Epithelioid Sarcoma: Radiologic and Pathologic ...

Epithelioid

Sarcoma:

Radiologic

HING-HAR

LO,’

LESTER

and

KALISHER,’

The radiologic and pathologic manifestations of epithelioid sarcoma are presented based on an analysis of five cases. and the literature is reviewed. This rare entity tends to orginate in the extremities and metastasizes primarily via the lymphatics. Although the lesion grows slowly. it recurs with high frequency. Males are affected three times as often as females. The differential diagnosis includes both malignant and benign entities. The natural history and methods of treatment are reported. In 1970, nant

Enzinger

sarcoma

tendons

and

have

been

sembles

[1]

first

which fascial reported

aspects

rare cases. and

review pathologic

in

[2-11].

Histologically,

not

25

the

and

aspects

We

describe

report

re-

section

and

the

new

radiographic

and

Methods

Since 1974. five cases of histologically proved epithelioid sarcoma were seen at Massachusetts General Hospital. All pertinent radiographic studies, clinical records, and pathologic specimens were reviewed. All patients underwent routine chest radiography, plain and/or xerographic radiography of the lesions, and chest tomography. In addition, lymphangiography was performed in three patients, venography in one, bone scans in three, and a 67Ga scan in one. Routine stains were performed on all specimens. Three were stained for glycogen and mucin, and one was studied by electron microscopy. Case Case

Reports

epithelioid

1

A 55-year-old woman first noted a firm tender nodule on the dorsum of the right hand over the second metacarpal. About 7 months later it was excised at a local hospital. The slides were reviewed at our hospital, and a diagnosis of epithelioid sarcoma was made. Xerography of the right arm. chest radiography, tomography, lymphangiography, and a 67Ga scan were all negative. The patient received radiation therapy (7,000 rad over 46 days). Three months after diagnosis. four distinct subcutaneous nodules had developed in the midportion of the volar aspect of the forearm. All were excised and proved to be metastatic. Six months after diagnosis, forearm amputation and right axillary node dissection were performed. Residual tumor was noted at

Received 1

December

Department

to L. Kalisher. 2 Department Am

J Roentgenol

13. 1976;

accepted

of Radiology.

Massachusetts

of Pathology.

Massachusetts

128:1017-1020.

June

showed

after

revision

March

General

Hospital

General

Hospital

1977

hyperplasia

in all 53

nodes.

The

thumb

base of the first metacarpal. Eight months subcutaneous nodules were noted above were excised; all showed recurrence. The to follow-up.

sarcoma.”

right

inguinal

lesion

was

radiation

Lymph

area, too

was to

node

thought

extensive

therapy

intravenous

for

.

be

rad

over

obtained

from

malignant

amputation

6. 1 20

adriamycin

biopsy.

to

sarcoma.

and 49

Aetroperitoneal

was

days.

the The

treated

by

followed

by

extension

which

developed about 3 months after completion of radiation therapy. included obstruction of the inferior vena cava and concomitant right hydronephosis. The patient is presently alive with disease. Case

4

A 25-year-old woman had thenar eminence. She was month before admission, two in the area of pain. Biopsy of lioid

1. 1977.

and Harvard and

reactive

Case 3 A 54-year-old female had pain and a lump in the medial aspect of the right thigh. Radiography of the thigh showed fusiform enlargement and periosteal reaction in the midshaft of the femur. A biopsy performed at an outside hospital 6 months later, was reported to show normal bone and “proliferative myositis. “ Review of slides showed them to be consistent with epithelioid sarcoma. Radiography of the right femur 6 months after the biopsy. showed fusiform swelling of the midshaft with defect at the biopsy site (fig. 2); there was also mottling and local periosteal reaction extending distally to the condyles. Extensive radiologic workup included chest x-ray and tomography. intravenous pyelography, barium enema, and upper gastrointestinal and small bowel series, all negative. Bone scan and venography were positive. Open biopsy of the right femur was reported as “malignant lesion-undifferentiated carcinoma versus

features. Materials

site in the amputation specimen; seven of 13 nodes for metastasis. The patient received 4,400 rad at the nodal resection area. She is presently free

was amputated at the after diagnosis, three the operative site and patient has been lost

of this five

D. FAIX2

A 1 5-year-old male had a small hard nontender skin elevation of the right hand for 6-7 years. There was no apparent growth. but delayed healing after multipe episodes of surface abrasions was noted. Excisional biopsy was diagnosed as epithelioid sarcoma. Chest radiography and lung tomography were negative. Lymphangiography (fig. 1 ) demonstrated two enlarged nodes. one of which showed destruction of the marginal sinus, thought to be consistent with metastatic disease. Right axillary node dis-

of

lesion

primary

Manifestations

Case 2

cases

melanoma,

radiologic

described.

the literature,

vicinity

time,

JAMES

were positive over 40 days of disease.

of a malig-

the

AND

the

to its proximity to tendons, soft tissue synovial lesions,

The

been

that

malignant

Due other

sarcoma.

have

cases

anise

Since

of granuloma,

synovial

tumor

62

to

structures.

squamous cell carcinoma. it may be confused with especially

reported

seemed

Pathologic

Harvard

1017

Medical Medical

sarcoma.

School. School.

Chest

Boston. Boston.

a 7 month history of pain in the left initially treated for synovitis. One small lumps developed with edema the lesion was diagnosed as epithe-

radiography

Massachusetts Massachusetts

and

tomography

021 14. Address 021 14

and

reprint

bone

scan

requests

1018

LO El

AL.

:1

S;.

“,

Fig.

1.-Case

2. Lymphangiogram

lymph

nodes,

one

were

negative.

wrist

demonstrated

with

of right

destruction

Plain

films a soft

of

and

arm

marginal

xerography

tissue

mass

showing

two

enlarged

sinus.

of the and

left hand

destruction

and

of

the

greater multangular (fig. 3). Partial amputation of the left hand was performed with excision of thumb, index, and middle fingers. Pathologic study reported complete excision of the tumor. Five months after diagnosis. midforearm amputation was performed due to recurrence of tumor on the ulnar aspect of the hand. Axillary node dissection 2 months later showed all nodes free of tumor; 7 months previously, left arm lymphangiography (fig. 4) had showed enlarged and irregular lymph nodes in the distal upper arm suggesting extension of disease. The patient is presently alive with disease. Case 5 A 13-year-old boy had a 7 week history of a painless lump in the palm of his right hand. Excisional biopsy at a local hospital showed epithelioid sarcoma (fig. 5). Radiography of the chest and right hand, bone and liver scan, chest tomography. and xerography of the right hand were all negative. Midforearm amputation was performed and axillary node dissection showed one positive node. The patient is being treated with chemotherapy (vincristine. prednisone, adriamycin, cyclophosphamide).

film and xerographic studies of the lesion, is usually seen in the soft tissues adjacent

a bony structure. bone, producing bone

destruction

bone

is produced

periosteum

(fig.

(radiognaphyand disease. In our evaluating

The soft a scalloped (fig.

3).

as the 2).

Occasionally,

and

all chest were

the by

peniosteal

invades

Interestingly,

tomography)

a to

tissue mass may invade cortical margin followed

tumor

negative

new

elevates

the

studies

to

date

for

metastatic

lymphangiography of this tumor.

Two

is unreliable patients (cases

in 2

xerogram

of right

of muscle

thigh

showing

subcutaneous

planes anteriorly.

and exuberant perioat middle to proximal femur. Calcifications from periosteal soft tissue muscle planes. Note biopsy defect in cortex

anteriorly.

and 4) were yet pathologic

considered studies

(figs. 1 and compression vanicosities tive had

to have showed

positive lymphangiognams. only reactive hyperplasia

4). Venognaphy (case 3) showed of the iliac vessels with retrograde of the

pelvis.

Bone

until peniosteal reaction a 67Ga scan which was

Pathologic

extrinsic filling of

scans

tend

to remain

occurs. negative.

One

patient

nega(case

1)

Findings features in all five cases were similar by Enzinger [1]. Four of the lesions

in the hand, and excision. Gross

amputation

specimens

all of these examination revealed

to oc-

patients had had preof the subsequent recurrent

tumor

in

the

area of excision which was firm, nodular, and rubbery with a glistening grayish tan appearance. Tumor was associated with

tendon

or with

tissue

metastases

mens.

In case

lower extremity, Microscopically, of

experience, the extent

3. Lateral

steal reaction surface into

curned vious

Findings

On plain mass density

2.-Case

The pathologic those described

Results Radiologic

Fig.

edema and thickening

plump

3 the

tendon

sheath

were

present

lesion

occurred

and only biopsy the characteristic

spindle-shaped

gonal cells containing (fig. 5). In the case with

cells increased involvement

in only in

all in the

was

two soft

done. findings

merging

cases.

amputation tissue were

with

eosinophilic in the lower

Soft speciof the

nodules

more

poly-

cytoplasm extremity

EPITHELIOID

Fig. 3.-Case 4. A, Radiograph of left hand showing of left hand showing soft tissue mass in thenar eminence multangular.

(case 3), these the lesion, and sidered.

more epithelioid-appearing a metastatic carcinoma

This

lesion,

as well

as one

lesions, was reviewed by F. M. with the diagnosis of epithelioid munication). cases, In

Necrosis

special case

stains

5,

polyhedral

nuclei.

nibosomes,

prominent

shaped

Cytoplasm

and

rough

seen, These

but occasional tight findings are consistent

Lymph

node

dissections

neoplastic

were

typical

In three negative.

rich

revealed

cells

with

in pinocytic

performed

were reports.

noted.

in all five

cases.

Metastatic

tumor,

described, the lymph

was found in three (cases 1 , 3, and 5). Although nodes in cases 2 and 4 were moderately en-

larged,

microscopic

hyperplasia

and

examination no evidence

to that

revealed

at base of thumb with destruction of greater multangular. B, Xerogram surface. Note invasion of subcutaneous tissue and destruction of greater

21 -64 series

previously

only

of tumor.

years

age 32).

distribution In Enzinger’s

(median,

23),

and

in

our series

particularly

head

and

neck

vulva

[6,

7]

in that

of

Santiago

et

years 4-58 al.

[5],

25 were the tumor on the

females occurred

volar

and 76 males. in the upper

surface,

and

in one,

series [1], 38 were in the lower extremities, and two

region.

and

Age ranges in other reported [2-4, 6-11]. In the cases

Unusual

two

in the

lesions

buttock

in the [10,

penis

11]

in

upper in the

have

and been

reported. Recurrence after

diagnosis

after

initial

rates

were

high,

and

initial

therapy,

surgery

for

usually

within

3-7

compared

Enzinger’s

series

months

to 6 months [1

1. None

of our

patients had lung metastases, even when extensive netropenitoneal involvement was present. In Enzinger’s series [1]. 16% manifested pulmonary and pleural metastases, all had repeated recurrences. The differential diagnosis must

and

benign

sheath

entities.

xanthomas, and bone

sarcoma, series was 1 3-55 [1 1. the range was

since 1970. of our patients In Enzinger’s 22 in the

without The (mean,

39). years

the thigh. extremities,

synovitis.

Discussion

(mean. 8-66

extremities,

but

reactive

years were

documented In four

Varying

to the nucleus. formation was

cell junctions with previous

in appearance

large

vesicles,

reticulum.

aligned parallel membrane

were

similar

more

who concurred (personal com-

examination

was

to skin

cells dominated seriously con-

the

and mucin

endoplasmic

quantities of microfilaments, were present. No basement

extends

in all cases.

microscopic

spindle

of

Enzinger sarcoma

for glycogen

electron

and

indented

was

was

soft tissue prominence which

1019

SARCOMA

the

foreign fibromas

will

invasion.

Malignant

origin

both

synovial

present

tissue include

carcinoma,

Kaposi’s

tendon

nodular

as soft lesions

malignant

cysts,

granuloma.

metastatic

angiosarcoma,

rhabdomyosarcoma

include

latter, body

fibrosarcoma,

melanoma, The

Of

tenomasses synovial

malignant

sarcoma.

.

of this

tumor

is unknown.

Ultrastructurally,

and

Fig. plump

5. -Case spindle

5. Typical microscopic cells irregularly merging

field with

from original biopsy showing polygonal cells (X385).

ACKNOWLEDGMENT We thank

Selma

Surman

manuscript,

and

Janice

also

Dr.

John

thank

clinical

information

for

and

Raker on

assistance

Ladonna

for

these

preparation

in

Cecile

Fanti

cooperation

for in

of the

typing.

We

providing

the

patients.

REFERENCES 1. Fig. showing upper

4.-Case enlarged

4.

Lymphangiogram

and

irregular

of

left

Iymphnodes

in

Enzinger

FM:

granuloma

arm

2. Soule

distal

EH,

nant

arm.

it relates

to

synovial

sarcoma

and

fibrohistiocytic

[21 suggest

Enriquez atypical

fibrous

cytoma,

malignant

The

a common

histiocytoma,

presenting

symptom

extremities.

The

examination. tumor, only

While 67Ga one of our

final

areas tumor

of involvement is most likely

focus

from

to rule

At present, surgical

the surgical

scalp

is quite

is a poorly diagnosis showed cases

tumor. false positive radiography

out

pulmonary

optimal

treatment

excision.

near

and is

common.

followed by interval examination of the

sarcoma.

defined made

mass by

in the

histologic

Enriquez

may

results in reactive and chest tomography and seems

pleural

metastases.

recurrence that

suggest

that

chest radiographs surgical site and

and scalp.

be

is

local

common

metastasis all

to the

patients

careful

physical

be

J

H, Feinerman and pathologic SW.

masquerading

1975 7. Piver

hyperare

to be vigorous

reports.

Bone

malig-

histiocytoma, study

sarcoma

Joint

and

65

of

tumors.

in X0/XX

1972 SMK: Epithelioid Surg

a

histiocytoma,

comparative

Pathol94:214-216, RB, Yvars MF, Chung

simulating

1970

fibrous

Arch

case

1972 5. Santiago A clinical 6. Moore

A

sarcoma

26:1O291O41,

malignant

30:128-143, 1972 JL, Lain KC: Epithelioid

syndrome. 4. Heppenstall Two

P: Atypical

sarcoma.

A

Cancer

histiocytoma,

3:133-147,

bone scan, the primary sarcoma but metastatic

It is of interest We

epithelioid

for

Cancer

3. Males

histio-

Lymphangiography

Unfortunately, site.

origin fibrous

no definite affinity to this had this study. If multiple

are seen on not epithelioid

another

unreliable due to plasia. Plain chest essential

histiocystic malignant

histiocytoma,

san-

but Fisher Soule and

sarcoma.

a carcinoma.

fibrous

eptithelioid

comas. Gabbiani et al. [9] favor synovial origin. and Horvat [10] favor fibroblastic origin.

Epithelioid

or

[Am]

Turner’s

sarcoma.

54:802-808,

LK, Lattes A: Epithelioid sarcoma. study of nine cases. Hum Pathol

1972

Wheeler JE, as Peyronie’s

Hefter LG: Epithelioid sarcoma disease. Cancer 3:1706-1710,

Y, Barlow J: Epithelioid sarcoma of the 40:839 -842, 1972 8. Nelson FR, Crawford BE: Epithelioid sarcoma: a case report. J Bone Joint Surg [Am] 54:798-801, 1972 9. Gabbiani G. Fu YS, Kaye GI, Lattes A, Majno G: Epithelioid sarcoma. A light and electron microscopic study suggesting a synovial origin. Cancer 30:486-499, 1972 10.

MS. Tsukada

vulva.

Obstet

Fisher

ER,

Gynecol

Horvat

B:

The

fibrocytic

called epithelioid sarcoma. Cancer 1 1 . Frable WJ, Kay S. Lawrence W, sarcoma. An electron microscopic 8-12.

1973

derivation

of

the

so-

30:1074-1081, 1972 Schatzki PF: Epithelioid study. Arch Pathol 95: