General Joseph

Case

Casuio,

MD

of the

Day1

#{149} Andr#{233}Lisbona,

MD

#{149} MaxJ.

Palayew,

MD

of the chest (Fig 1) and a ventilation-perfusion study (Fig 2) were performed. Computed tomography (CT) with contrast material enhancemcnt (Fig 3) and pulmonary angiography (Fig 4) were subsequently performed. U FINDINGS The chest radiograph cardiac gos

u;..

I

Figure

1. Posteroanterior chest radiograph enlarged cardiac sithouette and diminished pulmonary vasculature in the left lung.

shows

U HISTORY A 75-year-old man presented with a 4-month history of progressive shortness of breath and substernal chest pain. At physical examination, the chest was clear. There was a loud pansystolic

murmur

systolic ejection right-sided heart

electrocardiogram

showed

strain.

Initially,

Index

Pulmonary

arteries,

terms:

RadloGraphic.s the

1992; Department

ber 7, 1991; revision partment ofRadiology,

March

at the

murmur failure

ventricular

C

RSNA,

1992

vein

and

with

blunting

an enlarged

dilatation of the

of the

azy-

costophrenic

an-

gles. There was diminished lature in the left lung (Fig The ventilation-perfusion stnated normal ventilation

pulmonary vascu1). scans demonwith almost no pen-

fusion

2).

to the

left

lung

(Fig

The

right

lung

was

‘;‘;

From

showed

silhouette,

apex

and

a softer

at the base. were present.

Signs The

evidence plain

neoplasms,

normal. Dynamic contrast-enhanced CT scans nevealed partial occlusion of the main pulmonary artery and proximal right pulmonary artery by a soft-tissue mass, which also cornpletcly filled the left pulmonary artery (Fig 3). There was a moderate right pleural effusion, and a small loculated pleural fluid collection was seen along the left lateral chest wall. The pulmonary angiogram demonstrated an irregular polypoid filling defect in the pulmonary trunk and right pulmonary artery. The left pulmonary artery was occluded at its ongin (Fig 4).

of

of right

radiography

564.32

Sarcoma,

#{149}

564.32

12:401-404 of Radiology,

McGill

University,

requested November 25 and Sir Mortimer B. Davis-Jewish

Montreal,

receivedJanuary General

Que,

Canada.

7, 1992; Hospital. 3755

From

the

1991

acceptedJanuary Cote St Catherine

RSNA

scientific

13. Address Rd. Montreal,

assembly. reprint Quc,

requests Canada

Received

Novem.

to M.J.P., Il3T 1E2.

Dc.

1992

Casulloeta!

U

RadioGraphics

U

401

VE N TI LATION

,

RPO

POST

R.LAT

‘V

i

ANT

L.LAT

LPO

a.

PE RFUSION

POST Figure perfusion normal

Scans from a ventilationstudy show ventilation is (a) but almost no perfusion to the left lung (b). ANT = anterior, L.LAT = left lateral, LPO = left postenor oblique, POST = posterior, R.LAT = right lateral, RPO = right

posterior

I

-

I

R.LAT

2.

S ANT

oblique.

1.1 AT

b.

Figure 3. Dynamic CT scans obtamed after bolus injection of contrast material show complete occlusion of the left pulmonary artery and partial occlusion of the main

and proximal

right

pulmonary

arter-

ies.

DIAGNOSIS: monary artery

Primary sarcoma (chondrosancoma).

U DISCUSSION Primary sarcoma of the pulmonary rare. The first reported case was Mandelstamm in 1923 (1); since

of the

pul-

artery is described by that time,

about

105

cases

have

including

undifferentiated

sarcoma, rhabdmyosarcoma, coma, and mesenchymoma.

402

U

RadioGraphics

U

Casullo

et a!

appeared

in the

litena-

tune (2-6). The tumor originates in the main pulmonary artery, where it is closely related to the intima. However, from histologic and immunohistochcmical studies, the tumor does not appear to arise from intima endothehal cells (7). Multiple histologic patterns exist, sarcoma,

leiomyo-

chondrosarThe diversity

Volume

of

12

Number

2

senting

symptoms,

the

most

frequent

clinical

diagnoses considered include pulmonary thromboembolism, primary lung carcinoma, and primary pulmonary arterial hypertension (2,4).

The

diagnosis

coma

may

be suggested

ings

from

ofpulmonary

artery

on the

a variety

of different

radiography

showed

basis

san-

of find-

imaging

modal-

ities.

Chest findings

Figure

4.

such

.,..

I

I--.-

Pulmonary

angiogram

in the pulmonary

artery,

with

trunk

occlusion

of the

and left

shows

a filling

right

pulmonary

pulmonary

extension

of tumor

have

also

been perfusion

those

caused However,

attachment are observed (2-4). ally spreads into both pulmonary their branches along the intima

sion

through the on pcricardium

media;

into

ventricular

the

to 25% ofcases (3). Involvement pid valve and the right atrium described (3,4). The metastatic tumor

distant

is low;

the

metastases

The

mean

52 years,

most

is the

common

wide.

Pulmonary

slightly

more

the

age

artery common

sarcoma

is only with

of

is

evidence quently

of right-sided a loud precordial

The

prognosis

vival time symptoms. cal excision tion

therapy

examination

usually

1 year

Treatment with

with after

consists

or without

a mean the

of local

on chemotherapy

sun-

onset

adjuvant

Given the rarity of pulmonary coma and the nonspecific nature

1992

reveals

after

of

sungiradia-

less

perfu-

and

involves

lung

than

a

(as was is an perfu-

1% ofcases

of

demonstrate

absent

with

perfusion

normal

lung

entities,

most

the

common

however,

this may

The

cific

sign

The

plaquelike

intima

of large

the pulmonary (11). Direct

arteries

as large

pulmonary

in seven

trast-enhanced

minal

extent

mon

is evident

filling cases

was

performed

of

pruning artery defects

ventricle

ofeight CT

CT demonstrates tumor and enables

distal

right

the

tapering

of pulmonary

and

spe(10).

along

smooth

polypoid

arteries

possible

cinean-

sarcoma

with

visualization

sarcoma

le-

in the

of tumor

produce

ap-

polypoid

to be a more

artery

growth also

specific,

during

shown

or

identical

motion

arteries

been

ofpulmonary may

is not an

a to-and-fro

has

appear-

finding produce

pulmonary

giography

cause

(9).

sarcoma is one in the pulmonary

presence

showing

frequent

angiographic artery defects

thrombus

peanance.

most

carcinoma

of pulmonary large filling

proximal a ratio

heart failure and fresystolic murmur.

is very poor,

of about

bronchogenic

sions

.

Physical

other

being

arteries;

of 1 .3: 1 Clinically, the symptoms and physical findings associated with this tumor are related to its extensive intravascular growth, which produces concentric luminal narrowing of the pulmonary arteries. The most common symptoms are dyspnea, chest pain, and cough. The development of these symptoms is often insidious.

in fact,

many

The

is quite

in women,

.

embolism opposite

since

range

san-

the

portion of the lung Pulmonary embolism of a large, unilateral

cause

to the

ance more

lung.

at presentation

disartery

when

is unilateral

to one

show

thromboembolic

considered

case)

defect;

may

(9). It should be noted, however, that a large unilateral perfusion defect is not common and may be caused by

in up

site

in our

to

indistinguishable

pulmonary

contiguous

perfusion

ofthe tnicushas also been potential of

age of patients

although

occur

abnormality

pulmonary

of the

may

sion

by

be

uncommon

the mediastinum Retrograde exten-

tract

should

seen

invasion

endocardium

outflow

coma

large,

Tumor usuarteries and and superfi-

tumor

adventitia into is unusual.

of tumor

this

of

(8).

defects

(5,6).

right

and

arteries

studies

ease

sion

enlarge-

peripheral

observed

from

of the

into

Ventilation-perfusion

artery.

cellular differentiation suggests the existence of a plunipotential progenitor cell (7). Pulmonary artery sarcomas appear as intraluminal polypoid masses fixed at the base of the pulmonary trunk or in the pulmonary valve region where typically multiple sites of

layers

hilar

.

defect

cial

nonspecific

and

ment, areas of oligemia, pleural effusion, pulmonary metastases in 95% of 93 cases pulmonary artery sarcoma (5) Branching, tubular areas of opacity that corresponded

multiple

March

as cardiac

in the

was

in which

con-

(5,8,12,13).

the multicentnic origin of the evaluation of the extralu-

ofdisease.

However,

elsewhere,

the

unless

tu-

CT appearance

surgery.

artery sanof the pre-

Casullo

et

a!

U

RadioGraphics

U

403

of pulmonary nary arteries to thnombus. also

been

artery sarcoma in the pulmomay mimic that ofocclusion due Magnetic resonance imaging has used

to image

pulmonary

artery

sar-

coma (5,8). not specific nary artery

Although the signal intensity is for tumor, the diagnosis of pulmosarcoma may be suggested on the basis oftumon extent and pattern of growth. Identification of tumor masses in the pulmonary trunk and main pulmonary arteries with the use of two-dimensional cchocardiography has been reported in two cases (1 2, 14). This modality more commonly may demonstrate only night ventricular dilatation and hypokinesis. At surgery, our patient was found to have a large lobulated mass that obstructed the right ventricular outflow tract, was attached to the right

ventricular

septum

on

adherent to the pulmonary large mass, found in the itself,

was

virtually

a pedicle,

valve. pulmonary

occluding

and

was

Another artery

the

main

(Fig

5).

Despite

excision

mately

95%

of the

shortly

after

surgery.

An

nature

of the

formed. sion

The and

tumor,

loculated

metastatic

U

Kruger I, Borowski A, Horst M, Dc Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Sung

6.

Britton coma:

1990; 38:91-95.

of approxi-

the

patient

autopsy

pleural

7.

died

was

not

right

pleural

fluid

collection

per-

effuon

the left remains unknown as there had been no attempt at thonacentesis. The effusions, particularly that on the night, may have occurred as a result of right-sided heart failure. Although there was no radiographic evidence of lung or pleural nodules to suggest metastases, the effusions may have been caused by

5.

pulmo-

nary arteries. On the left side, the mass cxtended into the loban branches and subbranches. Histologic analysis of the mass showed a markedly pleomorphic population of spindle-shaped and fusiform cells with prominent regions of chondroid differentiation

Figure 5. Photomicrograph (original magnification, X 350; hematoxylin-eosin stain) shows the wall of the pulmonary artery (left) involved by spindie-shaped neoplastic cells (middle) and regions of chondroid differentiation (right).

8.

9.

disease.

Mandelstamrn

10.

M.

gen des heriens. 2.

primare

Virchows

Arch

Pathol

Bleisch

Anat 11.

VR, Kraus

FT.

trunk.

Polypoid

Cancer

sarcoma

1980;

Baker sarcomas.

PB, Goodwin RA. Pulmonary Arch Pathol Lab Med 1985;

39.

404

U

RadioGrapbics

of

Casullo

et a!

in pulmonary

artery

of four MR

sarcoma.

diagnosis artery.

ofprimary Circulation

sarcoma 1982;

of 66:

13.

Use of CT in the evaluation of primary cardiac tumors. Cardiovasc Intervent Radiol 1986; 9:132-135. Fitzgerald PM. Primary sarcoma ofthe pul-

46:3 14artery 109:35-

ultrastruc-

J Comput Assist Tomogr 1989; 13:906-909. Cho SR, TisnadoJ, Cockrell CH, Beachley MC, Fratkin MJ, Henry DA. Angiographic evaluation of patients with unilateral massive perfusion defects in the lung scan. RadioGraphics 1987; 7:729-745. HynesJK, Smith HC, Holmes DR, Edwards WD, Evans TC, Orszulak TA. Preoperative

14.

monary

trunk:

Tomogr

1983; 7:52 1-523.

Wright

CT findings.

EC, Wellons

pulmonary vasively maphy.

U

a clinicopathologic,

immunohistochemical study Mod Pathol 1989; 2:486-494. SW, Lesar MS, Travis WD, et al.

12.

324.

4.

and

Schermoly M, Overmann J, Pingleton 5K. Pulmonary artery sarcoma: unusual pulmonary angiographic findings-a case report. Angiology 1987; 38:617-621. ChaloupkaJC, Fishman EK, Siegelman 55.

63:263-272.

the pulmonary

sarcoma:

tural, cases. Smith

angiographic the pulmonary 672-674.

neubildun-

1923; 245:43-54. Shmookler BM, Marsh HB, Roberts WC. Primary sarcoma ofthe pulmonary trunk and/or right and left main pulmonary artery: a rare cause of obstruction to right ventricular outflow-report on two patients and analysis of 35 previously described patients. Am J Med

1977; 3.

Ueber

trunk

and CT findings

REFERENCES 1.

PD. Primary pulmonary artery sara report of two cases, with special emphasis on the diagnostic problems. Clin Ra. diol 1990; 4 1:92-94. McGlennen RC, ManivelJC, Stanley SJ, Slater DL, Wick MR, Dehner LP. Pulmonary artery

artery

J Comput

HA, Martin

sarcoma

by two-dimensional Circulation 1983;

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Volume

12

Number

2