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ORTHOPAEDIC ONCOLOGY

Surgical management of sphere of STS due to locally aggressive or rarely metastasizing


behaviours.

soft tissue sarcoma STS are a heterogeneous group of tumours with variable
clinical, prognostic and therapeutic features. They can occur
anywhere with the extremities being the most common site,
Kathryn H Steele followed by the trunk.1 Of the peripheral and truncal tumours,
Anna Raurell two thirds arise beneath the deep fascia and have an average
diameter of 9e11 cm at diagnosis; the remainder are more su-
Robert U Ashford perficially located with an average diameter of 5e6 cm at pre-
sentation.2,3 Retroperitoneal, intra-abdominal, gynaecological
and pelvic tumours are often large at time of presentation and
Abstract require management by a specialist sarcoma surgeon with the
Soft tissue sarcomas (STS) are a heterogeneous group of tumours and appropriate site-specific expertize. The management of these is
surgery is the principal treatment. The majority occur in the limbs and outside the scope of this article.
trunk wall and this article refers to management of these anatomical Guidelines for management of STS are available from various
sites. STS is managed within a specialist multidisciplinary team and sources4e7 The principal treatment of primary STS is surgical
an individualized treatment plan is produced for each patient. Histo- excision often combined with adjunctive radiotherapy. Chemo-
logical, anatomical and patient factors will all inuence the strategy. therapy or other pharmacological agents are also utilized for
In localized disease, the surgical aim is to achieve a wide excision specific tumour sub-types and within therapeutic trials.
with negative margin and preserve function. Radiotherapy may be indi- The surgical management of peripheral and trunk wall STS is
cated to reduce the risk of local recurrence and can be given pre- and/ described in this article.
or post-operatively. Planned marginal excision combined with radio-
therapy is an acceptable approach in order to preserve critical struc- Diagnosis
tures. Neo-adjuvant radiotherapy can reduce tumour burden and
improve operability. However, amputation is sometimes the only treat- Whilst this article does not focus on diagnosis, it is important to
ment option. Reconstructive surgery may be required to achieve establish the diagnosis prior to treatment and manage the patient
wound healing and restore function. The approach to management in a multidisciplinary team (MDT) setting. Image-guided core
of local recurrence is much the same as for primary disease. Advanced needle biopsy following MRI is the standard pathway. Occa-
disease is predominantly treated by systemic therapy but metastatec- sionally incisional and rarely excisional biopsy is required and
tomy is utilized in some circumstances. Follow-up is required to performed under the guidance of the MDT. Unplanned excision
monitor for recurrence, metastasis and complications of treatment. of a potentially malignant soft tissue mass is undesirable.
Keywords margin; radiotherapy; reconstruction; sarcoma; surgery
Surgical planning
Definitive resection of an STS should be performed at a desig-
Introduction nated STS treatment centre by an STS surgeon or a surgeon with
Soft tissue sarcomas (STS) are rare malignant mesenchymal tu- tumour site-specific skills in consultation with the sarcoma
mours arising in fat, muscle, nerve, blood vessels and other MDT.
connective tissues. The World Health Organization classification Treatment recommendations will be made by the MDT
of STS includes more than 50 different sub-types, which are for patients who have followed the prescribed referral pathway.
classified by tissue of origin and biological potential. Whilst the The MDT will typically have access to the following information:
term STS refers to malignant tumours, there are also some in-  clinical features and functional status (history and exami-
termediate soft tissue tumours that may be managed under the nation by STS surgeon)
 MRI with contrast (performed in conjunction with and
reported by STS radiologist)
 core needle biopsy (ideally image-guided, performed by
STS service, processed by a specialist sarcoma pathologist)
 staging CT chest (further imaging sometimes indicated
Kathryn H Steele PhD FRCS(Plast) Locum Consultant Plastic Surgeon,
depending on tumour site and type).
Department of Plastic Surgery, Nottingham City Hospital,
Nottingham, UK; East Midlands Sarcoma Service, UK. Conicts of All of these factors will influence the surgical procedure and
interest: none declared. its timing.
The aim of surgical resection is to achieve oncological clear-
Anna Raurell LMS FRCS(Plast) Consultant Plastic Surgeon, Department
ance with minimal morbidity, hence maximizing long-term sur-
of Plastic Surgery, Nottingham City Hospital, Nottingham, UK;
East Midlands Sarcoma Service, UK. Conicts of interest: none vival, avoiding local recurrence and preserving function. An
declared. algorithm of basic principles for treatment of localized STS is
outlined in Figure 1.
Robert U Ashford MD FRCS(Tr & Orth) Consultant Orthopaedic &
Where possible the aim should be to achieve a wide excision
Musculoskeletal Tumour Surgeon, Department of Plastic Surgery,
Nottingham City Hospital, Nottingham, UK; Leicester Orthopaedics, with negative surgical margin. In some cases this is possible
Leicester Royal Inrmary, Leicester, UK; East Midlands Sarcoma without a significant impact on function. When this is not
Service, UK. Conicts of interest: none declared. possible consideration should be given to the impact of resecting

ORTHOPAEDICS AND TRAUMA --:- 1 2017 Elsevier Ltd. All rights reserved.

Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

STS

Resectable & Not resectable


functional or functional
RT*

Chemo
WLE RT ILP

Negative Positive Not


Resectable Borderline
margin margin resectable

Low grade High grade


Superficial Deep Re-excision WLE PPM Amputation
< 5 cm > 5 cm

RT* Chemo Debulking


ILP
Chemo
Follow Up Best support

Figure 1 Algorithm for management of localized soft tissue sarcoma (STS). *Radiotherapy is given either pre-operatively or post-operatively in
resectable STS that are high-grade, deep or large. Chemotherapy may be used pre-operatively in chemo-sensitive STS sub-types that are difcult
to resect. Isolated limb perfusion is also a pre-operative treatment option for limb STS that are difcult to resect. Post-operative chemotherapy
may be offered in selected high-grade STS. RT, radiotherapy; WLE, wide local excision; Chemo, chemotherapy; ILP, isolated limb perfusion;
PPM, planned positive margin.

structures, the possibilities for reconstruction, the prognostic were excised with a wide margin of muscle or areolar tissue
implication of a more conservative excision and suitability for compared with those that had a wide margin of fascia or sub-
neo-adjuvant or adjuvant treatments, along with patient wishes. cutaneous tissue.9 Kawaguchi et al. analysed the width and
Although primary amputation is now unusual, it must still be quality of tissue comprising the margin and suggested that
considered according to patient preference and if reconstruction smaller margins were acceptable in the presence of a barrier such
of a functional limb is not possible or sensible. It must also be as periosteum or fascia.10 In addition specific STS sub-types (eg.
remembered that some STS are not resectable and in addition myxofibrosarcoma) have high risk of local recurrence despite
patient factors such as fitness for surgery or advanced dissemi- wide margin excision, whilst others have low risk despite mar-
nated disease may result in a decision not to operate. ginal excision.11 Therefore both tumour biology and the inherent
resistance of tissues to invasion also influence the planned
Margins margin.
Although excision margins of up to 5 cm have been suggested
Surgical
for STS, the introduction of adjuvant therapy has changed
There remains a lack of consensus regarding the desirable exci-
practice. The effect of radiotherapy on microscopic tumour has
sion margin in STS. Enneking et al. first described a model for
allowed substantial reduction in the surgical margin with some
sarcoma resection in 19818 based on the anatomical and patho-
logical features of sarcoma growth (Figure 2). This took account
of the potential presence of microscopic tumour in the reactive Radical
zone, or pseudocapsule around the tumour, and the possibility Pseudocapsule Wide
of microsatellites within adjacent normal tissues. Excision was Tumour Marginal
classified into four categories: Intralesional
 intralesional: the tumour is breached macroscopically
 marginal: the pseudocapsule forms at least part of the
periphery of the specimen
 wide: non-reactive normal tissue forms the entire periph-
ery of the specimen
 radical: all normal tissue of the anatomical site is removed
(e.g. fascial compartment and its sheath).
Figure 2 Schematic diagram of Ennekings description8 of soft tissue
Other groups have attempted to quantify the amount of
sarcoma excision. Cross-section of thigh showing tumour in adductor
normal tissue required for a safe margin in wide excision and in magnus with surrounding reactive zone or pseudocapsule. Four
doing so realized the importance of tissue composition. Rydholm different classes of surgical excision are depicted, indicating tissue
and Rooser observed that local recurrence was higher in STS that excised with each type of resection.

ORTHOPAEDICS AND TRAUMA --:- 2 2017 Elsevier Ltd. All rights reserved.

Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

studies showing that as little as 2 mm may be equivalent to wide certainly influence quality of life. PPM resection is illustrated in
excision in terms of local recurrence and metastasis.12 There Figure 3.
remains variation in the planned surgical margin between
Unplanned positive margins
different centres and many centres make exceptions depending
An unplanned positive margin may occur for two different rea-
on tumour sub-type. Approximately 1 cm of normal tissue or a
sons. It may be a completely unplanned excision in a non-STS
barrier layer (e.g. fascia) is commonly accepted as a wide local
institution, or it may be a planned excision with an unexpected
excision (WLE).
positive margin.
UK soft tissue sarcoma guidelines recommend that lumps that
Histological
are larger than 5 cm diameter, increasing in size, deep to fascia
The histological resection margin is classified according to the
or those that are painful are referred to an STS service for
Union for International Cancer Control (UICC):
assessment.5,6 Despite this cases of unplanned excision still
 R0: Microscopically clear
occur.2 In this situation the reporting pathologist will usually
 R1: Microscopically involved
refer the case to a specialist sarcoma pathologist and via them to
 R2: Macroscopically involved.
the STS MDT. Following appropriate staging investigations a re-
An R1 resection is therefore defined as microscopic presence
excision will be offered in the STS centre unless there is a strong
of tumour cells at an inked margin of the specimen. There has
contra-indication to this.
however been a lack of agreement regarding what constitutes a
The surgical approach to re-excision of a residual mass is the
negative margin. Whilst the absence of tumour cells at the
same as primary resection, aiming for either negative margin or
margin is classified as R0, some published series have required
planned positive margin. When no residual mass is evident
2 mm of uninvolved tissue in their classification of a negative
clinically or radiologically, an estimation needs to be made of the
margin,12 and therefore direct comparisons within the literature
potential involved area of tissue. Pre-operative images of the
are difficult. Since the introduction of adjuvant therapy it is more
mass (if available) and post-operative images of oedema and
usual to describe the margin as simply positive or negative. The
scarring will aid this assessment. It can be difficult to define the
probability of a negative margin is influenced by tumour biology,
operative field accurately and in most cases the area is widely
with some sub-types such as myxofibrosarcoma being particu-
excised. Success of this procedure depends on the degree of
larly difficult to excise due to a diffusely infiltrative pattern. A
spillage at the original surgery, tumour biology and size
negative margin can yield 5-year local recurrence-free and
of margin; the Toronto group reported a local recurrence rate of
disease-specific survival rates of 92% and 87% respectively.13 A
21e32% within 5 years if the re-excision yielded a positive
typical sarcoma histopathology report should define the clear-
margin, but the disease-specific survival was only slightly lower
ance in millimetres of the closest margin, the type of tissue at the
than in negative margin resection at 85%.13 Patients in this
margin, whether the tumour is infiltrative or pushing at its
category have smaller mean tumour size with a higher propor-
border, presence of vascular invasion and response to any in-
tion of low grade and superficial lesions than seen in STS inci-
duction therapy.
dence overall.15 These data demonstrate significantly inferior
local control for a group that should have better than average
Planned positive margins
prognosis and highlight the influence of tumour biology on
The emphasis on limb salvage and function preservation does
survival.
not always permit a negative margin surgical resection. A posi-
A truly unexpected positive margin occurring during excision
tive margin may be planned to permit preservation of a vital
at an STS centre may be due to inadvertent visualization of
structure, provided that adjuvant radiotherapy is utilized. The
tumour in the case of rapid growth, or due to underestimation of
Toronto group have published most extensively on positive
tumour burden and microscopic spread along tissue planes.
margins. Gerrand et al. reported a local recurrence rate of 3.6%
Identified risk factors for positive margins include age over 50
at 3 years with planned positive margins.14 Subsequently
years, low-grade tumours, tumours with diameter more than 7
ODonnell et al. have performed a more extensive analysis with
cm, deep tumours or tumours involving the trunk.16 In contrast
larger patient numbers and longer follow-up time.13 Planned
to patients who had unplanned excision, the 5-year local recur-
close margins for preservation of critical structures with pre-
rence rate was 38% and the disease-specific survival 59% in the
and/or post-operative radiotherapy resulted in 15% local recur-
Toronto series.13 If the positive margin is recognized intra-
rence and 59% disease-specific survival rate at 5 years in re-
operatively, it should be revised to a wider margin where
sections that proved to be R1, versus 3% and 80% in resections
possible. If the positive margin is recognized after histological
that were classified as R0. Patients who underwent resection of a
analysis, a further wide excision should be considered if
critical structure due to encasement had a 5-year local recurrence
anatomical constraints permit it.
rate of 9% and a disease-specific survival of 64%. As the
improvement in recurrence and survival with resection of the
Peri-operative adjuvant treatment
critical structure is only limited, it is likely that tumour invasion
of critical structures indicates biological aggressiveness and Radiotherapy has become standard practice for intermediate- and
supports the practice of planned positive margins (PPM) for high-grade tumours, deep tumours, tumours with diameter more
preservation of function. There is no doubt that a negative than 5 cm, and close resection margin or R1 resection.4,5,7 Its
margin is preferable but the MDT must weigh the benefits of purpose is to reduce the incidence of local recurrence.
wider excision against morbidity and functional outcome, as it Isolated limb perfusion (ILP) with melphalan and tumour
may not particularly influence disease-specific survival but could necrosis factor-alpha (TNF-a) has a specific pre-operative role in

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Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

Figure 3 Excision of undifferentiated pleomorphic sarcoma left thigh. (a) Axial MRI image showing sarcoma in adductor compartment with planned
marginal resection on femoral vessels and femur. (b) Tumour bed post-excision with vascular slings around femoral vessels and arrow indicating
femur. (c) Marked specimen for histopathological analysis. (d) Primary wound closure.

attempting to reduce the size of selected limb tumours that would  marginal excision of vital structures is not possible and
otherwise require amputation.17 ILP requires inflation of a tour- reconstructive outcome would be poor, neo-adjuvant
niquet proximal to the tumour and is only presently available at therapy is given followed by repeat imaging and re-
two centres in the UK. Chemotherapy is more controversial, with evaluation of the patient
some studies suggesting no survival benefit and others showing  marginal excision is possible following neo-adjuvant
some difference. At present, it may be considered by the MDT for therapy, PPM resection is performed
large high-grade tumours and specific chemo-sensitive sub-types  marginal excision remains impossible, amputation is per-
such as synovial sarcoma.4,5 The role of adjuvant therapy in the formed or palliation.
management of STS is discussed in the article on Non-surgical
management of musculoskeletal malignancy elsewhere in this Procedure
issue (http://dx.doi.org/10.1016/j.mporth.2017.03.011). Informed consent is sought from the patient prior to surgery in the
UK for inclusion of blood and resected tissue samples in a Human
Principles of resection Tissue Authority-regulated biobank. The purpose of this is to
enable research and permit re-analysis if new molecular patho-
The plan for resection will be agreed by the STS MDT. Due to the logical assessment techniques become available in the future.
heterogeneity of STS there will always be exceptions and each The surgical procedure begins with marking of the intended
case is considered individually, but in broad terms, if: skin incision. This will take account of previous biopsy sites, the
 wide excision is straightforward and does not require presumed extent of the tumour, access to critical structures and
reconstruction, WLE is performed the proposed reconstruction. Dissection proceeds including en
 wide excision can be performed with satisfactory recon- bloc excision of the biopsy tract through normal uncontaminated
struction of resected structures, WLE is performed (the tissue with minimal manipulation of the tumour.
need for radiotherapy should be considered when planning In a WLE the tumour surface will not be seen. When there is a
the reconstruction) planned marginal excision against a critical structure, the
 wide excision would result in sub-optimal reconstruction outermost layer of the structure (adventitia, epineurium or
of resected structures, but marginal excision is possible, periosteum) is excised with the specimen. If major vessels are to
PPM resection is performed following neo-adjuvant be resected or stripped of adventitia, proximal and distal control
radiotherapy is gained prior to resection.

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Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

The resected specimen should be orientated for the patholo- the presence of radiotherapy due to damaged microcirculation.
gist according to its anatomical relationships with identification Therefore, flap reconstruction (tissue with its own blood supply)
of any areas of planned positive margin. It is acceptable to re- is often indicated for reconstruction of sarcoma defects. Flaps
approximate surrounding tissues over the tumour if they have may be composed of immediately adjacent tissues (local),
splayed following resection and no longer represent the in-situ regional tissues mobilized on their vascular and/or neural
appearance. It is vital to note for the pathologist any spillage of pedicle (pedicled), or distant tissues reconnected to a local blood
tumour, revision of planned margin intra-operatively and previ- supply (free). Pre-operative radiotherapy decreases the threshold
ous radiotherapy. Clinical photography of the specimen and for importing tissue due to higher risk of wound complications.18
tumour bed is a useful adjunct to this process.19 Placement of Post-operative radiotherapy discourages extensive dissection
surgical clips in the cavity can aid targeting of radiotherapy when adjacent to the resection margin as the whole operative site re-
a positive margin is suspected. quires radiation, increasing the risk of morbidity to uninvolved
If surgical drains are required they should exit from the tumour tissues. It may be necessary to utilize additional tissues in
cavity immediately adjacent to the incision to avoid seeding. addition to the principal soft tissue flap in order to replace
particular functions. Specific scenarios are discussed below.
Intermediate lesions
Vascular reconstruction
Intermediate lesions treated under the sphere of STS due to Resection of the arterial inflow to either the upper or lower limb
locally aggressive behaviour have different treatment protocols requires reconstruction using either autogenous reversed vein
as described below. graft or synthetic polytetrafluoroethylene (PTFE) graft unless
sufficient collateral circulation has already established. Autoge-
Atypical lipomatous tumour (ALT)
nous graft is preferred by many surgeons due to risk of wound
It is widely accepted that well-differentiated liposarcomas or
complications and infection in the irradiated field. The timing of
atypical lipomatous tumours (ALT) have a low rate of dedif-
arterial division must be coordinated with graft harvest to enable
ferentiation, and although they do have a propensity for local
expeditious reperfusion and prevent prolonged limb ischaemia. In
recurrence, the rates of this are reported to be as low as 4% at 3
many cases of vascular involvement, there is already collateral
years despite marginal or microscopically-positive resection.14
venous circulation owing to presence of compression or deep
Therefore, most of these tumours are planned to be excised
venous thrombosis and reconstruction of the principal draining
marginally and treated as a distinct entity that is separate from
vein is not required. It would be highly unusual to perform venous
other types of STS.
reconstruction as the limb can often be temporized with elevation
Desmoid (aggressive) bromatosis (DF) and compression until collateral circulation is well established
DF is a benign, locally aggressive condition managed in sarcoma and venous grafts have a significant incidence of thrombosis.
units. Its behaviour is unpredictable and it may grow, stabilize
Chest wall reconstruction
or regress and therefore initially it should be managed expec-
The general principle is that skeletal chest wall defects involving
tantly with active observation and interval MRI.20 The treatment
more than four ribs or larger than 5 cm require restoration of
pathway for lesions that are symptomatic and progressive can be
stability. However posterior defects are better tolerated than
either medical or surgical, but considering that negative margin
anterior or lateral defects, and pre-operative pulmonary function
surgical resection has a local recurrence rate as high as 50%, the
and comorbidities must also be considered. The chest wall is
primary interventional treatment is often medical. First-line
likely to be stiffer in patients that have had previous radiotherapy
drugs include non-steroidal anti-inflammatory drugs (NSAIDs)
and therefore a slightly larger defect may be tolerated in recur-
and tamoxifen, progressing to more potent immunomodulatory,
rent disease. Reconstruction is usually by means of a synthetic
cytotoxic and biologic drugs. When surgery is undertaken pri-
polypropylene mesh-methylmethacrylate sandwich, which is
marily or after failed medical treatment, function-preserving
fashioned to fit the defect intra-operatively and secured to the
excision is utilized and adjuvant radiotherapy may be offered
surrounding ribs. Alternatives are available including custom-
depending on the histopathology results. Patients must be
made titanium plates.
counselled carefully about the risk of recurrence.
Autogenous flap cover will certainly be required if there is a
concurrent skin deficit, but is also advisable as an interposition
Reconstruction after sarcoma resection
layer regardless of skin deficit in order to reduce the risk of
Reconstruction of the surgical defect is determined by what has infection or extrusion. There are several large loco-regional flaps
been excised, what is required and what is available. Resection within pedicled reach of the chest wall including pectoralis
may include skin, muscle, tendon, nerve, artery, vein, bone, joint major, latissimus dorsi, rectus abdominis, serratus anterior and
or a whole limb. What is required will depend on healing and omentum. Some of these can be fashioned with a skin island and
functional requirements and will also be influenced by radio- all are suitable for an overlying split skin graft. In exceptional
therapy. What is available depends on the amount of tissue cases where none of these options are adequate, free tissue
required, previous surgery and functional requirements of the transfer is necessary.
individual patient.
A wound that can be primarily closed with slight tension or Abdominal wall reconstruction
resurfaced with a split skin graft is likely to heal when no Full-thickness defects of the musculo-aponeurotic abdominal
radiotherapy is required, but would be at risk of breakdown in wall require reinforcement to prevent gross herniation. There are

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Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

allograft, xenograft, allograft and autogenous options. Poly- ensuring that a wide margin is achieved when planning the
propylene mesh is durable and strong but can result in adhesions wound closure. When there is uninvolved soft tissue on a
and requires robust soft tissue cover due to infection risk, and particular aspect of a limb it can be preserved and used as a flap
therefore is suited to smaller defects. Acellular dermal matrices for closure. When there is no clear uninvolved soft tissue an
are reported to allow host tissue remodelling with fibrous assessment needs to be made as to whether there is adequate soft
ingrowth and replacement with a reduced incidence of adhe- tissue to provide cover of the remaining bone. In some situations,
sions, although rates of recurrent herniation vary widely.21 Fas- shortening of bone to permit closure may not result in any
cia lata is the classic autogenous material used for abdominal functional difference, but in others it may have a dramatic impact
wall reconstruction. It can be used as a graft or incorporated into and importation of additional soft tissue should be considered. In
a pedicled or free flap reconstruction when harvested with the the case of major limb amputation such as shoulder and hip
anterolateral thigh flap (ALT) or tensor fascia lata (TFL) mus- disarticulation or forequarter and hindquarter amputation there
culocutaneous flap. Ideally the reinforcing material should be will be no choice but to incorporate pedicled or free flaps if the
secured as an inlay to the peritoneum with substantial overlap local tissues are insufficient. In other cases where the distal part
and appropriate tensioning to reduce the risk of bulging and of an amputated limb is not involved by disease, use can be made
herniation. of the available tissues either as free flaps or grafts.
There are several regional options available to provide cover It is well-recognized that distal amputations such as single
of the reconstructed abdominal wall when there is skin or sig- rays in the upper limb or below-knee in the lower limb can have
nificant muscle deficit. The ALT or TFL flaps provide a large excellent functional outcomes. This option may provide radical
amount of skin and can be used on their pedicle or transferred excision, avoid radiotherapy and shorten the reconstructive and
free. The latissimus dorsi can be turned over on its origin to rehabilitation process and hence may be preferred by some pa-
reach posterior defects or transferred free with split skin graft tients. Proximal amputations have a more significant functional
resurfacing. impact and may only provide a wide excision. In the case of large,
proximal high-grade tumours that involve multiple different tis-
Upper limb reconstruction sue types and/or multiple compartments, it may either not be
The upper limb requires a concerted effort to reconstruct the possible to reconstruct a functional limb or the reconstructive
functional elements and avoid hand dysfunction. There are process may be beyond the physiological reserve of the patient,
numerous local, regional and distant flap options to fill soft tissue and the only reasonable decision may be amputation.
defects. Attention should be focused on function. Many series of The rehabilitation team should be involved in decision-
tendon transfers have been described in the upper limb to restore making and evaluate rehabilitation potential prior to surgery.
hand function. Where there is more extensive musculotendinous This may influence the decision to proceed with reconstruction or
loss with lack of donor transfers, free-functioning muscle transfers amputation and the level of amputation. Whilst patients who are
such as rectus femoris or gracilis can be used to power a physically able will ambulate on prosthetics for trans-femoral or
compartment and permit composite movements. Although a free- more distal amputations, they will tend to mobilize on crutches
functioning muscle never regains normal power, the weight- with a more proximal lower limb amputation.22 It is possible that
bearing demands of the upper limb are far less than the lower very proximal trans-femoral amputees who cannot currently
limb, and a relatively weak motor can make a functional difference. tolerate a prosthesis will be suitable for osseo-integrated implants
for prosthesis attachment in the future. Patients who are less
Lower limb reconstruction physically able may be confined to a wheelchair with anything
Soft tissue defects in the lower limb can be addressed using the but the most distal of lower limb amputations. The function
armamentarium of local, regional and free flaps depending on gained with upper limb prosthetics is less dramatic, particularly
site and size. The key functional goals are to preserve knee for above elbow and more proximal amputations, and most
extension, avoid foot drop and restore sensation, thus main- people become unidextrous in this eventuality.
taining ambulation. Loss of function in a single compartment can
usually be addressed with local transfers or grafts. The hamstring Recurrent disease
to quadriceps transfer will restore knee extension, whilst the
tibialis posterior to tibialis anterior transfer will restore ankle Relapses usually occur in the first 3 years after surgery in high-
dorsiflexion. Gastrocnemius tendon can be harvested to replace risk patients, but may occur later than 10 years in low-risk pa-
patella tendon. tients. It is recommended that clinical examination is performed
Reconstruction is more challenging when multiple compart- every 3e4 months for the first 2e3 years, every 6 months until 5
ments are involved, limiting the available donors, or when there years and annually until 10 years in intermediate or high-grade
is resection of the sciatic nerve. A free functioning muscle STS. For low-grade STS the frequency is reduced to every 4e6
transfer can be considered for restoration of knee extension or months for the first 3e5 years and then annually until 10
ankle dorsiflexion, typically using the latissimus dorsi or rectus years.4,5 Imaging of the primary site is not usually performed
femoris, although the power achieved may still require orthotic repeatedly, but it is routine to obtain a baseline post-operative
support. MRI for deep tumours or sites that are difficult to examine.
Despite regular clinical examinations, the majority of patients
Amputations who develop recurrence detect it themselves between appoint-
It is important to recognize that amputation does not always ments. Local recurrence is often associated with metastatic dis-
result in radical excision of STS and thought needs to be given to ease. Therefore, any patient who is suspected to have local

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Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012
ORTHOPAEDIC ONCOLOGY

recurrence requires MRI of the primary site with or without bi- sometimes protracted despite best efforts and it is particularly
opsy, followed by restaging. important to address this when adjuvant radiotherapy is
Treatment of locally recurrent disease in the absence of required.
distant metastases is similar to primary disease. Adjuvant As with any cancer, patients with sarcoma need psychological
radiotherapy may not be an option depending on previous support to cope with their diagnosis, treatment and rehabilita-
treatment. An attempt should be made to excise the recurrence tion. In the UK this is usually organized by an STS clinical nurse
with adequate margins if this is feasible. Where functional limb specialist. A
preservation is not possible, isolated limb perfusion may render
the disease operable or at least provide palliation. Alternatively
amputation may be the only means of local control. Key points
Advanced disease C A soft tissue mass that is increasing in size, more than 5 cm in
Treatment of metastatic disease depends on the length of disease- diameter, whether or not painful, should be referred for urgent
free period, number and location of metastases, progression of ultrasound scan or directly to an soft tissue sarcoma (STS)
disease and functional status. The lung is the primary site of centre.
metastases in STS, except in specific tumour sub-types such as C The STS multidisciplinary team will make treatment recom-
myxoid liposarcoma that has a propensity for metastasizing to mendations for STS based on tumour biology, anatomical
soft tissues. Although chest CT scan would likely detect metas- extent, staging and functional status.
tases earlier than chest radiograph, there is no evidence that CT C Surgery is the principal treatment for localized STS.
is more beneficial to outcome, and therefore patients are moni- C Wide excision through normal tissue with a 1 cm margin or
tored with regular chest radiographs following primary staging intact structural layer is the standard approach in resectable
and treatment. disease.
A tenth of patients with STS have detectable metastases at the C A planned close margin against a critical structure with uti-
time of presentation. In general synchronous pulmonary or extra- lisation of radiotherapy is acceptable in order to preserve
pulmonary metastases will be treated with systemic chemo- function.
therapy. Resection of the primary tumour for palliation remains C Neo-adjuvant radiotherapy, chemotherapy or isolated limb
an option in patients with advanced disease depending on pre- perfusion may render a tumour of borderline operability
dicted symptom relief balanced against surgical morbidity, resectable.
functional status and prognosis. Surgery will often be performed C Amputation is sometimes the only possibility for local control.
following adjuvant chemotherapy unless there is a need for ur- C Either pre- or post-operative radiotherapy is indicated for STS
gent intervention such as for a fungating tumour. that are intermediate or high grade, large, deep to fascia or
Metachronous pulmonary metastases, detected later than a have a close excision margin.
year after STS diagnosis, will be considered for resection. Staging C Neo-adjuvant radiotherapy imparts a higher risk of wound
positron emission tomography-CT is required to confirm absence complications but lower late toxicity.
of other metastases prior to surgery and the lesions must be C Reconstruction is individualized and focused on function in
amenable to excision with adequate remaining lung function. addition to wound healing.
Diagnosis of pulmonary metastases after a shorter disease-free
interval requires more caution and typically an interval CT
scan is performed 3 months later. If there are no further metas-
tases resection may be offered at this stage.
Lymph node metastases are rare in STS and are linked to poor
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dx.doi.org/10.1016/j.mporth.2017.03.012
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ORTHOPAEDICS AND TRAUMA --:- 8 2017 Elsevier Ltd. All rights reserved.

Please cite this article in press as: Steele KH, et al., Surgical management of soft tissue sarcoma, Orthopaedics and Trauma (2017), http://
dx.doi.org/10.1016/j.mporth.2017.03.012