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WHO Classification of Soft Tissue Tumors

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DOI: 10.1007/978-3-319-46679-8_11

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 WHO Classification of Soft Tissue
Tumors
Joan C. Vilanova
Contents
11.1  Introduction   
11.2  Adipocytic Tumors   
11.3  Fibroblastic/Myofibroblastic Tumors   
11.4  So-Called Fibrohistiocytic Tumors   
11.5  Smooth Muscle Tumors   
11.6  Pericytic (Perivascular) Tumors   
11.7  Skeletal Muscle Tumors   
11.8  Vascular Tumors   
11.9  Chondro-Osseous Tumors   
11.10  Gastrointestinal Stromal Tumors   
11.11  Peripheral Nerve Sheath Tumors   
11.12  Tumors of Uncertain Differentiation   
11.13  Undifferentiated/Unclassified
Sarcoma   
References   
J.C. Vilanova, MD, PhD
Department of Radiology, Clínica Girona. Institute
Catalan of Health – IDI. University of Girona,
Girona, Spain
e-mail: kvilanova@comg.cat
© Springer International Publishing AG 2017
F.M. Vanhoenacker et al. (eds.), Imaging of Soft Tissue Tumors, DOI 10.1007/978-3-319-46679-8_11
kvilanova@comg.cat
11
11.1 Introduction
187
Soft tissue tumors (STT) represent a complex
188 group of lesions that may show a broad range of
189 differentiation. The WHO (World Health
190 Organization) classification was established and
up-to-dated in 2013 for the purpose of uniformity
190
[1]. The WHO system helps to unify the lexicon
191 for the performance of clinical trials and to serve
191 as a guide for the multidisciplinary working
192
group of specialists such as radiologists, patholo-
gists, and orthopedic oncologists, to improve the
192 patient’s management and outcome. The nomen-
193 clature has been adapted by the American Joint
193 Cancer Commission (AJCC) for sarcoma staging
and by the College of American Pathologists
193
Cancer protocols for soft tissue sarcomas. The
2013 classification is the 4th edition and replaces
195
the previous edition in 2002 [2]. The major modi-
195 fications from the previous edition are the addi-
tion of three new chapters: gastrointestinal
stromal tumors (GIST), nerve sheath tumors, and
undifferentiated/unclassified sarcomas.
The WHO classification incorporates detailed
clinical, histological, and genetic data. There are
also imaging features in the new edition of soft
tissue tumor classification.
The classification of soft tissue tumors of the
WHO includes the following groups: (1) adipo-
cytic tumors, (2) fibroblastic/myofibroblastic
tumors, (3) so-called fibrohistiocytic tumors, (4)
smooth muscle tumors, (5) pericytic (perivascu-
lar) tumors, (6) skeletal muscle tumors, (7) vas-
187
188 J.C. Vilanova

Table 11.1  Changes and update relevant for radiologists in the 2013 WHO classification of tumors of the soft tissue
Tumor category Major changes
Adipocytic Mixed-type liposarcoma removed
Fibroblastic DFSP and giant cell fibroblastoma included for the first time
Myofibroblastic “Hemangiopericytoma” removed as a synonym for SFT
Recognition of nodular fasciitis and variants as true neoplasms
So-called fibrohistiocytic “Malignant fibrous histiocytoma” removed
Smooth muscle Angioleiomyoma reclassified as pericytic tumor
Pericytic Angioleiomyoma reclassified as a pericytic tumor
Myofibroma now classified as pericytic tumor
Skeletal muscle Spindle cell/sclerosing rhabdomyosarcoma classified together and
separated from others subtypes
Vascular Pseudomyogenic (epithelioid sarcoma-like)
Hemangioendothelioma added as a new entity
Gastrointestinal stromal GIST included in the volume on STT for the first time
Nerve sheath Peripheral nerve sheath tumors included volume on STT for the first time
New hybrid benign nerve sheath tumors included (schwannoma/
perineurioma)
Tumors of uncertain differentiation New tumors: acral fibromyxoma, hemosiderotic fibrolipomatous tumor
differentiation
Phosphaturic mesenchymal tumor
Atypical fibroxanthoma now included
PNET removed as synonym for Ewing’s sarcoma
Undifferentiated/other category Includes tumors that cannot be classified into any unclassified sarcoma
Abbreviations: WHO World Health Organization, DFSP dermatofibrosarcoma protuberans, SFT solitary fibrous tumor,
GIST gastrointestinal stromal tumor, STT soft tissue tumor, PNET primitive neuroectodermal tumor

cular tumors, (8) gastrointestinal stromal 11.2 Adipocytic Tumors

tumors, (9) nerve sheath tumors, (10) chondro-
osseous tumors, (11) tumors of uncertain differ-
entiation, and (12) undifferentiated/unclassified
sarcomas.
Generally, the WHO classification divides
each group in four categories according to bio-
logical behavior: benign, intermediate (locally
aggressive), intermediate (rarely metastasizing),
and malignant.
The new classification has incorporated more
detailed cytogenetic and molecular data in accor-
dance with the rapidly increasing knowledge of
genetics of tumors.
This chapter reviews the main features of the
classification with the major changes within the
different categories of soft tissue tumors.
Table  11.1 summarizes all relevant changes of
the 2013 WHO classification of STT.
Due to the high incidence of lipomas, angiolipo-
mas, and liposarcomas, adipocytic tumors repre-
sent the largest single group of mesenchymal
tumors. The WHO classification persists to con-
sider atypical lipoma (atypical lipomatous tumor)
and well-differentiated liposarcoma as essen-
tially synonymous (Table 11.2) and considered
locally aggressive with no potential for metasta-
sis [1]. The terms “mixed-type liposarcoma” and
myxolipoma have been deleted.
In children diffuse lipoblastoma is now the
preferred term for lipoblastomatosis.
The definition of dedifferentiated liposar-
coma has been slightly modified and is cur-
rently subdivided into four groups (see
Table  11.1). Chondroid lipoma may resemble
myxoid liposarcoma. Unlike myxoid liposar-

kvilanova@comg.cat
11  WHO Classification of Soft Tissue Tumors 189

Table 11.2  Group 1 – Adipocytic tumors Table 11.3 Group 2 – Fibroblastic/myofibroblastic
tumors
Benign
Lipoma Benign
Lipomatosis Nodular fasciitis (proliferative fasciitis/proliferative
myositis)
Lipomatosis of the nerve
Myositis ossificans
Lipoblastoma
Elastofibroma
Angiolipoma
Fibromatosis colli
Myolipoma of the soft tissue
Fibrous hamartoma of infancy
Chondroid lipoma
Juvenile hyaline fibromatosis
Spindle cell lipoma/pleomorphic lipoma
Inclusion body fibromatosis
Hibernoma
Fibroma of the tendon sheath
Intermediate (locally aggressive)
Desmoplastic fibroblastoma
Atypical lipomatous tumor/well-differentiated
liposarcoma Mammary-type myofibroblastoma
Malignant Calcifying fibrous tumor
Dedifferentiated liposarcoma Angiofibroma
Myxoid liposarcoma Angiomyofibroblastoma
Pleomorphic liposarcoma Gardner fibroma
Liposarcoma, not otherwise specified Calcifying fibrous tumor
Intermediate (locally aggressive)
Superficial fibromatosis (plantar/palmar)
coma chondroid lipoma usually shows calcifi- Desmoid-type fibromatosis
cations, which are best demonstrated by Lipofibromatosis
radiographs, CT, or US [3]. Giant cell fibroblastoma
Intermediate (rarely metastasizing)
Dermatofibrosarcoma protuberans
11.3 Fibroblastic/Myofibroblastic Solitary fibrous tumor
Tumors Inflammatory myofibroblastic tumor
Myofibroblastic sarcoma/atypical myxoinflammatory
Fibroblastic/myofibroblastic tumors represent a fibroblastic tumor
very large group of mesenchymal tumors, many Infantile fibrosarcoma
of which contain fibroblastic as well as myofi- Malignant
broblastic elements (Table 11.3). The current Adult fibrosarcoma
WHO classification recognizes that nodular fas- Myxofibrosarcoma
ciitis, proliferative fasciitis, and proliferative Low-grade fibromyxoid sarcoma
Sclerosing epithelioid fibrosarcoma
myositis (Fig. 11.1) are neoplastic [4], whereas
previously they were believed to be reactive
lesions. locally aggressive (intermediate) category
Other changes were the inclusion of the because it recurs in approximately 50 % of
closely related giant cell fibroblastoma and der- cases, but does not metastasize [6].
matofibrosarcoma protuberans (DFSP), for- Hemangiopericytoma has been removed from
merly included in volume on skin lesions. DFSP the classification of “extrapleural solitary
is categorized as a rarely metastasizing (inter- fibrous tumor” because it is well established that
mediate) tumor, although it should be noted that this outdated term included tumors that repre-
metastatic potential is gained only when a com- sented cellular examples of SFT [5]. Lipomatous
ponent of a fibrosarcomatous change is present hemangiopericytoma and giant cell angiofi-
[5]. Giant cell fibroblastoma is classified in the broma have also been included as SFT. The term

kvilanova@comg.cat
190
Fig. 11.1 Proliferative myositis of the thigh. Axial
T2-WI image shows a well-defined mass (arrow) within
the adductor muscle with inhomogeneous mild high sig-
nal intensity with areas of low signal within the lesion due
to the fibrous content
“atypical myxoinflammatory fibroblastic tumor”
was introduced for as synonym for “myxoin-
flammatory fibroblastic sarcoma.” This new
term better reflects the extremely low potential
for metastasis for this tumor type.
11.4 So-Called Fibrohistiocytic
Tumors
As the concept of fibrohistiocytic differentiation
has been a matter of debate [7], malignant fibrous
histiocytoma (MFH) has been finally deleted
from the current WHO classification. MFH and
its subtypes have been reclassified in the new
separate group of unclassified/undifferentiated
sarcomas (group 12). Thus, group 3 now does
not include malignant tumors (Table 11.4). It
includes the common tenosynovial giant cell
tumors, the uncommon giant cell tumor
kvilanova@comg.cat
J.C. Vilanova
Table 11.4  Group 3 – The so-called fibrohistiocytic
tumors
Benign
Tenosynovial giant cell tumor
 Localized type
 Diffuse type
 Malignant
Deep benign fibrous histiocytoma
Intermediate (rarely metastasizing)
Plexiform fibrohistiocytic tumor
Giant cell tumor of the soft tissue
Fig. 11.2  Giant cell tumor of the soft tissue with Paget’s
disease. Axial T2-WI image shows an intermediate signal
nodular lesion in the pretibial subcutaneous space (thin
arrow). Note the cortical thickening due to Paget’s disease
(thick arrow)
(Fig.  11.2) of soft tissues, and the plexiform
fibrohistiocytic tumor.
11.5 Smooth Muscle Tumors
Smooth muscle tumors arising at non-cutaneous,
non-uterine locations have been the focus of a
considerable conceptual shift in the past but
there were no major changes in this category
(Table  11.5). The only change is that
11  WHO Classification of Soft Tissue Tumors 191

Table 11.5  Group 4 – Smooth muscle tumors Table 11.6  Group 5 – Pericytic (perivascular) tumors
Benign Benign
Leiomyoma of the deep soft tissue Glomus tumors (and variants)
Malignant  Glomangiomatosis
Leiomyosarcoma (excluding the skin)    Malignant glomus tumor
Myopericytoma
 Myofibroma
a­ ngioleiomyoma (vascular leiomyoma) was real-  Myofibromatosis
located to the category of pericytic (perivascular) Angioleiomyoma
tumors (group 5). There are no changes on the
deep leiomyosarcoma, although some tumors
Table 11.7  Group 6 – Skeletal muscle tumors
classified as undifferentiated pleomorphic sar-
coma closely resemble a subset of leiomyosar- Benign
coma, which may suggest the existence of Rhabdomyoma
 Adult type
“dedifferentiated leiomyosarcoma.”
 Fetal type
 Genital type
Malignant
11.6 Pericytic (Perivascular)
Embryonal rhabdomyosarcoma
Tumors
Alveolar rhabdomyosarcoma
Pleomorphic rhabdomyosarcoma
The lesions in the pericytic/perivascular category
Spindle cell/sclerosing rhabdomyosarcoma
were first included in the 2002 edition. At the
same time, hemangiopericytoma was removed
from this group and listed as synonym for soli-
tary fibrous tumor (group 2). Although rare, the
most common tumor analyzed on imaging in this
group is glomus tumor [8], composed of cells
resembling the modified smooth muscle cells of
the normal glomus body. It has been acknowl-
edged that myofibroma/myofibromatosis repre-
sent morphological features along the spectrum
of myopericytic neoplasms (Table 11.6) instead
of fibroblastic/myofibroblastic tumors (group 2),
which were included in the past. Angioleiomyoma
(vascular leiomyoma) which typically presents as
a painful lesion in the distal extremities of
middle-­aged adults has now been included in this
group [9, 10].

Fig. 11.3 Rhabdomyoma of the neck. Axial T2-WI

11.7 Skeletal Muscle Tumors
Two entities have been added: rhabdomyoma and
rhabdomyosarcoma (Table 11.7).
Rhabdomyoma is subdivided into cardiac and
extracardiac types and pathologically as adult,
fetal (immature skeletal muscle fibers), and geni-
tal (female or male genital tract). Extracardiac
image shows a large well-circumscribed lesion of inter-
mediate signal intensity within the parapharyngeal space
extending anteriorly bilaterally in the subfascial planes of
the muscles and showing a lobulated appearance (arrows)
adult rhabdomyoma (Fig. 11.3) is rarely reported
on imaging [11]. Spindle cell/sclerosing rhabdo-
myosarcoma is recognized as a separate tumor
different from embryonal rhabdomyosarcoma.

kvilanova@comg.cat
192
The prognosis of spindle cell rhabdomyosarcoma
in adults is worse than in children, and recent
insights into genetics of this tumor suggest that
there may be underlying genetic differences
between pediatric and adult cases [12].
11.8 Vascular Tumors
Benign vascular tumors are very common and
most frequently occur in the skin. It is often dif-
ficult to determine whether they represent mal-
formations, true neoplasms, or reactive
processes [13]. Hemangiomas are classified his-
tologically as synovial, venous, arteriovenous,
mixed malformations, and intramuscular
(Table 11.8). “Pseudomyogenic (epithelioid sar-
coma-like) hemangioendothelioma” [14] has
been added as a rarely metastasizing neoplasm.
This tumor commonly arises in young adult
males, often presents as multiple discontiguous
nodules in different tissue planes of a limb
(Fig. 11.4), and may involve the skin as well as
deep soft tissues and the bone. This tumor tends
Table 11.8  Group 7 – Vascular tumors
Benign
Hemangioma
 Synovial
 Venous
 Arteriovenous hemangioma/malformation
Epithelioid hemangioma
Angiomatosis
Lymphangioma
Intermediate (locally aggressive)
Kaposiform hemangioendothelioma
Intermediate (rarely metastasizing)
Retiform hemangioendothelioma
Papillary intralymphatic angioendothelioma
Composite hemangioendothelioma
Pseudomyogenic (epithelioid sarcoma-like)
hemangioendothelioma
Kaposi sarcoma
Malignant
Epithelioid hemangioendothelioma
Angiosarcoma of the soft tissue
kvilanova@comg.cat
J.C. Vilanova
to be highly FDG-­avid, and therefore PET-CT is
useful for the detection of deep lesions.
There are no changes on the classification of
angiosarcoma and epithelioid hemangioendothe-
lioma (EHE) although new genetic data has
shown that multifocal EHE most likely arises as
a result of metastatic disease rather than repre-
senting synchronous primary tumors as previ-
ously thought.
Immunohistochemical analysis can differenti-
ate postradiation vascular proliferation from
angiosarcoma [15].
11.9 Chondro-Osseous Tumors
There are no changes on the chondro-osseous soft
tissue tumors (Table 11.9). Myositis ossificans was
regarded as a (myo)fibroblastic lesion in the 2002
version, and extraskeletal myxoid chondrosarcoma
(EMC) was also classified in the tumors of uncer-
tain differentiation, since it shows little evidence of
cartilaginous differentiation [2], despite the name.
A synonym for EMC is chordoid sarcoma.
Fig. 11.4 Pseudomyogenic (epithelioid sarcoma-like)
hemangioendothelioma of the hand. Axial STIR MR
image of the hand shows two nodular high signal intensity
lesions at the subcutaneous tissue of the hypothenar
region (arrows)
Table 11.9  Group 8 – Chondro-osseous tumors
Benign
Soft tissue chondroma
Malignant
Mesenchymal chondrosarcoma
Extraskeletal osteosarcoma
11  WHO Classification of Soft Tissue Tumors
Mesenchymal chondrosarcoma of soft tissues
occurs less frequently than the EMC, but nearly
half of the mesenchymal chondrosarcomas are
extraskeletal in location [16].
Extraskeletal osteosarcoma, soft tissue osteo-
sarcoma, shows similar histologic features of bone
osteosarcoma without systematic genetic differ-
ences [1].
11.10 Gastrointestinal Stromal
Tumors
This group has been added, gastrointestinal stromal
tumor (GIST), which is the most common primary
mesenchymal tumor in the gastrointestinal tract.
Histologically GIST are classified as benign,
uncertain malignant potential, and malignant.
Prognostic factors are tumor size, mitotic activity,
and anatomical site [17]. Almost 50 % arise in the
stomach, 30 % in the small intestine, and the rest
for the colon, rectum, and esophagus and primary
disseminated with unspecified site of origin.
Isolated cases have been reported in the appendix.
As GIST present with abdominal symptoms and
are detected by ultrasound or CT scans of the abdo-
men (Fig. 11.5), these lesions are not referred to as
musculoskeletal tumors as such.
Fig. 11.5  Gastrointestinal stromal tumor (GIST) of the
stomach. Non-contrast-enhanced CT shows a bulky exo-
phytic gastric mass with well-circumscribed outer con-
tours (arrow). No calcifications are present
kvilanova@comg.cat
193
11.11 P
 eripheral Nerve Sheath
Tumors
Nerve sheath tumors were previously included
in the 2007 WHO classification of tumors of the
central nervous system. Although imaging
reviews on soft tissue tumors already regarded
nerve sheath tumors previously as typical soft
tissue tumors [18], nerve sheath tumors have
been included for the first time in the WHO
classification of soft tissue tumors since 2013
(Table 11.10). Hybrid nerve sheath tumors, such
as schwannoma/perineurioma [19] and neurofi-
broma/schwannoma, have been included in the
group of peripheral nerve sheath tumors. The
latter might be related to NF-2, NF-1, or schwan-
nomatosis [20].
11.12 T
 umors of Uncertain
Differentiation
Tumors with unknown clear line of cell differentia-
tion are included in this group. It includes a long list
of tumors (Table 11.11). New entities have been
Table 11.10  Group 10 – Peripheral nerve sheath tumors
Benign
Schwannoma (including variants)
Melanotic schwannoma
Neurofibroma (including variants)
Perineurioma
 Malignant intermediate perineurioma
Granular cell tumor
Dermal nerve sheath myxoma
Solitary circumscribed neuroma
Ectopic meningioma
Nasal glial heterotopia
Benign triton tumor
Hybrid nerve sheath tumors
Malignant
Malignant peripheral nerve sheath tumor
Epithelioid malignant peripheral nerve sheath tumor
Malignant triton tumor
Malignant granular cell tumor
Ectomesenchymoma
194 J.C. Vilanova

Table 11.11 Group 11 – Tumors of uncertain

differentiation
Benign
Acral/digital fibromyxoma
Intramuscular – juxtaarticular myxoma
Deep (“aggressive”) angiomyxoma
Pleomorphic hyalinizing angiectatic tumor
Ectopic hamartomatous thymoma
Intermediate (locally aggressive)
Hemosiderotic fibrolipomatous tumor
Intermediate (rarely metastasizing)
Atypical fibroxanthoma
Angiomatoid fibrous histiocytoma
Ossifying fibromyxoid tumor
Mixed tumor
Myoepithelioma/carcinoma
Phosphaturic mesenchymal tumor
Fig. 11.6 Aggressive angiomyxoma of the pelvis.
Malignant Coronal T2-WI shows an hyperintense mass (long arrows)
Synovial sarcoma extending through the left ischiorectal fossa with a lay-
Epithelioid sarcoma ered appearance (short arrows)
Alveolar soft part sarcoma
Clear cell sarcoma of the soft tissue
Extraskeletal myxoid chondrosarcoma
Malignant mesenchymoma
Desmoplastic small round cell tumor
Extraskeletal Ewing sarcoma
Extrarenal rhabdoid tumor
Neoplasms with perivascular epithelioid cell
differentiation (PEComa)
Intimal sarcoma

included: acral fibromyxoma (digital fibromyx-

oma), hemosiderotic fibrolipomatous tumor, phos-
phaturic mesenchymal tumor, and atypical
fibroxanthoma. Among the group of malignant
lesions, primitive neuroectodermal tumor (PNET)
has been dropped as a synonym for Ewing’s sar-
coma in order to minimize confusion with similarity
named lesions in the CNS and female genital tract.
Extraskeletal myxoid chondrosarcoma is
included in this category as there is no convinc-
ing evidence of cartilaginous differentiation.
Deep (“aggressive”) angiomyxoma is an uncom-
mon slowly growing neoplasm with a predilection
for pelvic and perineal regions [21] and tendency to
local recurrence and characteristic features on MR
(Fig. 11.6).
Ossifying fibromyxoid tumor of the soft tissue is
a well-circumscribed lobulated hard tumor cov-
ered by a thick fibrous pseudocapsule (Fig. 11.7).
Fig. 11.7  Ossifying fibromyxoid tumor of the foot. Axial
T2-WI shows a large mass (long arrows) of low signal
intensity extending within the subcutaneous tissue. The
low signal intensity can be attributed to calcification
(short arrows)
It was surprising that the term “synovial sar-
coma” remained unchanged in the current
updated classification. As the lesion is not
derived from true synovial cells and may
involve virtually any body part (Fig. 11.8) [21,
22], the term “synovial sarcoma” is indeed a
misnomer. Therefore, future revisions of the
WHO classification on STT should consider to
abandon the confusing term “synovial
sarcoma.”

kvilanova@comg.cat
11  WHO Classification of Soft Tissue Tumors
Fig. 11.8  The so-called synovial sarcoma of the lower
leg. Axial fat-suppressed T1-WI after contrast shows
homogeneously enhancing lesion in the subcutaneous tis-
sue extending through the muscular fascia to deep com-
partment (arrow). As no nearby synovial tissue can be
related to the mass in this location, the term “synovial” is
a misnomer for this lesion which is derived from undif-
ferentiated mesenchymal stem cell
11.13 Undifferentiated/
Unclassified Sarcoma
This new category of tumors, introduced for
the first time in the 2013 classification, recog-
nizes the fact that a small, but significant, sub-
set of sarcomas cannot be classified into any
presently defined categories [23]. This group
of tumors was previously included in the fibro-
histiocytic group (group 3), namely, “malig-
nant fibrous histiocytoma.” This group of
tumors might have spindle cell, pleomorphic,
round cell, or epithelioid morphology
(Table 11.12). A subset of radiation-­associated
sarcomas falls into this category. These lesions
show no definable line of differentiation
using currently available technologies.
Dedifferentiated types of specific sarcomas are
not included in this category. Undifferentiated/
unclassified sarcoma accounts for up to 20 %
of all sarcomas and about a quarter of these are
radiation-­associated tumors [24]. It is likely
that this group of lesions will be subject to
future reclassification along with ongoing
progress in molecular genetics.
kvilanova@comg.cat
195
Table 11.12  Group 12 – Undifferentiated/unclassified
sarcoma
Undifferentiated spindle cell sarcoma
Undifferentiated pleomorphic sarcoma
Undifferentiated round cell sarcoma
Undifferentiated epithelioid sarcoma
Undifferentiated sarcoma, not otherwise specified
Key Points
1. The WHO classification of soft tissue
tumors has been up-to-dated in 2013 for
the purpose of uniformity.
2. The new WHO classification has
included three new groups of tumors:
gastrointestinal stromal tumors (GIST),
nerve sheath tumors, and undifferenti-
ated/unclassified sarcomas.
3. Each group of tumors is divided in four
categories regarding the biological
potential: benign, intermediate (locally
aggressive), intermediate (rarely metas-
tasizing), and malignant.
4. Radiologist should know the WHO
classification to serve as a guide to work
in a multidisciplinary committee with
clinicians, surgeons, and pathologists.
5. Imaging (especially MRI) plays a pivotal
role in the work-up of soft tissue tumors.
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