Pediatric Manuel

Case

P. Meza,

MD

of the

#{149} Beverley

Day1

Newman,

MD

#{149} Paul

S. Dickman,

MD

#{149} Richard

B. Towbin,

MD

t

Figures 1, 2. mothorax-induced “cystic” mass demonstrate (arrowheads)

(1)

Chest radiograph collapse of the

shows tension night lung, giving

pneurise to a

(arrows). (2) Axial 3-mm-thick CT sections multiloculated, septated, air-containing mass arising from the night lower lobe.

1.

2.

U HISTORY A full-term female infant was noted to have supnaventnicular tachycardia without nespinatory difficulties shortly after birth. After medical therapy, the patient was discharged. At 5 days of age, she experienced respiratory dis-

U FINDINGS The chest radiograph

tress

lung base. Findings from the CT scans confirmed the presence of the mass, which contamed septa ofvarying thicknesses, in the antenon aspect of the right lower lobe (Fig 2).

and

was

partment.

brought

to the

Mild cyanosis

emergency

and

pneumothonax,

diminished

breath sounds oven the right hemithorax were noted at physical examination. A portable chest radiograph (Fig 1) was obtained. Three days later, computed tomography (CT) of the chest

Index

was

performed

terms:

Hamartoma,

RadioGraphics 1

From

burgh,

1992;

the

(Fig

Departments

PA 15213.

From

63.314

heart

and

mediastinum

right

lung

had

air-containing,

de-

showed

with

1). Most

except ovoid

tension

shift

(Fig

collapsed 4-cm

a night

contralatenal

mass

for

of the

of the a septated,

in the

right

2).

Infants,

#{149}

newborn,

respiratory

system.

63.314,

63.7893

Lung

#{149}

neopla.sms,

63.314

12:843-844 of Radiology the

1991

RSNA

(M.P.M., scientific

B.N.,

R.B.T.)

assembly.

and Received

Pathology March

(P.S.D.),

Childrens

6. 1992;

accepted

Hospital March

of Pittsburgh. 30. Address

3705 reprint

Fifth requests

Ave.

Pittsto

M.P.M. C

July

1992

RSNA,

1992

Mezaetal

U

RadioGraphics

U

843

Figures

(3) Gross

3, 4.

The cysts eosin stain) epithelium.

shows

septa

DIAGNOSIS: tic

specimen

shows

vary in size and wall thickness. formed

a multicystic

by dense

Pulmonary

bands

mesenchymal

of primitive

cys-

U DISCUSSION The pneumothonax

most

ofthe

hemorrhage lower lobe

for 3 days

of a chest

night thoracotomy 4.0-cm rubbery

tube.

magnification,

mesenchymal

cells

age, with

was removed from (Fig 3). Microscopic

epithelium.

bands and chromatic

and The

A 3.5 x focal areas

of

postoperative

of the

were

was

sizes noncil-

formed

of primitive cells (Fig

course

Mesenchymal

by

age range

the

to CAM,

with

of 1.5-53

MCH period,

youngest

reported

and

appears

bly examples Surgical

these

to be

potential prophylactic

MCH.

in

hemoptysis,

to primitive In retrospect, at reported cases

were

(5,8). is the primary

Because

of MCH, Bove chemotherapy

and,

complicated

complications

ofMCH resection

case.

bilateral

pneumothorax,

of CAM with

years

has not been and our pa-

and malignant transformation sarcomas of the lung (1,3-7). least one and possibly several

for CAM and

hyper4). The

unremarkable,

contrast

by respiratory

proba-

treatment

of the malignant

has

suggested in these

use of cases (5).

pneumothorax. was

U

REFERENCES

first described in 1986 (1). Radiographically and pathologically, MCH is similar to cystic adenomatoid malformation (CAM). Both typi-

1.

Mark EJ. Mesenchymal cystic hamartoma of the lung. N EnglJ Med 1986; 3 15:1255-1259. Rosado-de-Chnistenson ML, StockerJT. Congenital cystic adenomatoid malformation. RadioGraphics 1991; 11:865-886. Hedlund GL, Bisset GS III, Bove KE. Malignant

cally

cystic

manifest

differ

in that

of fibrous

hamartoma

as a cystic

both are characterized lumnar nonciliated CAM

tissue

contains

and

(MCH)

pulmonary

by low epithelium. other

2.

mass;

cuboidal These

or coentities

stroma

composed

mature

elements,

whereas the stnoma in MCH bers of primitive mesenchymal

has striking cells.

3.

neoplasms

arising

Meza

et al

of the

5.

arising in mesenchymal Pediatr Pathol 1987; Bove KE. Sarcoma

chymal

cystic

173:77-79.

sarcomas

hamartomas

cystic

(abstr).

7:478.

arising

hamartoma.

in pulmonary Pediatr

Pathol

C, Catsaras H. a cystic hamartoma

of the

mesen1989;

9:785-792.

6.

Stephanopoulus complicating

Myxosarcoma lung.

Tho-

i-ax 1963;

7.

8.

18: 144-145. Weinberg AG, Currarino G, Moore GC, Votteler TP. Mesenchymal neoplasia and congenital pulmonary cysts. Pediatr Radiol 1980; 9:179-182. Ueda K, Gruppo R, Unger F, Martin L, Bove K. Rhabdomyosarcoma of lung arising in congenital cystic adenomatoid 40:383-388.

U

hamartomas

4.

.

RadioGraphics

in cystic

lung in childhood. Radiology 1989; Becroft DM, Jagusch MF. Pulmonary

num-

most commonly occurs in neonates, who present with nespiratony distress secondany to compression of normal lung and other intnathonacic structures by a large air- on fluidcontaining cystic mass (2) In a small percentage of cases, CAM can occur later in life as an unresolving ‘ ‘infiltrate’ ‘ or as smaller pulmonary cysts. In contrast, MCH can occur at any CAM

U

lined

may be multiple

by spontaneous

the night examination

cysts ofvarying tall columnar

septa

rare nodules mesenchymal

resolution

a

parenchyma.

100; hematoxylin-

X

and

a reported

represents

MCH

de-

As a result,

was performed. mass containing

revealed multilobulated lined by low cuboidal

844

pulmonary

(original

tient

persisted

placement

iated

replacing

(1,3). To our knowledge, reported in the neonatal

hamartoma.

spite

lesion

(4) Photomicrograph

malformation.

Cancer

Volume

1977;

12

Number

4