147

Downloaded from www.ajronline.org by 206.214.9.223 on 10/05/15 from IP address 206.214.9.223. Copyright ARRS. For personal use only; all rights reserved

:

.

Pulmonary

Blastoma

in a Child

Thomas E. Sumner,1 Charles R. Phelps, James E. Crowe,1 Stephen P. Poolos,2 and Louis DeS. Shaffner3

blastoma

Pulmonary

nard

]

[1

first

is a rare

described

malignant

it in

1 952

of its histologic similarity Spencer [2] reported four

because 1 961 term

blastoma.

mal

pulmonary

He

postulated

11,1

lung

as

tumor.

a lung

ithelial

to fetal lung tissues. cases and introduced

tumor

origin

Microscopically

Bar-

embryoma

from

In the

mesenchy-

analagous to renal nephroblastoma originating from renal blastema. Pulmonary blastoma is considered to be a primary lung tumor and capable of producing metastases. When Fung et al. [3] reviewed the literature in 1 977 and added

blastema,

a new

case

of their

own,

they

found

only

39 reported

cases of pulmonary blastoma. The average age reported is 39 years with the youngest 2 months and oldest 77 years. The entity is extremely rare in children. Martinez et al. [4] recently with

reported

unilateral

case

cystic

a pulmonary blastoma lung disease. We

of pulmonary

the

blastoma

in a child

in a 2-year-old girl have seen a second with

cystic

disease

the

components.

tumor

of undifferentiated

cells.

ponents

variable

exhibited

mesenchymal and

was

anaplastic

smooth

chondrocytes. The

structures tunes

and

varied with

cuboidal

to

The

previous

tissue

amounts were

lung

suggested

cartilaginous two

present material

cysts

of the

a relation

to

component

The

(fig.

lining

with

2B).

was

reviewed

peripheral

of the

struc-

cytoplasm These

were

stratification and

type.

pulmonary

2A).

tubular

scanty

cytoplasm. was

(fig.

the

pleomorphic

of branching

cells there

The vas-

chondrocytes

structures.

focally

com-

chondromatous,

of eosinophilic

and

epithelial

differentiation.

composed

differentiated

surgical

congenital

was

ep-

composed

hypenchnomatic,

contained

glandlike

columnar No cilia

The

large,

tumor

poorly

larger

epithelium. with

simple

from

to cells

elements.

of the

and

myxoid,

and

areas

mesenchymal

contained

Iacunae

part

large

of histologic

abnormally

Many

epithelial

the

mesenchymal

were

degrees

muscle

with

both

there

Both

component

culan,

contained

In addition

of

was

the

consistent

No mesenchymal blastoma

that

later

appeared.

of

lung. Discussion

Case

Report

In addition

A 4-year-old

after a

right

white

middle

congenital

‘

lobe

studies

within (fig.

mass

calcification,

pleural After

biopsy was A partial segment. 2.8

cyst.

were

circumscribed

localization

bands.

The The

in

the

right

lung

3 cm

of

routine

focally

showed with

No

of lung

a well

rare

surface,

x

fibrous

circumscribed

a lobulated

but up

to

2.3 cm in diameter grossly consistent

and composed of a firm tan tissue with foci with cartilage. There was no grossly demonstra-

ble

bronchial

relation

to any

Received 1

Salem, 2

Salem,

November

Department

of Radiology,

NC

Address

27103.

Department NC

of Pathology,

accepted

Bowman reprint

after Gray

requests

Bowman

Gray

revision

School

March

of Medicine,

5, 1979. Winston-

to T. Sumner. School

of Medicine,

Winston-

Bowman

AJR 133:147-148, July 1979 © 1 979 American Roentgen Ray Society 0361-803X/79/1331-0147 $00.00

Gray

School

of

Medicine,

in pediatric

Winston-

[3,

cases 4].

patients.

of pulmo-

Only

about

Blastoma

one

tends

to

subpleunal location and lacks bronchial Therefore it is most often an incidental finding patients. Chest nadiognaphs chanactenisti-

[5].

Upper

lobe

infrequent

[6].

Hilan

peripheral

location,

cytology,

bronchoscopy,

mass lesion ranging from a small an entire lobe; bilateral lesions are

predominance nodes

are

is common; rarely

preoperative and

cavitation

enlarged

[7].

evaluation scalene

Due

with

node

biopsy

is to its

sputum is usually

nonnevealing. Although

course

considered

of

a

pulmonary

malignant

blastoma

tumor,

varies.

shortest 3 months. to pneumonectomy; have

also

The enchymal

been

used.

No

clinical

distant

resection. is 1 9 years;

The the

ranges from wedge resection irradiation and chemotherapy particular

therapy

seems

to en-

curability.

origin

of

whether

the

entity

remains

it is

derived

from

pulmonary

Some authors cancinosarcoma

to fetal

Treatment external

the

Frequently

mestastases have occurred after surgical longest reported survival after resection

known Surgery,

40 additional reported

in a peripheral

hance

27103.

Department of Salem, NC 27103. j

structure.

1 4, 1 978;

been

cally reveal a solitary nodule to one occupying

x 5.0

by

have

been

was needle

5.0

have

connections. in asymptomatic

malignancy. in the anterior

roughened

quarter occur

cavitation,

fluoroscopy,

to our patient,

blastoma

well

solitary

adenopathy

suggested the lesion

a portion

was tumor

resection and

base.

and

histology included

surface

subpleural

x

on mediastinal

was

surface

sectioned

a 3

ultrasound

specimen

nary

3 years

limits.

the

with

lobe

examinations

showed

and biopsy lobectomy

pleural

unencapsulated

normal

or hilar

cough

night lower

Physical

‘ ‘

1)

lesion

fluid,

performed night upper Grossly

cm.

a nonproductive

and segmental

radiographs

evident.

developed

multilocular

laboratory Chest

girl

lung

have

blastema,

as Spencer

classified

it as

It is not of messuggested.

rests

[2]

a distinctive

type

a coincidental histologic resemblance 0]. However, pulmonary blastoma differs

with

[8-1

controversial. embryonic

of

CASE

148

REPORTS

AJR:133,

July

1979

Fig. 1 -Frontal (A) and lateral (B) views. Solitary round mass lesion in

Downloaded from www.ajronline.org by 206.214.9.223 on 10/05/15 from IP address 206.214.9.223. Copyright ARRS. For personal use only; all rights reserved

right

cystic

malformation

segmental

3

right

lower

years

after

lobe

resection

cyst. A histologic relation between blastoma has been suggested pulmonary

mas

blastomas

nate

from

are

right

middle

lobe

and

for a multiloculated

mesothelium and pulmonary [1 1 ]. Spencer [2] believes

the

counterpart

of

nephnoblasto-

B 2.-

A, Mesenchymal

smooth muscle lined by poorly tumor composed

portion

epithelial

with

chondrosarcomatous

component with and surrounding

and

glandlike spaces solid portion of

1

.

in

that

and

tumors.

it remains

mesothelium

and

Therefore,

of

origin

attractive,

Barnard

GW:

carcinosancoma

fibrous

although

pulmonary

blastoma

unproven.

it occurs

peripherally

Embryoma

Pulmonary

Martinez

of lung.

JA,

of a case. 5.

blastoma.

JC,

Manzano

rather

than endobronchially and has a glandular rather than squamous epithelial component. Prognosis also differs in that carcinosancomas have a uniformly poor prognosis. Of particular interest in our case was the development of a tumor at the site of previous resection for a multiloculated congenital cyst. Kern and Stiles [1 1 ] reported a pulmonary blastoma in a 29-year-old man 31 months after excision of a benign night middle lobe mesothelium-lined cyst. They suggested a histologic relationship may exist between mesothelium and blastoma. Martinez et al. [4] reported excision of a left congenital lung cyst which contained epithelial and mesenchymal elements consistent with a pulmonary bIastoma. Their case, a 2-year-old girl, resembles ours in that the excised tumor was located within ‘ ‘cystic lung. ‘ Our experience is somewhat similar in that a pulmonary blastoma occurred within a portion of night upper lobe having focal

WG:

FC,

Gutierrez

Corral

TF,

Abnil

J Pediatr

Han

55,

and

literature

Wills

JS,

Surg

review.

Peacock

MJ,

8.

monary Barson Patho/2i 10.

1 2.

.

a form

1977 del

blastoma:

report

1978 blastoma: 049, malignant 1977

Pulmonary

case

report

1976 tumors

blastoma.

of the

Thorax

31:

ER, Beland

AH, Judson

HA: Pul-

Am

Pathol

of cancinosarcoma.

J C/in

1966 AL,

Jones

AW,

:480-485, MP,

electron

microscopic

Fu YS,

Lodge

WR:

Organoid

47 : 227-247,

KV:

Pulmonary

blastoma.

J C/in

1968 Kay

5:

study.

Kern WH, Stiles QR: Pulmonary Surg 72:801-808, 1976 Pathol

Pulmonary

1 27 : 1 048-i

OE, Jennings

McCann

Waddell

JL:

Pulmonary

F:

63,

de Ia Pe#{241}aC, Gutiennez

Stank P: Other 12:225-237,

Whitwell

blastoma,

46:322-329,

1 1

1952

,

1976

Bauenmeisten

9.

OS: AJR

7.

39 : 1 53-i

1 3 : 93-94,

Allen

Greene R, McLoud TC, lung. Semin Roentgenol 197-204,

Cancer

Pecero

6.

‘

7 : 299-301

Thorax

Spencer H: Pulmonary blastomas. J Pathol 82 : 1 61 -1 65, 1961 Fung CH, Lo JW, Yonan TN, Milloy FJ, Hakami MH, Changus

2. 3. 4.

from

mesoderm and

REFERENCES

of tumor

differentiation. B, Epithelial differentiated epithelium of undifferentiated cells.

existence

embryonal-mesothelial

seems

Fig.

their

pleunipotential

form

an

A

that

base.

can be explained only by assuming that the peripheral part of the lung is derived from mesothelium. The peripheral location of pulmonary blastomas is consistent with Spencer’s explanation. In addition, Waddell [1 2] feels that peripheral bronchi and alveoli onigimay

and

lung

Pulmonary Cancer

: 789-797,

blastoma.

differentiation 1949

blastoma: 38

J Thorac of

the

a light

and

1976

fetal

Cardiovasc lung.

Arch