Pulmonary adenomatoid malformation presenting as unilobar cysts in an adult

RESPIRATORY

MEDICINE

(1998)

92,

1364-1372

Case Reports

Pulmonary adenomatoid malformation unilobar cysts in an adult D. HELLMLJTH*, J. C. GLERANT*, H. SEVESTRE+,A. REMOND* “Pulmonary Division, ‘Pafhology 80054 Amiens ceder 1, France

Division

*Radiology

Division,

presenting

as

AND V. JOUNIEAUX*

Centre Hospitalier

Universitaire

Sud,

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 performed 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
Case Report A 23-year-old woman presented in July 1996, with haemoptysis. Her medical history included a surgical resection of a right ovarian cyst, and tobacco use (1 pack-year). On admission, the patient was asymptomatic and haemoptysis spontaneously resolved. Physical examination was unremarkable. Chest X-rays showed an air-fluid level in the right upper lobe (Fig. 1). During fibre bronchoscopy a clot obstructing the right upper bronchus was observed. High resolution CT scan of the chest revealed multiple cysts in the right upper lobe surrounded by parenchyma with ground-glass appearance (Fig. 2). The size of the visible cysts ranged from 5 to 30 mm. Air-fluid levels could be observed in the three largest cysts and the fluid density corresponded to the density of blood. Routine laboratory tests were normal. Intradermal test with 10 tuberculin units was negative. Examination of bronchial secretions showed no abnormal cells or

1. Chest X-ray showing an air-fluid level in the right upper lobe.

FIG.

micro-organisms. Pulmonary function tests and diffusion capacity were normal as were arterial blood gas values. In August 1996, a complete surgical resection of the right upper lobe was performed. Pathological examination showed multiple cystic lesions lined with a cuboidal ciliated epithelium and mucigenic cells (Fig. 3). Inflammation was noted but no malignant cells were observed. Final diagnosis was a type 1 CCAM according to the modified Stocker’s classification (Table 1) (1).

Discussion Received 20 May 1998 and accepted in revised form 13 July 1998. Correspondence should be addressed to: V. Jounieaux, Service de Pneumologie et Unit& de R&animation Respiratoire, Centre Hospitalier Universitaire Sud, 80054 Amiens cedex 1, France. 0954-6111/98/121364+09

$12.00/O

Congenital cystic adenomatoid malformations of the lung (CCAM) result from a disturbed lung embryogenesis. Simonet et al., who studied pulmonary malformations in transgenic mice expressing human keratinocyte growth 0

1998 W. B. SAUNDERS

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LTD

CASE REPORE

1365

1. Expanded classification of congenital cystic adenomatoid malformation of the lung modified by Stocker in 1994

TABLE

Type 0 Composed of bronchial-like structures, appears to Type 1 Type 2 Type 3 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 predilection. 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 casesof 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

Type 4

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 midacinar 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 casesof CCAM have been reported in adulthood. These cases are listed in Table 2. Sixteen out of these 23 casescorresponded to a type 1 CCAM according to Stocker’s modified classification (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 caseswere revealed by spontaneous pneumothorax, three by a haemoptysis and one by dyspnoea. 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 controversial. Some authors recommend surgical resection for diagnosis but also as a therapeutic procedure because of a substantial risk of later neoplasia (9). Others consider this approach too aggressivein 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 rhabdomyosarcoma, 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). However, four casesof 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 adulthood than in childhood (P
2

et al.

1

1

1 1

et al. 1997 (26)

Salmon et al. 1997 (25) Plit et nl. 1997 (7)

Jordan

1996 (8)

et al.

Lackner

I 7

Ribet et al. 1995 (14) Patz et al. 1995 (24)

1995 (17)

I 1

Akiba et al. 1992 (22) Han et ~1. 1994 (23)

Morresi

1 1

Pulpeiro et ul. 1987 (21) Sheffield ef al. 1987 (16)

Not precise 29 27

22

Man Man

Man

Womzan

3 Women

Woman 4 Men

Woman Woman

Man Woman

Womzan Woman Woman Woman Man

Man Man

Sex

literature

42 From 21 to 61

20 41

18 19

65 23 44 18 18

I

2

Chen 1985 (19) Sagawa et al. 1985 (20)

35 24

2

et al. 1984 (I 8)

Avitabile

AS (years)

Cases

2. Review of international

Author references

TABLE

39°C

Recurrent Dyspnoea

infections

Pneumothorax

0 Six recurrent pneumonia One recurrent pneumothorax Occasional haemoptysis

0

0 Haemoptysis and impairment of exercise tolerance Recurrent pneumonia Multiple air-fluids levels on CT

Haemoptysis Pneumothorax

Fever 0

Symptoms

middle

and lower

Left lower lobe Bilateral lung involvement

Not precise

Right

lobe

lobes)

lobes

lower

lobe lower

(4 left, 3 right

Left lower Exclusively

Middle lobe Left lower lobe

Right upper lobe Left upper lobe

Left lower lobe Air fluid level at the right lung base Right lower lobe Right lower lobe

Side and site

1 2

Not

1

2:2

1 1 1 5:l

1

1 1

1

1 1

precise

Stocker’s classification

0 0

Cavernous transformation of the portal and splenic vein presacral Not precise

Slightly enlarged and tortuous bronchial artery feeding the multilobulated cystic lesion 0 0 0 0

0 0

Bronchectiasis

0 0

Associated abnormalities

tumorlets

trigeminism

colonization

precise Ventricular 0

Not

0

BAC BAC BAC 0

0

Fungus BAC

Pulmonary

0 0

Complications

lower lower

lobectomy lobectomy

Left 0

Not

Right lower

lower

precise

of the

lobectomy

middle and lobectomy

Lobectomy Cyst resection Segmentectomy Surgical resection abnormal tissues

Right pneumonectomy Not precise

Right upper lobectomy Cyst resection

Right Right

Left lower lobectomy Right lower lobectomy

Treatment

CASE REPOKTS

Acknowledgement The authors thank Professor Daniel 0. Rodenstein (Pneumology Unit, Cliniques Universitaires St Luc: Brussels, Belgium) for his valuable comments.

References 1. Stocker JT. Congenital and developmental diseases. In: Dali DH: Hammar SP, eds. Pul7m7za7y Pathology. 2nd edn. New York: Springer-Verlag, 1994: 155-90. 2. Simonet WS, DeRose ML; Bucay N: et al. Pulmonary malformation in transgenic mice expressing keratinocyte growth factor in the Lung. Proc Nat1 Acnd Sci USA 1995; 92: 12461-12465. 3. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung Classification and morphological spectrum. Hu7n Path01 1977; 8: 156-171. 4. Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest 1993; 103: 161-764. 5. Chin KY, Tang MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Path01 Lab iWed 1949; 48: 221-229. 6. Pages A, Kourie JP. Malformation adenomato’ide kystique congenitale du poumon: a propos de sept observations. Arch Anat Cytol Path 1994; 42: 91-95. I. 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. 8. 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. 9. Case Records of the Massachusetts General Hospital (case 20-1996). N Engl J Med 1996; 334: 172661732. 10. Ward SJ. Surgery for congenital cystic adenomatoid malformation of the lung. N Engl J &fed 1996; 335: 1689. 11. Cohen M, Emms M: Kaschula ROC. Childhood pulmonary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma. Pediat Path01 1991; 11: 137-149. 12. Ueda K, Grippo R, Unger F, et al. Rhabdomyosarcoma of lung arising in a congenital cystic adenomatoid malformation. Cancer 1977; 40: 383-388.

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13. Kaslovsky RA, Purdy S, Dangman BC, et al. Bronchiolo alveolar carcinoma in a child with congenital cystic adenomatoid malformation. Chest 1997; 112: 548-551. 14. Ribet ME, Copim MC, Soots JG; et al. Bronchioloalveolar carcinoma and congenital cystic adenomatoid malformation. Ann Thorax Sung 1995; 60: 112661128. DR, Cahill JL. Bronchioloalveolar carci15. Benjamin noma of the lung and congenital cystic adenomatoid malformation. Am J Clin Path01 1991; 95: 889-892. 16. Sheffield EA, Addis BJ, Conn B, et al. Epithelial hyperplasia and malignant changes in congenital lung cysts. J Clilz Path01 1977; 40: 612-614. 17. Morresi A: Wockel W, Karg 0. Adenomatoid cystic lung abnormality in adults with associated bronchioloalveolar carcinoma. Pathologe 1995; 16: 292-298. 18. Avitabile AM, Greco MA, Hulnick DH, et al. Congenital cystic adenomatoid malformation of the lung in adults. Am J Swg Path01 1984; 8: 193-202. 19. Chen KT. Congenital cystic malformation of the lung and pulmonary tumorlets in an adult. J Swg Oncol 1985;30: 106-108. 20. Sagawa H, Ebihara Y, Kuwabara T, et al. Two adults cases of pulmonary congenital cystic adenomatoid malformation. Nippon Kyobu Shikkan Gakkai Zasshi 1985; 23:593-598. 21 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. 22. 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. 23. 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. 24. Patz EF, Muller NL, Swensen SJ: et al. Congenital cystic adenomatoid malformation in adults: CT findings. J Conzput Assist Tonlogy 1995; 19: 361-364. 25. 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. 26. Jordan KG, Kwong S, Flint J, et al. Surgically treated pneumothorax: radiologic and pathologic findings. Chest 1997; 11: 280-285.