radiation therapy of desmoid tumors - AJR

JANUARY,

RADIATION By

DENNIS

R.

THERAPY

HILL,

M.D.,

OF

HARRY

NEWMAN, SAN

T

HE

desmoid

tumor

benign neoplasm musculoaponeurotic invasive lymph

but does nodes or via

tumor first

is

not

in its

and

by

and

Raffaele9

This was

Nichols

in

the

similarity befibroma and tumor. Recently,

I923.

classified

desmoid

PATHOLOGICAL

tu-

structures.

commonly

seen

in young

adults.

PATHOGENESIS

Various theories of the pathogenesis of desmoid tumors have been advanced. Booher and Pack2 have suggested that they are related to hormonal factors because of the frequent association of these tumors with pregnancy. They also postulated that a traumatic hematoma may form a nidus for

the

S From California. Supported

t

development the

Supported

Section in part by

of

of Radiation

a desmoid Oncology,

by Nationallnstitutes Fellowship

IF 03

ofHealthTraining

CA

tumor.

Department

52938

from

of Radiology, Grant

the National

M.D.

FEATURES

No

capsule

or

definite

line

of

cleavage exists between the tumor and adjacent muscle. The tumor may be attached to bone and cause erosion by pressure. Although the lesions have been found to surround vessels and nerves, to our knowledge invasion of these structures has not been reported-and no lymphatic or hematogenous metastases occur. The histologic appearance consists of uniform, elongated, slender cells surrounded and separated from one another by abundant collagen. The cells do not have atypical, hyperchromatic, or bizarre nuclei. The nuclei are small, pale-staining, and sharply defined. The nucleoli are not prominent. Occasionally, mitotic figures may be present, but they are typical in configuration. The cells and collagen fibers are arranged in swirling or interlacing bundles or fascicles, which often merge with one another and form a dense, solid tumor mass. The fibrous tissue extends into and between muscle bundles for considerable distances, often isolating islands of muscle fibers.

FEATURES

Most patients complain of a firm mass, usually fixed, which grows slowly and attains a large size before treatment is sought. Pain, if present, occurs as a late symptom. In 8 of a reported series of more than 6o cases of extra-abdominal fibromatosis, the shoulder girdle was the site of involvement.9 In this location the brachial plexus may be affected, resulting in signs of motor weakness, numbness, or hypoesthesia. The tumor occurs in both sexes and may occur at any age, although it is most

L. PHILLIPS,

The gross tumor is dense and hard. If the surface is cut the tumor bulges out and is white or pinkish in color. The interlacing bundles of white fibrous tissue are usually seen without difficulty and there is a remarkable tendency to invade surrounding

mors under the general pathological heading of fibromatoses. This group includes fibromas, keloids, Peyronie’s disease, plantar and palmar fibromatosis, fibromatosis colli, progressive myositis fibrosa, and desmoid tumors. CLINICAL

THEODORE

and

The microscopic studies of desmoid tumors, however, have revealed no hemosiderin content. It was further postulated that this tumor could represent an autoimmune disease and that the aggregates of lymphoid tissues, sometimes noted at the margins of the tumor, could be involved in the autoimmune process.

to

stream. location

TUMORS*

CALIFORNIA

uncommon,

metastasize

described

He emphasized tween the extra-abdominal the abdominal desmoid

M.D.,t

FRANCISCO,

arises from It is locally

the blood extra-abdominal

defined

Stout

an

which structures.

DESMOID

I973

University

in Radiation

Institute

84

of

Therapy

of Cancer,

California CA National

School

of

Medicine,

#{231}v. Institutes

of Health.

San

Francisco,

\O1..

117,

IIG.

I.

No.

Radiation

1

Case

I. (A) therapy.

Distinction apparent

Biopsy

incision

(B)

REPORT

radiation

OF

tumors

or consulting

surgeon.

A 69 year

old woman

I.

the right nation

it

of a slowly

had

attained

from

the

to the shoulder and posteriorly I, A and B). because

radial

pulse

and

there

was

some

medial

right neck of tumor

and shoulder, through the

performed before biopsy incision.

is readily of cases.

7:

.

radiation

‘:

definitive extensive

from

by

the

Samoa

enlarging

of the

right

anteriorly the trapezius

had

mass

ear

laterally

to the clavicle, muscle (Fig.

extent

arm

sensation

weakness

on

right

tumor.

were

normal,

in

studies the

of the

surface

of the

the

right

The but

arm.

demonstrated side

of

devoid of calcification (Fig. 2). gram of the right shoulder showed the

in

on At the time of examia huge size. The mass

of the

Roen tgenographic mass

herniation

Tumors

It was not tender but rock-hard. could scarcely move her cervical

patient

spine

huge

lobe

joint, into

mass

showing

unresectable

side of her neck.

extended

The

deemed

history

I year

of Desmoid

CASES

referring CASE

tissue

view

treated by means of therapy 4 patients with

desmoid

a

ofsoft

Profile

from a fibrosarcoma in the great majority

have

\Ve

Therapy

scapula

the

neck

1

be

A roentgenoan erosion on due

L

the to

to pres-

FIG.

2. Case

trachea

and

I.

The extends

large

mass displaces below the clavicle.

the

86

1).

R.

Hill,

H.

Newman

and

T.

L.

Phillips

tamed tumor

within

vessels

The

tumor

the

the

large

herniated

through

revealed radiation

benign therapy

Biopsy Definitive

with

use

of a cobalt

6o

mass

the

(Fig.

there

tensive

mass

neck

pain

Case

.

of the

i.

Erosion

scaptiIs

defect

caused

(arrows)

b

pressure

on

of

medial the

teletherapy

after

free

ofdisease CAsE

had

slow

appara-

to the

increased

At therapy, (Fig.

A

II.

old

resection

was

desmoid

tumor

with

One

year

the

tumor

the

and

fixed

of motion

a large

thin

medial

tumor

that

the

the

upper

medial

portion

thoracic

deemed (Fig. 6).

man

the

unresectable.

The patient

A

subsequently

the

left anmuscles diagnosis margins. 8 cm.

aspect

had

of the

progressively by the Tumor It was found

of the

spine;

2

It was

recommendation surgical exploration.

was

of

at

surgical

The

enlarged. Board

ex-

of the

mass

recurred.

at the

The

incision.

the

amount

The left clavicle, and both pectoral en bloc. The pathologic

diameter

surgical

follow-up of

Greek-American of

girdle. 3 ribs,

later

mid-

fractions

follow-up examination, the patient was clinically c, A and B).

left shoulder tenor upper were removed

in

in

range

year

32

a radical

32

resolution

decrease

spine.

years

bortumor.

seen

with

and

cervical

lic.

was

mci-

fibromatosis. was undertaken

the patient received #{231},500 rads plane of the mass, accomplished in over a 42 day period. On subsequent visits,

4).

biopsy

tus;

der

.

(Fig. 3). An axillary arteriogram was ohwhich revealed abnormal nonmalignant

sure

sion.

J;xuAR\

lesion mass

adhered was

biopsy

to

therefore

was

underwent

taken

therapy

at the Section of Radiation Oncology. He had a hard lobulated axillary mass, measuring approximately i#{231}cm.X7.5 cm., which extended from the lateral border of the left scapula, across the axilla to the anterior axillary line. In addition there was a diffuse, hard, supraclavicular

mass,

measuring

cm. The involvement pulse

was

absent.

definitive

therapy

The

radiation of

and

tumor,

was on

receiving

the

cm. dry,

X 5

indicating

nerves;

patient

therapy

apparatus,

mid-plane

approximately

arm was cold of sympathetic

left

the

radial

treated

a cobalt

with 6o

6,100 rads accomplished

tele-

to the in 3

fractions over a period of 53 days. The following 2 year period has revealed slow resolution of the

large

mass

radial

pulse

cal

FIG.

4.

Case

Abnormal seen within I.

nonmalignant the tumor.

vessels

and

decrease

is now

evidence

present

in shoulder and

pain.

The

is no clini-

of disease.

CASE III. Rectal and vaginal a 27 year old woman complaining revealed

there

a

palpable

mass.

examination of aching

A laparotomy

of pain was

Vo,.

No.

117,

5. Case

l’1G.

undertaken tumor

Radiation

I

in was

tion

fixed way ficing

mass

that the

NIarked

the

resolution

of

a large presacral fibrous the sacral hollow and pathological diagnosis was tumor. The patient did well when examination showed a from

on her left buttock. Further a 12 cm.Xio cm. desmoid

revealed

to

B)

of

which

removed

left sacral plexus.The that of a desmoid until 24 years later

large

(A and

I.

Therapy

surrounding

it could

not

sciatic

nerve.

structures be

excised

The

only

exploratumor, in such

without

sacri-

surgical

possi-

a

Desmoid

the

Tumors

mass

bilitv

with

a small

would

the

surgeon

may

not

deep

pelvic

quently treated tor, dose

87

persistent

have

been

postulated be

central

a hemipelvectomv that

even

successful

because

extension.

The

referred definitively receiving of 5,100

to

ulcer.

and

this of

procedure

the

patient

previous was

for radiation therapy on a mev. linear the

mid-plane

of

subse-

and was accelera-

the

tumor

rads in 32 fractions over a 48 day period (Fig. 7). On subsequent follow-up visits there was gradual flattening of the left buttock, and the buttocks and thighs became almost symmetric (Fig. At the present time, 2 years .

*jj ..; . ,‘

.

.

.

lIG.

,

6. Case II. Photomicrograph of biopsy specimen showing composition of tumor to be dense, relati vely avascular, hypocellular bands of connective tissue (H and E stain; X200). Adjacent skeletal muscle is seen to be surrounded in part by some of

the fascicles

ofconnective

tissue.

11G. tion

tour

7. Case of

iii.

radiation

and

radiation

Mass

on

left

treatment.

pigmentation.

buttock Note

near distortion

compleof con-

a

D. R. Hill,

88

H. Newman

and

T. L. Phillips

3973

JANUARY,

#{149}

--

#{149},-ci:-

-

,#{149}

I

&.

.-

.-

. .(

.

#{149}

.

.

‘(

,-

:

#{149}

S ,‘

8. Case left buttock

l”IG.

Two shows

111.

years nearly

after treatment. normal contour.

The

FIG.

Case

10.

tion

of

therapy, the of recurrent

patient disease.

gives

no clinical

bundles

man

had

nuclei

evi-

are

very

progressive CASE history

IV.

A 22

of a slowly portion of

old

year

enlarging his neck,

a

on the lower was painful

mass which

20

right (l’ig. 9). Biopsy was performed revealing fibromatosis (Fig. io). Surgical exploration was subsequently carried out which exposed a large, firm, pale, tumor mass that occupied the base of the neck on the right and extended under the right clavicle to the midline and the trapezius posteriorly. The mass appeared to fuse with the prevertebral roots injury not tion.

on

and

merge

laterally

with

dose day

linear

rads

of

period.

accelerator

Follow-up

in

with

30

of

the

over

have

shown

(H and

E stain;

flattened

and

of

of

the

its

in

The

X200).

pyknotic.

extensive

visit, 12 months the tumor had original size (Fig.

the

harmless

their recur,

potential and to

tumor.

after regressed

cornto

I I).

a

between

manner

suggestive

recurrence

rate

high,

ported

by Enzinger

-

I

their

appar-

appearance

of

and

and

surgical

(7

30

to

fibrosarcoma.

following of

17

cent) reported io 0129 per

r

probbecause

to attain a large size, infiltrate adjacent structures

is

and

a difficult

management

microscopic

cision per

present and

discrepancy

ently

in

a

tumors

in evaluation

The

a mean

fractions

visits

cent

Desmoid

the brachial plexus. The surgeon feared the brachial plexus and therefore did proceed with any aggressive surgical resecThe patient was referred to u and treated 4 mev.

per

lem

to

the

last patient of therapy,

proliferagrowing

DISCUSSION

of

tumor 43

fascia

thin,

showing

fibroblasts

resolution

At the pletion

month

of tumor

differentiated

interlacing

after dence

Section

Iv.

well

per

5 of

Shiraki,4

by Pack and cent) reported

ex-

cent)

reI

8

(27

Ehrlich,8 by Hunt

L

-f

11G.

9.

base

Case Iv. of neck

Frontal before

view oflarge treatment by

mass at right irradiation.

FIG.

I I

apy.

.

Case The

Iv.

Twelve

tumor

months

has regressed

after

radiation

considerably.

ther-

VOL.

117,

No.

Radiation

i

Therapy

et al.5 This high recurrence rate had led sur-. geons to adopt more radical approaches in an attempt to control this benign but locally aggressive lesion. Das Gupta and associates3 feel that major amputation is the treatment of choice in recurrent large tumors of the iliac fossa, gluteal fold, or shoulder. Experience with irradiation in the treatment of desmoid tumor is limited. Musgrove and McDonald6 were not convinced of the value of radiation, but of 7 patients who had radiation therapy in their series of 35 showed no recurrence. Hunt et al.5 feel that radiation plays no part in the management of desmoid tumors; they admit, however, to no personal experience. Pack and Ehrlich8 indicate radiation therapy to be useful in inoperable tumors ( of 3 patients were free of recurrence at 5 years)

and

in

recurrent

tumor

after

radiation

therapy.

are

decreased

free of disease the tumor of

to

Dennis

Department

per

20

cent

years fourth

the its

after has

former

size

I

R. Hill, M.D. of Radiology

University

San

of California

Francisco,

San

California

Francisco

94122

REFERENCES I.

D., and of desmoid

BENNINGHOFF,

treatment

RAD.

GENOL.,

9!,

1964, 2.

BOOHER,

abdominal

R. Nature

ROBBIN5,

tumors. &

THERAPY

J.

AM.

and

ROENT-

MED.,

NUCLEAR

132-137.

R. J., and PACK, G. T. Desmomas wall in children. Cancer, 1951,

of 4,

1052-1065.

3.

DAS

T.

GUPTA,

K.,

BRA5FIELD,

J. Extra-abdominal

O’HARA,

pathological

study.

Ann.

R. desmoids:

Surg.,

1969,

D., and clinico170,

109-

121.

F. M., and SHIRAKI, M. Musculoaponeurotic fibromatosis of shoulder girdle (extra-abdominal desmoid): analysis of thirty cases followed up for ten or more years. Cancer,

4.

ENZINGER,

5.

HUNT,

6.

MUSGROVE,

1967,20,

1131-1140.

R. T., MORGAN, H. C., and ACKERMAN, L. V. Principles in management of extra-abdominal

desmoids.

Cancer,

J. E., and

abdominal diagnosis

1960,

13,

825-836.

J. R. Extra-

MCDONALD,

desmoid tumors: their differential and treatment. Arch. Path., 1948, 45,

513-540.

7.

R. W. Desmoid tumors: report of thirtyone cases. Arch. Surg., 1923, 7, 227-236. 8. PACK, G. T., and EHRLICH, H. E. Neoplasms of anterior abdominal wall with special consideraNICHOLS,

tion cases

of desmoid and

ternat. Abstr.

SUMMARY

Four patients with extensive, unresectable desmoid tumors were treated with definitive radiation therapy.

89

year after therapy by irradiation. The authors feel that surgery is the treatment ofchoice in cases ofsmall lesions, but radiation therapy should be attempted in the more extensive tumors. Radical amputation should be reserved for only those patients in whom desmoid tumors failed to respond to radiation treatment.

Presently,

the fourth patient has shown considerable regression of a huge tumor, just i months after therapy. The tumors usually recur during the first months after radiation treatment,4 but the first few months constitute too short a follow-up period to predict ultimate control. As a last resort, radical surgery (such as forequarter or hindquarter amputation) could be performed if properly administered therapy with modern megavoltage equipment has been unsuccessful.

Tumors

Three treatment;

following

surgery (, of patients showed good results). Benninghoff and Robbins’ advise radiation therapy for inoperable, incompletely removed, and recurrent tumors. Radical, mutilating surgery for this relatively benign lesion should be seriously questioned. In our modest series, 3 patients with extensive, unresectable tumor, have been free of disease for more than years

of Desmoid

9.

tumors:

collective

Surg.,

1944,

of

79,

with

literature.

391

In-

177-198.

L. Tumors of soft of Tumor Pathology. Second Series, Fascicle I. Armed Forces Institute of Pathology, Washington, D.C., 1967.

STOUT,

tissues.

A. P., and In: Atlas

experience

review RAFFAELE,