FIBROSAKCOMA IN INFANTS AND CHILDREN

ARTHUK
PURDY

;UY \ L A R S the writer has been interested in the fibroblastic tumors. Since undertaking the study of the less comnion mesenthynial tiiiiiors in childrcm, it has become apparent to him, in coinnion with a majority of
pathologists, that the exact recognition of
niany 01 the nirsenchymal tumors niny be confusing because of the fact that many differtiated mesencliymal cells of other types are
cnp,ible of acting as facultative fibr<)blilsts.~30
r h u s lipohla\ts, synovioblasts, mesothelial
cc.Ils, histioc-ytcs, and other cells can and do
make fibroblastic tissue, and their malignant
tiiiiior~may be mistaken lor fibrosarcomas. A
fil)rol)lci~t
ic e l ~ ~ m c nmay
t
be prominent in a
malignant niesenchymoma or a mixed mcsenthynial tumor and is always prominent in
p w d o ~ i roinni
(
ous fasciitis,71 which has been
mistaken for various malignant mesenchpmal
tumors, including fibrosarcoma.
I%ut if a tuinor is puiely fibroiis in all itc

SrOIJT, W.D.

parts, if i t is made u p cntircly of spindleshaped cells accompanied by reticulin fibers

that are generally wrapped about each cell,
and if cells and fibers tend LO form sinuously
interlacing bands o r cords and thcre are no
other elements recognized i n the growth, i t
seems safe to assume that one is tiealing with
a genuine and not a spurious fibroblastic tumor. J i a fibroblastic tumor has cells that are
well diffrrentiatcd, with no evidence of anaplasia :md rare or no mitoses and if there is
considerable reticulin and/or collagen separaling the tumor cells in children less than 16
years of age when the growth first manifests
itself, the writer has used the term juvenile
fibroma tosis to designate such tumors28They
are considercd benign. T h e writer published
44 s i i t h cnses in 1954,28 none of which had
metastasized. A t the prcsent time (Scpt. I ,
1961) he has accumulated 241 juvenile fibromatoses. None of the cases, so far a s he is
aware, has metastasbed except the one reported by Prior and Sisson,24 which apparently
mctasta,\i,ed to Imth lungs in spite of having
no histological features that would permit one
to predict that it would (lo so. During the same
period, 23 cases of pseudosarcomatous fasciitis
in children haye been recorded in our Laboratory. There remain 2.5 fibrons tumors in
children that at the present time bear the
label fibrosiircoma. T h c y are all purely fibrous tiiinors that will be analyzed anti tliscusscci in order to try to determine whether
or not thc name is justified. Rut before doing
this it will be well to learn as much as possible
from the reported tumors in children that
have been called fibrosarcomas.

$'rani tlic 1,;iborator) of Surgical Iathology, Coluinhia IJniversity College of Physicians and Surgeons, 630
\V. 168th St., %:ew York 32, N.Y.
rhc nii111oris gratcfril to thr following physicians
who submitled the cases and when possible furnished
frillow-up information.
Dr. Henry Brody, Philadelphia, Pa., case 1; Dr.
C. 1:. C:artIrnas. Ilavana, Ciih;i, casc 2: Dr. Snul Kay.
Kichrnctnd. Va.. c a m 3 ;tnd 8; D r . K. M. Heard, Jackson, Miss.. case 1; Dr. M. DcA. Figurredo and Dr. HumImto lorloni, SPo Paulo, Braril, case 5; Col. F. M.
Iownscricl, Aimctl F o ~ ~ Iiistituie
cs
of Pathology, Washington, I).C.. and Ilr. K. I. Fuller. Tucson, A r k , casc
(i: I h . P. .\. R o w , Alhuqoerquc, N.M., antl Dr. A. I.
McQiieency, Sanra Harlm-a, Calif., casc 7; Dr. D. F.
Ilabl), Prmcc, Poeito Rico, casc 9; Dr. Erccnian, Dr.
D. C. Watlcr, and Ilr. L. E. Arnold, Kingston, Jamaica, B.W.1.. case 1 0 Dr. R. W. Alexander, Chicago,
Ill., and Dr. J. T. H o w ~ l l ,New York, N.Y., case 11:
Ilr. D. I,i1q)liarcl, I.lrnitington, Pa.. and I h . F. W.
Brason, Harrishurg, Pa., case 12; I)r. E. Ialik, Tulsa,
Dr. F. W. Wiglesworth, Montreal,
REVIEW
OF THE LTTERATURE
I , case 14; Dr. G. N. Strmmrrinanri,
FIntiolulii. Hawaii, case 15; 1 h . E. r. Srandley, San
One has to hunt far arid wide in the literaAntonio, Texas, case 1 6 Ilr. C.. B. Elliott, Calgxy. Alture to find cases of tumors called fibrosarberta, Canada, case 17; Col. W. J. Redner, Jr., New
York, N.Y., antl Dr. W. 7. Thorpe. Clrvclant
comas that have developed in children. T h e
casc 18; I)r. P. F. Ashlr!;, Wilrnington, &I.,
authors
sometimes use that name, sometimes
Dr. C. A. McNiml, Huntington, N.Y.,case 20: Dr.
R. E. Weland, Cedar Rapids, Iowa, case 21; Dr. C. W. they are called spindle cell sarcomas and someKc*tchiirn. Tallahassce. Fla.. a n d Dr. J. H . Parkcr, IT.,times the name chosen is merely sarcoma. AfPcrry, Fla., case 22; Dr. H. H. Iitts and Dr. T. R. Oster potential candidates have been found they
Icr. Vancouver, British Columhia, Canada. case 23.
The author is indebted t o Mr. Edward Haijar, Pho- have to be evaluated to see if they can be actographcr of thc Francis Iklafield Hospital, New York,
cepted as such. One has to depend upon phoN.Y., for the photomicrographs.
tomicro~graphs,histological descriptions, or ocRcccivc-cl for pn1,lication Oct. 30, 1961.

1028

No. 5

FIBROSARCOMA
IN INFANTS
& CHILDREN Stout

1029

FIG. 1. Case 5. A, Appearance of the original tumor in thc palm. T h e cells are spindle shaped and grow in
bundles. No mitoses were detected, The cells are slightly smaller than the average. ( X 2 3 0 . ) B, Silver reticulin stain
of the original tumor. This shows that the cells are not wrapped about with fibers. This could not have been suspected without this stain. ( ~ 2 3 0 . )C, Metastasis in the axillary lymph node. T h e cells now appear longer than in
D, Silver reticulin stain of the metastasis. Most of the cells are not surrounded by
the primary tumor. (~230.)
fibers. Again this could not have been determined without a fiber stain. ( X 2 3 0 . )

1030

CANCER
September-October 1962

Fie. 2. Case 7. This is the average appearance of a

dilFcrcniiatcd Librosarconia. The cells are sliglitly larger
than those shown in Fig. 1 (case 5). This is the more
~ i m i l tcll sirc that is dcsc.rit)cct in thc table by the
word "inctliuin." ( ~ 2 5 0 . )

ca4onally the knowledge that the reporter is

qualified to recognize a fibroblastic tumor. If
there seeins to be serious doubt, the case is
rejected. The results of this survey are interestiiig becauw they parallel almost exactly our
experience with the cases collected in this
Laboratory.
There is an interesting report of 129 socalled malignant fibroblastic tumors involving skin and subcutaneous tissues Erom the
Radiumhemniet by Gentele.12 Among the patients were 8 children whose ages ranged from
8 months to 14 years. All were treated by excision and most of them had radiothcrapy as
well. There w a s recurrence in only I case. The
follow-up on 7 ranged from 9 to 22 years. One
Imy drowned after 2% years. All were without
cvidence of tumor at last report. This is most
valuable infornia tion.
Wil~on35reported upon 11 I tumors called
fibrosarcoma that were treated at Duke Univenity. Eight of thcse were found in children between l and 10 years of age, and 19
~7ereincluded in he 11- to 20-year group. Rut
there are no follow-up data regarding the chil-

Vol. 15

dren and no way of telling whether the tumors were fibromatoses or fibrosarcomas.
Warren and SommeF studied 163 cases of
fibrosarconia of the soft parts. Two developed
in the first decade, and 2 others were i n children IS and 14 years of age respectively. Three
of the patients were alive and well 12.7, 12.7,
and 1.3 years after treatment. This last patient
had had 2 recurrences beforc the tumor was
finally eliminated. The fourth child had a
congenital fibrosarcoma of the hand. There
were 20 recurrences, and after 8 gcars the child
died of other causes without evidence of tumor.
In an extensive study of fibrosarcoma of the
soft tissucs by Pack and hie123 there were 2
patients who were less than 20 year4 of agc.
Both were considered cured for more than 5
years. One othcr patient, a 2-month-old infant,
hat1 a fibrosarcoma of the arm. This tumor
shrank and disappcared completely 7 months
aftcr radiotherapy.
There were Y children aged 2, 15, and 16
years respectively reported by Kaplan and Rubenfeldls None died and only 1 had a recurrence. T h e follow-up periods however were
only 6 months to 2 years.
'There were 3 children in the group reported
by Bick.3 In 1 the tumor was said to have
ended Eatally, but without any histological
details one cannot feel sure of the real nature
of any of these tumor$.
T h e writer27 reported the case of a boy who
5ometime before he was 9 years old had a back
injury that left a scar. At the age of 9 years,
a lump appeared in the scar. When he was
35 years old, it was excised but promptly recurred. It was again widcly excised at age ti5
because of rapid growth and ulceration. T h i s
was successful, and at the age of 91 years, he
was free from evidencc ol' recurrcnce. This
was called a fibrosarcoma but the writer now
believes it w a ~
merely a fibroniatosis.
Collins and Annpachs described thc case of
a boy who had a tumor in the plantar aspect
of the foot that appeared when he was 1 4 years
of age and was biopsied whcn he was 19 ycars
of age. He was treated by radiotherapy, and
there was improvement for 2 pears. Then the
tumor metastasized to the groin and later to
the lungs. He tliecl 3 years after radiotherapy
was begun. At autopsy, tlic only metastases
omti were those in the lungs.
Thc congenital fibrosarcomas form a most
intcresting group. It is a great pity that some
of them were not studied by pathologists ex-

No. 5

FIBROSARCOMA
IN INFANTS
& CHILDREN Stout

pert in the interpretation of mesenchymal tumors, because, in many instances, it is impossible t@ determine the exact nature of the tumors.
The remarkable collection of congenital
tumors assembled by Wells33 unfortunately is
of no value because the author merely accepted the labels used by the various authors
and made no critical analysis. Of 13 cases
called either spindle cell sarcoma or fibrosarcoma, 4 of the patients died of tumor, 5 recovered, and the result is unknown in the
other 4 cases. It would be unsafe to accept any
of these as fibrosarcomas. The case reported
by Warren and Sommer,32 involving the hand,
has already been referred to. There were no
metastases.
The case reported by Hudson,l7 involving
the foot, resembles case 18 in our own series
both histologically and because in both cases
amputation was necessary. During the short
follow-up of 9 months there was no evidence
of reappearance of the tumor.
Comparable to Hudsons case is the one reported by Dreyfuss.8 I t also involved the dor-

I031

sum of the foot, and when the child was 2y2

months old a mid-thigh amputation was done.
Dreyfussg called the tumor a spindle cell sarcoma, but it may have been a fibrosarcoma.
Shores2jpatient is remarkable. T h e tumor
was present at birth in the girls interscapular
region. It was excised twice and recurred each
time. Then it disappeared spontaneously. This
tumor I would now classify as a fibromatosis.
Heckersl5 patient had a congenital tumor
in the gluteal muscle removed at the age of 3
months and called a spindle cell sarcoma rich
in cells. As the follow-up period was only 3
months and the nature of the tumor uncertain, the case is valueless.
Hartz and Guerrero Tablante14 reported
the case of a congenital tumor of the mastoid
region in a baby girl. When she was of 2
months of age, the rapidly growing tumor was
excised and the tumor bed electrocoagulated.
Seven days after treatment the child died with
symptoms of meningoencephalitis. The careful histological study justifies the diagnosis of
fibrosarcoma.
A 3-monrh-old infant had a congenital tu-

FIG. 3. Case 8. A, This congenital tumor removed from the trapezius muscle has the same general appearance in this illustration as the nonmetastasizing tumor shown in Fig. 2. The nuclear markings are slightly more
distinct, and the mitotic count was slightly greater but not significantly so. (X230.) B, The recurrence of the
tumor. The cells are now distinctly smaller, many are rounded instead of spindle shaped, and a mass of tumor
cells have indented one side of a vascular tube. This is probably evidence of loss of differentiation. This patient
was the child who died with evidences of lung metastases. (X230.)

1032

CANCEK Sept ernber-0 ct o bey 1962

inor ol the toot. I t W ~ Sexcisecl, re( urrecl locally, and a n amputation was carried out after 4 months. Seven months later, the child
died with probable lung metastases. Fahey ancl
Bollingci9 called the tumor sarcoma. It may
have bcen a fihrosai coiiin.
An exircniely interesting casc was reported
by IVilliani~antl Schrum34 as a congenital fibrosarconia with widespread metastases i n the
skin, S U ~ C U L ~ I ~ ~ tissues,
OUS
and viscera. T h e
boy died on the twenty-wc-ond day after birth.
Ihis caw was later slutlied by the writer.28
Wc belie\ed it to be a n example 01 congenital
gencraliJet1 fibrornatoscs with multiple foci
and not a metastasizing fibrosarcoma.
Ihis intomplcte survey oE probably congenital fibrosarcomas s h o w how completely frustrating the data concerning this subject have
proved to be. But it seems significant that in
only 1 cnse. that o E Fahey and Bollinger,9 was
there ekitlenc e of distant metastasis in the
lungs, aiirl in that case the diagnosis of fibrosarconia is yuestionablc.
Fibrosarconias sometimes develop in the
deepcr tissue? ancl the viscera of children. A
numbcr have been described that have involved the bronchi and lungs.
I , c ~ i s cabe
~ ~ was in the bronchus of il 14year-old bo) . Following bronchoscopic removal
FIG 4. Case 9. A, The gross appearance of
thc bisected tumor in the vastiis mcdialis
niu\clr. B, The illustration shows cells of
irietlircin siie and spindle shape arrangement
in inteilacing bands. In other parts of this
tiinlor. them were larger and smaller cells.
In some aicas, reticulin fibers were wiapped
about each rell; in otheis they were sparse.
Mitoses average 12 in 50 high power fields.
1 1 i i $ tumor recurred and was re-excised. In
spite 01 tlie suggestion\ of possible malignanc\, the tliilcl was a 5-year cure. ( ~ 2 3 0 . )

YIJI. 15

and radiotherapy, he ieminerl well lor 2%

years. Feldmanslo casc 1 was in an 11-year-old

girl. After segmental resection of the lung
tumor in the left lower lobe, she remained well
for more than 3 years. In the photornicrograph, the tunior looks like a fibrosarcoma.
Donoghue ct al.s7 patient was a girl 5% years
of age. She had a n endobronchial fibrosarcoma
treated by right pneumonectoniy. She was well
after 3 months but could not be traced after
that. Curry ancl FuchsG patictit was ;I 15-yearold girl who coughed up ;F fibrosarconia. She
received no treatnient and was well 4 years
later. Holinger et al.slo patient was a 51/-yearold boy with a bronchial fibrosarcoma. T h e
right middle and lower lobe5 were resected.
He recovcred itom the opelation, hut there
was no follow-up. In the case ol Gerbasi et al.lR
the child died with metastases in the opposite
lung. T h e 2-year 9-month-old boy was treated
by lobectomy antl radioactive cobalt therapy.
Although called fibrosarcoma, this case cannot be accepted be( ;iuw the pliotomitrographs
leave the nature of the tumor uncertain, and
there is no report of proper histological investigation. Hence reporled fibrosarcoma of the
lung in children has not proved to be any
more nialignaiit than fibros;ircoma elsewhere.
Fi brosarcoina has occahiia lly becn rcportcd

No. 5

FIBROSARCOMA
IN INFANTS
& CHILDREN Stout

1033

by Snyder et a1.,26 but since these authors have

given no photomicrographs or histological descriptions, the cases cannot be evaluated.
Gardner and Turner11 described a fibroblastic tumor that arose in the choroid plexus
of the lateral ventricle of a 3G-year-old child.
It was excised, and 9 months after operation
the child was symptomatically improved.
From these case reports concerning presumably acceptable juvenile fibrosarcomas it is
found that, of 61 patients, 29 were followed
for various periods and did not have evidence
of metastasis, 80 were not followed but presumably did not show evidence of metastasis
at the time of reporting, and only 2 gave evidence of metastases. It is quite true that several of the tumors infiltrated extensively and
incomplete removal resulted in recurrences
that led to amputation and sometimes were
directly responsible for the death of the patient, but, since they were not known to have
metastasized, they were potentially curabsle.
Moreover, 2 of the tumors disappeared, I after radiotherapy and 1 spontaneously. It is
quite evident that so-called juvenile fibrosarFIG.5. Case 13. The cells of this tumor of the ton- coma differs very little from juvenile fibrosillar fossa have the usual interlaced arrangement and matosis, and it becomes of great importance
vary in size from medium to slightly smaller. Reticulin to learn how to recognize the ones that are pofibers surround each cell. The mitotic rate is negligible, and there is little to distinguish this tumor from tentially metastatic from all of the others. Since
many of the others. In spite of this, the child died. sections of the reported tumors that metasta(~230.)
sized are not available to the writer, it will be
necessary to glean what information is possible
in the upper respiratory tract of children. Bah- from the new cases to be reported from this
gat1 described a 13-year-old girl who had a institution.
mass filling the naso- and oropharynx and encroaching on the laryngopharynx. It was exPERSONAL
CASES
cised, and after 3 months there was no eviEtiological Features. Sex. Fourteen of the
dence of recurrence.
Berard and Sargnon2described a tumor that patients were boys, 7 were girls, and the sex of
filled the nasopharynx and the maxillary sinus 2 is unknown.
Age at Onset. As has sometimes been the
and invaded the orbit, displacing the globe,
and made the palate bulge into the mouth. I t case in other mesenchymal tumors, a majority
was excised, and 2 radium treatments were first manifested themselves in either the first
given. T h e patient was well 20 years later. or third quinquennia. The onset of 11 cases
This seems more descriptive of osteofibroma was in the first quinquennium (birth to 5
years), and 4 of these were congenital. Five
(fibrous dysplasia) than of fibrosarcoma.
Cabots* patient (case 16302) was a 6-year- were first noted in the second quinquennium
old child in whom the tumor started in the (ages 6 to 10 years), and 7 in the third (11 to
temporal region; it was excised and treated by 15 years).
Site of Tumor. The tumors were widely
X ray and radium but recurred in the cheek
and extended to the maxilla, maxillary sinus, scattered: head and neck, 7; trunk, 4; upper
malar, temporal, and ethmoid bones, and the extremity, 4 (arm 1, forearm 1, hand 2); lower
ethmoid sinus. It was called a fibrosarcoma extremity, 5 (thigh 1, leg 1, foot 3); oral cavity,
3 (tongue, gum, pharynx): bronchus, 1; spinal
but did not metastasize.
Fibrosarcomas have been reported in the meninges, 1. None of the tumors was primarily
retroperitoneum of children by Melicow22 and in the skin. Intramuscular tumors included

I034

Vol. 15

CANCEK
Sept embt:r-October 1962

1 in the external soft tissues and 1 in the

t.ongue. One tumor was attached to the periosteirni of the scapula.
l+trt Ii ologicuI Findings. Gross Description.

__--

_-

PERSONAL CZSES
I
_

It. age, yr.

-_

Reference to Table 1 will show that. in cases in Case p&s

Ses
4t
At
which the size was known, most of the tumors 110.
no.
Pt.
onset
treat.
Site turn.
were small. However, it must be realized that
?
5
Scalp
1
29461
?
74/12
Scap. reg.
5
~ . h c . none is dealing with infants, a tumor that
2
30949
F
1% o d d be considered small or not very large
11
1 - 1 4 /12
Bronchus
3* 35430
M
i t i ;in adult would assume tiiore formidable
Birth
3/12
Gastpc4
39421
?
Iiemius
~xoportions.For example, in case 18, the child
muscle
was born with a tumor enlarging arid deforrning an entire foot. Compared with the size of
10
11
Pal rn
M
5
43104
462 1 7
thc L h i J d , the 9 x 8 7-cm.
~
lumorr appeared
2-5/12
2-6/12
Over lacr.
6
47245
M
C ~ O I I ~ ~ U The
S.
consistency of the tumors
Sac
8-6/12
8
Forcarm
7
49176
M
varied; soinc were hard, others soft; there was
c onipletc variabilily among them. They were
11/12
Birth
Trapezius
8
50572
M
seltlom painful, and the nature of the tumor
muscle
51312
2-6/12
24/12
Vastus
9
51944
F
coultl not bc deduced from the clinical exniedinlis
aniiii~iti~~~i.
muscle
hlicroscopic Desa iption. The great major13
12
Foot
ity of the tumors were composed of spindle5h;ipetl cells with a tendency to be arranged
,
6/12
Pcrios54311
F
i n sinuous bands or cords that interlaced. The 11
teum;
elongated cell bodies tended to be pointed,
scap.
.inti the nutlei conformed to the shape of the 12
a
3
Tongue
58330
F
b o d y and were also pointed. There were no
in t r<icc.1I u I <tr fibrils, b 11t reticulin fibers coni- 13
12
12-1/12
Tonsillar
60212
F
fossa
nioiily surrounded each cell and there were
6
4
Pa1m
60846
kl
also often elongated reticiilin fibers parallel- 14
ing the long axes of the cells (Fig. 6A and B). 15
16
15+
61366
M
Lymph
iiodc a t
Very occaGonally the reticulin fibers were not
lat. dorsi
wrapped about each individual cell but were
muscle
o n l y p r s c l y scattcred among them. Since this 16t 62230
7
7
Mast. reg.
ill
?
12
Axilla
63326
M
was found in cases 5 and 8, the 2 cases known 17
t o have inetastasixd, it is probably an indiBirth
1 day
Foot
18
6.3875
M
c;ttion of increased malignancy (Fig. 1K and
11). The usual length of the cells is shown in 19
1-5/12
14/12
Nas. reg.
61512
M
Figs. 2 and 6.4, and this length is labeled
11-2/12
11
Gum;
F
nictliuiii in the cable. Tumors were some- 20* 64713
maxilla
times composed of cells either larger or smaller
than medium (Figs. .SU, 7, and 8A). In true 21
?
8
Epidural;
64718
M
L-2 & 3
human fibroblastic tumors, real giant cells
with either single or multiple nuclei are hardly 22
11
12
I h l toid
ti5057
M
reg.
ever found. If any appreciable number of gixnt cells are found in a rpindle cell tumor, it 23
10
654.59
F
9
Sole foot
is probably ci ther a liposarcoma, a rhabdomyosarcoma, or a leiomyosarcoma. All of the fibro*The patient was a Negro.
sarcomas infiltrated their environs. If the nuc-lcus resembled the nuclei of thc cells in fibroniatoses, it was recorded as no anaplasia. If hoped that the relative number of mitoses
the nuclear markings were more accentuated, might have some relationship to malignancy.
i t was recorded as slight or definite ana- Therefore in each case their number in 50 high
plasia. In definite anaplasia, the cell body us- power fields has been recorded. Apparently
ually was well defined. Often in fibromatoses this has not been a reliable criterion, and one
the nucleus is not too clearly defined. It was is forced to assume that it is simply an indica-

FIBROSARCOMA
IN INFANTS
& CHILDREN
*
Stout

No. 5

1035

TABLE
1
OF JUVENILE FIBROSARCOMA
Size
turn.,
cm.

Treat.

Anaplas.

Reticulin

Mitoses/
50
HPF

Ar.
each
cell

Sparse

Size
cell

Recur.

Metast.

Died

0
?

0
0

0
0

Remarks

?
2.

Excis.
Excis.

Sl.

1
10

+
+

. ..
. ..

?
Lge.

Biopsy
Excis.;
amput.

S1.
S1.

0
13

+
+

...
...

Med.
Med. to
Ige.
Sm.
Sm.

1.5X1.

Excis.

No

...

Sin.

Pea

Excis.

+
...

Med.

Axil.
node
0

...

Med.

+
+

Med. &
sm.
Med.,

+
+

? reexcis.
scar
0

g e d 6 nio.;
lung metast.
Recur. 8 mo.;
well 5-5/12
yr. aft. 2d
OP.
Well 5 yr.

Well 4 yr.
Well 3-9/12
Yr-

?
3.5+
4. X2.5

Excis. ;
re-excis.
Excis.
Excis. &
excis.
recur.

+
Sl.

0
0
1

SI.

+
+
SI.

2
12

+
+

Ige., &

3 mo.

+
0

sin.

Well 12 yr.
No roll.-up
Well 8 yr.
Well 8 mo.
aft. amput.
then lost to
f0ll.-up
Died 2-2/12
yr. ; no autop.
Well 6-7/12
Yr.
Well 6-3/12

Excis.

KO

Excis. ;
X ray

N O

...

Med. &
sm.

Ptl. excis.;
compl.
excis.
Excis.

No

...

Med.

s1.

...

Died 1-7/12
Y I-.
Well 2-9/12

Med. &
sni.
Med.

NO

+
+

19

...

Med. &
sm.

Well 3 yr.

.. .

Med.
Med. &
sm.
Sm.

+ aft.

?
0

Tum.
back
removed

Well 3 yr.
Well 2-1/12
Yr.
Well 2 yr.

Sm.

4
?
3.2

?
?
9.X8.X7,
1.5

Lge.
5.

1.

Excis.;
re-excis.
Excis.

...

Med.

NO

No

...
.. .

Excis. ;
X ray
Excis.

No

.. .

Med.

NO

10

...

Med.

Excis. ;
recur. ;
X ray
Forequarter
amput.
Excis.

NO

_..

No

12

...

Excis.
Excis.; reems.
Amput.
foot

No

...

2 yr.

Med.

Med. &
sm.

Med &
sm.

YI--

Well 1-10/12
YrOss. metalas.; no
roll.-up
Sympt. rel.
aft. 3 mo.
Well 1-9/12

Yr-

Recur. scar
1-10/12 yr.

tThe patient was a Chinese.

tion of rapidity of reproduction of cells. Since

tumor cells usually do not multiply at the
same rate in all parts of a tumor, the failure
to find mitotic activity in a tumor may simply
apply to the area of the tumor examined.
One of the extremely difficult problems of

differential diagnosis concerns the differentiation between fibrosarcoma and the fibroblastic
element of a synovial sarcoma. T h i s problem
has not arisen in the present collection of tumors in children but it does arise in adults.
The writer realizes that a section taken from

1056

CANCER
Scptem ber-Octobel- 1962

1'01. 1.5

Frc. G. Case 14. A, This is the recurrence of the tumor in the palm. The cclls are of medium sizc. have thc usual
iriter1;icccl appi'armce, and iriGItrate thc I I I ~ I C U ~ ~ I I C O Ifat.
I S (~230.)
13, The silver reticulin stain shows fine reticitlin tilwrs siirroui~diiigeach cell. aud some paralleling the long axes of the spintlle-shapedcells. (~230.)

1 pait oi a s)novial sarcoma may reveal only

the f i b i o~arcomatouselcrnent. He is unable to
rccogniie this when i t occurs antl knows of
no~hingshot t of tiswe culture tliat could cieL i d e thc choice. He would prefer to err b y
calling ;I syiiovial s'ircoma a fibrosarcoma, becauw apparently the tumor is more apt to
display thc less malignant characteristics of a
fibrowrcom;i c w n if it should be a disguised
synovial 5arcoma.
iliiother cutrenicly difficult problem has
becii to try to decide where to draw a dividing
li tie be tu een fib1 onia~o5cs,ind fibrosarcomas.
In selecting this group of cases, fibroblastic tumors with a marked degree of cellularity were
arbitrarily chosen. The writer is well aware
that this is far irom exact since obviously the
tei in "marked" will bc differently interpreted.
Actually it has very little significance, since,
in children, the proportion of fibroblastic tumors that will metastasize is very small. This
que5tion will be further discussed under indications for treatment.
Results of Treaiment. It has been possible
for the majority of those who consulted the
writer about these tumors to furnish him with
reliable follow-up data. At the timc of writing,

15 patients were reported lree lrom evidences

of tumor. Six were well from 5 to 12 years after last treatment and 9 others from 1 to 5
years. Onc was well for 8 months and then was
lost to iollow-up. Onc (case 23) was well 22
months alter excision with iecurrcnce in the
scar. T h e child with tumor involving the spinal
meninges (case 21) was syrnptoiriatically improved 3 months after treatment by radiotherapy. Three children died: 2 (cases 5 and 8)
were known to have had rnetmtasis, antl the
cause of death of the third (case 13) is not
known. Two patierits were lost LO follow-up.
One child (case 15) had a tumor involving the
latissimus dorsi muscle with involvement of an
axillary lymph node. Whether this was due to
metastasis or due to direct invasion is not clear,
but the child was well 3 years after treatment.
Three children had extremities amputated
(cases 4, 18, and 22) but are not known to have
had any metastases.

Drscr fswm
As a result of studying the cases of lesions
called fibrosarcomas in children reported in
the literature and a further study of the pres-

No. 5

FIBROSARCOMA
IN INFANTS
& CHILDREN* Stout

ent series of 23 cases of juvenile fibrosarcoma

available to the writer, it is apparent that extremely few fibroblastic tumors metastasize.
If we include our 241 juvenile fibromatosis
cases, 1 of which metastasized, with all 54 of
our own and reported juvenile fibrosarcoma
cases, a total of 295 cases in children, only 5,
or 1.6%, are known to have metastasized.When
we include with our 23 cases of fibrosarcoma
the 31 reported in the literature with followup, it is found that only 4, or 7.6%, of the tumors are known to have metastasized. Just as
is the case with the fibromatoses, the development of a juvenile fibrosarcoma in certain
key sites can lead to serious or even fatal results because all of these tumors infiltrate insidiously and a failure to realize this can lead
to repeated recurrences, amputations and
other disabling major operations, and, occasionally, even to death. But these untoward
events are only the result of persistent invasive
growth and are not the result of embolic
metastasis, which is generally regarded as the
true test of a malignant neoplasm.
T h e present study was undertaken also in
the hope of learning first, how to distinguish
between juvenile fibromatoses and fibrosarcomas and second, how to detect those that
are potentially metastastic. I t must be
confessed that the study has failed in both attempts. If a pure fibrous tumor is so well differentiated and has so much collagen and
reticulin that it looks like hyperplastic scar
tissue, it is easy to call it a fibromatosis. But as
cellularity increases, the uncertainty grows as
to whether a tumor should be called a fibromatosis or fibrosarcoma. If one uses such terms
as marked cellularity it is obvious that this
leaves the choice up to each individual. If one
attempts to use the number of mitoses in 50
high power fields as a criterion of malignancy,
it is found that in the present series there are
11 cases listed as fibrosarcomas in which none
or only 1 mitosis could be counted in 50 fields,
and 1 of these children died with metastases.
It is therefore not a reliable evidence of malignancy. Evidence of anaplasia and failure of
the tumor cells to form much reticulin are
both suggestive, but the former is hard to
recognize, and a silver reticulin stain is necessary to be sure of the latter, so that they are
not readily applicable to biopsies in most instances. T h e same difficulty applies to cell size;
without a definite standard of comparison it
is not easy to determine whether the tumor
cells are average, Smaller, or larger. Finally, a

1037

single biopsy specimen may be deceptive because these tumors do not always maintain a
uniform appearance throughout.
It has seemed to the writer, therefore, in
dealing with the fibrous tumors, that it would
be wise to apply a general standard of treatment whether it is elected to call a tumor a
fibromatosis or a fibrosarcoma. All of them
infiltrate and can cause plenty of local damage
if not completely removed. Even the tumors
that can be called fibrosarcomas have only a
statistical chance of metastasis in 7.6%, which
is 1 in 13 cases. Treatment should be planned
with the realization that all of these tumors
infiltrate insidiously and excisions passing well
outside of the palpable limits of the tumor
have the best chance of cure. For small tumors,
this can be carried out without biopsy because
it is the best treatment for all varieties of
small soft tissue tumors. If biopsy is elected,
this should be carried out with care and no
further action taken until a report on it has
been received. The worst approach to all soft

FIG.7. Case 15. This tumor is anomalous because it

had some of the characteristicsof malignancy. The cells
are small in this area with little tendency to be arranged in bundles. The mitotic rate of 19 per 50 high
power fields is the highest in the series, and the tumor
had either directlv invaded or possibly metastasized to
an immediately adjacent lymph nod& In spite of all
this there was no recurrence after 3 years. (~230.)

1038

CANC,ER
Septem bey-October 1962

Vol. 15

FIG. 8. Caw 18. Yibrosarcoma of foot. A, Characteristic field showing the relatively small spindle-shapcd cells
occasionally arranget! in vague bundles. The edcma and the many infiltrating lymphocytes confuse the picture.
(~230.)B, Silver retirillin stain showing that many of the cells are wrapped ahout by the very delicate reticulin
lihcrs. (~230.)

tissue tumors in children is to remove them

piecemeal o r in a single piece but close to the
tumor :is if it were a cyst. These fibrous tumors are never encapsulated and always infiltrate to some degree. If, after biopsy or incomplete excision, the tumor proves to be a
fibrosarcoma, one should not sacrifice extremities without making every attempt to achieve
a cure by less drastic measures. The fact that
the chances of metastasis are SO remote in a
given case should influence the surgeons judgment in planning treatment.

CASEREPORTS
Case 5. When this Brazilian white boy was
10 years old, a tumor was noted in the thenar
region of the right hand. When he was 11
years old, the tumor occupied the entire thenar region and extended into the thumb as
far as the distal phalanx. A wide local excision was carried out because the tumor invaded
muscle. A chest roentgenogram at this time
was negative. Eights months later, a n axillary
node dissection was carried out. One among
44 nodes had a metastasis. Seventeen months
after this axillary opcration, the patient died
at home, and no autopsy was obtained. His-

tologically, there was nothing to suggest malignancy in h e hematoxylin and eosin-stained

sections. T h e sections stained with Laidlaws
stain showed that reticulin fibers were not
wrapped about each cell. T h e metastasis in
the lymph node showed exactly the same picture (Figs. lA, B, C, and D).
Case 7. This 8-year-old boy developed a
painless mass on the anterolatcral aspect of
the upper left forearm. Six months after its appearance, a 2-cm. circumscribed tumor was removed from the subcutaneous tissue. After 4
months, the area was re-excised because of a
recurrence. Six years and 3 months later, the
boy was well and free from any evidence of
tumor. T h e tumor was well differentiated and
quite vascular. Mitoses were rare, and the tumor was on the borderline between a welldifferentiated fibrosarcoma and a fibromatosis (Fig. 2).
Case 8. This boy was a difficult breech delivery, and the neck was supposed to have been
injured during it. A lesion developed in the
trapezius muscle. 4t the age of 11 months, the
growth was removed piecemeal from the muscle. I n less than 3 months, there was a recurrence, and roentgenograms at this time
showed that the lungs were riddled with metas-

KO.

FIBROSARCOMA
IN INFANTS
8c CHILDREN Stout

tases. I n 3 more months the child was dead.

The original tumor looked in part like a differentiated fibromatosis but had areas in
which the tissue was more cellular, the nuclei
suggested anaplasia, and some cells were
rounded instead of spindle shaped. Recurrence
showed a reproduction of the more active
part of the primary tumor (Fig. 3A and B).
Case 9. At the age of 2% years, this girl was
found to have a 5-cm. tumor embedded in the
muscles of the right thigh. A local excision
was carried out, but the tumor recurred. After 8 months, the recurrence was excised.
There was no further reappearance of the
tumor, and the child was reported well 5 years
and 5 months after the second operation. The
closely packed spindle cells showed some nuclear anaplasia and a considerable number of
mitoses and gave the impression of malignancy
(Fig. 4A and B).

1039

scribed, 3.2 cm.in diameter, and was not removed intact but broken into during the operation. In spite of this, there has been no
evidence of recurrence or metastasis during
the 3 years that have elapsed since the op'
eration. This tumor had a mixture of cords
of spindle-shaped cells and undifferentiated
rounded and spindle-shaped cells. Mitoses were
frequent, and some cells appeared anaplastic
(Fig. 7). The tumor was partly surrounded by
lymphoid tissue, suggesting a compressed
lymph node.

Case 18. This boy, a first child, delivered

normally after an uneventful pregnancy, was
born with a large tumor mass involving the
entire right foot anterior to the heel. A midleg amputation was carried out the next day.
During the 2 years that have elapsed since
the operation there has been no evidence of
recurrence or metastasis. During this period, a
tumor of unknown type was removed from the
Case 13. A 12-year-oldgirl was found to have back. The tumor of the foot, measuring 9X8X
a growth in the left tonsillar fossa 5 weeks af- 7 cm., involved all the soft tissues of the foot
ter tonsillectomy. When the tonsils were re- and almost obliterated the toes. There was
moved, a 1-cm. nodule was exposed in the left extensive necrosis and ulceration of the tumor
fossa. In 5 weeks, it had reached a diameter on the plantar surface. The tumor was comof 4 cm.It was removed piecemeal. It has been posed largely of rather small spindle-shaped
possible to learn only that the child died 19 cells that sometimes formed bundles but elsemonths later. Whether there were metastases where had n o definite arrangement. In many
or recurrence is unknown. Histologically, the areas, inflammatory cells and engorged capiltumor was quite cellular but with most of the laries confused the picture. Very fine reticulin
nuclei appearing normal or with only a slight fibers surrounded each cell. Mitoses were rare
suggestion of anaplasia and very few mitoses (Fig. 8A and B).
(Fig. 5).
Case 14. A four-year-old Jewish boy was
found to have a swelling of the right hypothenar eminence. Two years later, a tumor was
excised and recurred almost at once. Its original size was not recorded, but the tumor was
reported as a dermatofibrosarcoma protuberans. T h e recurrence was excised about 3 weeks
after the first operation. Two years and 9
months after the second operation the child
was well and free from evidence of tumor. The
recurrence appeared relatively well differentiated, with reticulin around each cell, no anaplasia, and a moderate number of mitoses
(Fig. 6A and B).

SUMMARY

A study of available reports of probable juvenile fibrosarcomas in the literature and of

23 personal cases suggests that this is an uncommon tumor type in children. It can develop in the soft tissues and rarely in the viscera in many parts of the body. The tumor is
difficult to distinguish from the fibromatoses
and seldom metastasizes, probably not more
than once in every 13 cases. It has not been
possible for the writer to be able to distinguish
those that will probably metastasize from the
others, although certain features are suggesCase 15. This 16-year-old boy first noticed a tive. Since all fibrous tumors in children infilswelling in the latissimus dorsi muscle 15 a. trate, the indication for treatment is directed
lateral to the osterior mid-line at the level of
the twelfth orsal vertebra. The lesion was entirely toward complete removal of the local
painless and was first noticed at stretching ex- lesion. If this is accomplished, almost all cases
ercises during football practice. It was circum- will be cured.

REFERENCES

1. BAHGAT,
H,:Interesting clinical cases with operations. J . Egyptian M . A . 16: 678-686,1933; 678-679.
and SARGNON:Fibro-sarcorne du naso2. B~RARD,
pharynx guCri depuis 1914 par le traitement chirurgi-

A la curietherapie. Lyon chir. 3 0 728-729,

1933.
3. BICK,E. M.: Fibrosarcoma of extremitics. Ann.
S U T ~ 101:
.
759-765,1935.
cal associk

I040

CANC,ER
Septembel--October 1962

4. Cnwr, C. K.,Ed.: Case 16902. In Case rccords of

Massachusetts General Hospital, ante-mortem and postiiiorlem records as used in weekly clinico-pathological
rsercisrr. blew England /. Itfed. 203: 174-180, 1930; 177-

Vol. 15

Zentmlbl. Chir. 62: 2126-2127, 1935.

20. LEWIS,I.: Sarcoma of bronchus. Proc. Roy. SOC.
Med. 40: 119-120, 1947.
21. MALLORY,
T. B., Ed.: Case 24202. In Case rec180.
ords of M'assachusetts General Hospital; antemortem
5. COLLINS,
N. C., and .~SSIWX, W. E.: Fibrosarconia and postmortem rccords as used i n weekly clinimpathOf p1;iiitar tissues. .4nn. .I. Cancer 40: 465-470, 19.10.
ological exercises. New England J. Med. 218: 838-843,
G . (;I'KKY, J. I., and FUCHS,J. E.: Expectoration o f
1938; 843-845.
I~bros:ircoma:paricmt wrll 4 years 1at.er. J. Thorncic
22. M E L I C ~ W
M., M.: Primary tumors of retroperito,slug. I!): 135-137, 1'150"
nrum; clinicopathologic analysis of 162 cases; review of
7. I ) o ~ o c r - i u ~P., E.; ANDEKSI'N,
EL A,, and M c I ~ N - literature and tables of classification. J. Internat. C o l l .
.+LD, J. K.: Unusual bronchial tumors.
Ann. O l d . Surgeons 1 9 401-449, 1953.
R l i i n , b Luiyng. 65: 820-828, 1956.
23. PACK,G. T.. and ARIEL,I. M.: Fibrosarcoma of
8. I)RIXFUSS,M. L.: Congenital sarcoma. J . Pediat. soft somatic tissues; clinical and pathologic study. Surgery 31: 443-478, 1952.
583-587, 1049.
24. PRIOR,J. T., and Srsso~,B. J.: Dermal arid fascia1
9. FAHEY,J. J., and BOLLINWR,
J. A.: Congenital
Sarcoma of foot; case report and review of 1it.erature. fibromatosis. Ann. Surg. 1 3 9 453-467, 1954.
-4. M .A. A m . J . Dis. Child. 86: 23-27, 1953.
25. SHORE,B. R.: Spontaneous cure of congenital recurring connective-lissoe tumor. A7n. .I. Cancer 27:
10. FH.DMAN,P. A: Sarcoma of lung; report of 3
736-739, 1936.
rases. Brit. J. 'I'uberc. 51: 331-334, 1957.
26. SNYDE:R,'CV. H., JR.; KRUSE,C. A.; GREANEY,
11. ( . ~ w N F , W.
R , I,, and TURNKR,
0.A,: Primary
1it)rol)lastic tumors bf choroid plcxus of I;it.t.ral veil- E. M., and CIIAFFIN,I,.: Retroperitoneal tiiniors in infants and children; report of 88 cases. A . M. A . Arch.
tricks; clirticopatholo~icalstudy of 3 cases. Swg. Gy nrc.
StLTg. 65: 26-38, 1951.
i O ~ S /66:
. 804-8019, 1!).38.
27. s'rour, A. P.: Fibrosarcoma; nialignarit tunior of
12. GENI'EL'~:, 1-1.: Malignant, fibroblastic: tumors of
fibroblasts. Cancer 1: 30-63, 1948.
skin; clinical and pathological-anatomical stridies of
129 cases of malignant, fibroblastic tumors from cutane28. STOUT, A. P.: Juvenile fibromatoses. Cancer 7:
ous and suljcutancous layers observed at Radiumhein953-978, 1954.
met during period 1927-1917. Acta derrnat.-veitereol.
29. STOIJT,A. P.: Fibrous tumors of soft tissues.
wppl. 27: 1-180, 1951.
Minnesota Med. 43: 455-459, 1960.
13. GERRASI,
F. S.; MARGILETII,
A. M., and KIRLER,
30. STOUT,A. P.: Fibromatoses. Clin. Orthopaed. 19:
R. S.: Primary fibrosarcoma of lung in young child:
11-18, 1961.
case treated by lobectomy and cobalt therapy. J. fediut.
31. STOUT,A. P.: Pseudosarcomatous fasciitis i n chil5.1: 488-495, 1959.
dren. Cancer 14: 1216-1222, 1961.
14. 1fARrz. 1'. I{,, and GUEKRERO ?'AIILAN'Il?, 'l'.:
32. WARREN,
S., and SOMMEK,G. N. J., JR.: Fibrosar(:ongenital fi1)rosarcoma. Docr~m.nzrd. geog. et trop.
coma of soft parts: with special refcrence to recurxence
i:98-102, 1053.
and metastasis. Arch. Surg. 33: 425-450,1956.
1.5. EiFLKm, 1'. G.: Ein Fall von angebrbrenem Sdr33. WELLS,H. G.: Occurrence and significancc of
hom. Zentral112. Chir. 55: 842-843, 1928.
congenital malignant neoplasms. Arch. Path. 30: 53516, HOLINGFR,
P. H.; JOHNSTON, K. C.; GOSSWEILI~R,
601, 1940.
Y.. and HIRWI, E. C.: Primary fibrosarcoma of bron34. WILLIAMS,J. 0.. and SCHRUM,
D.: Congcnital
ctltls. Dk.Chest 37: 137-143, 1960.
fibrosarcoma; rcport of case i n newborn infant.
I i. HunsoN, 0. C:.: Intrd-uterine fibrosarcoma of fool;
A. A4. A. Arch. Path. 51: 548-552, 1951.
c-ase report. A m . J . Cancer 26: 568-570, 1936.
35. WIISON, D. A.: Tumors of subcutaneous tissue
18. KAPLAN,I. I.. and RI!BENFEI.D,S.: Sarcoma of
and fascia, with spccial reference to fibrosarcoma
soft tissue. A?)?..I. Iioenigenol. 37: 53-69, 19.37.
-clinical study. S7rrg. Gynec. &- Obst. 80: 500-508,
I!!. K I W A X S I ,E.: Ein Fall von angcborenem Sarkom.
19t5.