Skeletal Radiology

https://doi.org/10.1007/s00256-021-03836-2

REVIEW ARTICLE

Update of pediatric soft tissue tumors with review of conventional

MRI appearance—part 1: tumor‑like lesions, adipocytic tumors,
fibroblastic and myofibroblastic tumors, and perivascular tumors
Jack Porrino1 · Khalid Al‑Dasuqi1 · Lina Irshaid2 · Annie Wang1 · Kimia Kani3 · Andrew Haims1 · Ezekiel Maloney4

Received: 17 December 2020 / Revised: 22 May 2021 / Accepted: 2 June 2021

© ISS 2021

Abstract
There are numerous soft tissue tumors and tumor-like conditions in the pediatric population. Magnetic resonance imaging is
the most useful modality for imaging these lesions. Although certain soft tissue lesions exhibit magnetic resonance features
characteristic of a specific diagnosis, most lesions are indeterminate, and a biopsy is necessary for diagnosis. We provide
a detailed update of soft tissue tumors and tumor-like conditions that occur in the pediatric population, emphasizing each
lesion’s conventional magnetic resonance imaging appearance, using the recently released 5th edition of the World Health
Organization Classification of Soft Tissue and Bone Tumors as a guide. In part one of this review, pediatric tumor-like lesions,
adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors are discussed. In part two, vascular
lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and
undifferentiated small round cell sarcomas are reviewed. Per the convention of the WHO, these lesions involve the connective,
subcutaneous, and other non-parenchymatous-organ soft tissues, as well as the peripheral and autonomic nervous system.

Keywords Pediatric · Soft tissue mass · Tumor · Sarcoma · Vascular malformation

Introduction
* Jack Porrino
jack.porrino@yale.edu
There are numerous soft tissue tumors and tumor-like condi-
Khalid Al‑Dasuqi tions that may present in the pediatric population. Magnetic
khalid.aldasuqi@yale.edu
resonance imaging (MRI) is the most useful modality for
Lina Irshaid imaging these soft tissue lesions as a result of its detailed
lina.irshaid@yale.edu
anatomic portrayal of the mass, including its relation to adja-
Annie Wang cent anatomic structures such as nerves, arteries, fascia, and
annie.wang@yale.edu
periosteum [1, 2].
Kimia Kani Certain soft tissue lesions may exhibit MR imaging fea-
kimia.kani@umm.edu
tures characteristic enough to allow for diagnosis without
Andrew Haims biopsy. However, soft tissue lesions are often indetermi-
andrew.haims@yale.edu
nate based on clinical examination and MRI, and biopsy is
Ezekiel Maloney required for a diagnosis [1, 3]. While imaging can suggest
eze@uw.edu
the diagnosis of more aggressive pediatric soft tissue tumors,
1
Yale Radiology and Biomedical Imaging, 330 Cedar Street, it is rarely definitive. The overall success of differentiating
New Haven, CT 06520, USA a benign from malignant soft tissue mass on MRI varies
2
Yale School of Medicine Department of Pathology, 333 from 30 to 90% in the literature [4]. Malignant lesions are
Cedar Street, New Haven, CT 06520, USA more likely to exceed 5 cm in size, show central necrosis
3
Department of Radiology, University of Maryland Medical and appear heterogenous, and are also more often deep. As
Center, 22 S Greene St, Baltimore, MD 21201, USA a general rule, most soft tissue sarcomas are hypointense
4
Seattle Children’s Hospital, 4800 Sand Point Way NE, on T1, hyperintense on T2-weighted imaging, and exhibit a
Seattle, WA 98105, USA pseudocapsule [4]. Notably, MRI for the evaluation of a soft

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 Skeletal Radiology

tissue mass is often performed utilizing T2-fat-suppressed
and short tau inversion recovery fluid sensitive sequences. In
this regard, signal is routinely described in relation to mus-
cle. Tumor enhancement and perilesional soft tissue edema
are not of particular value in differentiating benign from
malignant [4]. MRI determines the extent of a lesion, which
helps in assessing resectability. Accurate diagnosis requires
a combination of light microscopy, immunohistochemical
analysis, and molecular genetics [4].
We provide a detailed update of soft tissue tumors and
tumor-like conditions that occur in the pediatric population,
emphasizing each lesion’s conventional magnetic resonance
imaging appearance, using the recently released 5th edition
of the World Health Organization Classification of Soft Tis-
sue and Bone Tumors as a guide [5]. Many new entities
and tumor subtypes, with newly recognized molecular and
genetic alterations, are detailed in the 2020 WHO classifi-
cation, and these are highlighted where appropriate in our
descriptions. For more information on updates provided in
the 2020 WHO classification, we direct readers to a sum-
mary by Choi et al. [6].
Beyond our own category of “tumor-like” lesions
(hematoma, abscess), our review categorizes lesions by
WHO convention, including adipocytic tumors, fibroblas-
tic and myofibroblastic tumors, perivascular and vascular
lesions, fibrohistiocytic tumors, muscle tumors, peripheral
nerve sheath tumors, tumors of uncertain differentiation,
and selected undifferentiated small round cell sarcomas
as summarized in Table 1. In selecting specific tumors
for inclusion in our discussion, we relied on institutional
experience, past literature addressing this topic, and the
WHO-defined epidemiology of each tumor. Genetic tumor
syndromes with characteristic soft tissue tumors are also
integrated into the discussion, including Li-Fraumeni
syndrome, neurofibromatosis types 1 and 2, PHACE
(Posterior fossa and supratentorial brain malformations,
Hemangioma of face and neck, Arterial abnormalities,
Cardiovascular defects, Eye abnormalities, supraumbili-
cal raphe, and sternal clefts/defects), LUMBAR (Lower
body infantile hemangiomas and other cutaneous defects,
Urogenital anomalies and ulceration, Myelopathy, Bony

Table 1  Overview of WHO classes of pediatric soft tissue tumors, excluding vascular lesion subsection
Adipocytic tumors Fibroblastic and myofibroblastic Muscle tumors Tumors of uncertain differentiation
tumors

Lipoma (part 1, Fig. 2) Nodular fasciitis (part 1, Fig. 6
and 7)
Lipomatosis (part 1, Fig. 3) Proliferative fasciitis and myositis Fetal rhabdomyoma
Lipoblastoma and lipoblastomato- Myositis ossificans (part 1, Fig. 8) Embryonal rhabdomyosarcoma
sis (part 1, Fig. 4)
Myxoid and myxoidpleomorphic Fibrous hamartoma of infancy
liposarcoma (part 1, Fig. 5) (part 1, Fig. 9)
Fibromatosis colli
Fibrohistiocytic tumors Juvenile hyaline fibromatosis
Plexiform fibrohistiocytic tumor Inclusion body fibromatosis
Calcifying aponeurotic fibroma
(part 1, Fig. 10)
Perivascular tumors Gardner fibroma
Myopericytoma, including infan- Desmoid fibromatosis (part 1,
tile myofibroma (part 1, Fig. 17) Fig. 11)
Lipofibromatosis (part 1, Fig. 12)
Giant cell fibroblastoma (part 1,
Fig. 13)
Dermatofibrosarcoma protruber-
ans (part 1, Fig. 13)
Inflammatory myofibroblastic
tumor (part 1, Fig. 14)
Infantile fibrosarcoma (part 1,
Fig. 15)
Low-grade fibromyxoid sarcoma
(part 1, Fig. 16)
EBV-associated smooth muscle NTRK-rearranged spindle cell
tumor neoplasm
Synovial sarcoma (part 2, Fig. 9)
Epithelioid sarcoma (part 2,
Fig. 10)
Alveolar rhabdomyosarcoma (part Alveolar soft part sarcoma (part 2,
2, Fig. 5) Fig. 11)
Spindle cell/sclerosing rhabdo- Desmoplastic small round cell
myosarcoma tumor
Ectomesenchymoma Extrarenal rhabdoid tumor
Peripheral nerve sheath tumors Undifferentiated small round cell
sarcomas
Schwannoma Round cell sarcoma with EWSR1-
non-ETS fusions
Neurofibroma (part 2, Figs. 6 CIC-rearranged sarcoma
and 7)
Benign triton tumor/neuromuscu- Extraskeletal Ewing sarcoma
lar choristoma
Malignant peripheral nerve sheath Sarcoma with BCOR alterations
tumor (part 2, Fig. 8) (part 2, Fig. 12)

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Skeletal Radiology

deformities, Anorectal malformations, Arterial anomalies,
Renal anomalies), and overgrowth spectrum syndromes.
In part one of this review, pediatric tumor-like lesions,
adipocytic tumors, fibroblastic and myofibroblastic tumors,
and perivascular tumors are discussed.
Tumor‑like lesions
Hematoma
Hematomas can occur as a result of trauma or may be spon-
taneous, such as that seen in those on anticoagulant therapy
or with an underlying soft tissue neoplasm [7].
Hematomas exhibit variable signal on MRI based on the
age of the lesion, as a result of hemoglobin breakdown [7, 8].
The best discriminatory feature is that of increased signal on
T1-weighted imaging as a result of methemoglobin, which
is seen during the early and late subacute phase, as well as
low signal on T1- and T2-weighted imaging within the wall
of the lesion in the late phase as a result of hemosiderin.
Additionally, if gradient echo imaging is performed, there
is characteristic signal drop or blooming. While hematomas
may exhibit peripheral enhancement, they should not dem-
onstrate central enhancement [7].
To assist with differentiation of a hematoma from a soft
tissue neoplasm, a history of trauma or predisposition to
bleeding should be investigated. Hematomas should progres-
sively decrease in size as well [7]. The caveat being that of a
chronic expanding hematoma, which can persist long after
the initial trauma, and may present as a slowly enlarging
mass [9].
Abscess
On conventional MRI, an abscess classically presents as a
well-defined fluid collection, hypointense on T1 and hyper-
intense on T2 weighted imaging, with an enhancing rim.
Restriction is commonly seen on the advanced MRI tech-
nique of diffusion-weighted imaging. However, depending
on the abscess content, specifically the presence of blood
or protein within the abscess, the signal characteristics may
vary [8, 10, 11] (Fig. 1).
The penumbra sign is an area of relatively hyperintense
signal between the intermediate to low signal intensity
abscess cavity and the adjacent edematous or sclerotic bone

Fig. 1  Abscess. A 5-year-old

male with knee cellulitis, severe
tenderness, and inability to
bear weight. Axial T1 (A),
T2-fat-suppressed (B), T1-fat-
suppressed (C), and T1-fat-
suppressed post-intravenous
gadolinium (D) MR images
of the knee demonstrate a rim
enhancing soft tissue abscess
within the subcutaneous fat of
the posterior knee (arrow)

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marrow on unenhanced T1-weighted imaging. The sign is
also useful for abscesses within the soft tissue [12].
Purely cystic lesions
There are a variety of truly cystic soft tissue lesions, which
include synovial cysts/ganglia, bursae, skin appendage
lesions/epidermal inclusion cysts, and lymphatic malfor-
mations [13].
A purely cystic lesion, such as a ganglion/synovial cyst,
parameniscal cyst, post-operative seroma, or lymphocele, is
homogeneously low in signal on T1 and exhibits increased
signal on T2-weighted imaging, and with a thin rim of
peripheral enhancement but no internal enhancement [8, 13].
The presence of thick and irregular peripheral rim
enhancement suggests alternative diagnoses, such as an
inflamed or infected ganglion cyst, abscess, hematoma, or
necrotic neoplasm. If internal enhancement is present in a T2
hyperintense lesion, differential considerations include soft
tissue masses such as myxoma, myxoid sarcoma, peripheral
nerve sheath tumor, and synovial sarcoma [13]. Notably,
pre-contrast T1 images can be vital in differentiating internal
enhancement from intrinsic high T1 signal such as that seen
with proteinaceous or hemorrhagic cysts.
Adipocytic tumors
The presence of internal macroscopic fat allows for a lim-
ited differential diagnosis. Furthermore, lesions with internal
macroscopic fat are usually benign [14]. Notably, fat is not
only present in adipocytic tumors, but can also be seen in
fibroblastic/myofibroblastic tumors, vascular anomalies, and
other mass-like lesions.
Lipoma
The lipoma is a benign tumor comprised entirely of mature
fat and can occur anywhere in the body where fat is normally
found, most commonly at the upper back, neck, proximal
extremities, and abdomen. They can occur in the subcuta-
neous soft tissue or may be deep within the inter- or intra-
muscular compartments, beneath the muscle investing fas-
cia. Lipoma is the most common fatty tumor in children and
adolescents and accounts for approximately two-thirds of
adipocytic tumors in the first two decades of life. The signal
is that of fat on all MR pulse sequences, occasionally with
a low signal thin fibrous capsule. If a capsule is not present,
the lesion may be impossible to distinguish from the adja-
cent fat. Thin (< 2 mm) internal septa may enhance, but as
a general rule, there should be no significant enhancement
following contrast administration. Notably, intramuscular
lipomas can be well-circumscribed and encapsulated or
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Skeletal Radiology
infiltrative insinuating between muscle fibers and creating a
striated appearance [3, 8, 14, 15] (Fig. 2).
Lipomatosis
Lipomatosis is defined by overgrowth of adipose tissue.
This may involve any part of the body in regional fashion,
including nerves (this is classified separately as “lipomatosis
of nerve” by the WHO), or may be diffuse [5]. Underly-
ing etiologies of lipomatosis in children include exogenous
steroid therapies, increased endogenous steroid produc-
tion, liver disease, genetic disorders, or may be idiopathic.
“Congenital lipomatosis,” including lipomatosis of nerve,
can be seen in young children with overgrowth syndromes.
Understanding of the mechanistic pathway underlying the
overgrowth disorder spectrum has expanded significantly
in recent years, and these can be grouped by the portion(s)
of the “PI3K-AKT-mTOR pathway” where somatic activat-
ing mutations occur, as detailed in Table 2 [16–18]. Genes
in this pathway include those named, as well at PTEN and
TSC1/2, and ultimately control cell growth and metabolism.
Some genes have stronger expression in different parts of the
body (e.g., AKT3 is expressed most strongly in the nerv-
ous system), while others have more widespread expression
throughout the body (e.g., AKT1, PIK3CA, and mTOR) and
thus can have more variable and widespread effects when
dysregulated. Imaging can help to define the extent of fat
accumulation and any localized mass effects, to characterize
additional features of associated syndromes, and to exclude
other causes of clinically identified masses (Fig. 3).
Lipoblastoma and lipoblastomatosis
Lipoblastoma is a benign tumor that differs from lipoma
in that it is composed of both mature and immature adi-
pocytes. Additionally, lipoblastoma is almost exclusively
found within the first 3 years of life and most commonly
within the extremities and trunk. The tumor can be well-
circumscribed, superficial, and encapsulated (lipoblastoma)
or infiltrative non-encapsulated, growing in deeply situated
adipose (lipoblastomatosis). The tumor can be found in the
subcutaneous tissue, or may be deep seated with extension
into the musculature. The tumor contains variable amounts
of fibrous septa and myxoid matrix, which determines the
MR appearance. Some lesions contain very little fat, and
therefore appear heterogenous on MRI with enhancing soft
tissue components. Typically, lipoblastoma cannot be differ-
entiated from liposarcoma on MRI, although liposarcoma is
exceedingly rare in children younger than 5 years and tumors
with features of lipoblastoma can be comfortably diagnosed
as such in this setting [8, 14, 15] (Fig. 4). A minority of pedi-
atric patients with lipoblastoma have additional central nerv-
ous system disorders, congenital malformations, or familial
Skeletal Radiology

Fig. 2  Lipoma. A 16-year-old

male with right hip pain. Axial
T1 (A), T2-fat-suppressed
(B), T1-fat-suppressed (C),
and T1-fat-suppressed post-
intravenous gadolinium (D)
MR images through the right
hip demonstrate a large,
well-defined soft tissue mass
following fat signal on all pulse
sequences, with internal septa-
tion but no internal enhance-
ment, abutting the anterior
aspect of the right femoral
head and neck. Grossly (E), the
tumor is well circumscribed and
exhibits a homogenous, yellow
cut surface with no areas of
hemorrhage or necrosis. Micro-
scopic examination (F) reveals
sheets of mature adipocytes
with small, bland, peripherally
located nuclei (^) and scattered
small-sized vessels (arrow)

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 Skeletal Radiology

Table 2  Clinical overgrowth

disorders associated with the
PI3K-AKT-mTOR pathway
PI3KCA related
Congenital infiltrating lipomatosis of the face (CILF)
Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal
(CLOVES)
Fibroadipose overgrowth
Megalencephaly-capillary malformation (MCAP)
Hemihyperplasia multiple lipomatosis (HHML)
Macrodactyly
Muscle hemihypertrophy
Skin disorders (e.g., benign lichenoid keratosis, epidermal nevi, and seborrheic keratosis)
Hemimegalencephaly
PTEN related
Bannayan-Riley-Ruvalcaba syndrome
Cowden syndrome
Lhermitte-Duclos disease
AKT related
Proteus syndrome
Megalencephaly polymicrogyria polydactyly hydrocephalus (MPPH) syndrome
Lipodystrophy syndrome — Hypoglycemia
Hemimegalencephaly (also mTOR-related)
Focal cortical dysplasia (also mTOR-related)
TSC1/2 related
Tuberous sclerosis complex

lipomas potentially related to chromosome 8q alterations,
including the PLAG1 gene [19–21].
Liposarcoma
Liposarcoma implies a malignant tumor with adipocytic
differentiation; however, there are five histologic subtypes
described by the WHO that alter the imaging appearance,
including the well-differentiated (preferred term for tumors
in deep anatomical sites, such as the retroperitoneum, medi-
astinum, or spermatic cord)/atypical lipomatous tumor (pre-
ferred term for lesions arising at anatomical sites that are
amenable to curative, complete surgical resection, such as
the extremities), myxoid, dedifferentiated, pleomorphic, and
myxoid pleomorphic forms (a new and distinct variant per
the 2020 WHO classification) [5, 22].
Although a common mesenchymal neoplasm in adults,
liposarcoma is rare in children, with only 0.7% of cases
occurring in those under the age of 16 years old. The major-
ity of fatty tumors in those younger than 3 years of age are
lipoblastoma, while the majority in those over the age of
3 years are lipomas. Almost all cases of liposarcoma in chil-
dren are of the myxoid subtype, which has myxoid stroma
and little mature fat, and therefore appears predominately
low on T1 and high on T2-weighted imaging, with possible
interspersed areas of macroscopic fat. Myxoid liposarcomas
are typically large (> 10 cm), but can present at any size,
and often demonstrate heterogeneous enhancement. They
occur most commonly in the lower extremities, and more
commonly metastasize to other soft tissue sites and bone
(particularly the spine) than the lungs [4, 8, 22–25] (Fig. 5).
Myxoid pleomorphic liposarcoma is an exceptionally rare
and aggressive tumor subtype that typically occurs in chil-
dren and adolescents. This tumor has a predilection for the
mediastinum and has been associated with Li-Fraumeni syn-
drome, inactivation of the RB1 tumor suppressor gene, and
other chromosomal aberrations [26–31].
The differentiation of a lipoblastoma from a liposarcoma
is not possible on MRI, and even at histologic analysis, the
diagnosis may not be conclusive. Cytogenetic analysis with
tumor karyotyping helps in the differentiation of these two
tumors [8]. Age is an important discriminator in this context.
Fibroblastic and myofibroblastic tumors
Fibroblastic and myofibroblastic tumors are a subset of mes-
enchymal neoplasms that range from benign to malignant
[32, 33]. They account for about 12% of pediatric soft tissue
tumors [32]. There are numerous musculoskeletal fibrous
tumors that tend to be seen in the pediatric population. Nota-
bly, myositis ossificans, considered by some to be a “tumor-
like lesion,” is classified by the WHO as a benign fibro-
blastic/myofibroblastic tumor [32, 34]. We detail the more
common and unique types of lesions within this category.

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Skeletal Radiology

Fig. 3  Lipomatosis. A 9-month-

old female with a left facial
mass, slowly enlarging over
time and present at birth. MRI
including axial T1 (A), axial
STIR (B), coronal T1-fat-
suppressed (C), and coronal
T1-fat-suppressed post-intrave-
nous contrast (D) demonstrates
lipomatosis involving the left
cheek and ear, with fatty infil-
tration of the left parotid gland
and masseter muscle, as well as
overgrowth of the left hemi-
mandible, the left side of the
tongue, and a left lingual tonsil
that obstructed the oropharyn-
geal inlet

Nodular fasciitis
Nodular fasciitis is the result of a self-limiting clonal neo-
plastic pseudosarcomatous proliferation of fibroblastic/
myofibroblastic cells. Nearly all cases of nodular fasciitis
are due to balanced translocations resulting in overexpres-
sion of USP6 gene, most commonly in the form of recurrent
translocations of MYH9-USP6 [32, 35, 36]. Up to 20% of
cases of nodular fasciitis occur in children, and although
these tumors may be seen in infants and young children, they
more frequently affect adolescents [32]. Nodular fasciitis
typically presents as a rapidly enlarging solitary mass, and
can be seen in all age groups, but most commonly in the
young and middle-aged adults [37]. Intravascular fasciitis
and cranial fasciitis are two rare subtypes of nodular fas-
ciitis that are most commonly seen in people aged less than
30 years old and less than 2 years old, respectively. These
subtypes are similar to nodular fasciitis histologically [38,
39]. Nodular fasciitis can occur anywhere in the body, with
a predilection for the upper extremities and shoulder girdles
that is usually subcutaneous, but can be dermal, deep fascial,
intramuscular, or intermuscular [32, 40, 41]. Cranial fasciitis
typically involves the outer table of the calvarium and can
extend into the superficial soft tissues of the scalp, or deep
through the inner table of bone to the meninges [38]. The
imaging appearance is non-specific, but in general, the lesion
is usually oval, well-circumscribed, approximately 2 cm in
diameter, with a broad-based fascial contact and fascial tail
[32] (Fig. 6). On MRI, the lesion appears isointense to mus-
cle on T1, heterogenous intermediate to high in signal on
T2-weighted imaging, and with heterogeneous enhancement.
Some lesions exhibit an “inverted target” sign, in which
there is high T2 signal centrally, and low signal peripher-
ally, with the central component non-enhancing (Fig. 7). The
tumor may rarely appear locally aggressive with transcom-
partmental spread and intraosseous involvement [32, 40];

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 Skeletal Radiology

Fig. 4  Lipoblastoma. A
15-month-old female with new
posterior occiput mass. Axial
and sagittal T1 (A, B), coronal
T2-fat-suppressed (C), and
coronal T1-fat-suppressed post-
intravenous gadolinium (D) MR
images demonstrate a right sub-
occipital soft tissue mass with
increased T1 signal and signal
suppression on fat-suppressed
sequences with internal septa.
Grossly (E), the tumor demon-
strates a lobulated, pale yellow
and glistening cut surface,
devoid of hemorrhage, necrosis,
or calcification. Fibrous bands
(^) divide the mass into variably
sized nodules. Microscopic
examination (F) demonstrates
lipocytes in various stages of
development including spindled
preadipocytes (*), univacu-
olated lipoblasts (^), and large,
mature-appearing adipocytes
(**)

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Skeletal Radiology

Fig. 5  Liposarcoma (myxoid

type). A 14-year-old male with
an anterior left thigh mass.
Axial T1 (A), T2-fat-suppressed
(B), T1-fat-suppressed (C),
and T1-fat-suppressed post-
intravenous gadolinium (D)
MR images through the thigh
demonstrate a T2 bright lesion
that is slightly hyperintense
to muscle on T1, and with
heterogenous enhancement
situated within the anterior
subcutaneous fat below the
marker. There is no demon-
strable fat signal within the
myxoid lesion. Grossly (E), the
mass is well-circumscribed,
encapsulated, and displays a
tan-red, gelatinous cut surface.
Microscopic examination (F)
shows abundant myxoid stroma
including small, spindled tumor
cells (^), and delicate arborizing
capillary vasculature (arrows)

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 Skeletal Radiology

Fig. 6  Nodular fasciitis.

A 16-year-old male with a
solid palpable mass adjacent
to the left clavicle. Axial
T1 (A), T2-fat-suppressed
(B), T1-fat-suppressed (C),
T1-fat-suppressed post-
intravenous gadolinium (D),
and coronal T1-fat-suppressed
post-intravenous gadolinium
(E) MR images of the clavicle
demonstrate a 2-cm well-
circumscribed oval-shaped
subcutaneous mass abutting the
superficial fascia of the deltoid
with T1 signal isointense to
muscle, high T2 signal, and dif-
fuse enhancement. The coronal
image highlights the enhanc-
ing fascial tail sign (arrow).
Microscopic examination (F)
demonstrates a loose storiform
growth of monomorphic, spin-
dled myofibroblastic cells with
vesicular nuclei and indistinct
nucleoli. Scattered extravasated
erythrocytes are noted (arrow)

13
Skeletal Radiology

Fig. 7  Inverted target sign of nodular fasciitis. Sagittal T1-fat-suppressed MR image following intravenous gadolinium demonstrates a mass
within the soft tissues of the right arm compatible with nodular fasciitis on pathology that exhibits peripheral enhancement, and central non-
enhancement, compatible with an inverted target sign

in this unusual context, it can be mistaken for a soft tissue
sarcoma on imaging [41, 42].
Proliferative fasciitis and proliferative myositis
Proliferative fasciitis, occurring subcutaneously, and pro-
liferative myositis, occurring intramuscularly, are rare
conditions characterized by rapid growth of masses of
ganglion-like cells (pathologically distinguishing them
from more common nodular fasciitis) and plump myofibro-
blastic cells that rarely recur after spontaneous resolution
or conservative local excision but do not metastasize [5].
In children, the masses can have better delineated borders,
greater cellularity, and greater frequency of focal necrosis
than in adults [5, 43]. Imaging features have infrequently
been reported, are non-specific, and unfortunately often

13

overlap with more worrisome/aggressive tumors such as
rhabdomyosarcoma and result in aggressive resection [44].
Proliferative myositis may also contain metaplastic bone,
demonstrating some semblance to acute/intermediate phase
myositis ossificans [5, 45]. Particularly in children, biopsy
of these lesions is necessary to obtain a definitive diagnosis
and exclude malignancy [44]. Within both adult and pedi-
atric populations, the lesions have been described on MRI
as having non-contrast T1-weighted signal isointense to
muscle, hyperintense T2-weighted signal, and generalized
enhancement on T1-weighted images post-intravenous con-
trast, including enhancement along the peri-lesional fascia
[44, 46–50]. A “checkerboard” pattern created by isointense,
preserved muscle striations, has been reported on MRI as
suggestive of proliferative myositis in few reported cases
[46, 50].
Myositis ossificans
Myositis ossificans, fibro-osseous pseudotumor of digits,
and soft tissue aneurysmal bone cyst are considered parts
of the same neoplastic spectrum by WHO classification [5].
While myositis ossificans may occur in individuals of any
age, it is more common in adolescents and young adults and
is not typical in children younger than 10 years of age [32].
These lesions have overlapping imaging and pathologic fea-
tures [5, 51]. Myositis ossificans typically develops within
skeletal muscle, but similar lesions can occur in the subcutis
(panniculitis ossificans), the tendons/fascia (fasciitis ossifi-
cans), and the subcutaneous tissues of the fingers and toes
(fibro-osseous pseudotumor of digits) [5]. Myositis ossifi-
cans is an aberration of muscle repair that occurs as a result
of disordered satellite cell differentiation. It is the result of
direct trauma, non-traumatic causes (burns, immobilization,
and neurologic dysfunction), or may be genetic (myositis
ossificans progressiva) [32, 40, 52]. Up to 75% of cases har-
bor the genetic fusion COL1A1-USP6 [51, 53–57]. USP6
fusions are also frequently seen in aneurysmal bone cyst
and nodular fasciitis. Clinically, myositis ossificans can be
subdivided into three types: post-traumatic, non-traumatic,
and myositis ossificans progressiva [32]. Post-traumatic
myositis ossificans is the most common subtype, and com-
monly affects the extremities, with the quadriceps femoris
and brachialis the most commonly affected muscles [32].
In its early proliferative stage, within 2–3 weeks of the
initial insult, the active vascular and cellular proliferation
of myositis ossificans manifests as a soft tissue mass on
MRI, sometimes with aggressive features such as intense
perilesional soft tissue and marrow edema, ill-definition,
and periosteal reaction. Notably, the adjacent bone cortex
should always remain intact [32, 40, 52]. At the intermediate
13
Skeletal Radiology
calcification and ossification phase, occurring at 3–7 weeks,
lesions are composed primarily of fibroblasts. At the acute
and intermediate phases, myositis ossificans may resem-
ble nodular fasciitis, proliferative myositis, or be confused
with a more concerning soft tissue mass such as rhabdo-
myosarcoma [45]. Extensive perilesional edema of more
than double the size of the central lesion in these phases
has been reported as highly specific, but not pathognomonic
for myositis ossificans [58]. Marginal calcification develops,
but this is difficult to discern on MRI. As such, MRI alone
may not be an optimal imaging strategy for the evaluation of
myositis ossificans — radiographs or CT are complimentary.
By 6 or more weeks, a peripheral ring of mature ossification
develops, surrounding a central acellular core, in what is
considered the mature ossification phase [32, 52] (Fig. 8).
Myositis ossificans is typically isointense to muscle on
T1, hyperintense on T2-weighted imaging, and with diffuse
or peripheral enhancement. With lesion maturity, the periph-
erally calcified rim becomes apparent, and is best visual-
ized on T2-weighted imaging [32] (Fig. 8). Imaging-based
differentiation of myositis ossificans from the exceptionally
rare soft tissue aneurysmal bone cyst is difficult. Both can
peripherally calcify/develop mineralization, and have sur-
rounding edema; however, multi-cystic/cavitary and septated
appearance of a lesion, with multiple fluid levels, have been
described on imaging in up to 40% of soft tissue aneurysmal
bone cyst cases, and if evident, can help to make this dif-
ferentiation [51, 59–61].
Fibrous hamartoma of infancy
Fibrous hamartoma of infancy, historically considered
a hamartoma, was recently reclassified by the WHO as a
neoplastic process when recurrent EGFR exon 20 insertion/
duplication mutations were discovered in the lesion [5, 62].
It is a rare, benign tumor comprised of fibroblastic/myofibro-
blastic cells admixed with mature adipose tissue and myxoid
nodules [5]. It most often occurs in children less than 2 years
old (but can occur in older children) and is congenital in
15–25% of cases [63, 64]. Patients typically present with
a painless, solitary subcutaneous mass at the axilla, trunk,
upper extremities, or genital regions, and may have overly-
ing edema, skin discoloration, dimpling, or hypertrichosis
[63]. Rarely, multiple lesions have been reported in the same
patient or in those with tuberous sclerosis complex or Wil-
liams syndrome [63, 65, 66]. On MRI, the lesion shows a
variable amount of fat, as well as tissue T1 and T2 iso- to
hypointense relative to muscle corresponding to mesenchy-
mal and fibrous tissue. If present, in addition to subcuta-
neous location of the mass, these “parallel” or “whirling”
strands of tissue can suggest the diagnosis, but is non-spe-
cific [67, 68] (Fig. 9).
Skeletal Radiology

Fig. 8  Myositis ossificans. An

18-year-old female with right
hip pain following an episode of
trauma. Frontal radiograph (A)
and reconstructed coronal CT
of the right hip (B) demon-
strate a well-defined, rounded
lesion with a peripheral ring of
mature ossification surround-
ing a central acellular core, in
what is considered the mature
ossification phase, adjacent
to the lesser trochanter. Axial
T1 (C) and T2-fat-suppressed
(D) MR images of the right
hip demonstrate a relatively
non-descript appearing rounded
mass that is T1-isointense and
T2-hyperintense to skeletal
muscle. The gross specimen (E)
demonstrates a central smooth-
walled unilocular cyst (*)
with peripheral red-tan, firm,
and granular tissue (arrow).
Microscopic examination (F)
demonstrates central zones of
spindled fibroblastic cells (*)
transitioning into osteoblast-
lined immature woven bone
(arrow) which then matures into
lamellar bone (**)

13
 Skeletal Radiology

Fig. 9  Fibrous hamartoma of

infancy. A 1-year-old female
with enlarging mass at her
left upper arm, first noted at
7 months of age. On physical
exam, an area of the overlying
skin had bluish discoloration
and was slightly dimpled with
overlying hypertrichosis. MRI
demonstrates a mass contained
within the subcutaneous tissues
along the proximal left arm, not
involving the underlying mus-
cle. The mass has irregular mar-
gins. Axial (A) and sagittal T1
(B), and axial (C) STIR images
demonstrate interspersed fat and
T1/T2 isointense tissue rela-
tive to muscle, as well as thin
peripheral T2 hyperintensity.
D Sagittal T1-fat-suppressed
post-intravenous gadolinium
sequence demonstrates cor-
responding thin peripheral
enhancement with a few thin
internal enhancing septa

Fibromatosis colli hypoechoic, well-defined ovoid mass in continuity with

Fibromatosis colli is benign fibroblastic proliferation within
the sternocleidomastoid muscle of infants. Although the
reported incidence is only 0.3–2.0% of live births, it is the
most common cause of a neck mass in the perinatal period
[69]. Congenital muscular torticollis develops in 10–20%
of affected patients, with head tilt toward the involved
sternocleidomastoid muscle, and chin directed toward the
contralateral shoulder [70, 71]. The lesion may grow for
several weeks, then stabilizes and spontaneously resolves
in the majority of cases [69–72]. Ultrasound is the imag-
ing assessment of choice, which demonstrates an iso- to
the sternocleidomastoid muscle [73, 74]. In one series of
patients who underwent MR imaging, affected sternocleido-
mastoid muscles showed enlargement at their lower third,
where there was diffuse T2 signal hyperintensity relative to
muscle, without a well-defined mass [75].
Juvenile hyaline fibromatosis
Juvenile hyaline fibromatosis is an extremely rare, auto-
somal recessive syndrome resulting from loss of function
mutations including the ANTXR2 (CMG2) gene that may
mediate collagen VI degradation [76–80]. The syndrome

13
Skeletal Radiology

Fig. 10  Calcifying aponeurotic

fibroma. An 8-year-old female
with a mass over the posterior
aspect of her left ankle, first
noticed 2 months prior, and not
causing symptoms. A Lateral
radiograph of the ankle demon-
strates a 2-cm oval soft tissue
mass projecting anterior to the
Achilles tendon with faint cal-
cifications at its upper posterior
aspect. B Axial T1-weighted
image reveals the homogene-
ously isointense mass to be
centered within Kager’s fat pad,
abutting the Achilles tendon
posteriorly and the flexor hal-
licus longus tendon anteriorly.
C Sagittal T2-weighted fat-
suppressed image demonstrates
heterogeneous hyperintense sig-
nal within the mass. D Sagittal
T1-weighted image post-intra-
venous contrast demonstrates
diffuse enhancement. Punctate
areas of low signal intensity on
all sequences within the upper
posterior aspect of the mass cor-
responded to faint calcifications
seen on radiograph

is characterized by progressive, abnormal deposits of hya-
linized fibrous material in the skin, subcutaneous soft tis-
sues, and gingiva. Patients typically present in infancy, most
commonly with subcutaneous soft tissue nodules, gingival
hypertrophy, and progressive joint contractures, but in severe
cases, there can be systemic involvement which is associated
with early mortality. Treatment is largely supportive. The
imaging appearance is non-specific. On MRI, multiple large
subcutaneous nodules, some with overlying skin ulceration,
others with underlying bony erosion, have been described
in a few children and young adults with juvenile hyaline
fibromatosis [81–85]. These were isointense on T1-weighted
sequences, hyperintense on T2-weighted sequences, dem-
onstrated diffusion restriction on diffusion-weighted imag-
ing, and enhanced on T1-weighted imaging performed after
administration of intravenous gadolinium.
Inclusion body fibromatosis
Inclusion body fibromatosis is a benign myofibroblastic
tumor most commonly less than 2 cm and seen along the
dorsal and lateral aspect of the toes or fingers in children

13

less than 5 years old, with approximately 30% present at
birth [86–88]. Extra-digital location has been reported in
rare cases involving the extremities, tongue and breast [89].
There are limited reports regarding MRI appearance of these
lesions — in one instance described as a heterogenous sub-
cutaneous soft tissue mass with non-specific signal charac-
teristics [90]. Asymptomatic lesions are often observed as
they may spontaneously regress. Symptomatic lesions can
be treated with function-preserving excision or steroid injec-
tion, but can recur [91–93].
Calcifying aponeurotic fibroma
Calcifying aponeurotic fibroma is a rare benign tumor
comprised of spindle cells within a collagenous matrix
that can harbor a recurrent FN1-EGF gene fusion [94].
Most patients present between 5 and 15 years old with
a painless soft tissue mass along the palmar surfaces of
the hands and fingers or the plantar surfaces of the feet
and toes [95, 96]. Most lesions are subcutaneous and are
commonly connected to tendons and aponeuroses. Calci-
fication is often seen within the mass on radiographs, and
on MRI, a meta-analysis of 18 cases found the lesions to
most commonly have low to intermediate signal intensity
on T1-weighted imaging, heterogeneously hyperintense
T2 signal, heterogeneous enhancement following intra-
venous contrast, and minute foci of low signal intensity
on all sequences corresponding to areas of calcifica-
tion [97]. The tumors have limited growth potential, are
excised for preservation of function, and can recur in as
many as 50% of cases, often requiring re-excision [95,
96] (Fig. 10).
Gardner fibroma
Gardner fibroma is a benign lesion that usually manifests
as a painless plaque-like mass in the superficial or deep
soft tissues, most commonly along the back and paraspi-
nal region. Most cases present in the first decade of life
with mean age of 5 years at first presentation in patients
with germline APC mutations [98, 99]. Gardner fibro-
mas are strongly associated with desmoid fibromatosis,
and their detection may represent the sentinel event for
identifying the adenomatous polyposis coli mutation,
familial adenomatous polyposis, or Gardner syndrome.
The lesion consists of thick haphazardly arranged col-
lagen bundles with interspersed bland fibroblasts [5].
On MRI, the lesion appears as a T1 and T2 hypointense
plaque-like mass with variable enhancement. Coexisting
desmoid fibromatosis may be present, which appears as
a round mass contiguous with the plaque-like Gardner
fibroma [32, 98].
13
Skeletal Radiology
Desmoid fibromatosis
Fibromatosis is a benign, but locally aggressive, prolif-
eration of fibroblasts. Desmoid fibromatosis is a locally
aggressive neoplasm that accounts for up to 60% of fibrous
tumors seen in children [32]. Sporadic somatic CTNNB1
mutations and less frequently germline APC mutations in
Gardner syndrome contribute to the etiology of desmoid
fibromatosis, although physical factors, including trauma,
surgery, and pregnancy, have also been implicated [100].
The tumor can be solitary or multifocal, more commonly
involving deep versus superficial musculoaponeurotic
structures of the extremities or trunk when extra-abdomi-
nal [32]. A small subset of desmoid tumors occur in pedi-
atric and young adult patients with familial adenomatous
polyposis/Gardner syndrome and in this context are often
intra-abdominal and multifocal [101]. Due to their poten-
tial to arise in and infiltrate deep soft tissues, complete
surgical excision can be difficult to achieve and up to 25%
of patients required more than one operative procedure in
one previously studied cohort [102]. Recent studies have
shown systemic medical treatment, such as hormonal ther-
apy, anti-inflammatory drugs, tyrosine kinase inhibitors,
or cytotoxic chemotherapy, is associated with improved
patient outcomes [103].
On MRI, the signal reflects the extent of collagen and
overall cellularity, being most commonly isointense to
muscle on T1 and hyperintense with bands of low signal
on T2-weighted imaging, and with heterogeneous enhance-
ment. The fascial tail sign refers to linear extension of the
lesion along fascial planes [32, 40] (Fig. 11). A recent
study by Crombe et al. showed prognostic value in radi-
omic scoring based on volumetric MRI changes on fat-
suppressed T1-weighted imaging following intravenous
gadolinium administration for predominantly adult patients
with desmoid fibromatosis undergoing chemotherapy [104].
Others have also explored correlations between RECIST
(Response Evaluation Criteria in Solid Tumors) scoring, a
maximum dimension size-based criteria of determining dis-
ease improvement, and decreasing tumor volume or decreas-
ing T2 hyperintensity [103, 105, 106]. To the author’s
knowledge, these prognostic findings have not yet been
shown in a large pediatric population study, although small
series in pediatric populations undergoing therapy have
shown similar short-term imaging-based findings [107–109].
Lipofibromatosis
Lipofibromatosis is a rare benign fibrous tumor com-
posed of lobules of mature fat, short fascicles of bland
spindle cells, and lipoblast-like cells [5]. It occurs almost
Skeletal Radiology

Fig. 11  Desmoid fibromato-

sis. A 16-year-old male with
a palpable mass in the thigh.
Axial T1 (A), T2-fat-suppressed
(B), T1-fat-suppressed (C),
and T1-fat-suppressed post-
intravenous gadolinium (D) MR
images of the thigh demonstrate
a large well-circumscribed
intramuscular lesion isointense
to muscle on T1 and hyper-
intense in signal on T2, and
with enhancement on post-
contrast imaging. Note regions
of stippled hypointense signal
throughout the tumor related
to collagenous stroma. Grossly
(E), the mass is firm, well-cir-
cumscribed, encapsulated, and
exhibits tan-white, trabeculated,
fibrous tissue (*), with no evi-
dence of hemorrhage or cystic
change. Microscopic examina-
tion (F) demonstrates fascicles
of cytologically bland spindle
cells with moderate cellularity
(arrows) and intervening colla-
genous stroma. No evidence of
cytologic atypia, pleomorphism,
hyperchromasia, or necrosis is
present

13
 Skeletal Radiology

Fig. 12  Lipofibromatosis. A
2-year-old female with right
knee pain. Axial T1 (A), T2-fat-
suppressed (B), T1-fat-sup-
pressed (C), T1-fat-suppressed
post-intravenous gadolinium
(D), and sagittal T1-fat-sup-
pressed post-intravenous gado-
linium (E) MR images of the
leg demonstrate a non-specific
and ill-defined T1 hypointense,
T2 heterogenous, and enhancing
mass within the subcutaneous
fat of the anterior leg (arrow)

exclusively in children, usually younger than 2 years of age
(20% congenital), with a 2:1 male predilection [110, 111].
Fusion abnormalities have been identified involving ligands
to EGFR, EGFR itself, or other receptor tyrosine kinases,
suggesting the pathogenesis of lipofibromatosis involves
activation of the PI3K/AKT/mTOR pathway, as mentioned
during the discussion of lipomatosis [111]. Lipofibromato-
sis is most commonly found in the distal extremities, but
also known to involve the trunk, head, and neck. The tumor
is typically infiltrative and is known to recur locally requir-
ing limb amputation in some cases, but with no reported
metastases.

13
Skeletal Radiology

Fig. 13  Hybrid giant cell fibro-

blastoma and dermatofibrosar-
coma protuberans. A 9-year-old
female with lifelong history
of skin changes/discoloration
over the anterior neck, initially
thought to be a port-wine stain.
A mass developed underly-
ing the area of discoloration,
prompting further evaluation. A
Targeted ultrasound revealed a
mixed echogenicity mass with
scattered internal vascularity.
Axial (B, C) and coronal (D)
STIR MR sequences demon-
strate a multifocal (small satel-
lite nodule within the anterior
left chest, white arrow), hyper-
intense mass centered within
the superficial soft tissues of the
anterior neck. The mass extends
from the subdermal fat through
the plane of the platysma
muscle, displacing the sterno-
cleidomastoid muscles laterally
and abutting the strap muscles
at its deep aspect. There was
a single dominant flow void
within the mass suspected to
represent a venous branch from
the right internal jugular vein
(black arrows demonstrate the
flow void within the dominant
mass). The mass is isointense
on coronal T1 (E) and enhances
mildly on T1 post-intravenous
contrast (F). Incidental note is
made of an arachnoid cyst in the
right middle cranial fossa

On MRI, the lesion is poorly circumscribed within the
subcutaneous or deep soft tissue, with variable degrees of fat
and fibrous tissue. As such, this tumor should be considered
as part of the differential diagnosis of fat-containing tumors
in children, along with lipomatosis, lipoblastoma, lipoblasto-
matosis, fibrous hamartoma of infancy, and the exceptionally
rare liposarcoma [32, 112] (Fig. 12).
Giant cell fibroblastoma
Giant cell fibroblastoma is a rare, locally aggressive neo-
plasm arising predominantly in children, characterized
by hypo-cellularity, myxoid or collagenous stroma, the
presence of bland spindle cells and scattered multinu-
cleated giant cells lining pseudovascular spaces, and
often formation of a COL1A1-PDGFB gene fusion [5,
113, 114]. Patients present at mean age of 6 years old,
most commonly with a slow-growing, plaque-like pain-
less cutaneous/subcutaneous lesion at the trunk, groin,
or axillary region, less commonly the extremities, head
or neck [114]. Rare reports of these lesions including
MRI features have described subcutaneous location, T1
hypointensity, T2 hyperintensity, and contrast enhance-
ment [115, 116].

13
 Skeletal Radiology

Fig. 14  Inflammatory myofi-

broblastic tumor. A 16-year-old
male wrestler and football
player with slow onset progres-
sive left hip pain and decreased
range of motion. MRI dem-
onstrates an infiltrative mass
within the left ischofemoral
space and involving the com-
mon hamstring origin. The mass
is predominantly isointense on
axial T1-weighted images with
a few discrete foci of internal
fat (A), hyperintense on axial
T2-fat-suppressed images
(B), and enhances following
administration of intravenous
gadolinium on axial T1-fat-
suppressed images (C)

Giant cell fibroblastoma is closely related to dermatofi- Dermatofibrosarcoma protuberans

brosarcoma protuberans. The two tumor types share
chromosomal and molecular features. Areas of the con-
ventional dermatofibrosarcoma protuberans are present
in approximately 15% of giant cell fibroblastoma cases
(hybrid lesions) [114, 117] (Fig. 13).
Dermatofibrosarcoma protuberans is a superficial, locally
aggressive, fibroblastic neoplasm, seen most commonly
on the trunk and proximal extremities, followed by the
head and neck. Although it is a rare tumor and most

Fig. 15  Infantile fibrosarcoma.

A 6-month-old female with
rapidly enlarging left thigh mass
over 2 months. A T1-weighted
coronal image of the left lower
extremity demonstrates a large,
lobulated isointense mass
extending between the super-
ficial subcutaneous fat and the
deep medial thigh musculature.
B Coronal T2-weighted fat-
suppressed image demonstrates
heterogenous T2 hyperintensity.
C Coronal T1-fat-suppressed
image post-intravenous gado-
linium administration demon-
strates diffuse, avid enhance-
ment of the mass

13
Skeletal Radiology
common in middle aged adults, a substantial number of
cases are seen in children, and can be seen congenitally
[117, 118]. It typically presents as a slowly growing nodu-
lar or multinodular cutaneous mass with red discoloration.
The tumor typically has a COL1A1-PDGFB or related
fusion, and can be multicentric in children with adenosine
deaminase-deficient severe combined immunodeficiency
[5, 119].
On MRI, these lesions are typically well defined, and
centered within the superficial soft tissues, just deep to
the dermis but with enlargement can infiltrate underly-
ing tissues (Fig. 13). They are usually iso- to hypointense
relative to muscle on T1-weighted images, hyperintense
on T2-weighted images, and show uniform or patchy
enhancement with administration of intravenous gadolin-
ium contrast [40, 120, 121]. In children, these lesions can
be confused with mixed venolymphatic malformations,
given the distribution and overlying skin discoloration
[40]. Unless widely excised, these lesions are often char-
acterized by repeated local recurrences [122–124]. There
are several histopathologic subtypes. The fibrosarcoma-
tous subtype exhibits more aggressive behavior and can
metastasize, typically to the lungs [5, 125].
Inflammatory myofibroblastic tumor
Inflammatory myofibroblastic tumors are composed of
myofibroblastic and fibroblastic spindle cells, with an
inflammatory infiltrate, and primarily affect children
and young adults [126–128]. As many as 60% of tumors
have genetic abnormalities resulting in overexpression
of the anaplastic lymphoma kinase (ALK) protein [5].
Fig. 16  Low-grade fibromyxoid sarcoma. A 15-year-old female pre-
sented with an asymptomatic soft tissue mass in her left forearm, pre-
sent for approximately 3 years. On MRI, sagittal T1-weighted image
(A) demonstrates a predominantly isointense mass centered within
the musculature, as well as a “split fat” sign. B Sagittal T2-weighted
Inflammatory myofibroblastic tumor has a wide anatomi-
cal distribution, and the site of origin determines the pre-
senting symptoms. Up to a third of patients present with
fever, weight loss, malaise, and laboratory abnormalities
including elevated erythrocyte sedimentation rate and
C-reactive protein [127, 129, 130].
Imaging features are variable. On MRI, tumors are com-
monly reported to be isointense to muscle on T1-weighted
images, T2 signal intensity is inversely proportional to
fibrotic content, and tumors enhance avidly with intra-
venous gadolinium contrast administration [8, 131–133]
(Fig. 14). Some demonstrate coarse calcification. Distant
metastases are rare, and approximately 25% of extrapul-
monary inflammatory myofibroblastic tumors recur, with
risk dependent on anatomical site and resectability [127,
128, 134]. Surgical excision is the primary treatment, but
for inoperable cases where ALK gene rearrangements are
identified, Crizotinib, a small-molecule tyrosine kinase
inhibitor targeting ALK, can be of benefit [135].
Infantile fibrosarcoma
Infantile fibrosarcoma is a locally aggressive, rapidly grow-
ing malignant fibroblastic tumor most commonly seen in
the first 2 years of life that frequently harbors an ETV6-
NTRK3 fusion, although alternative fusions involving other
kinases such as NTRK, BRAF, and MET are also reported
[136–139]. One-third of cases are found at birth, and may
be detected by prenatal ultrasound [140, 141]. Tumors most
commonly involve the superficial and deep soft tissues of
the extremities, but have a wide range of anatomic distribu-
tion [142]. Histopathologically, the tumor is characterized
fat-suppressed imaging demonstrates heterogeneous T2 hyperinten-
sity, admixed with hypointense foci that were also T1 hypointense. C
Sagittal T1-fat-suppressed image post-intravenous gadolinium admin-
istration demonstrates heterogeneous enhancement
13
 Skeletal Radiology

Fig. 17  Infantile myofibroma.

A 2-year-old male with a finger
mass, first noticed at 1.5 years
old, with slight increase in size.
A T2-weighted fat-suppressed
coronal MR image reveals a
hyperintense, multilobulated
mass along the radial index
finger, spanning between the
skin surface and the underlying
bone. The mass is relatively
homogenously isointense on
T1-weighted images (B) and
enhances avidly following
administration of intravenous
gadolinium on T1-weighted fat-
suppressed images (C)

by spindle to primitive ovoid round cells, often arranged in
interweaving bundles [143].
Infantile fibrosarcoma is usually large, can be poorly cir-
cumscribed and infiltrating surrounding tissues with encase-
ment of neurovascular structures, or well-demarcated. The
mass is isointense to muscle on T1, and heterogeneously
high in signal on T2-weighted imaging owing to necrosis,
hemorrhage, myxoid material, and cystic areas. Intense,
heterogeneous enhancement may be present [4, 40, 144]
(Fig. 15). The tumor can cause disseminated intravascular

13
Skeletal Radiology
coagulopathy, and this can be confused with Kasabach-Mer-
ritt phenomenon that is seen with kaposiform hemangioen-
diothelioma or tufted angioma [40]. Treatment involves vari-
ous combinations of surgery and/or standard chemotherapy,
with local recurrence rates of 25–40% [136, 140]. Targeted
tyrosine kinase inhibitor therapies may also be of benefit in
advanced or intractable disease [145, 146].
Low‑grade fibromyxoid sarcoma
Low-grade fibromyxoid sarcoma is characterized by alter-
nating fibrous and myxoid areas, bland spindle cells in
whorled or short fascicular pattern, and commonly FUS
gene fusions with resultant strong expression of MUC4 (an
epithelial glycoprotein) [5, 147, 148]. The tumor is seen in a
wide age range, but as many as 20% of patients are less than
18 years old [5, 149, 150]. Patients typically present with
a painless mass, most commonly involving the proximal
extremities and trunk at subcutaneous or subfascial depth,
less commonly the abdominal cavity, retroperitoneum and
mediastinum [149–152]. The tumor can metastasize, most
commonly to the lungs. On long-term follow-up, one study
of both pediatric and adult patients found recurrences after
excision and death from disease in 45% and 42% of patients,
respectively [150].
On MRI, the tumor may appear solid or mixed solid/
cystic depending on the ratio of fibrous and myxoid com-
ponents. On T1-weighted images, tumors are isointense,
occasionally showing internal hemorrhage, and for intra-
muscular tumors, a thin rim of peripheral “split” fat can be
seen [153, 154] (Fig. 16). On fluid sensitive sequences, a
“gyriform” pattern of T2 hyperintensity mimicking brain
gyri can be seen, as can peri-tumoral edema [154, 155]. On
T1-weighted fat-suppressed images post-intravenous con-
trast, heterogenous enhancement is typically seen [154–156].
Perivascular tumors
Myopericytoma, including myofibroma
Myopericytomas, on a spectrum with myofibromas, are
perivascular myoid-appearing spindled cell neoplasms that
can be associated with PDGFRB mutations [157, 158].
Myopericytomas can be seen at any age, but are most com-
monly seen in adults. Myofibromas may be present at birth
or appear in the first 2 years of life [159]. Myofibromas
may present as a solitary lesion, or with multiple lesions
in patients with myofibromatosis — germline mutations in
PDGFRB often underlie the latter [160].
In infants, solitary infantile myofibromatosis is the most
common benign fibrous tumor, with almost 90% of cases
occurring within the first 2 years of life, and 50% occur-
ring in newborns [32, 40, 161]. While the tumor can present
in solitary, multicentric without visceral involvement, and
generalized with both cutaneous and visceral involvement
forms, the solitary type is most common, presenting as a
firm nodule on the skin and in the subcutaneous tissue at the
head, neck, or trunk or extremities. The lesion has a vari-
able appearance on MRI, with a central high signal typically
present on T2-weighted imaging (Fig. 17). The target sign
describes peripheral enhancement that is apparent follow-
ing intravenous gadolinium administration, thought to be
related to central necrosis [32, 40]. In a recent report corre-
lating the MRI and histopathologic features of these lesions,
the authors found variable T1- and T2-weighted signal but
with universal enhancement following intravenous contrast
administration, with tumor size, extent of enhancement, and
tumor margins correlating with variable degrees of cellular-
ity, collagen, and myxoid changes [161].
Conclusion
There are numerous soft tissue tumors and tumor-like condi-
tions in the pediatric population. The recently updated WHO
classification scheme for these tumors provides a useful
standardized nomenclature in the context of evolving under-
standing of underlying genetic aberrations and emerging
targeted therapies. MRI is widely employed when imaging
these soft tissue lesions due to its superior soft tissue con-
trast and characterization, ability to identify any underlying
bone marrow signal abnormalities, and in select instances
provide quantitative or semi-quantitative data. While certain
soft tissue lesions may exhibit conventional MRI features
characteristic of a specific diagnosis without biopsy, most
lesions are often indeterminate based on clinical examina-
tion and MRI, and biopsy is required.
Declarations
Conflict of interest Author Andrew Haims, MD, discloses a financial
relationship with Pfizer.
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