European Journal of Radiology 83 (2014) 47–56

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European Journal of Radiology

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Review

Pulmonary Langerhans cell histiocytosis in children: A spectrum of

radiologic findings
Shahina Bano a,∗ , Vikas Chaudhary b,1 , Mahender Kaur Narula a,2 , Rama Anand a,3 ,
Bhuvaneswari Venkatesan a,4 , Shramana Mandal c,5 , Kaushik Majumdar c,6
a
Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001, India
b
Department of Radiodiagnosis, Employees’ State Insurance Corporation (ESIC) Model Hospital, Gurgaon 122001, Haryana, India
c
Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002, India

a r t i c l e i n f o a b s t r a c t

Article history: Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare
Received 2 February 2013 in children. It is better described in adults than in children. We describe the current understanding of
Received in revised form 19 March 2013 PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high
Accepted 11 April 2013
resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of
disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre
Keywords:
in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH
Pulmonary Langerhans cell histiocytosis
is similar in adult and paediatric populations with the exception that lung base near the costophrenic
(PLCH)
Langerhans cell histiocytosis (LCH)
angle is spared in adults but almost always involved in children.
Paediatric population © 2013 Elsevier Ireland Ltd. All rights reserved.
High resolution computed tomography
(HRCT)
Chest X-ray (CXR)

1. Introduction and imaging features of PLCH in children, formulates the appropri-

ate differential diagnosis and demonstrates the etiological, clinical
“Primary” pulmonary Langerhans cell histiocytosis (PLCH) refers and radiological differences of PLCH between the adult and child.
to LCH isolated to the respiratory system, notably the lungs [1].
PLCH can occur as a part of disseminated LCH (multifocal and sys-
2. Epidemiology and etio-pathogenesis
temic forms) usually seen in infants and children, where pulmonary
involvement is often not a prominent feature; or more frequently
“Primary” or isolated pulmonary LCH (PLCH) is a well described
as a distinct entity in adult smokers. Isolated PLCH is exceedingly
entity in young adults between 20 and 40 years of age; but it is
rare in children [2–11]. This article describes the etio-pathogesis
exceedingly rare in children younger than 18 years of age [9,12].
Pulmonary involvement, however, is not unusual in systemic forms
of LCH seen in children [13]. Isolated pulmonary involvement was
first reported in 1951 in two adults and in 1976 in a child as
∗ Corresponding author at: Room no.: 13, Department of Radiodiagnosis, Lady
“eosinophilic granuloma of the lung” [2,14]. The gender distribu-
Hardinge Medical College and Associated Smt. Sucheta Kriplani & Kalawati Hospi-
tals, New Delhi 110001, India. Tel.: +91 9868244786.
tion of PLCH is variable both in children and adults; however, it
E-mail addresses: dr shahinaindia@yahoo.com (S. Bano), more commonly affects the Caucasians [13]. The exact incidence
dr vikaschaudhary@yahoo.com (V. Chaudhary), narulamk@gmail.com and prevalence of PLCH is unknown because a significant num-
(M.K. Narula), rama home@yahoo.co.in (R. Anand), ber of affected patients may be asymptomatic and the disorder
venkatesanbhuvana@yahoo.com (B. Venkatesan), shramana@hotmail.co.in
may undergo spontaneous resolution or may remain underdiag-
(S. Mandal), drkaushik.m@gmail.com (K. Majumdar).
1
Employees’ State Insurance Corporation (ESIC) Model Hospital, Gurgaon nosed as it may be difficult to identify the LCs from lung biopsy
122001, Haryana, India. Tel.: +91 9968338008. specimens in patients with advanced end stage disease (LCs are
2
Tel.: +91 011 25459098. rare or absent in patients with lung fibrosis) [1,13]. Pathogenesis
3
Tel.: +91 011 25259248. of PLCH in childhood differs from that of adult where more than
4
Room no.: 13, Department of Radiodiagnosis, Lady Hardinge Medical College
and Associated Hospitals, New Delhi 110001, India. Tel.: +91 9711152157.
95% of affected patients are cigarette smokers [12,15]. In children,
5
Tel.: +91 9718599072. the pathology probably reflects an uncontrolled immune response
6
Tel.: +91 7838918518. to an unknown stimulus/antigen [10]. It has been suggested that

0720-048X/$ – see front matter © 2013 Elsevier Ireland Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ejrad.2013.04.044
48 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56
Fig. 1. (a and b) PLCH in a 4-year old male child. Broncho-fibreoptic Lung biopsy
[H&E stain, 200×] shows collection of histiocytes (arrow), along with lymphoid
aggregates and pneumocytes (a). Immunohistochemistry for S100 protein highlight-
ing the histiocytes (arrows) in the lung lesion [200×] (b).
in response to an unknown stimulus, there is rapid proliferation of
Langerhans cell almost exclusively in the bronchial and bronchio-
lar epithelium, with formation of destructive granulomas adjacent
to the small airways. Later, these cellular granulomas evolve and
undergo cavitation; the peripheral fibrosis causes traction on the
central bronchiole which becomes cyst like. The cysts have varied
wall thickness and sizes, and often bizarre shapes. This explains the
presumed evolution from nodule, through cavitating nodule and
cysts with variable wall thickness [9,12,13]. Definitive diagnosis
of PLCH is established with lung biopsy or bronchoalveolar lavage
(BAL). Histopathologically, the disease is characterized by prolifer-
ation of Langerhans cell histiocytes (Fig. 1a). CD1a positivity and
presence of cytoplasmic S-100 protein (Fig. 1b) are other useful
indicators of this histiocytic disorder [16,17]. Electron microscopy
may reveal characteristic Birbeck granules [12].
3. Clinical presentation
Up to a quarter of patients are asymptomatic. Presentation
is usually with dyspnoea or a non-productive cough [1]. Other
symptoms include constitutional symptoms like fatigue, malaise
and weight loss or pleuritic chest pain [6,9,11,13]. Wheezing and
haemoptysis are uncommon [12]. Clubbing is an exceptional find-
ing [12]. Spontaneous pneumothorax (Figs. 2 and 3) resulting
from rupture of a subpleural cystic lesion occurs in approximately
10–20% of children [8]. Advanced disease may show signs of
Fig. 2. (a and b) PLCH in a 4-year old male child (same patient as in Fig. 1). Pos-
teroanterior (PA) chest radiograph shows bilateral pneumothoraces (white arrows)
and partially collapsed lungs (a); and diffuse, bilateral, symmetrical, small nodular
opacities (curved arrows) throughout the re-expanded lung fields after intercostal
chest tube drains insertion (b). Mild left sided pleural effusion (black arrow) is also
present (b). The patient had skin lesions and radiological evidence of LCH involving
hepatobiliary system (hepatosplenomegaly with portal triaditis due to histiocytic
infiltration) (not shown).
Fig. 3. PLCH in a 3-year old male child. Chest X-ray PA view shows bilateral retic-
ulonodular opacities (curved arrows) and spontaneous pneumothorax (minimal)
(straight arrow) along the left side of mediastinal pleura.
corpulmonale and severe pulmonary insufficiency [6,9,11,13]. Pul-
monary function test may show great variability depending on
the duration of disease and nature of parenchymal abnormali-
ties present. Normal, obstructive, restrictive and mixed patterns
have been described [9,12,13]. Screening echocardiography for
pulmonary hypertension should be considered in all dyspnoeic
patients [15]. Results of a respiratory examination and laboratory
blood tests are usually normal or nonspecific in patients with PLCH
[6,9].
Fig. 4. (a and b) PLCH in a 7-year old female child. Chest radiograph PA view (a)
shows normal lung fields. HRCT of the chest, axial image (lung window) at level of
tracheal bifurcation (b), early in the course of the disease demonstrates micronodu-
lar (≤0.5 mm size) interstitial opacities (arrows) involving both the lung fields, not
evident on chest X-ray.
 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56 49

Fig. 5. PLCH in a 17-year old male child. HRCT of the chest, axial image (lung win-
dow) at lung base, early in the course of the disease demonstrates small (<5 mm
size), nodular interstitial opacities (arrows) predominantly involving right lung field.
Note, the presence of nodular opacities at lung base near the costophrenic angles. Fig. 7. PLCH in an 1-year old female child. HRCT of the chest, axial image (lung
window) at level of heart, early in the course of the disease reveals relatively
larger (>5 mm size), discrete, irregular nodules (arrow heads) randomly distributed
4. Imaging findings in both the lung fields; some of these nodules demonstrate early sign of cavita-
tion/excavation (arrows).
Imaging finding of pulmonary LCH vary depending on the stage
of the disease at the time of diagnosis. Chest X-ray has limited sen-
sitivity and specificity in detecting and characterizing early and
subtle changes (Fig. 4). High-resolution CT (HRCT) is most useful
and sensitive for demonstrating the spectrum of imaging features
of PLCH [18,19]. The major advantage of HRCT is in assessing the
lung nodules that cannot be adequately seen on CXR. CT findings
of PLCH are similar in adult and paediatric population, with the
exception that lung base near the costophrenic angle is almost
always involved in children (Fig. 5) but is spared in adults [20].
The HRCT pattern of disease demonstrates nodular (Figs. 5–7)
or reticulonodular (Fig. 8) infiltrates in early course of the dis-
ease; whereas, cystic lesions predominate in advanced disease
(Fig. 9). Few patients may show a combination of nodular or retic-
ulonodular opacities and cystic lesions. The lesions are typically
diffuse, bilaterally symmetrical and have predilection for upper
and mid-zones. The nodules usually have ill-defined margins, are

Fig. 8. PLCH in a 3-year old male child (same patient as in Fig. 3). HRCT of the chest,
axial image (lung window) at level of right pulmonary artery, early in the course of
the disease demonstrates reticulonodular interstitial opacities (arrows), along with
left sided pneumothorax (asterisk).

Fig. 6. PLCH in a 3-years old male child. HRCT of the chest, axial image (lung window)
at hilar level, early in the course of the disease demonstrates small (<5 mm size),
nodular interstitial opacities (arrows) evenly distributed in bilateral lung fields. The
nodules have ill-defined margins and are typically cetrilobular, peribronchial and
peribronchiolar in distribution. Left sided pneumothorax (asterisk) present.
Fig. 9. PLCH in a 14-year old female. HRCT of the chest, axial image (lung window),
apical region shows a combination of nodular (straight arrows) and cystic lesions
(curved arrows). The nodules have irregular borders; the cysts are more in number
have thin walls and are of variable sizes and shapes.
50 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56

Fig. 10. (a and b) PLCH in a 12-year old female. HRCT chest, coronal thick MPR (a) and MinIP (b) images demonstrate multiple thin walled lung cysts (white arrows) with
upper and middle lobe predominance. Few ill-defined subpleural nodules (arrow heads) are also seen.

typically cetrilobular, peribronchial, or peribronchiolar in distribu- cavitation (Figs. 4–6) [21–24]. The cysts are often seen in asso-
tion (Fig. 6), and surrounded by normal lung parenchyma. They ciation with nodules and occur predominantly in upper lung
may be few or innumerable in numbers, measuring between zones (Figs. 9–11). Occasionally, cysts may be the only find-
0.5 and 10 mm in size (Figs. 4–7), and with (Fig. 7) or without ing on HRCT (Fig. 12). They are usually less than 10 mm in

Fig. 11. (a–e) PLCH in a 3-year old male child in advanced disease. Chest radiograph PA view (a) shows bilateral upper zone nodules (straight arrows) and mid/lower zone
cystic (curved arrows) changes (more prominent on right side). HRCT of the chest, axial image (lung window), upper zone (b) shows a combination of nodular (straight
arrows) and cystic (curved arrows) lesions, while middle and lower zone (c) lesions are purely cystic. The cysts are more in numbers and better appreciated on coronal MinIP
(d) image, when compared to routine HRCT image (e). Cysts are diffuse, thin walled, of variable sizes and shapes and associated with marked parenchymal destruction. The
lung volume appears normal.
 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56 51

Fig. 12. (a–c) PLCH in a 1-year old male child. HRCT of the chest in advanced disease, (a) axial image (lung window), demonstrates purely cystic lesions (curved arrows)
causing extensive parenchymal destruction. Cysts are diffuse, thin walled and discrete to confluent (bizarre) in nature. Right middle lobe consolidation with air bronchogram
(straight arrows) also seen. Coronal (b) and sagittal (c) MPR images (lung window), demonstrate normal lung volume inspite of extensive parenchymal destruction.

diameter, but may measure up to 20–30 mm in size. Major-
ity of cysts have round or ovoid shapes but some may appear
to coalesce into larger, more irregular structures with bizarre
configuration exhibiting bilobed, cloverleaf and branching mor-
phologies [12,21–24] (Figs. 9–13). Cysts are usually thin walled
(wall thickness up to 2 mm), but rarely may be thick walled
(wall thickness >2 mm) (Fig. 14). Reduction in size of PLHC cysts
at end of expiratory film (when compared with their size on
inspiratory scan) suggests communication between the cysts and
the airways [12]. Minimum IP (MinIP) images are excellent in
characterizing occult cystic lesions (Fig. 15) not evident on con-
ventional HRCT. It is required to state that the MinIP should be
routinely used as the optimum imaging technique for diagnos-
ing PLCH of the paediatric patients. In end-stage PLCH, the cysts
and residual parenchyma tend to undergo fibrosis over time (e.g.
stellate scars and surrounding cystic spaces of variable diameter),
eventually leading to coarse reticular opacities or changes of honey-
combing with irreversible architectural distortion [12,25,26]. The
appearance of new nodules later in the disease when the cystic
change is well established, is a rare finding, and indicates disease
progression [9,18,27].
Rare radiographic manifestations of PLCH include ground
glass opacities such as desquamated interstitial pneumonia (DIP)-
like changes (Fig. 16), mosaic attenuation pattern (Fig. 17),
emphysema (traction) [9,19,27], air space consolidation (mul-
tifocal fibrotic consolidation) [10], solitary pulmonary nodule,
endobronchial mass with distal consolidation [28–31], pleural
effusion/hydropneumothorax (Fig. 18) and mediastinal/hilar lym-
phadenopathy (Fig. 18) [9,12,25,32]. Very rarely, PLCH has been
reported to be associated with active pulmonary tuberculosis
[33,34]. Therefore, it should always be kept in mind that, in areas
with high prevalence of tuberculosis, LCH and pulmonary tubercu-
losis might coexist in the same patient. The lung volume, unlike that
in other interstitial lung diseases, mostly remains normal or may
be increased. Reduced lung volume is uncommon and only seen in
end-stage fibrotic PLCH [12,19,25,27].
Spontaneous pneumothorax from rupture of peripherally sit-
uated lung cyst is a recognized complication of PLCH, seen in
approximately 10–20% of patients. Pneumothorax can occur at
any time throughout the evolution of the disease and can be
recurrent and bilateral. Bilateral and large pneumothorax usually
presents as an acute surgical emergency requiring urgent inter-
vention [6,8,14,19]. Rarely, pneumomediastinum due to leakage
of air from pulmonary interstices may also occur [9]. Pulmonary
arterial hypertension and cor pulmonale may result from exten-
sive parenchymal destruction due to fibrosis, eventually leading to
death from respiratory failure in end stage PLCH [14,18].
The high-resolution CT demonstration of cysts and nodules
in a characteristic distribution, in correlation with demographic
and clinical factors, allows a confident prospective diagnosis of
PLCH with a diagnostic accuracy of 72% [35]. However, the diag-
nostic accuracy of HRCT falls short when only nodules or cysts
alone are present. Early in the disease, when nodules are the only
CT manifestation of PLCH, the differential diagnosis may include
granulomatous disease (e.g. sarcoidosis), silicosis, tuberculosis and
metastases. The anatomic distribution of nodules may be use-
ful in narrowing the differential. The nodules of PLCH typically
follow a centrilobular, peribronchial and peribronchiolar distribu-
tion and thus differ from the nodules of sarcoidosis, silicosis and
lymphangitic carcinomatosis, which are perilymphatic in distri-
bution [9,12,36]. Later in the disease, when cysts are the only CT
manifestation, the other differentials of primary cystic lung disease
52 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56

Fig. 13. (a–d) PLCH in a 10-month old male child. HRCT of the chest in advanced disease, axial image (a), coronal (b)/sagittal (c) images (lung window) and coronal MinIP
(d), demonstrates extensive cystic lesions distributed throughout both the lung fields. Cysts are of variable sizes and shapes, varying from tiny thin walled and discrete
(straight arrows) to large and confluent in nature (curved arrows). Cysts in bilateral perihilar region appear to coalesce into larger, more irregular structures with bizarre
configuration exhibiting bilobed, cloverleaf and branching morphologies. They appear to engulf the perihilar vessels. Bilateral perihilar patchy consolidation also seen. Note,
cysts in bilateral CP angle region best appreciated on coronal MinIP image. The patient had skin lesions and radiological evidence of LCH involving hepatobiliary system
(hepatosplenomegaly with portal triaditis due to histiocytic infiltration) (not shown).

such as lymphangiomyomatosis, emphysema (centrilobular), cys-
tic bronchiectasis, pneumocystis jiroveci pneumonia and idiopathic
pulmonary fibrosis should be excluded. The cysts in LCH are often
variable in size and wall thickness and tend to be more numerous
in upper lung zone. Cysts of lymphangiomyomatosis can resemble
those of PLCH, but they occur diffusely throughout the lungs and
affect women almost exclusively. Cystic changes in emphysema
represent foci of destroyed lung parenchyma that typically lacks
perceptible walls. Cysts in bronchiectasis demonstrate communi-
cating branching pattern on contiguous CT images. The lung cysts
or pneumatoceles in patients with pneumocystis jiroveci pneumonia
may be indistinguishable from cystic lesions of PLCH. Honeycomb
cysts of idiopathic pulmonary fibrosis are basal and subpleural in
distribution, and are associated with reduced lung volume and
adjacent lung parenchymal abnormalities (such as ground-glass
opacity or architectural distortion); whereas PLCH cysts are usu-
ally surrounded by normal lung [9,12,36]. Pinpoint centrilobular
nodules in the background of diffuse ground-glass opacification of
the lung are a rare observation and should be differentiated from
active interstitial pneumonias in children like DIP [27].
Different patterns of disease evolution have been recognized
on radiological examination. The radiographic abnormalities may
regress or completely resolve, remain stable over several years or
progress to fibrosis and end-stage PLCH [19,24]. In PLCH cases, it
has been observed that nodular and ground glass opacities typi-
cally exhibit regression; while, the cystic lesions, emphysema and
fibrotic changes are either persistent or may show progression on
serial imaging studies [19,37].
 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56 53

Fig. 14. (a and b) PLCH in two different patients, (a) 13-year and (b) 15-year old female. HRCT of the chest, axial image (lung window), shows multifocal nodules and cysts. The
nodules (arrow heads) have irregular borders, the cyst walls have variable thickness ranging from thin and uniform (curved arrow) to thick, nodular and irregular (straight
arrow).

HRCT not only allows a confident prospective diagnosis of PLCH
that obviates the need for biopsy in some patients, but also helps
in selecting an optimum site for lung biopsy in doubtful cases
[21,23]. Periodic evaluation with HRCT thorax helps in monitor-
ing the disease in very young children, who are too small for lung
function tests. It has been observed that the degree of pulmonary
involvement depicted at HRCT correlates well with the severity
of functional impairment. Moreover, HRCT provides radiographic
correlates of pathologic findings [21,38].
5. Treatment
Children with diffuse LCH or with rapidly progressive
PLCH are treated with chemotherapeutic agents, such as
vinblastine, methotrexate, cyclophosphamide, etoposide and 2-
chlorodeoxyadenosine [39,40]. The long-term clinical course of
childhood PLCH is not well established. Hence, a long-term follow-
up is recommended in these patients to evaluate the eventual
clinical course by serial imaging studies and pulmonary function
test [41,42].
Lung transplantation is a therapeutic option in some selected
patients with progressive disease unresponsive to planned
chemotherapy [15,43]. Recurrent pneumothoraces are treated by
standard techniques such as drainage and surgical pleurodesis [44].
Aggressive treatment of secondary pulmonary infections is also
important in the management of patients with PLCH, which oth-
erwise may increase the morbidity and mortality associated with
the disease [15].
6. Prognosis
Prognosis of primary PLCH is unclear in paediatric age group
because of limited number of cases. Poor outcome is associated with
extremes of age, prolonged constitutional disturbances, multiorgan
involvement, severely reduced diffusion capacity, extensive cysts
and honeycombing on imaging, recurrent pneumothoraces, associ-
ated pulmonary hypertension and prolonged treatment. Death may
result from respiratory insufficiency or pulmonary hypertension or
both [36,44,45].
7. Role of passive smoking in development of PLCH in
children
Based on the pathogenesis of PLCH in response to cigarette
smoke, not only active smoking, but passive smoking also has a
role in the development of PLCH [1,21]. Pulmonary LCH has been
observed in children exposed to second-hand smoke [46]. Although
prospective data suggesting that stopping exposure to second-
hand smoke may improve the disease manifestation are lacking,

Fig. 15. (a and b) PLCH in a 4-year old male child (same patient as in Fig. 2). HRCT of the chest, coronal (a) MinIP image shows multiple occult cystic lesions (white arrows),
not evident on (b) MPR image. Also note interstitial micronodules (black arrows) throughout the lung field and consolidation along the course of ICTDs bilaterally (asterisk). In
addition, the patient had skin lesions and radiological evidence of LCH involving hepatobiliary system (hepatosplenomegaly with portal triaditis due to histiocytic infiltration)
(not shown).
54 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56

Fig. 16. (a–d) PLCH in a 6 month old male child. Chest X-ray PA view (a) shows ground glass opacification of bilateral lung fields. HRCT of the chest (lung window), axial
(b) and coronal MPR (c) images demonstrate bilateral pinpoint interstitial opacities (arrows) in the background of hyper-attenuated lung field. Coronal MinIP image (d), in
addition, demonstrates tiny cystic opacities in peribroncial/peribronchiolar location (on right side) not appreciated on routine HRCT image.

Fig. 17. PLCH in a 16 year old female child. HRCT chest axial image (lung window) demonstrates mosaic attenuation pattern of the lung along with multiple small cysts
(arrows) sparsely distributed in bilateral lung fields. Multiple small (1–3 mm size) ill-defined nodules were also seen in right apical region (not shown).
 S. Bano et al. / European Journal of Radiology 83 (2014) 47–56 55

Fig. 18. (a–c) PLCH in a 1 year old female child. Chest radiograph PA view (a) shows right sided loculated hydropneumothorax (asterisk) with collapse-consolidation of right
upper lobe (straight black arrow). Left lung field reveals multiple ill-defined nodular parenchymal opacities (straight white arrow). HRCT of the chest, axial image (b) lung
window and (c) mediastinal window confirms the chest X-ray findings; and also demonstrates tiny cysts (curved arrow) in the left lung in addition to irregular nodules
(white arrow head). Right intercostal chest tube drain (white arrow head in a) and nasogastric tube (black arrow head in a, b) are seen in situ. Patient had disseminated LCH
(multifocal and systemic forms) with involvement of skin, lung, hepatobiliary (hepatosplenomegaly with portal triaditis) and central nervous system (pontine granuloma,
mastoiditis with bone destruction, otitis media and posterior auricular abscess) (not shown).

stabilization or complete remission of the disease has been docu-
mented in smokers after treatment with chemotherapeutic agents
and cessation of smoking. Moreover, some individuals may have
disease progression despite smoking cessation [1,21,46].
8. Conclusion
HRCT allows a confident prospective diagnosis of PLCH in the
appropriate clinical setting. The HRCT pattern of nodular and cystic
changes involving the upper and middle lobes with involvement of
lung bases is highly characteristic of PLCH in children; the adults
however show relative sparing of the costophrenic angles. We
emphasize on use of MinIP in all cases of PLCH for demonstration
of occult cystic lesions. The clinical course and prognosis is variable
with frequent regression or stability of the abnormalities.
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