pulmonary adenomatoid malformation presenting as unilobar … · 2017. 1. 5. · respiratory...
Post on 25-Jan-2021
5 Views
Preview:
TRANSCRIPT
-
RESPIRATORY MEDICINE (1998) 92, 1364-1372
Case Reports
Pulmonary adenomatoid malformation presenting as unilobar cysts in an adult
D. HELLMLJTH*, J. C. GLERANT*, H. SEVESTRE+, A. REMOND* AND V. JOUNIEAUX*
“Pulmonary Division, ‘Pafhology Division *Radiology Division, Centre Hospitalier Universitaire Sud, 80054 Amiens ceder 1, France
Introduction
We report the case of a patient with congenital cystic adenomatoid malformation of the lung (CCAM) diagnosed during adulthood. High resolution computed tomography (CT) scan of the lungs revealed type 1 disease according to Stocker’s classification. A surgical procedure was per- formed and pathological examination of the lobe involved showed inflammation but no neoplasia. When analysing the literature, CCAM are more frequently associated with neoplasia in adulthood as compared to childhood (P
-
FIG. 2. High resolution CT scan showing the largest cyst in the right upper lobe surrounded by smaller cysts with a cluster organization. Note the air-fluid level in this cyst and the ground-glass appearance of the surrounding parenchyma. The fluid density corresponded to the density of blood.
FIG. 3. Numerous small cysts separated by fibromuscular walls. Note the cartilage plate embedded in the fibrous wall of a large cyst at the upper right corner of the figure (H & E, magnification x 32, reproduced here at 55%).
factor in the lung, suggested that CCAM could correspond to an arrest of lung development at a premature stage (2). No specific factors have been identified with regard to pathogenesis and CCAM shows no sexual or racial predi- lection. CCAM was first classified by Stocker et al. into three types after pathological examination (3); however, this author modified his classification in 1994 and separated CCAM into five pathological types according to their resemblance to normal anatomical structures from proximal to distal (Table 1) (1).
Apart from four cases of bilateral CCAM (447), the lesions are unilateral. Right and left lungs are affected almost equally. The malformation is usually limited to one lobe [82% for Cloutier et al. (4) with a slightly more frequent lower lobe involvement (5.5%)]. More than 150 cases of CCAM have been described: approximately 61% in newborns, 22% in infants under 6 months of age and 17% in older children (4). Visceral malformations associated with CCAM have been reported in up to 18% of children in association with type 2 CCAM in 56% of cases (3). In only
CASE REPORE 1365
TABLE 1. Expanded classification of congenital cystic adenomatoid malformation of the lung modified by Stocker in 1994
Type 0
Type 1
Type 2
Type 3
Type 4
Composed of bronchial-like structures, appears to be malformation of proximal tracebronchial tree Composed of bronchial-like structures and proximal bronchiolar-like structures, mimics distal bronchial tree and proximal acinus Composed of bronchiolar-like structures, resembles bronchiolar section of acinus Composed of structures resembling terminal bronchioles and alveolar ducts, suggests mid- acinar malformation Composed of thin-walled structures lined by alveolar lining cells, suggests origin from distal components of acinus
one adult patient, several congenital malformations have been described in association with a type 1 CCAM (8).
To the best of our knowledge, until now only 23 cases of CCAM have been reported in adulthood. These cases are listed in Table 2. Sixteen out of these 23 cases corresponded to a type 1 CCAM according to Stocker’s modified classi- fication (1). Like other congenital lung lesions, CCAM may be asymptomatic for years. In eight patients, CCAM was asymptomatic and diagnosed from routine chest X-rays whereas recurrent respiratory infections led to diagnosis in eight other cases. Three cases were revealed by spontaneous pneumothorax, three by a haemoptysis and one by dysp- noea. Type 1 CCAM can be accurately diagnosed through CT scan showing large cystic lesions (more than 1 cm in diameter). Bronchography adds no further information and should not be routinely performed.
Management of CCAM of the lung remains controver- sial. Some authors recommend surgical resection for diag- nosis but also as a therapeutic procedure because of a substantial risk of later neoplasia (9). Others consider this approach too aggressive in the absence of symptoms and/or complications and prefer watchful waiting (10). We agree that, in children, CCAM and especially type 1 CCAM are rarely associated with neoplasia. One case of rhabdomyo- sarcoma, one case of pulmonary blastoma and another one of bronchioloalveolar carcinoma have been reported out of more than 150 cases reported in childhood (11-13). How- ever, four cases of bronchioloalveolar carcinoma have been reported in 23 adults with CCAM (14-17). When analysing these data using Fisher’s exact test, neoplasia in type 1 CCAM appears to be significantly more frequent in adult- hood than in childhood (P
-
TABL
E 2.
Rev
iew
of i
nter
natio
nal
litera
ture
Auth
or
AS
refe
renc
es
Case
s (ye
ars)
Sex
Sym
ptom
s Si
de
and
site
Stoc
ker’s
As
socia
ted
class
ificat
ion
abno
rmal
ities
Co
mpl
icatio
ns
Trea
tmen
t
Avita
bile
et
al.
1984
(I
8)
Chen
19
85 (
19)
Saga
wa
et a
l. 19
85
(20)
Pulp
eiro
et
ul.
1987
(2
1)
Shef
field
ef
al.
1987
(1
6)
Akib
a et
al.
1992
(22
) Ha
n et
~1.
199
4 (2
3)
Mor
resi
et al.
19
95 (
17)
Ribe
t et
al.
1995
(1
4)
Patz
et
al.
1995
(24
)
Lack
ner
et al.
19
96 (
8)
Jord
an
et a
l. 19
97 (
26)
Salm
on
et al.
19
97
(25)
Pl
it et
nl.
1997
(7
)
2 I 2 1 1 I 1 2 I 7 1 1 1 1
35
Man
Fe
ver
39°C
24
M
an
0 Hae
mop
tysis
Pneu
mot
hora
x 65
W
omza
n
23
Wom
an
44
Wom
an
18
Wom
an
18
Man
0 H
aem
optys
is an
d im
pairm
ent
of
exer
cise
tole
ranc
e Re
curre
nt
pneu
mon
ia
Mul
tiple
ai
r-flu
ids
leve
ls on
CT
18
Man
19
W
oman
20
41
42
From
21
to
61
0 W
oman
W
oman
Wom
an
4 M
en
3 W
omen
22
Wom
zan
0 Six
recu
rrent
pn
eum
onia
On
e re
curre
nt
pneu
mot
hora
x Oc
casio
nal
haem
optys
is
Not
prec
ise
29
27
Man
Pn
eum
otho
rax
Not
prec
ise
Man
Re
curre
nt
infe
ctio
ns
Man
Dy
spno
ea
Left
lowe
r lo
be
Air
fluid
le
vel
at
the
right
lu
ng
base
Ri
ght
lowe
r lo
be
Righ
t lo
wer
lobe
1 0
1 0
0 0 Pul
mon
ary
tum
orle
ts
Left
lowe
r lo
bect
omy
Righ
t lo
wer
lobe
ctom
y
1 Br
onch
ectia
sis
Righ
t lo
wer
lobe
ctom
y Ri
ght
lowe
r lo
bect
omy
Righ
t up
per
lobe
1
0 Fu
ngus
co
loni
zatio
n Ri
ght
uppe
r lo
bect
omy
Left
uppe
r lo
be
1 0
BAC
Cyst
rese
ction
Mid
dle
lobe
Le
ft lo
wer
lobe
Ri
ght
pneu
mon
ecto
my
Not
prec
ise
1 Sl
ight
ly en
larg
ed
and
tortu
ous
bron
chia
l ar
tery
fe
edin
g th
e m
ultil
obul
ated
cy
stic
lesio
n 1
0 1
0 1
0 5:
l 0
0 BAC
BAC
BAC
0
Lobe
ctom
y Cy
st re
secti
on
Segm
ente
ctom
y Su
rgica
l re
secti
on
of t
he
abno
rmal
tis
sues
Left
lowe
r lo
be
Exclu
sively
lo
wer
lobe
s
(4 l
eft,
3 rig
ht
lowe
r lob
es)
Righ
t m
iddl
e an
d lo
wer
lobe
2:2 1
Cave
rnou
s tra
nsfo
rmat
ion
of
the
porta
l an
d sp
leni
c ve
in
pres
acra
l No
t pr
ecise
No
t pr
ecise
1 0
2 0
0 Ri
ght
mid
dle
and
lowe
r lo
bect
omy
Not
prec
ise
Not
prec
ise
Vent
ricul
ar
trige
min
ism
Left
lowe
r lo
bect
omy
0 0
Left
lowe
r lo
be
Bila
tera
l lu
ng
invo
lvem
ent
-
Acknowledgement
The authors thank Professor Daniel 0. Rodenstein (Pneu- mology Unit, Cliniques Universitaires St Luc: Brussels, Belgium) for his valuable comments.
References
1.
2.
3.
4.
5.
6.
I.
8.
9.
10.
11.
12.
Stocker JT. Congenital and developmental diseases. In: Dali DH: Hammar SP, eds. Pul7m7za7y Pathology. 2nd edn. New York: Springer-Verlag, 1994: 155-90. Simonet WS, DeRose ML; Bucay N: et al. Pulmonary malformation in transgenic mice expressing keratin- ocyte growth factor in the Lung. Proc Nat1 Acnd Sci USA 1995; 92: 12461-12465. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung Classifi- cation and morphological spectrum. Hu7n Path01 1977; 8: 156-171. Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest 1993; 103: 161-764. Chin KY, Tang MY. Congenital adenomatoid malfor- mation of one lobe of a lung with general anasarca. Arch Path01 Lab iWed 1949; 48: 221-229. Pages A, Kourie JP. Malformation adenomato’ide kystique congenitale du poumon: a propos de sept observations. Arch Anat Cytol Path 1994; 42: 91-95. Plit ML, Blott JA, Lakis N, et al. Clinical, radiographic and lung function features of diffuse congenital cystic adenomatoid malformation of the lung in an adult. Eur Respir J 1997 10: 1680-1682. Lackner RP, Thompson AB, Rikkers LF, et al. Cystic adenomatoid malformation involving an entire lung in a 22 year old woman. Arm Thorac Surg 1996; 61: 1827-1829. Case Records of the Massachusetts General Hospital (case 20-1996). N Engl J Med 1996; 334: 172661732. Ward SJ. Surgery for congenital cystic adenomatoid malformation of the lung. N Engl J &fed 1996; 335: 1689. Cohen M, Emms M: Kaschula ROC. Childhood pul- monary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma. Pediat Path01 1991; 11: 137-149. Ueda K, Grippo R, Unger F, et al. Rhabdomyosar- coma of lung arising in a congenital cystic adenomatoid malformation. Cancer 1977; 40: 383-388.
13.
14.
15.
16.
17.
18.
19.
20.
21
22.
23.
24.
25.
26.
CASE REPOKTS 1367
Kaslovsky RA, Purdy S, Dangman BC, et al. Bron- chiolo alveolar carcinoma in a child with congenital cystic adenomatoid malformation. Chest 1997; 112: 548-551. Ribet ME, Copim MC, Soots JG; et al. Bronchiolo- alveolar carcinoma and congenital cystic adenomatoid malformation. Ann Thorax Sung 1995; 60: 112661128. Benjamin DR, Cahill JL. Bronchioloalveolar carci- noma of the lung and congenital cystic adenomatoid malformation. Am J Clin Path01 1991; 95: 889-892. Sheffield EA, Addis BJ, Conn B, et al. Epithelial hyperplasia and malignant changes in congenital lung cysts. J Clilz Path01 1977; 40: 612-614. Morresi A: Wockel W, Karg 0. Adenomatoid cystic lung abnormality in adults with associated bronchiolo- alveolar carcinoma. Pathologe 1995; 16: 292-298. Avitabile AM, Greco MA, Hulnick DH, et al. Con- genital cystic adenomatoid malformation of the lung in adults. Am J Swg Path01 1984; 8: 193-202. Chen KT. Congenital cystic malformation of the lung and pulmonary tumorlets in an adult. J Swg Oncol 1985;30: 106-108. Sagawa H, Ebihara Y, Kuwabara T, et al. Two adults cases of pulmonary congenital cystic adenomatoid mal- formation. Nippon Kyobu Shikkan Gakkai Zasshi 1985; 23:593-598. Pulpeiro JR, Lopez I, Sotelo T, et al. Congenital cystic adenomatoid malformation of the lung in a young adult. B J Radio1 1987; 60: 1128-1130. Akiba T, Yamazaki Y, Yasukawa S, et al. A case of congenital cystic adenomatoid malformation of the lung in an 18 year old male. Nippon Kyobu Gekn Gakkai Zasshi 1992; 40: 161-164. Han YM, Lee DK, Lee SY, et al. Adult presentation of congenital cystic adenomatoid malformation of the lung: a case report. J Korean Med Sci 1994; 9: 86-91. Patz EF, Muller NL, Swensen SJ: et al. Congenital cystic adenomatoid malformation in adults: CT find- ings. J Conzput Assist Tonlogy 1995; 19: 361-364. Salmon JP, Coibion-Jossa V, Guiot-Ramault M. DCgCnerescence adenomatoi’de kystique congenitale du poumon et trigeminisme ventriculaire a l’age adulte. Peut-on trouver une relation de cause & effet? Rev Pneumol Clin 1997; 53: 33-36. Jordan KG, Kwong S, Flint J, et al. Surgically treated pneumothorax: radiologic and pathologic findings. Chest 1997; 11: 280-285.
top related