Hernia (2014) 18:9197
DOI 10.1007/s10029-013-1156-x
REVIEW
A guide to oncological management of soft tissue tumours
of the abdominal wall
K. J. Williams A. J. Hayes
Received: 1 April 2013 / Accepted: 6 September 2013 / Published online: 17 September 2013
Springer-Verlag France 2013
Abstract
Introduction An abdominal mass is a common clinical
presentation, and a small percentage of such patients will
have an abdominal wall tumour with the two most common
pathologies being fibromatosis and soft tissue sarcoma.
Methods Here we present the available literature on the
diagnosis and management of both fibromatosis and soft
tissue sarcoma, in the context of our experience in a tertiary
referral centre for sarcoma.
Results and discussion Appropriate cross-sectional imaging
and a pre-operative tissue diagnosis by percutaneous core biopsy
are necessary to define management. Desmoid fibromatosis can
be managed initially by observation with serial imaging, with
surgery being reserved for those patients who demonstrate pro-
gression. Soft tissue sarcoma can display a range of pathologies
from relatively indolent tumours to locally aggressive sarcomas
that can readily metastasise. An accurate pre-operative histo-
logical diagnosis and staging enables a multidisciplinary
approach to management. This may include chemotherapy and
radiotherapy, but the mainstay of treatment remains wide sur-
gical resection and abdominal wall reconstruction. Patient out-
comes are worse if referral is delayed or if the sarcoma is
incompletely resected without an initial tissue diagnosis.
Keywords Abdominal wall
Fibromatosis
Soft
tissue sarcoma
Imaging
Biopsy
K. J. Williams
Academic Section of Vascular Surgery, Imperial College
London, London, UK
e-mail: k.williams@imperial.ac.uk
A. J. Hayes (&)
Department of Sarcoma and Melanoma, The Royal Marsden
Hospital, Fulham Road, London SW11 6JJ, UK
e-mail: andrew.hayes@rmh.nhs.uk
Introduction
The presentation of abdominal mass is common, and the
diagnosis of hernia is usually clinically apparent. However,
a proportion of these clinical presentations will be soft
tissue tumours and management strategies will be com-
pletely different.
The most common tumours affecting the abdominal wall
are benign simple lipomas. However, other less common
pathologies may also present in a very similar way. The
next most common abdominal wall tumours are desmoid
fibromatosis and soft tissue sarcoma (STS).
In this review, we discuss the diagnosis and manage-
ment of abdominal wall tumours, with particular focus on
STS and fibromatosis, from the viewpoint of a tertiary
referral sarcoma specialist centre.
Diagnosis
The annual clinical incidence of benign soft tissue tumours
is estimated at 3,000/million population [1, 2] and that
95 % of those presenting to primary care with a clinically
suspicious soft tissue lump will be benign. Even within
secondary care, a majority of patients seen with soft tissue
tumours are likely to have a benign lesion. However, it has
also been recognised that delay in diagnosis at presentation
of malignant disease, particularly sarcoma, is a significant
cause of morbidity [3, 4]. Therefore, differentiation of
these two groups is of paramount importance, both to
patient and clinician.
Clinical criteria have been developed to raise the sus-
picion of possible soft tissue malignancy, specifically: (1)
size greater than 5 cm, (2) a mass deep to deep fascia, (3) a
mass increasing in size, and (4) recurrence at the site of
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previous excision [3]. However, these guidelines have not
been validated in the general population and ultimately
clinical examination may not be sensitive or specific [5, 6].
When there is a high index of suspicion of a soft tissue
malignancy as per these clinical criteria, most specialist
sarcoma centres encourage referral on clinical suspicion
alone [7]. However, the majority of soft tissue masses will
prove not to be malignant and, therefore, imaging can be
helpful in reinforcing the impression that the mass is
indeed benign in nature.
The main aim of imaging is to raise the possibility of
soft tissue malignancy such that inadvertent enucleation of
sarcoma can be avoided.
Imaging of suspicious soft tissue masses
While in the majority of cases, no form of imaging can be
sufficiently sensitive or specific to diagnose a STS, imaging
can both raise the diagnostic possibility of STS, as well as
providing crucial anatomical information for surgical
planning.
Ultrasound
USS can provide excellent evaluation of dimension, vas-
cularity, and relationship to other soft tissue planes. It is
also widely available, quick, and well tolerated by patients
[5]. Lakkaraju et al. recommend its use as a first-line
investigation of patients with a clinical soft tissue mass and
as a tool for triaging patients requiring further investiga-
tion, allowing timely patient reassurance, or fast-tracking
as appropriate. Their prospective study of 358 patients over
6 months showed 100 % specificity for malignancy, with 6
out of the 95 cases evaluated as suspicious further identi-
fied via biopsy and MRI as malignant tumours.
Computed tomography/magnetic resonance imaging
Some form of cross-sectional imaging of abdominal wall
tumours is necessary both to aid diagnosis, but more
importantly for surgical planning. The two commonest
forms of cross-sectional imaging are computed tomography
(CT) and magnetic resonance imaging (MRI). MRI has
superior diagnostic specificity than CT scanning because of
better delineation of soft tissues. This is particularly true
for tumours in the extremities, but is less important for
tumours arising in the abdominal wall. MRI can be prone
to movement artefact. It should be noted that diagnosis of
solid soft tissue tumours arising in the abdominal wall and
at other sites is made by core needle biopsy. Accordingly,
the diagnostic superiority of MRI over CT is not particu-
larly relevant because neither has sufficient diagnostic
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accuracy to define management, and all patients with a
solid mass will require a pre-operative core biopsy for
diagnosis. A benefit of CT scanning is that as well as
delineating size, location, and relationships to adjacent
vital structures and intra-abdominal viscera, it can stage the
chest for possible metastatic disease at the same time [8].
Positron emission tomography
Positron emission tomography (PET) scanning is rarely
necessary for the diagnosis of primary abdominal wall
tumours, and is reserved for the assessment of possible
recurrent disease, particularly when a biopsy is not possible
due to anatomical considerations.
Tissue diagnosis
The mainstay of diagnosis of possible malignancy of the
abdominal wall is by pre-operative core needle biopsy [2,
3, 9]. Primary enucleation or marginal excision of a solid
soft tissue mass as a diagnostic manoeuvre is an error in
management, and results in increased rates of local recur-
rence if the mass is malignant [1012].
The diagnosis of STS is more accurate by core needle
biopsy when compared to fine needle aspiration (FNA),
and can accurately define histological subtype [13, 14].
Strauss et al. have shown an accuracy of 97 % for char-
acterising benign versus malignant lesions, and a sensi-
tivity of 81 % for high versus low grade sarcoma. Due to
the extensive range of mesenchymal tumour subtypes, and
the heterogeneity of histology that may be present in the
tumour, FNA has been shown to have an inferior diagnostic
accuracy when compared to core needle biopsy. FNA also
precludes immunohistochemistry or molecular genetic
analysis.
Multiple core needle biopsies can be taken either free-
hand or under image guidance with local anaesthetic.
Biopsy should be undertaken through skin which will be
subsequently resected, as needle tract recurrence has been
reported, although it is rare [15]. Biopsy should not be done
over the dome of the tumour if the skin is considered at
risk, as skin breakdown and tumour fungation may occur.
Most specialist sarcoma centres prefer that biopsies of
possible sarcomas are undertaken at a sarcoma unit; both
because of the availability of specialist pathological
expertise to assess the biopsy, but also such that the
operating surgeon can place the biopsy at an appropriate
site for future surgical resection.
If core biopsy fails to secure the diagnosis, which is rare,
then open incisional biopsy may be employed, but inter-
ruption and contamination of surgical planes may affect
further surgical management. CT scanning can assist in
Hernia (2014) 18:9197
targeting the biopsy needle to informative sampling areas,
and is particularly helpful in large heterogeneous tumours
where a small random sample may not be representative of
the whole.
Management
The evaluation of any abdominal wall tumour involves
clinical examination, radiographic evaluation to charac-
terise the size and anatomical location of the tumour, and
identification of possible metastatic disease and a pre-
operative histopathological diagnosis made by percutane-
ous core biopsy. The diagnosis can include a heterogeneous
group of pathologies with a wide array of biological
behaviour as well as primary pathologies such as sarcoma
and fibromatosis, secondary pathologies can also present as
abdominal wall masses (e.g. metastatic carcinoma, mela-
noma, lymphoma, arteriovenous malformation, iatrogenic
implantation of endometriosis or intra-abdominal malig-
nancy at laparoscopic port site). The management path-
ways of each will vary widely, therefore diagnosis is
paramount prior to intervention.
Desmoid fibromatosis
These are benign slow-growing tumours of myofibroblast
origin causing local invasion but not distant metastasis. The
incidence of this tumour is 24 new cases per 1,000,000
per year [16, 17]. They can be classified as superficial or
deep. Superficial fibromatosis of the hand is well known as
Dupuytrens contracture, or of the foot as plantar fibro-
matosis. It is often described as firm, well defined, and
smooth to palpation, arising from musculoaponeurotic
structures. Deep fibromatosis arising in muscle or apo-
neurotic tissues presents as hard often painless masses that
can attain considerable size. In the abdominal wall these
tumours have a predisposition to affect the rectus abdo-
minis muscle although any muscle of the abdominal wall
can be affected. Deep fibromatosis of the extremities can
affect any muscle in the extremities, but has a tendency to
affect proximal muscle groups of the limb girdle. Macro-
scopically they commonly resemble scar tissue, often
exhibiting gross infiltration of adjacent structures [17].
Microscopically they are composed of collagen-rich spin-
dle cells, and immunohistochemistry often shows distinct
nuclear accumulation of beta-catenin. The aetiology of
desmoid fibromatosis has not been established, but it can be
associated with familial adenomatous polyposis (FAP) and
Gardners syndrome [9, 18, 19]. Those associated with
FAP are more likely to have intra-abdominal involvement,
and their growth and compression of neighbouring struc-
tures can be a cause of significant morbidity [20]. Other
93
risk factors include oestrogen exposure and abdominal wall
trauma. Indeed, deep fibromatosis of the abdominal wall
commonly occurs in young women following pregnancy,
where both these risk factors may combine [21]. In Fiores
2009 paper, he reports pregnancy in 51 % of his reported
abdominal desmoid fibromatosis cases [22]. Abdominal
wall fibromatosis carries a better prognosis than that arising
in the extremities [9, 23]. The behaviour of deep fibro-
matosis is not readily predictable, except for its inability to
metastasise. They are typically slow growing; however,
reports of rapidly progressive and aggressive tumours are
noted by many in the literature, as are episodes of spon-
taneous regression. An individual treatment plan, with
multidisciplinary input, is important. Important factors to
consider are unequivocal diagnosis, severity of patient
symptoms, size, and rate of growth.
Evaluation for the possibility of FAP should be con-
sidered pre-operatively in patients with deep fibromatosis,
which includes a detailed family history, endoscopy of the
gastrointestinal tract, and sequencing of the APC gene.
Fibromatosis is known to occur in 10 % of FAP patients,
although this is usually intra-abdominal [17, 20] (Fig. 1).
Historically, surgical treatment of fibromatosis is wide
full-thickness circumferential excision with one uninvolved
anatomical plane in all directions, and control can be
achieved in 70 % of cases [9, 24]. This becomes difficult in
the context of the abdominal wall, especially when the
tumour is large, infiltrates important anatomical structures,
or when intra-abdominal involvement exists. The goals of
reconstruction are to restore skin and musculofascial
integrity. If the peritoneum is excised, a neoperitoneum
can be created by suturing the greater omentum to the
margins of the defect to separate bowel from mesh, to
prevent incorporation of small bowel with possible en-
terocutaneous fistulation and complex wound management
Fig. 1 Deep desmoid fibromatosis of the abdominal wall
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problems. In the absence of contamination, the abdominal
wall defect may be closed with single or double layers of
prolene mesh [25]. Incisional hernias are more common
with lateral abdominal wall repairs, and careful mesh
placement with appropriate tension is important [17].
Myocutaneous flaps may be employed as an alternative to
mesh repair. Negative margins may not be achievable due
to anatomical or quality of life considerations, and this
must be balanced against increased risk of local recurrence
[26]. Adjuvant therapies, or salvage repeat resections for
recurrence, may be appropriate in this cohort [17]. In
Mullen et al.s retrospective case series, survival after
intention-to-treat margin positive resection was improved
with the use of adjuvant radio- and/or medical therapy
(p = 0.066) [26].
Current evidence seems to suggest that a watch and
wait policy with serial MRIs over 3 months can help to
identify those patients who would benefit most from
immediate resection, and those in which either medical or
conservative strategies may be employed [24]. Bonvalot
et al. [16] compared a prospective cohort of 112 patients
newly diagnosed with fibromatosis, and have identified a
potential cohort of patients where marginal excision
resulted in a much worse outcome when compared to non-
surgical strategies or R0 resection. They suggest that a less
aggressive local strategy may be considered first, and also
represents an alternative in situations where surgery or is
expected to be marginal, or would result in major func-
tional or cosmetic defects. Where a watch and wait risk increasing with dose [14, 33]. They become more
policy is employed, documented progression of disease on
two serial MRIs by 25 %, or appearance of a new lesion,
was used as their threshold for resection. Fiore et al.
identified retrospectively a group of 142 patients in whom a
deliberate frontline conservative policy was initially
employed. This represented 73 % of patients presenting
with primary tumour, and 43 % of those with local recur-
rence. They observed a 5-year progression-free survival
rate of abdominal wall desmoids of 49.9 % [22]. The watch
and wait group was characterised by having smaller or non-
symptomatic tumours, being younger, and having a female
preponderance. Local recurrences were more likely to be
given systemic medical treatment. Mullen makes the point
that inherent tumour biology may have an overarching
impact on growth and recurrence over other potential
prognostic factors. If a policy of initial observation is
applied and surgery is limited to patients who progress,
then quality of surgical margins is important and should be
properly factored into the decision [24].
After wide excision and mesh reconstruction, recurrence
of abdominal wall fibromatosis is unusual, with reported
rates of less than 10 % at 5 years in some carefully selected
series [23, 2729]. This may be contrasted to that of the
limbs and shoulder girdle, where recurrence rates seem to
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Hernia (2014) 18:9197
be higher and do not correlate closely with resection
margin [21, 30]. With reference to abdominal wall fibroma-
tosis, extra-abdominal site has a reported recurrence hazard
ratio 4.66 (p \ 0.05), and an independent hazard ratio of 3.28
of worse 5-year recurrence-free survival after controlling for
tumour size and margin status [31]. In cases of local recur-
rence, repeat excision should be considered [32].
Non-surgical therapies for desmoid fibromatosis are also
used with varying levels of success. These include anti-
inflammatory agents, hormonal therapy, systemic chemo-
therapy, and Imatinib. Radiotherapy is increasingly used to
treat recurrence of fibromatosis, and surgical outcomes for
recurrence are reported to be worse when compared to
surgery for primary lesion [16].
Soft tissue sarcomas
Sarcomas are a rare and diverse group of cancers that arise
from mesenchymal cells, a lineage that normally produces
connective tissue structures such as bone, cartilage, muscle,
blood vessels, nerves and fat. They are classified as either
bone or STSs. STSs are rare malignancies with an inci-
dence of around 1,0002,000 people in the UK per annum
[3, 14]. 10 % of sarcomas occur in the abdominal wall,
with much higher rates in the limbs. Most are sporadic, but
can be associated with familial cancer syndromes (Gardner
Syndrome, neurofibromatosis, Li Fraumeni Syndrome, and
familial retinoblastoma) and previous radiation exposure,
common with increasing age and have a slight male pre-
ponderance. A common presentation is of an incidental
painless mass having no apparent deleterious effect on
general health or function. This, combined with the rarity
of STS often leads to misinterpretation as a benign con-
dition. Swedish epidemiological data has shown that
superficial soft tissue masses greater than 5 cm, and all
deep-seated soft tissue lesions, have a 10 % risk of being a
sarcoma [1, 34]. In addition to the potential for locally
destructive growth and recurrence, STS carries a significant
risk of distant metastases, ranging from 20 to 100 %
depending on histological type and grade. Sarcoma grading
is dependent on histological features, but there is no cur-
rently agreed single system by which to do this [14]. Most
use a scoring system that assesses degree of necrosis,
mitotic count, cellularity, differentiation/pleomorphism,
and myxoid areas [3538]. The score for each is added up
and the totals divided into three or four groups. A low score
represents grade 1, and the highest scores grade 3 or 4.
Intermediate and high grade tumours have significantly
poorer outcomes for recurrence, metastasis and survival
than those of low grade. At first presentation of sarcoma,
10 % of patients will have lung metastases, which is a
common cause of death in this population [2] (Table 1).
Hernia (2014) 18:9197 95

Table 1 The World Health Organization lists the following cell types can be useful in the characterization of STS due to its
in its classification of soft tissue sarcomas (adapted from AJCC ability to distinguish tumour tissue from adjacent muscle
Cancer Staging Manual [39])
and fat, as well as define relationships to neurovascular
Adipocytic tumours structures. It also provides information on haemorrhage,
Dedifferentiated liposarcoma necrosis, oedema, cystic and myxoid degeneration, and
Myxoid/round cell liposarcoma fibrosis.
Pleomorphic liposarcoma Several staging systems have been published in an
Fibroblastic/myofibroblastic tumours attempt to predict outcome. In the UK, the French
Fibrosarcoma FNCLCC system assesses tumour differentiation, mitotic
Myxofibrosarcoma, low grade count, and tumour necrosis to generate a grade (13). A
Low grade fibromyxoid sarcoma multidisciplinary team of specialist medical oncologists,
Sclerosing epithelioid fibrosarcoma radiation oncologists and specialist surgeons will then
So-called fibrohistiocytic tumours construct a treatment plan tailored to the lesions predicted
Undifferentiated pleomorphic sarcoma/malignant fibrous pattern of local growth, risk of metastases, and likely sites
histiocytoma (including pleomorphic, giant cell, myxoid/high of distant spread. A properly executed wide local excision
grade myxofibrosarcoma, and inflammatory forms) with negative histopathological margins in all directions
Smooth muscle tumours remains the most important part of a curative treatment
Leiomyosarcoma plan [40]. However, as previously discussed, abdominal
Skeletal muscle tumours wall resections may be complicated by involvement of skin
Rhabdomyosarcoma (embryonal, alveolar, and pleomorphic or intra-abdominal viscera. The very different biological
forms) behaviours of sarcomas, depending on histological subtype
Vascular tumours and grade, will also greatly affect management.
Epithelioid hemangioendothelioma Mesh reconstruction of the abdominal wall and dia-
Deep angiosarcoma phragm can be achieved in a straightforward fashion;
Tumours of peripheral nerves however, reconstruction of the thoracic wall may require
Malignant peripheral nerve sheath tumour the input of a specialist thoracic surgeon. Again, if peri-
Chondro-osseous tumours toneum is resected, formation of an omental neoperitoneum
Extraskeletal chondrosarcoma (mesenchymal and other variants) may reduce the incidence of enteric complications.
Extraskeletal osteosarcoma Surgical excision is normally combined with radiother-
Tumours of uncertain differentiation apy and/or systemic chemotherapy (agents such as doxo-
Synovial sarcoma rubicin, ifosfamide) [41]. The optimum combination is
Epithelioid sarcoma controversial, and must balance the goal of minimising
Alveolar soft part sarcoma recurrence against preserving function and quality of life
Clear cell sarcoma of soft tissue [42, 43]. The majority of randomised trials of chemother-
Extraskeletal myxoid chondrosarcoma apy have shown no significant impact on overall survival,
Primitive neuroectodermal tumour (PNET)/extraskeletal Ewing but some have shown improved local control [4446]. The
tumour high variability of patient characteristics, regimens, ana-
Desmoplastic small round cell tumour tomical sites, histological grading, variable follow-up and
Extrarenal rhabdoid tumour differing endpoints mean that the results of these trials
Undifferentiated sarcoma; sarcoma, not otherwise specified must be interpreted with caution [41]. Post-operative
(NOS) adjuvant radiotherapy has been shown to be beneficial in a
subset of truncal sarcomas [47]. Palliation may be appro-
priate for high grade or irresectable tumours, or those with
Careful physical examination and radiographic evalua- multiple metastases. Lung metastasectomy may be bene-
tion to evaluate the size, depth and location, along with ficial for both palliation of symptomatic pulmonary disease
signs of neurovascular involvement are essential for and improving long-term survival, although data is scarce
designing the best therapeutic approach [2]. Spiral CT is [48] (Figs. 2, 3).
preferable for examining sarcomas of the chest and abdo- Local recurrence rates are high (25 %) with a 5-year
men, since MRIs of this region are degraded by air/tissue survival rate between 50 and 60 % [49]. These are closely
interface, motion artefact, and the presence of calcium. It related to tumour grade, size, depth, site, and incomplete
can also provide staging information, as the lungs are the surgical resection margins [9]. Unplanned sarcoma resec-
most common site of metastases, and for this reason is the tion has been shown in some centres to increase the chance
most common imaging modality in this population. MRI of local recurrence despite re-excision, and be detrimental

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Fig. 2 Grade 2 sarcoma in rectus abdominis muscle treated by
surgical resection, mesh reconstruction, and neo-adjuvant treatment
Fig. 3 Large high grade sarcoma treated successfully by pre-
operative adjuvant radiotherapy, surgical resection, and mesh
reconstruction
to long-term survival due to an increased rate of distant
metastases [10, 12, 50].
Conclusion
An abdominal mass is a common clinical presentation, and
a small percentage of such patients will have an abdominal
wall tumour with the two most common pathologies being
fibromatosis and STS. Appropriate cross-sectional imaging
and a pre-operative tissue diagnosis by percutaneous core
biopsy are necessary to define management.
Desmoid fibromatosis can be treated initially by obser-
vation with serial imaging, with surgery being reserved for
those patients who demonstrate progression. STS can dis-
play a range of pathologies form relatively indolent
tumours to locally aggressive sarcomas with that can
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Hernia (2014) 18:9197
readily metastasise. An accurate pre-operative histological
diagnosis and staging enables a multidisciplinary approach
to management. This may include chemotherapy and
radiotherapy, but the mainstay of treatment remains wide
surgical resection and abdominal wall reconstruction.
Patient outcomes are worse if referral is delayed or if the
sarcoma is incompletely resected without an initial tissue
diagnosis.
Conflict of interest K.J.W and A.J.H declare no conflict of interest.
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