Pe d i a t r i c I m a g i n g • R ev i ew

Restrepo et al.
Imaging Evaluation of Neonatal Soft-Tissue Tumors

Pediatric Imaging
Review

Up-To-Date Practical Imaging

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Evaluation of Neonatal Soft-Tissue

Tumors: What Radiologists Need
to Know
Ricardo Restrepo1 OBJECTIVE. The purposes of this article are to provide an up-to-date overview of neo-
Michael L. Francavilla2,3 natal soft-tissue tumors, including information regarding their unique nature, and to present
Robert Mas 4 practical imaging techniques and characteristic imaging findings.
Edward Y. Lee5 CONCLUSION. Neonatal soft-tissue tumors are a unique set of neoplasms that often
have characteristic clinical and imaging findings. Imaging evaluation, mainly with ultrasound
Restrepo R, Francavilla ML, Mas R, Lee EY and MRI, plays an important role in the initial diagnosis, staging, preoperative assessment,
and follow-up evaluation. Clear understanding of practical imaging techniques combined
with up-to-date knowledge of characteristic imaging findings can help the radiologist pro-
vide a timely and accurate diagnosis of these neoplasms and can lead to optimal neonatal pa-
tient care.

eonatal soft-tissue tumors are scribes their clinical presentation and charac-

N congenital neoplasms discovered

during the first month of life [1].
Because of their rarity, these tu-
teristic imaging findings.

Unique Nature of Neonatal Soft-Tissue

mors often pose diagnostic challenges for cli-
nicians and specialists, including radiologists.
From an epidemiologic point of view, there
has been no unified method of reporting neo-
natal soft-tissue tumors [1]. As a result, the ex-
Keywords: congenital tumors, neonatal tumors, act prevalence, sites of origin, and pathologic
soft-tissue tumors nature of the tumors are unknown [1–4].
Neoplasms, except for infantile hemangi-
DOI:10.2214/AJR.16.17576
omas, are uncommon in neonates. The most
Received October 24, 2016; accepted after revision commonly reported neonatal neoplasms, ex-
January 13, 2017. cluding hemangiomas, are neuroblastoma and
1
teratoma, followed by soft-tissue tumors [1, 3,
Department of Radiology, Nicklaus Children’s Hospital,
Miami, FL.
5, 6]. Neonatal soft-tissue neoplasms, which
have a reported incidence between 8% and
2
Department of Radiology, Children’s Hospital of Philadel- 10%, can be classified into three main catego-
phia, 34th St and Civic Center Blvd, Philadelphia, PA ries: benign tumors, which include infantile
19104. Address correspondence to M. L. Francavilla
hemangioma, congenital hemangioma, fibro-
(francavilm@email.chop.edu).
matosis coli, and lipoblastoma; tumors with in-
3
Perelman School of Medicine at the University of termediate behavior (i.e., histologically benign
Pennsylvania, Philadelphia, PA. but locally aggressive with little to no metastat-
4
ic potential), which include infantile myofibro-
Ross University School of Medicine, Miramar, FL.
ma, myofibromatosis, solitary fibrous tumor,
5
Department of Radiology, Boston Children’s Hospital kaposiform hemangioendothelioma, and fibro-
and Harvard Medical School, Boston, MA. sarcoma; and malignant tumors, which include
rhabdomyosarcoma, neuroblastoma, and con-
AJR 2017; 209:195–204 genital leukemia [1, 7, 8].
0361–803X/17/2091–195
This article provides an overview of the
unique nature of neonatal soft-tissue tumors
© American Roentgen Ray Society and the practical imaging approach and de-
Tumors
Neonatal soft-tissue tumors have three
main characteristics that make them differ-
ent from soft-tissue tumors presenting in old-
er children and adults.
First, the underlying cause is different in
that preconceptional and transplacental on-
cogenesis plays a major role in the etiopatho-
genesis of neonatal soft-tissue tumors. A
high incidence of chromosomal changes and
some inherited and spontaneous gene muta-
tions can also play a role in the inherited pre-
disposition to malignancy in this young pa-
tient population [1, 9–11].
Second, some neonatal soft-tissue neo-
plasms have well-established associations
with congenital malformations that probably
were initiated in utero by the transplacental
passage of mutagenic and carcinogenic stim-
uli [12–14]. Some of these associations are
also related to chromosomal abnormalities,
commonly trisomies [1].
Third, some neoplasms in neonates have a
unique neonatal oncogenic grace period, re-
ferring to the less aggressive biologic behav-
ior and better prognosis in which age per se
becomes an important prognostic factor inde-
pendent of the histologic features and extent of
the tumor. Neonatal neuroblastoma and infan-

AJR:209, July 2017 195


tile hemangioma are examples of neoplasms
that can completely regress [1, 4, 5, 15–17].
Imaging Modalities and Techniques
The two best imaging modalities available
for evaluating neonatal soft-tissue tumors are
ultrasound (US) and MRI, which are particu-
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larly useful at this age because they spare the
neonate from ionizing radiation.
After careful physical examination, US
should be the first imaging modality used to as-
sess soft-tissue tumors in neonates. US is read-
ily available and inexpensive. It also allows
real-time imaging, which can be performed
portably and does not require sedation. The
less ossified neonatal skeleton and relatively
little subcutaneous fat in this age group typi-
cally allow reasonable visualization of deeper
structures. For evaluation of neonatal soft-tis-
sue tumors, US images should be obtained with
a high-resolution transducer (7–15 MHz) with
either a linear array or sector transducer. Gray-
scale imaging is useful for characterizing the
nature of the lesions (i.e., cystic or solid) and
the relation to adjacent structures. Color Dop-
pler technique is helpful for differentiating vas-
cular from nonvascular soft-tissue tumors.
For further characterization after US,
MRI is the next imaging modality of choice.
The major advantages of MRI are its superi-
or tissue contrast resolution, multiplanar ca-
pability, and utility for evaluating extensive
lesions. MRI can further help to characterize
the tumor, accurately evaluate its relations to
adjacent vital structures, acquire information
for local staging, and plan subsequent treat-
ment. In addition, MRI is the imaging modal-
ity of choice for posttreatment local surveil-
lance of neonatal soft-tissue tumors. Three
main disadvantages of MRI in the neonatal
period are the limited availability, fairly high
cost, and potential need for sedation.
When a suspected neonatal soft-tissue tu-
mor is located in an extremity, coronal large-
FOV imaging of both extremities should be
followed by acquisition of axial small-FOV
images of the ROI to obtain both global and
detailed views of the abnormality. Gadolin-
ium-based IV contrast enhancement helps
to improve tumor conspicuity. DWI comple-
ments anatomic MRI by adding information
about the cellularity of the tumor. Such func-
tional information has been used with vary-
ing results and clinical benefits [18–23].
CT plays a limited role in the diagnosis
of soft-tissue tumors because of poor tissue
contrast resolution and the use of ionizing ra-
diation. However, in neonates in whom seda-
196 AJR:209, July 2017
Restrepo et al.
tion or MRI is contraindicated, CT may be
valuable in lieu of MRI.
Spectrum of Neonatal Soft-Tissue
Tumors
Benign Neonatal Soft-Tissue Tumors
Infantile hemangioma—Infantile heman-
gioma (IH) is a common benign neoplasm of
endothelial cells that reportedly occurs in ap-
proximately 3–10% of the pediatric population
[24–27]. Associated risk factors for IH include
female sex, white ethnic background, prema-
turity, and multiple gestations [24, 28]. IH has
been associated with several syndromes and
congenital malformations, such as PHACES
syndrome (posterior fossa malformations, hem-
angiomas, arterial anomalies, cardiac defects,
eye anomalies, sternal cleft, supraumbilical ra-
phe) and perineal PELVIS syndrome (heman-
gioma, external genitalia malformations, lipo-
myelomeningocele, vesicorenal abnormalities,
imperforate anus, skin tag) [29, 30].
Rarely fully developed at birth, IH typical-
ly arises from a precursor lesion and appears as
an area of dermal discoloration or telangiecta-
sia. IH follows a characteristic clinical course in
which it proliferates during the first few months
of life and then undergoes involution in the first
few years, leaving a scar of fibroadipose tissue
[28, 31]. The soft tissues of the head and neck
are the sites of origin in 60% of cases, followed
by the trunk, extremities, and viscera [32]. IH is
usually solitary, but as many as 15% of pediat-
ric patients with IH have multiple lesions [32].
IH presents typically as a focal nodule or
raised plaque with bright red discoloration
and associated skin thickening clinically
(Fig. 1). IH has a unique immunohistochemi-
cal marker, Glut-1, an erythrocyte-type glu-
cose transporter protein, which helps dif-
ferentiate IH from other vascular lesions in
atypical cases. The term “miliary hemangio-
matosis” is used when there are more than 30
papular punctate lesions, which can be red to
blue. Miliary hemangiomatosis is usually as-
sociated with extracutaneous hemangiomas,
which most commonly involve the liver [28].
Most IHs with a typical clinical history
and appearance at physical examination re-
quire no imaging evaluation. When atypical
or when treatment is contemplated, imaging
may be requested to assess the extent, depth,
and relation to adjacent structures. US usual-
ly suffices in the evaluation of focal lesions,
whereas MRI may be necessary for the more
extensive segmental type (Fig. 1). At US, IH
appears as a heterogeneous but predominant-
ly hyperechoic nodule with fairly well-delin-
eated borders. At color Doppler US imaging,
IH is markedly vascular with high- and low-
resistance arterial and venous waveforms
(Fig. 1). At MRI, IH is seen as circumscribed
nodules or plaques that are hyperintense (al-
though less than fluid) on T2-weighted MR
images and heterogeneous with fatty ele-
ments on T1-weighted MR images. Flow
voids and rapid, vivid contrast enhancement
are typical [28, 33] (Fig. 1). Calcifications are
typically not seen with IH.
Most IHs, because they involute sponta-
neously, require no treatment. Intervention is
reserved for lesions at risk of ulceration, dis-
figurement, or functional impairment. Pro-
pranolol therapy has become the cornerstone
of treatment of IH with less frequent alterna-
tive use of laser therapy, steroids, or surgical
procedures [28, 34].
Neoplasms potentially mimicking IH ow-
ing to skin discoloration and rapid growth
clinically and hypervascularity at imag-
ing include congenital hemangioma, myofi-
broma, kaposiform hemangioendothelioma,
congenital fibrosarcoma, neuroblastoma, and
congenital leukemia.
Congenital hemangioma—Congenital hem-
angioma is an uncommon benign vascular tu-
mor that, unlike IH, grows during fetal life and
is fully developed at birth [28, 32]. The follow-
ing three types of congenital hemangioma have
been described, according to natural course:
rapidly involuting, partially involuting, and
noninvoluting [32, 35, 36]. The rapidly involut-
ing type starts to involute earlier and faster than
does IH. The noninvoluting type has a static
course and never regresses. The partially in-
voluting type behaves like the rapidly involut-
ing initially, but the involution is subsequently
aborted [32, 35]. With an equal sex distribution,
the principal locations of congenital hemangi-
oma are the extremities near a joint and the su-
perficial soft tissues of the head and neck. Deep
soft-tissue involvement seldom occurs.
The diagnosis of congenital hemangio-
ma is typically made clinically when soli-
tary violaceous superficial nodules or bossed
plaques with superficial telangiectasia and
a distinct peripheral pale halo are detected
[32, 36] (Fig. 2). Pathologically, congenital
hemangioma is similar to IH, but congenital
hemangioma is Glut-1–negative [32].
The imaging features of congenital hem-
angioma and IH are similar. Congenital hem-
angioma, however, is usually confined to the
subcutaneous fat and dermis, has a more het-
erogeneous parenchyma, is less defined with
more infiltrative margins, and more common-
 Imaging Evaluation of Neonatal Soft-Tissue Tumors
ly exhibits perilesional edema and fat strand-
ing (Fig. 2). Calcification, not seen in IH, can
occasionally be present in congenital heman-
giomas [28, 36, 37]. Congenital hemangiomas
can be managed with laser therapy or surgery.
Fibromatosis colli—Fibromatosis colli is
a benign fibrous tumor arising in the ster-
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nocleidomastoid muscle of infants and neo-
nates. Histologically, it is composed of a dis-
organized proliferation of fibroblasts. The
mass usually arises in the first 2 weeks of
life and may be seen at birth. Approximately
20% of cases are associated with torticollis.
Fibromatosis colli is generally unilateral and
more common on the right. Neonates with
this anomaly typically have a history of birth
trauma, difficult delivery, developmental hip
dysplasia, or breech presentation.
US is the imaging modality of choice for the
diagnosis and follow-up of fibromatosis colli.
It shows focal or diffuse fusiform enlargement
of the sternocleidomastoid muscle, usually the
lower two-thirds, with normal adjacent soft tis-
sues. Fibromatosis colli has circumscribed bor-
ders and homogeneous or heterogeneous pa-
renchyma [38–40] (Fig. 3). Ninety percent of
cases have a thin, hypoechoic rim. Calcifica-
tions have been rarely reported [40]. Because
of the typical location and rapid regression, fur-
ther evaluation with other imaging modalities
or biopsy is not routinely performed. Fibroma-
tosis colli lesions enlarge over the first month of
life but usually resolve spontaneously or with
physical therapy [41].
Lipoblastoma—Lipoblastoma is a rare be-
nign tumor that occurs exclusively in early
childhood with several reports of congeni-
tal occurrences [42–48]. Lipoblastoma tends
to occur most frequently in the extremities
and trunk, followed by the cervicofacial re-
gion, retroperitoneum, mesentery, and medi-
astinum [42, 47, 49, 50]. Lipoblastoma typi-
cally is encapsulated and has no metastatic
potential. When unencapsulated, the tumor is
referred to as lipoblastomatosis [42, 47, 50].
Histologically, lipoblastoma is composed of
myxoid stroma surrounding adipocytes in
various stages of maturity [42, 50]. Young-
er patients have a larger myxoid component.
Clinically, lipoblastoma presents as a firm
but somewhat compressible superficial mass
without skin discoloration; lipoblastomatosis
is usually deeply seated and infiltrating [42].
The imaging characteristics of lipoblas-
toma parallel the histologic composition of
the lesion. Lipoblastoma is circumscribed,
but the margins are indistinct and infiltrat-
ing in lipoblastomatosis [49]. US shows a
AJR:209, July 2017 197
predominantly hyperechoic mass, reflecting
its fatty nature; however, more hypoechoic
lesions can be seen when the stromal myx-
oid component predominates. Anechoic cys-
tic spaces can also be present [49] (Fig. 4A).
At MRI, T1-weighted imaging is key, show-
ing a fatty mass with internal strands of myx-
oid tissue (Fig. 4B). The myxoid component
may be mildly enhancing [49]. On fat-sup-
pressed T2-weighted images, lipoblastoma
may remain hyperintense when the lesion
has an abundant myxoid component, poten-
tially mimicking a cystic lesion; however,
the presence of enhancement on T1-weighted
MR images confirms its solid nature. Intra­
lesional septations and cystic elements can
be seen on both T1- and T2-weighted MR
images [45, 49, 51, 52]. Calcification and os-
sification are not typical features of lipoblas-
toma. Complete resection is the treatment of
choice of lipoblastoma because incomplete
resection can lead to tumor recurrence [47].
Neonatal Soft-Tissue Tumors With
Intermediate Behavior
Infantile myofibroma and myofibroma-
tosis—Infantile myofibroma (solitary) and
myofibromatosis (multicentric) are the most
common fibrous tumors of infancy with an
incidence of 1 in 150,000 live births [14].
Approximately 50% of cases present in the
neonatal period [8]. Histologically, the le-
sions are composed of spindle-shaped fibro-
blasts [53]. The typical presentation is one or
more firm dermal or subcutaneous nodules
of different sizes and sometimes skin discol-
oration. The multicentric form is more fre-
quently seen in boys and involves the soft tis-
sues of the head and neck, trunk, and viscera.
In the absence of visceral involvement, this
tumor has a benign self-limiting course with
frequent spontaneous resolution and a low
recurrence rate [31, 54–56].
The US appearance, based on scant reports,
is a solid mass with an anechoic center and a
thick wall but no significant vascularity (Fig.
5). Associated calcifications have been report-
ed [53, 57]. At MRI, these tumors tend to be
of low signal intensity on T1-weighted im-
ages and of variable but predominantly high
signal on T2-weighted images [58]. Contrast-
enhanced T1-weighted MR images may show
targetlike peripheral enhancement [54, 55].
The borders of the tumor are usually sharp
but can be poorly defined, infiltrating adja-
cent soft-tissue structures and eroding bone.
Because infantile myofibroma and myofibro-
matosis often regress spontaneously, surgical
excision is usually reserved for tumors involv-
ing vital structures.
Solitary fibrous tumor—Solitary fibrous
tumor, formerly called hemangiopericytoma,
is a highly vascularized soft-tissue neoplasm.
These lesions were originally thought to arise
from pericytes, but more recent analysis fa-
vors a fibroblastic cell origin. Therefore, this
tumor is now included in the group of fibro-
blastic and myofibroblastic tumors. Approxi-
mately 10–20% exhibit aggressive behavior,
such as local invasion, recurrence, and me-
tastases [59–61]. There are two main types of
solitary fibrous tumor, according to age at pre-
sentation: infantile (< 1 year) and adult. The
infantile type has a less aggressive course and
sometimes spontaneously regresses [8, 62].
Solitary fibrous tumor can involve any loca-
tion, including the viscera. In the soft tissues,
it most commonly involves the lower extremi-
ties, followed by the craniocervical region [8].
At US, solitary fibrous tumor is typically hy-
poechoic and homogeneous in echogenicity.
As the tumor enlarges, it can become hetero-
geneous and may have an internal cystic com-
ponent. At MRI, a solitary fibrous tumor is ho-
mogeneous with low to intermediate signal
intensity on T1-weighted images and is inho-
mogeneous with variable signal intensity on
T2-weighted images. Enhancement tends to
be homogeneous in small tumors and hetero-
geneous in large tumors [59] (Fig. 6). Although
some tumors regress spontaneously, persistent
or enlarging tumors may require resection.
Kaposiform hemangioendothelioma—Ka-
posiform hemangioendothelioma (KHE) is a
rare locally aggressive vascular tumor in the
pediatric population. KHE contains endo-
thelium-derived spindle cells with minimal
atypia and infrequent mitosis without met-
astatic potential. It presents early in child-
hood and is often congenital [32, 63–65].
The most common location of tumor is the
trunk, followed by the extremities and cervi-
cofacial region. The typical presentation is a
large mass and thrombocytopenia, known as
the Kasabach-Merritt phenomenon. When it
involves the skin, KHE appears as a raised
plaque with bluish-reddish discoloration that
can be confused with a hemangioma [31, 66].
At imaging, KHE appears as an ill-de-
fined mass in the superficial or deep soft tis-
sues [66]. US shows a poorly defined, vascular
mass with variable (low to high) echogenici-
ty [67]. KHE tends to be deeply infiltrating,
and MRI is often required to assess the ex-
tent of the lesion. MRI typically shows an in-
distinct mass that is isointense or hypointense

on T1-weighted images and hyperintense on
T2-weighted images. KHE is avidly but het-
erogeneously enhancing with involvement of
multiple tissue planes (Fig. 7). Perilesional fat
stranding and edema (Fig. 7) and the presence
of Kasabach-Merritt phenomenon are impor-
tant features of KHE [68]. The treatment of
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KHE is challenging, requiring a multidisci-
plinary approach that includes pharmacother-
apy with steroids, vincristine, or interferon
and embolization or surgery [32].
Fibrosarcoma—Fibrosarcoma is a rare ma-
lignant mesenchymal neoplasm composed of
proliferating fibroblasts. This tumor is local-
ly invasive and has little or no metastatic po-
tential [69–71]. The incidence of fibrosarcoma
peaks twice, once in infants and young chil-
dren (infantile form) and again in children
10–15 years old (adult form) [69]. Approxi-
mately 30% of cases are congenital [15, 69].
The prognosis of the infantile form is more fa-
vorable because it is characterized by risk of
local recurrence, in contrast to the metastasis
associated with the adult form [15, 72]. Most
infantile fibrosarcomas are located in the ex-
tremities, particularly distally (Fig. 8), fol-
lowed by the trunk and neck. Neonates with
fibrosarcoma typically present with rapidly
growing large, hard, fixed masses that stretch
and discolor the skin, potentially simulating a
hemangioma [69, 73] (Fig. 8).
The imaging features of fibrosarcoma can
mimic those of congenital hemangioma and
other sarcomas [74–76]. At US, infantile fibro-
sarcoma typically appears as a fairly homoge-
neous hyperechoic solid hypervascular mass
(Fig. 8). Anechoic cystic spaces due to internal
necrosis can be seen. MRI shows a predomi-
nantly solid mass that is isointense to hyperin-
tense on T1-weighted images and hyperintense
on T2-weighted images [77]. Variable cys-
tic components are also often present [69, 73]
(Fig. 8). Infantile fibrosarcoma is intensely en-
hancing after gadolinium administration [69].
Bony invasion, poor margination, neurovascu-
lar involvement, and size larger than 6 cm are
poor prognostic factors [69]. The diagnosis of
infantile fibrosarcoma may be suggested when
a large, enhancing soft-tissue mass is present in
an extremity of a neonate [69]. Infantile fibro-
sarcoma is highly chemosensitive, and surgery
is reserved for cases in which complete nonmu-
tilating resection can be achieved [2, 4].
Malignant Neonatal Soft-Tissue Tumors
Rhabdomyosarcoma—Rhabdomyosarcoma
(RMS) is a malignant neoplasm arising from
embryonic mesenchyme that has the capa-
198 AJR:209, July 2017
Restrepo et al.
bility of differentiating into skeletal muscle.
RMS accounts for more than 60% of soft-tis-
sue sarcomas in older children, and it is rare as
a congenital tumor [7, 78, 79]. Neonatal RMS
is usually of the embryonal type and presents
as a rapidly growing firm mass with occasion-
al skin discoloration, which causes it to be
confused with a hemangioma [73, 80]. RMS
most commonly occurs in the gluteal region,
perineum, thighs, and craniofacial and geni-
tourinary regions [81–83] (Fig. 9). A distinct
presentation of neonatal RMS with typical fa-
tal outcome is characterized by alveolar his-
tology, multiple skin and subcutaneous nod-
ules, and skin discoloration due to metastasis
(Fig. 10), the so-called blueberry muffin syn-
drome [31, 84, 85]. Neonates with RMS usu-
ally have a worse prognosis than do older chil-
dren with RMS [86].
At imaging, neonatal RMS can be solid or
partially cystic owing to necrosis. It can be
well defined or infiltrative, but it usually does
not invade adjacent bone (Fig. 9). At US, cys-
tic and solid components with various de-
grees of internal vascularity are usually pres-
ent. At MRI, neonatal RMS typically has
intermediate signal intensity on T1-weighted
images and intermediate to high signal in-
tensity on T2-weighted images [87]. The sol-
id portion of the tumor is avidly enhancing
[87]. The current treatment of neonatal RMS
is resection, often after chemotherapy.
Neuroblastoma—Neuroblastoma is one
of the most common malignant neonatal
neoplasms, and 16% of cases are diagnosed
within the first month of life [4, 88]. Neuro-
blastoma is a small round blue-cell tumor of
neuroendocrine origin usually occurring in
the adrenal glands. However, the first mani-
festation in the neonatal period can be mul-
tiple firm superficial soft-tissue nodules (Fig.
10), sometimes with purple skin discolor-
ation, the so-called blueberry muffin syn-
drome. Neoplasms with blueberry muffin
and blueberry muffin–like manifestations
include IH, myofibromatosis, alveolar RMS,
metastatic neuroblastoma, and congenital
leukemia. Unlike the neuroblastoma found
in older children, neonatal neuroblastoma
may spontaneously regress or undergo be-
nign transformation [4, 16, 89, 90].
Metastases of neuroblastoma to the soft tis-
sues have no specific imaging features, but be-
cause of their small size and widespread dis-
tribution, US is the ideal imaging modality
for initial evaluation. Superficial neuroblasto-
ma metastases are typically seen as solid hy-
poechoic nodules involving the dermis and
subcutaneous soft tissues with variable vascu-
larity. MRI or CT is necessary to identify and
stage the primary tumor (Fig. 10A). Neonatal
neuroblastoma is often initially observed be-
cause many tumors regress or undergo benign
transformation. For enlarging tumors, chemo-
therapy followed by resection of the primary
tumor is the current management.
Congenital leukemia—Congenital leuke-
mia is rare but is one of the most common
malignancies and a leading cause of death of
malignancy among neonates [4]. Although
occasionally transient and with reports of
spontaneous remissions, neonatal leukemia
carries a worse prognosis than leukemia oc-
curring later in childhood. Although acute
lymphocytic leukemia is the most common
type in older children, acute myeloid leuke-
mia accounts for approximately two-thirds
of cases of acute leukemia in neonates. A tu-
moral syndrome with organomegaly is seen
in 80% of cases, and skin and subcutaneous
nodules (blueberry muffin syndrome) are ob-
served in approximately 60% of cases [4, 6,
91] (Figs. 10B and 10C).
There are no specific imaging findings
of neonatal leukemia, but US may show the
widespread solid nodules in the subcutaneous
soft tissues and dermis, which should prompt
evaluation of the internal organs. Organomeg-
aly due to diffuse organ infiltration can also
be present (Figs. 10B and 10C). The diagnosis
of neonatal leukemia is based on analysis of a
peripheral smear and bone marrow aspirate.
Chemotherapy is the treatment of choice, but
such management poses therapeutic, ethical,
and prognostic challenges in neonates [4].
Conclusion
Neonatal soft-tissue neoplasms are those
diagnosed in the first month of life. Imaging
evaluation is mostly performed with US and
MRI. Although some tumors may have char-
acteristic imaging findings that can point to
the correct diagnosis, biopsy or even excision
may be required for a definitive diagnosis.
Some neonatal soft-tissue tumors may spon-
taneously regress and carry a better progno-
sis. However, the management of benign but
locally aggressive and malignant neonatal
soft-tissue tumors is often challenging and
may require a combination of chemothera-
py and resection by a multidisciplinary team.
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 Imaging Evaluation of Neonatal Soft-Tissue Tumors
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A B C

D E F
Fig. 1—Infantile hemangioma.
A, 6-week-old premature girl with focal infantile hemangioma born with focal reddish macule in area that grew over time. Clinical photograph shows round raised
superficial red nodule (arrow) in midline of upper back.
B, Same patient as in A. Transverse gray-scale ultrasound image shows raised hypoechoic solid nodule (arrows) involving dermis and superficial subcutaneous soft tissues.
C, Same patient as in A and B. Transverse color Doppler ultrasound image shows markedly increased vascularity of nodule and its deep feeding vessels.
D, 25-day-old girl born with segmental infantile hemangioma. Clinical photograph shows large raised red plaque involving anterolateral aspect of forearm and wrist.
E, Same patient as in D. Coronal T1-weighted MR image shows extensive plaquelike mass involving dermis and subcutaneous soft tissues with component isointense to
muscle (asterisks) with interspersed hyperintense elements of fat (black arrow). Prominent flow voids (white arrow) correspond to arteries. Prominent arteries are also
present.
F, Same patient as in D and E. Coronal contrast-enhanced fat-suppressed T1-weighted MR image shows marked and homogeneous enhancement of hemangioma (arrows).

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 Restrepo et al.
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A B C
Fig. 2—1-day-old boy with rapidly involuting congenital hemangioma.
A, Clinical photograph shows large mass in right popliteal fossa with bluish and reddish discoloration with peripheral pale halo.
B, Transverse gray-scale ultrasound image shows solid mass (calipers) with indistinct borders (arrow) in some areas.
C, Transverse color Doppler ultrasound image shows marked internal hypervascularity of mass.

Fig. 3—3-week-old girl with fibromatosis colli who

presented with torticollis.
A and B, Longitudinal gray-scale ultrasound image
of right side of neck (B) shows marked fusiform
enlargement of sternocleidomastoid muscle (caliper
set 2) compared with normal contralateral (A) muscle
(caliper set 1).
A B

Fig. 4—6-week-old boy with painless foot lump since

birth that proved to be lipoblastoma.
A, Transverse gray-scale ultrasound image of
right foot shows well-defined solid mass (calipers)
in plantar aspect of mid foot. Mass is echogenic,
suggesting fatty composition, and has anechoic
cystic areas (arrows) containing mucous material.
B, Short-axis T1-weighted MR image shows mass
(calipers) that is isointense to subcutaneous fat,
confirming fatty nature of mass.
A B

Fig. 5—4-week-old boy with infantile myofibroma that presented as hard lump in upper back. Transverse high-
resolution gray-scale ultrasound image shows small nodule (arrows) with heterogeneous echogenicity but with
anechoic center (asterisk) involving dermis, subcutaneous soft tissues, and fascia of underlying paraspinal
muscle. D = dermis, M = muscle.

202 AJR:209, July 2017

 Imaging Evaluation of Neonatal Soft-Tissue Tumors

Fig. 6—3-week-old boy with visible painless neck

mass due to solitary fibrous tumor.
A, Axial STIR MR image shows solid round mass
(arrows) with hypointense center (asterisk) and
peripheral hyperintensity in subcutaneous soft tissues
anterior to left sternocleidomastoid muscle (M).
B, Axial gadolinium-enhanced T1-weighted MR image
shows mild heterogeneous enhancement (asterisks)
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by mass (arrows). M = left sternocleidomastoid

muscle.

A B

A B
Fig. 7—2-week-old girl with kaposiform hemangioendothelioma who presented with enlarged right thigh and violaceus discoloration.
A, Axial STIR MR image shows diffuse enlargement and edema of right thigh due to infiltrating soft-tissue mass (asterisks) with soft-tissue edema.
B, Axial gadolinium-enhanced T1-weighted MR image shows enhancement of infiltrating thigh mass (asterisks) and dermal thickening (arrow).

A B C
Fig. 8—3-week-old boy with congenital infantile fibrosarcoma that presented at birth as painless, rapidly growing hand mass. (Courtesy of Moreno LÁ, Fundacion
Hospital de la Misericordia, Bogota, Colombia)
A, Clinical photograph shows mass has bluish discoloration and tense overlying skin.
B, T1-weighted MR image shows large mass (white arrows) involving anterior and posterior hand compartments with homogeneous parenchyma. Mass encases
extensor tendons and erodes second and third metacarpals (black arrows).
C, Fat-saturated T2-weighted MR image shows mass to be homogeneously hyperintense (arrows). Because of congenital nature, rapid growth, skin discoloration, and
vascularity, lesion was initially thought to represent congenital hemangioma.

AJR:209, July 2017 203

 Restrepo et al.

Fig. 9—2-day-old girl with widespread metastatic rhabdomyosarcoma detected in utero. Coronal
T2-weighted MR image shows large mass (calipers) with solid and cystic (asterisk) component. Multiple
pulmonary and hepatic metastatic lesions (arrows) are also present. Patient also had many subcutaneous
nodules throughout body.
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A B C
Fig. 10—Soft-tissue metastases.
A, 12-day-old girl with neuroblastoma presenting as superficial soft-tissue nodules. Axial T2-weighted MR image of chest shows lobulated mass (NB) hyperintense to
muscle in left paraspinal region with extension into chest wall and spinal canal (asterisks). Intraspinal component is obliterating CSF space and compressing cord (black
arrow). Metastatic foci (white arrows) are present in subcutaneous soft tissues and muscle.
B, 3-week-old boy with leukemia who presented with palpable chest wall lumps. Transverse gray-scale ultrasound image shows two sharp round hypoechoic solid
nodules (arrows) in subcutaneous soft tissues of anterior chest wall. R = rib.
C, Same patient as in B. Longitudinal gray-scale ultrasound image shows hepatomegaly (caliper set 1) and nephromegaly (arrows).

204 AJR:209, July 2017