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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
Introduction An abdominal mass is a common clinical
presentation, and a small percentage of such patients will The presentation of abdominal mass is common, and the
have an abdominal wall tumour with the two most common diagnosis of hernia is usually clinically apparent. However,
pathologies being fibromatosis and soft tissue sarcoma. a proportion of these clinical presentations will be soft
Methods Here we present the available literature on the tissue tumours and management strategies will be com-
diagnosis and management of both fibromatosis and soft pletely different.
tissue sarcoma, in the context of our experience in a tertiary The most common tumours affecting the abdominal wall
referral centre for sarcoma. are benign simple lipomas. However, other less common
Results and discussion Appropriate cross-sectional imaging pathologies may also present in a very similar way. The
and a pre-operative tissue diagnosis by percutaneous core biopsy next most common abdominal wall tumours are desmoid
are necessary to define management. Desmoid fibromatosis can fibromatosis and soft tissue sarcoma (STS).
be managed initially by observation with serial imaging, with In this review, we discuss the diagnosis and manage-
surgery being reserved for those patients who demonstrate pro- ment of abdominal wall tumours, with particular focus on
gression. Soft tissue sarcoma can display a range of pathologies STS and fibromatosis, from the viewpoint of a tertiary
from relatively indolent tumours to locally aggressive sarcomas referral sarcoma specialist centre.
that can readily metastasise. An accurate pre-operative histo-
logical diagnosis and staging enables a multidisciplinary
approach to management. This may include chemotherapy and Diagnosis
radiotherapy, but the mainstay of treatment remains wide sur-
gical resection and abdominal wall reconstruction. Patient out- The annual clinical incidence of benign soft tissue tumours
comes are worse if referral is delayed or if the sarcoma is is estimated at 3,000/million population [1, 2] and that
incompletely resected without an initial tissue diagnosis. 95 % of those presenting to primary care with a clinically
suspicious soft tissue lump will be benign. Even within
Keywords Abdominal wall  Fibromatosis  Soft secondary care, a majority of patients seen with soft tissue
tissue sarcoma  Imaging  Biopsy 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
K. J. Williams
cause of morbidity [3, 4]. Therefore, differentiation of
Academic Section of Vascular Surgery, Imperial College
London, London, UK these two groups is of paramount importance, both to
e-mail: k.williams@imperial.ac.uk patient and clinician.
Clinical criteria have been developed to raise the sus-
A. J. Hayes (&)
picion of possible soft tissue malignancy, specifically: (1)
Department of Sarcoma and Melanoma, The Royal Marsden
Hospital, Fulham Road, London SW11 6JJ, UK size greater than 5 cm, (2) a mass deep to deep fascia, (3) a
e-mail: andrew.hayes@rmh.nhs.uk mass increasing in size, and (4) recurrence at the site of

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previous excision [3]. However, these guidelines have not accuracy to define management, and all patients with a
been validated in the general population and ultimately solid mass will require a pre-operative core biopsy for
clinical examination may not be sensitive or specific [5, 6]. diagnosis. A benefit of CT scanning is that as well as
When there is a high index of suspicion of a soft tissue delineating size, location, and relationships to adjacent
malignancy as per these clinical criteria, most specialist vital structures and intra-abdominal viscera, it can stage the
sarcoma centres encourage referral on clinical suspicion chest for possible metastatic disease at the same time [8].
alone [7]. However, the majority of soft tissue masses will
prove not to be malignant and, therefore, imaging can be Positron emission tomography
helpful in reinforcing the impression that the mass is
indeed benign in nature. Positron emission tomography (PET) scanning is rarely
The main aim of imaging is to raise the possibility of necessary for the diagnosis of primary abdominal wall
soft tissue malignancy such that inadvertent enucleation of tumours, and is reserved for the assessment of possible
sarcoma can be avoided. recurrent disease, particularly when a biopsy is not possible
due to anatomical considerations.

Imaging of suspicious soft tissue masses


Tissue diagnosis
While in the majority of cases, no form of imaging can be
sufficiently sensitive or specific to diagnose a STS, imaging The mainstay of diagnosis of possible malignancy of the
can both raise the diagnostic possibility of STS, as well as abdominal wall is by pre-operative core needle biopsy [2,
providing crucial anatomical information for surgical 3, 9]. Primary enucleation or marginal excision of a solid
planning. soft tissue mass as a diagnostic manoeuvre is an error in
management, and results in increased rates of local recur-
Ultrasound rence if the mass is malignant [1012].
The diagnosis of STS is more accurate by core needle
USS can provide excellent evaluation of dimension, vas- biopsy when compared to fine needle aspiration (FNA),
cularity, and relationship to other soft tissue planes. It is and can accurately define histological subtype [13, 14].
also widely available, quick, and well tolerated by patients Strauss et al. have shown an accuracy of 97 % for char-
[5]. Lakkaraju et al. recommend its use as a first-line acterising benign versus malignant lesions, and a sensi-
investigation of patients with a clinical soft tissue mass and tivity of 81 % for high versus low grade sarcoma. Due to
as a tool for triaging patients requiring further investiga- the extensive range of mesenchymal tumour subtypes, and
tion, allowing timely patient reassurance, or fast-tracking the heterogeneity of histology that may be present in the
as appropriate. Their prospective study of 358 patients over tumour, FNA has been shown to have an inferior diagnostic
6 months showed 100 % specificity for malignancy, with 6 accuracy when compared to core needle biopsy. FNA also
out of the 95 cases evaluated as suspicious further identi- precludes immunohistochemistry or molecular genetic
fied via biopsy and MRI as malignant tumours. analysis.
Multiple core needle biopsies can be taken either free-
Computed tomography/magnetic resonance imaging hand or under image guidance with local anaesthetic.
Biopsy should be undertaken through skin which will be
Some form of cross-sectional imaging of abdominal wall subsequently resected, as needle tract recurrence has been
tumours is necessary both to aid diagnosis, but more reported, although it is rare [15]. Biopsy should not be done
importantly for surgical planning. The two commonest over the dome of the tumour if the skin is considered at
forms of cross-sectional imaging are computed tomography risk, as skin breakdown and tumour fungation may occur.
(CT) and magnetic resonance imaging (MRI). MRI has Most specialist sarcoma centres prefer that biopsies of
superior diagnostic specificity than CT scanning because of possible sarcomas are undertaken at a sarcoma unit; both
better delineation of soft tissues. This is particularly true because of the availability of specialist pathological
for tumours in the extremities, but is less important for expertise to assess the biopsy, but also such that the
tumours arising in the abdominal wall. MRI can be prone operating surgeon can place the biopsy at an appropriate
to movement artefact. It should be noted that diagnosis of site for future surgical resection.
solid soft tissue tumours arising in the abdominal wall and If core biopsy fails to secure the diagnosis, which is rare,
at other sites is made by core needle biopsy. Accordingly, then open incisional biopsy may be employed, but inter-
the diagnostic superiority of MRI over CT is not particu- ruption and contamination of surgical planes may affect
larly relevant because neither has sufficient diagnostic further surgical management. CT scanning can assist in

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Hernia (2014) 18:9197 93

targeting the biopsy needle to informative sampling areas, risk factors include oestrogen exposure and abdominal wall
and is particularly helpful in large heterogeneous tumours trauma. Indeed, deep fibromatosis of the abdominal wall
where a small random sample may not be representative of commonly occurs in young women following pregnancy,
the whole. 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
Management wall fibromatosis carries a better prognosis than that arising
in the extremities [9, 23]. The behaviour of deep fibro-
The evaluation of any abdominal wall tumour involves matosis is not readily predictable, except for its inability to
clinical examination, radiographic evaluation to charac- metastasise. They are typically slow growing; however,
terise the size and anatomical location of the tumour, and reports of rapidly progressive and aggressive tumours are
identification of possible metastatic disease and a pre- noted by many in the literature, as are episodes of spon-
operative histopathological diagnosis made by percutane- taneous regression. An individual treatment plan, with
ous core biopsy. The diagnosis can include a heterogeneous multidisciplinary input, is important. Important factors to
group of pathologies with a wide array of biological consider are unequivocal diagnosis, severity of patient
behaviour as well as primary pathologies such as sarcoma symptoms, size, and rate of growth.
and fibromatosis, secondary pathologies can also present as Evaluation for the possibility of FAP should be con-
abdominal wall masses (e.g. metastatic carcinoma, mela- sidered pre-operatively in patients with deep fibromatosis,
noma, lymphoma, arteriovenous malformation, iatrogenic which includes a detailed family history, endoscopy of the
implantation of endometriosis or intra-abdominal malig- gastrointestinal tract, and sequencing of the APC gene.
nancy at laparoscopic port site). The management path- Fibromatosis is known to occur in 10 % of FAP patients,
ways of each will vary widely, therefore diagnosis is although this is usually intra-abdominal [17, 20] (Fig. 1).
paramount prior to intervention. Historically, surgical treatment of fibromatosis is wide
full-thickness circumferential excision with one uninvolved
Desmoid fibromatosis anatomical plane in all directions, and control can be
achieved in 70 % of cases [9, 24]. This becomes difficult in
These are benign slow-growing tumours of myofibroblast the context of the abdominal wall, especially when the
origin causing local invasion but not distant metastasis. The tumour is large, infiltrates important anatomical structures,
incidence of this tumour is 24 new cases per 1,000,000 or when intra-abdominal involvement exists. The goals of
per year [16, 17]. They can be classified as superficial or reconstruction are to restore skin and musculofascial
deep. Superficial fibromatosis of the hand is well known as integrity. If the peritoneum is excised, a neoperitoneum
Dupuytrens contracture, or of the foot as plantar fibro- can be created by suturing the greater omentum to the
matosis. It is often described as firm, well defined, and margins of the defect to separate bowel from mesh, to
smooth to palpation, arising from musculoaponeurotic prevent incorporation of small bowel with possible en-
structures. Deep fibromatosis arising in muscle or apo- terocutaneous fistulation and complex wound management
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 Fig. 1 Deep desmoid fibromatosis of the abdominal wall

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94 Hernia (2014) 18:9197

problems. In the absence of contamination, the abdominal be higher and do not correlate closely with resection
wall defect may be closed with single or double layers of margin [21, 30]. With reference to abdominal wall fibroma-
prolene mesh [25]. Incisional hernias are more common tosis, extra-abdominal site has a reported recurrence hazard
with lateral abdominal wall repairs, and careful mesh ratio 4.66 (p \ 0.05), and an independent hazard ratio of 3.28
placement with appropriate tension is important [17]. of worse 5-year recurrence-free survival after controlling for
Myocutaneous flaps may be employed as an alternative to tumour size and margin status [31]. In cases of local recur-
mesh repair. Negative margins may not be achievable due rence, repeat excision should be considered [32].
to anatomical or quality of life considerations, and this Non-surgical therapies for desmoid fibromatosis are also
must be balanced against increased risk of local recurrence used with varying levels of success. These include anti-
[26]. Adjuvant therapies, or salvage repeat resections for inflammatory agents, hormonal therapy, systemic chemo-
recurrence, may be appropriate in this cohort [17]. In therapy, and Imatinib. Radiotherapy is increasingly used to
Mullen et al.s retrospective case series, survival after treat recurrence of fibromatosis, and surgical outcomes for
intention-to-treat margin positive resection was improved recurrence are reported to be worse when compared to
with the use of adjuvant radio- and/or medical therapy surgery for primary lesion [16].
(p = 0.066) [26].
Current evidence seems to suggest that a watch and Soft tissue sarcomas
wait policy with serial MRIs over 3 months can help to
identify those patients who would benefit most from Sarcomas are a rare and diverse group of cancers that arise
immediate resection, and those in which either medical or from mesenchymal cells, a lineage that normally produces
conservative strategies may be employed [24]. Bonvalot connective tissue structures such as bone, cartilage, muscle,
et al. [16] compared a prospective cohort of 112 patients blood vessels, nerves and fat. They are classified as either
newly diagnosed with fibromatosis, and have identified a bone or STSs. STSs are rare malignancies with an inci-
potential cohort of patients where marginal excision dence of around 1,0002,000 people in the UK per annum
resulted in a much worse outcome when compared to non- [3, 14]. 10 % of sarcomas occur in the abdominal wall,
surgical strategies or R0 resection. They suggest that a less with much higher rates in the limbs. Most are sporadic, but
aggressive local strategy may be considered first, and also can be associated with familial cancer syndromes (Gardner
represents an alternative in situations where surgery or is Syndrome, neurofibromatosis, Li Fraumeni Syndrome, and
expected to be marginal, or would result in major func- familial retinoblastoma) and previous radiation exposure,
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 common with increasing age and have a slight male pre-
two serial MRIs by 25 %, or appearance of a new lesion, ponderance. A common presentation is of an incidental
was used as their threshold for resection. Fiore et al. painless mass having no apparent deleterious effect on
identified retrospectively a group of 142 patients in whom a general health or function. This, combined with the rarity
deliberate frontline conservative policy was initially of STS often leads to misinterpretation as a benign con-
employed. This represented 73 % of patients presenting dition. Swedish epidemiological data has shown that
with primary tumour, and 43 % of those with local recur- superficial soft tissue masses greater than 5 cm, and all
rence. They observed a 5-year progression-free survival deep-seated soft tissue lesions, have a 10 % risk of being a
rate of abdominal wall desmoids of 49.9 % [22]. The watch sarcoma [1, 34]. In addition to the potential for locally
and wait group was characterised by having smaller or non- destructive growth and recurrence, STS carries a significant
symptomatic tumours, being younger, and having a female risk of distant metastases, ranging from 20 to 100 %
preponderance. Local recurrences were more likely to be depending on histological type and grade. Sarcoma grading
given systemic medical treatment. Mullen makes the point is dependent on histological features, but there is no cur-
that inherent tumour biology may have an overarching rently agreed single system by which to do this [14]. Most
impact on growth and recurrence over other potential use a scoring system that assesses degree of necrosis,
prognostic factors. If a policy of initial observation is mitotic count, cellularity, differentiation/pleomorphism,
applied and surgery is limited to patients who progress, and myxoid areas [3538]. The score for each is added up
then quality of surgical margins is important and should be and the totals divided into three or four groups. A low score
properly factored into the decision [24]. represents grade 1, and the highest scores grade 3 or 4.
After wide excision and mesh reconstruction, recurrence Intermediate and high grade tumours have significantly
of abdominal wall fibromatosis is unusual, with reported poorer outcomes for recurrence, metastasis and survival
rates of less than 10 % at 5 years in some carefully selected than those of low grade. At first presentation of sarcoma,
series [23, 2729]. This may be contrasted to that of the 10 % of patients will have lung metastases, which is a
limbs and shoulder girdle, where recurrence rates seem to common cause of death in this population [2] (Table 1).

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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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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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