ORIGINAL PAPER

Clinicopathological study of three cases of infantilefibromatosis of the orbit

Kengo Hayashi • Nobutada Katori •

Yoshiro Otsuki • Kyoko Ohno-Matsui

Received: 10 August 2013 / Accepted: 2 February 2014

� Springer Science+Business Media Dordrecht 2014

Abstract We report the clinical and pathological

findings in three infants with infantile fibromatosis

that involved several extraocular muscles which led to

disorders of ocular motility. We also describe their

clinical features before and after surgery. This was a

retrospective interventional case study with clinico-

pathological correlations. We present three cases of

infantile fibromatosis that were diagnosed by clinical

features and histopathlogical examination of biopsy

specimens. The three patients were all female children

aged 1, 3, and 3 years at their initial visit. The orbital

tumor was unilateral in all patients. All three patients

had disorders of ocular motility because the tumors

involved C2 extraocular muscles. The margins

between the tumor and the involved extraocular

muscles were not distinct. We performed partial

resection of the tumors to preserve the extraocular

muscles. In all cases, the tumors partially remained,

but periodic postoperative magnetic resonance imag-

ing showed no enlargement of the tumors during the

follow-up period. All three patients had residual

limitations of eye movements. We should consider

the postoperative binocular function when we treat

infantile benign fibrous tumors involving the extraoc-

ular muscles.

Keywords Orbit � Infantile fibromatosis �Infantile myofibromatosis � Infantile

myofibroma � Juvenile fibromatosis

Introduction

Fibromatosis of the orbit is extremely rare, and only a

few single case reports have been published [1–5].

Thus, the clinical features of orbital fibromatosis in

children are not well known. Orbital fibromatosis is

usually treated by surgical excision; however, some

cases have been reported to have recurrences after

local excision [1] while other cases have been reported

to have no recurrence after wide excision [1, 2, 4].

Nevertheless, reference has not been made whether the

extensive excision should include the extraocular

muscles. Furthermore, there has been no report about

the postoperative eye position, eye movements, and

binocular vision after wide excision surgery. We

K. Hayashi (&) � K. Ohno-Matsui

Department of Ophthalmology and Visual Science, Tokyo

Medical and Dental University, 1-5-45 Yushima,

Bunkyo-ku, Tokyo 113-8519, Japan

e-mail: kengoh@mist.ocn.ne.jp

N. Katori

Department of Ocular Plastic & Orbital Surgery, Seirei

Hamamatsu General Hospital, 2-12-12 Sumiyoshi,

Naka-ku, Hamamatsu 430-8558, Japan

Y. Otsuki

Department of Pathology, Seirei Hamamatsu General

Hospital, 2-12-12 Sumiyoshi, Naka-ku,

Hamamatsu 430-8558, Japan

123

Int Ophthalmol

DOI 10.1007/s10792-014-9915-y

report our findings on three infants with infantile

fibromatosis that involved several extraocular muscles

which led to disorders of ocular motility. We also

describe the magnetic resonance imaging (MRI)

characteristics, histopathological findings, and clinical

features of the three patients before and after surgery.

Patients and methods

This was a retrospective interventional case study with

clinicopathological correlations. Approval of this

study was obtained from the Institutional Review

Board of Seirei Hamamatsu General Hospital, and

informed consent for examinations and surgery was

obtained from the parents.

We reviewed the medical records of the Depart-

ment of Ocular Plastic and Orbital Surgery of Seirei

Hamamatsu General Hospital in Japan for children

with an orbital tumor who were seen between 2003

and 2012. We found three cases that were diagnosed

with infantile fibromatosis by clinical features and

histopathlogical examination of biopsy specimens.

We present our findings of these three cases.

Results

The clinical features of the three infants with infantile

fibromatosis are summarized in Table 1. The three

patients were all female children aged 1, 3, and 3 years

at their initial visit. The orbital tumor was unilateral in

all patients. The medical histories were unremarkable

for tumors and ocular problems. They had no history

of trauma to the head or orbit. There was no family

history of tumors or other ocular or systemic disorders.

Clinical examination revealed no signs of infection.

General physical examination including the chest and

abdomen imaging revealed no other abnormalities.

The initial signs were proptosis at birth in Cases 1

and 2, disorders of ocular motility in Cases 2 and 3,

and ciliary entropion with lower eyelid retraction in

Case 1. MRI showed all the lesions had a low iso-

intensity signal relative to that of the extraocular

muscles on T1-weighted images (T1WI) and T2WI.

The lesions were homogeneously enhanced in the

T1WI after injection of gadolinium (Gd-DPTA).

Computer tomography (CT) revealed no bony destruc-

tion. In all three cases, the tumors involved C2Ta

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Int Ophthalmol

123

extraocular muscles, and the margins between the

tumor and the involved extraocular muscles were not

distinct.

We performed partial resection of the tumor to

preserve the extraocular muscles. The tumors in Cases

1 and 2 were located in the inferior orbit which led to

lower eyelid retraction and ciliary entropion. We

performed a modified Hotz procedure with recession

of the lower eyelid retractor (LER) simultaneously.

The ciliary entropion in these two cases improved.

The follow-up periods were 36, 46, and 90 months.

In all cases, part of the tumor remained, but periodic

postoperative MRI showed no enlargement of the

tumor during the follow-up period. All three patients

had residual limitations of eye movements. Case 2

developed amblyopia.

Case reports

Case 1

Case 1 was a 37-month-old female child who

presented with slight proptosis which had been

detected at birth and ciliary entropion from lower

eyelid retraction in her left eye (Fig. 1a, b). The

proptosis of the left eye was 2 mm at the first visit.

Ophthalmoscopy showed that the fundus was normal

in both eyes. MRI showed a 1.8 9 1.3 9 1.2 cm soft

tissue mass in the inferomedial quadrant of the left

orbit. The margin between the mass and medial rectus

(MR) muscle and inferior rectus (IR) muscle was not

distinct. The mass had a low iso-intensity signal

compared with the extraocular muscles in the T1WI

and T2WI which were enhanced homogeneously by

Gd-DPTA. The optic nerve was displaced superolat-

erally (Fig. 1c, d).

Medial orbitotomy was performed at 3 years of age.

On intraoperative observation, the tumor was firm,

pinkish-white, and nonencapsulated. The margins

between the tumor and the two extraocular muscles

were not distinct, and the tumor could not be

completely removed. Incisional biopsy of the tumor

between the MR and IR muscles was performed. A

modified Hotz procedure together with a recession of

the lower eyelid retractor was performed for the ciliary

entropion with lower eyelid retraction. The second and

third partial resection surgeries were performed at 4

and 5 years of age. A complete resection of the tumor

could not be performed at the first surgery because the

tumor involved the ocular muscles.

At the last examination when the patient was

6 years old and 3 years after the primary surgery, her

best-corrected visual acuity (BCVA) was 1.2 in both

eyes. The primary eye position was orthophoria, and

stereopsis was present as determined by the Titmus

stereo test. The ciliary entropion of the lower eyelid

was improved, but she had a severe limitation of

upward movements in the left eye (Fig. 2). MRI

showed no enlargement of the residual tumor.

Case 2

Case 2 was a 22-month-old female child who

presented with proptosis which had been detected at

birth. There was a limitation of ocular motility in

the upward and lateral directions in the left eye

(Fig. 3a, b). At 1 month old, CT examination in

another hospital showed a tumor in the left orbit

without bony destruction. A general physical exami-

nation including imaging revealed no other abnormal-

ities. At 2 years of age, her BCVA was 1.2 in the right

eye and 0.06 in the left eye. The refractive error

(spherical equivalent) was ?5.50 D in the left eye and

?0.50 D in the right eye. Thus, there was a hyperopic

anisometropia of 5.00 D. The color of the left disc was

already pale which suggested optic nerve atrophy had

developed probably due to compression by the orbital

tumor. We were not able to determine whether the

reduced BCVA in the left eye was due to anisometric

amblyopia or to the optic nerve atrophy.

MRI showed a 2.3 9 1.5 9 1.4 cm soft tissue mass

in the medial half of the left orbit involving the

superior rectus (SR), superior oblique (SO), MR, and

IR muscles (Fig. 3c–f). The lesion had a low iso-

intensity signal compared with the extraocular mus-

cles in the T1WI and T2WI, and the lesion was

enhanced homogeneously by Gd-DPTA. Periodic

MRI showed no enlargement of the lesion, and CT

revealed no bone erosion. Patching for 1 h/day was

performed since the age of 2 years assuming that the

reduced BCVA was due to amblyopia. At 4 years of

age, the patient was esotropic, the proptosis had

disappeared, and the eye was enophthalmic.

At 7 years of age, the globe was shifted inferome-

dially, and the patient had severely limited upward and

lateral gaze. The enophthalmos was more prominent.

Int Ophthalmol

123

Fig. 1 Findings in a 3-year-old female infant (Case 1) at the

initial visit. a Frontal photograph showing slight proptosis of the

left eye. b Frontal photograph of the left eye shows ciliary

entropion with lower eyelid retraction. c Coronal T1-weighted

MRI showing a mass of low iso-intensity signals compared with

extraocular muscles in the inferomedial quadrant. The mass was

continuous with the medial rectus muscle and inferior rectus

muscle of the left orbit. The optic nerve is displaced

superolaterally. d Coronal T1-weighted MRI after intravenous

gadolinium shows that the lesion is homogeneously enhanced

Fig. 2 Ocular motility 3 years after the primary surgery of Case 1 shows the residual limitation in upward gaze of the left eye. Primary

eye position is orthophoria

Int Ophthalmol

123

She gradually developed ciliary entropion with left

lower eyelid retraction (Fig. 4).

We performed medial orbitotomy with frontal bone

osteotomy and eyelid retraction for the lower ciliary

entropion. On intraoperative observation, the tumor

was firm, white, nonencapsulated, and involved the

four extraocular muscles. The margins between the

tumor and muscles were not clear. A partial resection

of the lesion between the SO and IR muscles was made

to preserve the extraocular muscles. We performed a

modified Hotz procedure with recession of the LER

simultaneously for the ciliary entropion.

At the last examination, she was 9 years of age

(2 years after the surgery and 7 years after her first

visit). Her BCVA was 1.2 in the right eye and 0.1 in the

left eye. The high hyperopic anisometropic and

strabismic amblyopia in her left eye remained.

Binocular function did not develop, and stereopsis

was not found. The left eye was displaced downward

and medially with an enophthalmos of 3 mm (Fig. 5).

The primary eye position was left hypotropia with

esotropia—3 prism diopters of hypotropia and 14

prism diopters of esotropia. She had severe limitation

of upward and lateral gaze in the left eye. MRI showed

no enlargement of the residual mass at the postoper-

ative follow-up examination 2 years later.

Case 3

Case 3 was a 44-month-old female child who had

presented with ocular motility in the right eye

which her mother first noted when she was 2 years

old. Her BCVA was 1.2 in both eyes. The primary

eye position was orthophoria, and stereopsis was

Fig. 3 Findings in a 1-year-old female child (Case 2) at the

initial visit. a Photograph of primary eye position shows

orthophoria. b Photograph shows disorder of ocular motility to

upward gaze of the left eye. c Coronal T1-weighted MRI shows

a large mass of low iso-intensity signals compared with

extraocular muscles in the medial half of the left orbit that

included the superior rectus muscle, superior oblique muscle,

medial rectus muscle, and inferior rectus muscle. d Coronal

T2-weighted MRI shows a wide lesion of low-intensity signals

in the medial half of the left orbit. e Coronal T1-weighted MRI

after intravenous gadolinium showing that the lesion is

homogeneously enhanced. f Axial T1-weighted MRI after

intravenous gadolinium shows that the lesion extends widely

and deeply in the orbit. There is a slight proptosis of the left eye

because of the tumor

Int Ophthalmol

123

present as determined by the Titmus stereo test.

Neither proptosis nor enophthalmos was present,

and ophthalmoscopy showed that the fundus of

both eyes were normal. She had a slight limitation

of lateral movements and mild ptosis in the right

eye. MRI showed a 1.4 9 1.0 9 1.0 cm soft tissue

mass in the superomedial quadrant of the right orbit

which included the SR, SO, and MR muscles.

The lesion had a low iso-intensity signal compared

with the extraocular muscles in the T1WI

and T2WI which were enhanced homogeneously

by Gd-DPTA.

At 6 years of age, she underwent medial orbitot-

omy. Intraoperatively, the tumor was firm, white,

nonencapsulated, and involved three extraocular mus-

cles. The margin between the mass and the muscles

was not distinct. We performed partial resection of the

lesion between the SO and MR muscles to preserve the

extraocular muscles.

At the last examination 9 months after the surgery,

her BCVA was 1.2 in the right eye, the primary eye

position was orthophoria, and stereopsis was present.

However, she had a residual mild limitation of lateral

movements in her left eye.

Histopathological findings

The tumors of the three cases showed similar

pathological features, which were ill-defined, scar-

like lesions that extended into the extraocular

muscle fibers (Fig. 6). All the lesions were com-

posed of fibroblast-like, spindle-shaped cells sur-

rounded by dense collagen matrix. The spindle

cells did not have malignant features, such as

cellular atypia, pleomorphism, and mitotic activity.

The cellularity of the lesions was relatively low. In

addition, the specimen obtained at 5 years of age

from Case 1 (Fig. 6i–l) was more fibrous with

lower cellularity than the specimens at 3 and

4 years of age (Fig. 6a–h). Inflammatory cells were

very sparse in all of the specimens.

Immunohistochemical stainings were not available

for the pathological diagnosis. However, the spindle

cells were focally positive for the estrogen receptor in

Cases 1 and 3.

Pathologically, we diagnosed these lesions as

benign fibrous lesions. We concluded that the lesions

of the three cases were infantile fibromatosis by the

clinical and pathological features.

Fig. 4 Preoperative photograph of Case 2 at 7 years of age. The left globe is enophthalmic. The ocular motility of the left eye is

severely limited in the upward and lateral directions. Ciliary entropion with left lower eyelid retraction can be seen

Int Ophthalmol

123

Discussion

Infantile fibromatosis represents the childhood coun-

terpart of abdominal or extra-abdominal fibromatosis.

It most commonly presents \2 years of age, and

typically originates in the muscles of the head and

neck, shoulder, and upper arm [6]. This lesion is so

aggressive locally that it can infiltrate and replace the

surrounding tissues such as muscle and fat. It tends to

recur locally, when inadequately excised, but not to

metastasize.

Infantile fibromatosis of the orbit is exceedingly

rare, and it has been rarely reported in the English

literature [7]. In addition, most earlier reports are of

single cases [1–5]. Therefore, clinical features of

orbital fibromatosis are not well known.

Our three cases of orbital infantile fibromatosis had

some common clinical characteristics. All cases had

disorders of ocular motility. Cases 1 and 2 had tumors

located in the inferior orbit, and both had ciliary

entropion with lower eyelid retraction. Both cases also

had proptosis at birth which gradually disappeared,

and in Case 2, an enophthalmos gradually developed.

We suggest that these findings resulted from a

retraction of the extraocular muscles and lower eyelid

due to the tumor.

Infantile fibromatosis has a wide morphological

spectrum reflecting the progressive stage in the

differentiation of the fibroblasts. There are two types

of infantile fibromatosis—diffuse type and desmoid

type [6]. The diffuse type has characteristic features of

primitive fibroblasts proliferating and separating stri-

ated muscles fibers. The desmoid type is histologically

indistinguishable from an adult extra-abdominal fibro-

matosis [8]. Fibroblasts infiltrate into muscle fibers,

and collagen matrix increases between the muscle

fibers. The morphological features of our three cases

appear to be consistent with the desmoid type.

Differential diagnoses of our cases are summarized

in Table 2 [6, 9, 10]. Differential diagnoses of our

cases should include a reactive fibrosis due to inflam-

mation and trauma, because the pathological findings

showed that the cellularity is relative low. Trauma is

reported as the cause of the abdominal and extra-

abdominal fibromatosis. Considering the cause of

fibromatosis coli, which is a benign fibrous prolifer-

ation of the sternocleidomastoid muscle, birth injury

may play a role in the pathogenesis. However, our

cases have no clinical history of trauma during

pregnancy and labor as well as after their birth.

Therefore, fibrosis due to reactive inflammation is

clinically excluded.

The appropriate treatment for infantile fibromatosis

has been considered to be wide excision of the lesion

because of the high tendency to recur [1]. When a

complete resection is not possible, conservative

Fig. 5 Postoperative photograph of Case 2 at 9 years of age.

a Photograph of the primary eye position shows the left eye is

displaced downward and medially. b Photograph shows a severe

limitation of ocular motility to upward gaze of the left eye.

c Photograph shows ocular motility to downward direction of

left eye is present. d Photograph taken from lower part shows

prominent left enophthalmos

Int Ophthalmol

123

debulking surgery should be considered [11, 12].

Harstein et al. [11] reported that the anatomical

location of orbital desmoid tumors did not allow wide

surgical margins, therefore they performed incisional

biopsy. Our findings are consistent with their report.

Some cases have been reported that did not have a

recurrence after wide excision of the orbital fibroma-

tosis or myofibroma in children [1, 2]. However, there

has been no report concerning whether a wide

excision including the extraocular muscles should be

Fig. 6 Histopathological examination of biopsies of all cases.

(a–l) Case 1 (a–d) Biopsy specimen collected at 3 years of age.

(e–h) Biopsy specimen collected at 4 years of age. (i–l) Biopsy

specimen collected at 5 years of age. (m–p) Case 2. (q–t) Case

3. (a, c, e, g, i, k, m, o, q, s) Hematoxylin & eosin staining. (b, d,f, h, j, l, n, p, r, t) Azan staining; the region stained red shows

muscle fiber, and blue shows collagenous structure. In all

images, the arrows indicate muscle fiber; asterisks indicate

collagenous structure. (a, b, e, f, i, j, m, n, q, r) Low-power

views showing collagen matrix and diffuse infiltrating into the

muscle fibers. (c, d, g, h, k, l, o, p, s, t) High-power views

showing spindle-shaped fibroblasts surrounded by an abundant,

dense collagen matrix. The fibrous tissue can be seen to extend

into the muscle fibers. The specimen from the second surgery

(e–h) is more fibrous than the specimen from the first surgery

(a–d). In addition, the specimen from the third surgery (i–l) is

more fibrous than that from the second surgery (e–h). (Original

magnification: a, b, e, f, i, j, m, n, q, r 9 30; c, d, g, h, k, l, o, p, s,

t 9 150)

Int Ophthalmol

123

performed. Furthermore, there is no report on the

postoperative eye position, eye movements, and

binocular vision after wide excision surgery.

Abdominal and extra-abdominal fibromatosis of

adulthood occurs in women 3–4 times more frequently

than in men [11, 13]. One- to two-thirds of these cases

of fibromatosis have been reported to express estrogen

receptor [14]. The successful administration of tamox-

ifen or similar anti-estrogen chemotherapeutic agents

for these patients with fibromatosis of adulthood has

been recently reported [15]. On the other hand, the

effects of anti-estrogen therapy for infantile fibroma-

tosis are not well known. However, Lackner et al. [16]

reported two childhood cases for which tamoxifen

administration was effective in treating unresectable

extra-abdominal fibromatosis. All of our cases were

female children, and in two of three cases, the tumor

cells showed a weak expression of estrogen receptors.

Although anti-estrogen therapy might be effective for

these patients, it is still an unapproved medical

treatment and may have serious side-effects. A large

trial is necessary to determine the efficacy of anti-

estrogen therapy.

We reported three cases of infantile fibromatosis

involving several extraocular muscles which caused a

disorder of ocular motility. Our long-term follow-up

clinical findings suggest that we should consider the

postoperative binocular function when we treat infan-

tile benign fibrous tumor involving the extraocular

muscles.

Acknowledgments The authors thank Dr. Duco I. Hamasaki

Professor Emeritus, Bascom Palmer Eye Institute, Department

of Ophthalmology University of Miami, School of Medicine

Miami, Florida, for his critical discussions and final manuscript

revisions.

References

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thalmol 75:313–316

3. Waeltermann JM, Huntrakoon M, Beatty EC Jr, Cibis GW

(1988) Congenital fibromatosis (myofibromatosis) of the

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(1999) Orbital (desmoid type) fibromatosis. Orbit 18:

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Hodgkins P (2006) Fibromatosis (desmoid tumor) involving

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Table 2 Different diagnoses of infantile fibromatosis: clinical

and histopathological features

Different diagnoses Clinical and histopathological

features

Infantile myofibroma/

infantile

myofibromatosis

Pathological findings are similar

to those of infantile

fibromatosis. However,

immunohistochemically they

are often positive for desmin

and smooth muscle actin, which

were not found in our cases

Fibrous histocytoma Pathological findings are similar

to those of infantile

fibromatosis. However, it often

has rounded histiocytes and

multinucleated giant cells

accompanied by spindle cells

Solitary fibrous tumor

(SFT)

SFTs have spindle cell

proliferation in the collagenous

background. However, SFTs

have relatively well-

circumscribed margins with a

characteristic

hemangiopericytomatous

pattern

Infantile fibrosarcoma It often shows moderate to high

cellularity, fascicles of

monomorphic spindle

fibroblastic cells in a uniform

herringbone pattern, cellular

atypia, mitotic figures,

hemorrhage, and necrosis. Our

cases did not have these

features.

Int Ophthalmol

123

12. Westfall AC, Mansoor A, Sulliyan SA, Wilson DJ, Dailey

RA (2003) Orbital and periorbital myofibromas in child-

hood: two case reports. Ophthalmology 110:2000–2005

13. Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig

EB (1990) Intra-abdominal fibromatosis. A pathologic

analysis of 130 tumors with comparison of clinical sub-

groups. Am J Surg Pathol 14:335–341

14. Coffin CM, Dehner LP, O’Shea PA (1997) Fibroblastic-

myofibroblastic tumors. Pediatric Soft Tissue Tumors.

Williams & Wilkins, Baltimore, pp 155–161

15. Hansmann A, Adolph C, Vogel T, Unger A, Moeslein

G (2004) High-dose tamoxifen and sulindac as first-

line treatment for desmoid tumors. Cancer 100:

612–620

16. Lackner H, Urban C, Kerbl R, Schwinger W, Beham A

(1997) Noncytotoxic drug therapy in children with unre-

sectable desmoids tumors. Cancer 80:334–340

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