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.