Paul Stark, MD #{149} Douglas Karen E. Chun, MD

Primary Sarcoma:

E

XTRAOSSEOUS

edge,

only

entity.

is

sarcoma

To our

kidney,

116 cases have

Primary

breast,

ported

and

(1-4).

teogenic

been

reported

of the

primary

cases

(5,7),

osteogenic

been

of primary

and

four

sarcoma

osteogenic

originated from the bly the left pulmonary

parenchyma,

os-

cases

of

from tery,

of the pleura

mediastinum artery),

the

(probalung

with right left hilan en-

ill-defined

and

several

lung

(Fig la). A lung

smaller

pulmonary

nodules

in

scan

of perfusion to defects on the

arteriograph-

pulmonary artery. On the well-penetrated arteriographic images the lung nod-

edly

calcified. (CT) scan

calcified

Index

terms:

A chest revealed

mediastinal

Radiology

I

From

Anderson

Lung

computed a mark-

mass in the me-

Mediastinum, neoplasms, 67.3255 neoplasms, 66.3255 #{149} Thorax, CT, 474.1211 #{149}Thorax, neoplasms, 474.375 #{149} Thorax, radiography, 474.11

Loma

No

specific

1990;

neoplasms,

University St.

Loma

radiograph

symptoms

of a 59-

revealed a lobe (Fig 2a).

related

to the

separate showed tion

mass

60.3255 #{149} Pleura,

left

#{149}

X

in the

The

lower

appeared

mass

lobe

bronchus

abnormality. 1 1-cm mock-hard left

ceral pleura. also present.

vealed

lower

lobe

a high-grade

only

the

the vis-

pleural fluid examination

osteogenic

from

was

at surgery.

to invade

Gelatinous Histologic

but

mass

lung

was me-

sarcoma

parenchyma.

A

postoperative bone scan revealed no othem skeletal or extraskeletal lesion. Case 3.-A 14-year-old boy presented with chest pain and dyspnea. He had undemgone radiation therapy for a left-sided Wilms tumor of the kidney 10 years previously. The lower left hemithorax had been included in the radiation field. The chest radiograph showed complete opaci-

fication

of the left hemithorax

geneously

calcified

mass.

by a homo-

Inversion

of the

left hemidiaphragm was also evident (Fig 3). A biopsy confirmed the diagnosis of osteogenic sarcoma, apparently originating

from

the pleural

space.

of Radiation CA

Center, 92354.

Sciences, 11234 Received

July 24, 1989; revision requested September 8; revision received October 12; accepted October 19. Address reprint requests to P.S. C RSNA, 1990

Extraosseous osteogenic sarcomas from soft tissues, without any evidence of skeletal involvement. They represent approximately 1% of all soft-

arise

tissue

sarcomas

and

ic sarcomas (1,2,6). tumors is unknown, sia of connective

4% of all osteogen-

The cause although tissue

and

of these metaplamalignant

somatic

tis ossificans, is a somewhat controvemsial predisposing risk factor (9). In contrast to primary osteogenic sarcoma of

buttock.

from the aorta. Bronchoscopy extrinsic compression and distor-

of the

of embryonal

remnants are believed by some authors to be responsible (1-4). Prior radiation therapy is a known risk factor and was probably causative in case 3 (2). Antecedent trauma, with subsequent myosi-

bone, which occurs most frequently in children and adolescents, the extraosseous variant is unusual before age 40 years. In two of our patients, the disease was discovered at ages 14 and 30 years; however, the majority of patients develop their tumors in the 5th or 6th decade of life (mean age, 53 years) (2-4). The most common sites of origin are the proximal lower extremity and the

aninto

DISCUSSION

Medical Linda,

The

left pulmonary embolization

chest

174:725-726

the Department

Linda

sarcoma.

originated

likely

speckles of calcification within (Fig 2b). A small accompanying pleural effusion was also detected. Because of the proximity of the mass to the descending thoracic aorta, an aontogram was obtained. It revealed the mass to be

found

in the

ic study demonstrated a smoothly contoured occlusion of the left pulmonary artery centrally and multiple large intraluminal filling defects in the right

ules appeared tomographic

osteogenic

year-old man with influenza large mass in the left lower

originating

A subsequent

extra-

mediastinum and ic). No primary

most

the wall of the with subsequent

no intraluminal A 6.5 X 7

nodular

lung,

absence multiple

degeneration

mass with the tumor

up to 3 cm in diameter

complete lung with

as well paren-

were present. A CT scan of the chest depicted a 7 X 8 X 10-cm left lower lobe

the right showed the left right.

with

tumor

Case 2.-The

which

REPORTS

multiple

in the (Fig

Osteogenic

the lung. three

sarcomas,

larged cardiac silhouette ventricular configuration,

left

#{149}

bone lesion could be demonstrated. A percutaneous needle aspiration biopsy of the largest left lung lesion yielded tissue primary

Case 1.-A 30-year-old woman was admitted to the hospital with an 8-month history of left chest and arm pain, fainting spells, progressively worsening dyspnea, and fatigue. A chest radiograph obtamed at admission showed a slightly en-

largement,

MD

left pulmonary artery, densely calcified lung

skeletal activity lung parenchyma

sar-

and the pleura. CASE

opacities

E. Watkins,

chymal lesions (Fig ib). A bone scan showed increased

consistent

osteogenic

(5,8-10) have also been described. We report our experience with

extraosseous

re-

mediastinum

of primary

lung

of the

has also

of the

(5,6), five cases coma

involvement heart

Three

sarcoma

#{149} Gregory

gion of the as multiple

knowl-

in the world literature. The majority of these lesions were found in the lower extremities, particularly in the soft tissues

of the thigh.

MD

Intrathoracic Eztraosseous Report of Three Cases’ osteogenic

an unusual

C. Smith,

Pain

is the

presenting

symp-

tom in one-third of cases; the median interval between onset of symptoms and diagnosis is 3-4 months. The typical extmaosseous osteogenic sarcoma is highly malignant and metastasizes early. Five-year survival rates of 13%-22% have been reported (5). There is a high rate of local recurrence after excision. Organs reported to have given origin to extraosseous osteogenic sarcomas indude the breast, kidney, thyroid gland, urinary bladder, and uterus (3,4). Five cases of osteogenic sarcoma of the heart have also been described (2). Including our three cases, four cases of mediastinal, six cases of lung pamenchymal, and five cases of pleural extmaosseous osteogenic sarcoma have been reported (5-10,12). The differential diagnosis of calcified mediastinal masses should include calcified lymph nodes from previous granulomatous disease, particularly tuberculosis and histoplasmosis. The latter is known to produce large calcified lymph nodes and to progress to mediastinal fibrosis. Calcification in pneumoconiosis, sancoidosis, and posttherapy lymphoma are other considerations. Calcified aneurysms, goiters, thymomas, and tematomas are further causes of mediastinal calcification. Calcification in pulmonary nodules on masses can be due to granuloma, hamartoma (5%), and bmonchogenic carcinoma (1% on plain nadiographs, 6% on CT scans). Metastatic disease from extrathoracic

drosarcoma

osteogenic

should

sarcoma

be included

or chon-

in the 725

.

a.

b.

Figure

1.

opacities

tion.

Primary

mediastinal

scattered

throughout

The left hilar

of contrast lung mass

osteogenic both

region

material. (arrow);

lungs,

is enlarged.

reveals

larger

metastatic on

(b) CT scan

Prominent mediastinal density approximates

ene diphosphonate

sarcoma,

markedly

the

left.

increased

The

of the chest

calcification that of osseous

cardiac

(a) Frontal silhouette

at the level

is seen structures.

activity

a.

c. to the lung.

chest is slightly

in the mediastinum

of the left scan obtained

and

reveals

enlarged

of the bifurcation

in the region (c) Bone

radiograph

bilateral

with

of the trachea, pulmonary artery after administration

ill-defined

a right

ventricular

obtained

without

and

nodular configura-

administration

in the peripheral of technetium-99m

subpleural methyl-

the left lung.

b.

Figure

2.

large

mass

Blunting

Lung

parenchymal

(arrowheads)

osteogenic

in the

of the left costophrenic

a large inhomogeneous tion (curved arrows)

and

sarcoma.

left lower lobe, sulcus is due

(a)

Frontal

chest

in close apposition to a small pleural

left lower lobe mass (open a small pleural effusion.

arrow)

with

radiograph

to the effusion.

mottled

reveals

descending (b) CT scan

islands

a

aorta. reveals

I

of calcifica-

Figure

3.

Frontal

differential diagnosis. Rarely, peniphenal bronchial carcinoid tumors can ossify. Idiopathic osseous metaplasia of the lung can lead to atypical feathery pattemns of pulmonary parenchymal calcification. Pleural calcifications have been observed after exposure to asbestos, after tubenculous on nonspecific bacterial empyema, and after traumatic hemothorax.

Pleural

an extrathoracic can ossify.

A classic

osteogenic

not

feature sarcomas mass.

up to 50% of these

definitely

calcified

on

is a

Radiology

#{149}

tissue

noteworthy

appearance, diagnosis that

but

in

the

left

masses plain

are

radio-

an-

5.

Ikeda

6.

7.

8. 9.

Wilson H. Extraskeletal ossifying tumors. Ann Surg 1941; 105:95-104. Allan CJ, Soule EH. Osteogenic sarcoma of the somatic soft tissues: a clinicopathological study of 26 cases and review of the literature. Cancer 1971; 27:1121-1133. Rao U, Cheng A, Didolkar MS. Extraosseous oxteogenic sarcoma: a clinicopathological study of eight cases and review of the literature. Cancer 1978; 41:1488-1496.

4.

Sardillo traosseous 51:727-734.

with by a with

inper-

PP. Hajdu SJ, Magill GB, Golbey RB. osteogenic sarcoma. Cancer 1983;

10.

11.

12. 13.

Ex-

1, Yoshimatsu

sarcoma

of the

H, et al.

mediastinum.

Primary Thorax

1974; 29:582-588. Tarr RW, Kerner T, McCook B, Page DL, Nance EP, Kaye JJ. Primary extraosseous osteogenic sarcoma

References

3.

T, Ishihara

osteogenic

Acknowledgments: We thank Dennis McDonald, MD, Donald Sauser, MD, and R. B. Keating, MD, for help with case 2 and Richard Saldana for excellent secretarial help.

2.

complete

It is

pulmonary

tery tumor in case 1 angiographically mimicked pulmonary artery agenesis on chronic endothelialized thromboembolic occlusion (13). #{149}

1.

sarcoma. reveals

most

is necessary.

of

the

niediastinum:

radiologic

correlation.

clinical,

South

pathologic.

Med

J 1988;

81:1317-1319. Nosanchuk

JS, Weatherbee L. Primary osteogenic sarcoma in lung. J Thorac Cardiovasc Surg 1969; 58:242-247. Pearson KD, Rubin D, Szemes CC, Preger L. Extraosseous osteogenic sarcoma of the chest. Br Dis

graphs (6), and CT may be necessary to detect calcification in such cases (eg, case 2). CT is also useful in determining the site of origin of the tumor, in depicting the full extent of the neoplasm, and in identifying any metastatic deposits. A pleural osteogenic sarcoma can simulate a malignant mesotheli-

726

cases

extnaosseous osmay have a highly

and

sarcoma

osteogenic or ossified

characteristic

osteogenic

radiograph

opacification of the left hemithorax version of the left hemidiaphragm densely calcified mass. (Reprinted, mission, from reference 1 1.)

from

plain-radiographic

of extraosseous densely calcified

However,

metastases

oma. When ossified, teogenic sarcomas

Pleural

chest

Chest

1969;

63:221-234.

Stauss HK. Osteogenic sarcoma arising in traumatic hemothorax and hematoma of the thoracic wall. Surgery 1951; 29:917-928. Cohn L, Hall AD. Extraosseous osteogenic sarcoma of the pleura. Ann Thorac Surg 1968; 5:545549. Stark P. Pleura. In: Taveras JM, Ferrucci JT Jr. eds. Radiology: diagnosis. imaging, intervention. Vol 1. Philadelphia. Lippincott. 1986. Reingold JM, Amromin GD. Extraosseous osteosarcoma of the lung. Cancer 1971; 28:491-498. Moser KM. Olson LK, Schlusselberg M, Daily P0. Dembitsky WP. Chronic thromboembolic occlusion in the adult can mimic pulmonary artery agenesis. Chest 1989; 95:503-508.

March

1990