverse CT image
upper lobe predominant lucencies (arrows) with shows cystlike lesions (arrows) contiguous with
no visible wall, caused by destruction of lung dilated bronchi.
parenchyma.

Pitfalls
Emphysematous changes sometimes can be mistaken for multifocal lung cysts. In contrast
to true cysts, the cystlike lucencies caused by the destruction of lung parenchyma in emphy-
sema do not have walls (Fig. 12). Nevertheless, emphysema can be accompanied by multiple
cysts, such as bullae, or in association with LCH.
Cystic bronchiectasis can also be mistaken for cysts at CT if thin-section images are not
reviewed. Multiplanar reformations can be helpful to 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. 13).
Suggested Reading
1. Armstrong P, Wilson AG, Dee P, Hansell DM, eds. Imaging of diseases of the chest, 3rd ed. St. Louis, MO:
Mosby, 2000
2. Bankier AA, Brunner C, Lomoschitz F, Mallek R. Pyrofluid inhalation in fire-eaters: sequential findings
on CT. J Thorac Imaging 1999; 14:303306
3. Dhnert WF, ed. Radiology review manual, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2003
4. Hansell DM, Bankier AA, MacMahon TC, McLoud TC, Muller NL, Remy J. Fleischner Society: glossary
of terms for thoracic imaging. Radiology 2008; 246:697722
5. Klein JS, Cohen HL, McAdams HP, eds. Chest disease 6th series test and syllabus. Reston, VA: American
College of Radiology, 2007
6. Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc 2003; 78:744752
7. Webb WR, Higgins CB, eds. Thoracic imaging: pulmonary and cardiovascular radiology, 3rd ed. Philadel-
phia, PA: Lippincott Williams & Wilkins, 2003
8. Weissleder R, Wittenberg J, Harisinghani MG, eds. Primer of diagnostic imaging, 3rd ed. St Louis, MO:
Mosby, 2002

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Residents Section Pat tern of the Month

Multiple Cystlike Lung Lesions

Keywords: cysts, lung, pattern

Received August 27, 2009; accepted after revision

AJR 2010; 194:W1W11 A B

AJR:194, January 2010 W1

Fig. 2Multiple upper

Blebs and Bullae

Congenital Pulmonary Airway Malformation

W2 AJR:194, January 2010

Fig. 3Congenital pulmonary airway malformation

Langerhans Cell Histiocytosis

AJR:194, January 2010 W3

Fig. 4Honeycombing. Multiple predominantly

Fig. 5Langerhans cell

W4 AJR:194, January 2010

AJR:194, January 2010 W5

Lymphocytic Interstitial Pneumonia

Lymphocytic interstitial pneumonia (LIP) is typically associated with collagenvascular

Fig. 7Lymphocytic interstitial

W6 AJR:194, January 2010

Fig. 8Multiple cystic colon metastases (arrows).

AJR:194, January 2010 W7

Pneumocystis Jiroveci Pneumonia

W8 AJR:194, January 2010

AJR:194, January 2010 W9

W10 AJR:194, January 2010

reviewed. Multiplanar reformations can be helpful to distinguish cystic bronchiectasis, in

AJR:194, January 2010 W11

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