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Extra-abdominal desmoid fibromatosis eA sarcoma unit
review of practice, long term recurrence rates and survival
N. Eastley
a,
*, R. Aujla
a
, R. Silk
b
, C.J. Richards
a
, T.A. McCulloch
b
,
C.P. Esler
a,b
, R.U. Ashford
a,b,c
a
Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square,
Leicester LE1 5WW, United Kingdom
b
Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road,
Nottingham NG5 1PB, United Kingdom
c
Academic Orthopaedics, Trauma & Sports Medicine, University of Nottingham, United Kingdom
Accepted 10 February 2014
Available online 22 February 2014
Abstract
Introduction: Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited
diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of
treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis
on active surveillance, function preserving resections, and non-surgical oncologic therapies.
Methods: We performed an 8-year retrospective review of all cases of extra-abdominal DF managed within our region to assess the con-
sistency of diagnostics, management and long-term outcome.
Results: 47 eligible cases were identified. Mean age at diagnosis was 41.3 years (1e81 years). Disease location and speciality of diagnosing
practitioners were varied. Management was generally inconsistent. Variation was seen in imaging, biopsy techniques, MDT involvement
and management. At a median follow up of 4.9 years our local recurrence rate was 19%.
Discussion: The optimal management of DF is unknown. This has led to a lack of formalised guidance for practitioners managing this
challenging condition, resulting in inconsistencies and areas for improvement in current management. We propose a diagnostic pathway
which may improve consistency of care, reduce potentially unnecessary surgery and the associated morbidity, and significantly increase
the rate of complete (R0) surgical resections when surgery is deemed appropriate whilst not significantly worsening oncological outcome.
Specifically we propose all cases should be imaged appropriately (usually with MRI), undergo a planned biopsy (by radiologically guided
core needle biopsy) and be managed centrally in conjunction with multidisciplinary sarcoma units.
Ó2014 Elsevier Ltd. All rights reserved.
Keywords: Desmoid; Tumour; Aggressive; Fibromatosis; Diagnosis; Excision
Introduction
Desmoid tumours are uncommon soft tissue neoplasms
with an incidence 0.2e0.4/100 000
1
accounting for approx-
imately 3% of all soft tissue tumours. Also known as
aggressive fibromatosis or desmoid type fibromatosis
(DF), these tumours generally originate from deep
musculo-aponeurotic structures but may occur intra-
abdominally or in the thoracic cavity. These tumours are
myofibroblastic in type and are regarded as intermediate
in their behaviour under the WHO classification of soft tis-
sue neoplasm, as a result of their unusual biology: locally
aggressive but never metastatic.
2
The mechanism of tu-
mourigenesis is deregulation of the wnt signalling pathway
usually due to mutation of the B-catenin gene, or less
frequently the APC gene.
3
The latter is the usual pathway
involved in the intra-abdominal fibromatoses associated
with Gardner’s syndrome. These mutations lead to an
* Corresponding author. Tel.: þ44 7890619380; fax: þ44 116 2585473.
E-mail addresses: neastley@doctors.org.uk (N. Eastley),
randeep.aujla@hotmail.co.uk (R. Aujla), Robert.Silk@nuh.nhs.uk (R.
Silk), cathy.richards@uhl-tr.nhs.uk (C.J. Richards), Tom.McCulloch@
nuh.nhs.uk (T.A. McCulloch), claire.esler@uhl-tr.nhs.uk (C.P. Esler),
robert.ashford@sky.com (R.U. Ashford).
0748-7983/$ - see front matter Ó2014 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ejso.2014.02.226
Available online at www.sciencedirect.com
ScienceDirect
EJSO 40 (2014) 1125e1130 www.ejso.com
accumulation of intracytoplasmic and intranuclear B-cate-
nin, which can be demonstrated immunohistochemically.
Patients are usually aged between 15 and 60 (with a
peak incidence of 25e35 years),
4
and have a 2:1 female
to male preponderance.
5
Histopathologically, desmoid tu-
mours consist of well-differentiated myofibroblastic cells
set in a variably collagenous matrix. Commonly reported
sites affected include the shoulder girdle, abdominal wall,
chest wall,
6
breast
7
abdominal mesentery,
8
and pelvis.
The disease is less frequent in the limbs (assuming the
shoulder and limb girdle are classified as part of the truck)
and in the head and neck. Occurrence in the distal extrem-
ities is distinctly unusual unlike the unrelated superficial
type fibromatoses. The majority of cases of DF are idio-
pathic or sporadic. The remaining are usually associated
with familial adenomatous polyposis (Gardner’s syn-
drome). Links with pregnancy, oestrogen exposure, trauma
and surgery
9
have been noted. Patients with DF classically
present complaining of an enlarging painless lump. DF tu-
mours have an aggressive tendency to infiltrate local struc-
tures. When this hampers the function of vital adjacent
organs or nervous structures, or impedes blood flow in
nearby vascular structures other signs or symptoms may
become evident. Historically this tendency to aggressively
invade local structures has led to comparisons being made
between DF and low-grade fibrosarcomas.
10
The optimal management of DF has not been estab-
lished. It has previously been accepted that wide local exci-
sion should form the cornerstone of DF treatment where
possible.
11e14
More recently a paradigm shift in manage-
ment has resulted in many clinicians adopting a more con-
servative therapeutic attitude,
15,16
placing emphasis on the
preservation of anatomical structures, avoidance of muti-
lating surgery and the use of adjuvant therapy.
17
There
has been a further evolution in care and the utilisation of
a period of active surveillance and observation before un-
dertaking any interventional management has become
adopted by many centres.
18
This change in mindset fol-
lowed the acknowledgement that desmoid tumours follow
a variable, unpredictable natural history with a significant
proportion of cases either not progressing, or undergoing
spontaneous regression when solely observed (allowing
for prolonged asymptomatic periods).
19e23
Recognition
that non-surgical oncologic therapies such as radiotherapy,
chemotherapy and hormonal therapies have a more central
role to play in the management of DF also supports this
shift in management.
Aims
The absence of a gold standard management for cases of
extra abdominal DF means that there is a lack of formalised
guidance for practitioners. This risks variance and inconsis-
tency in patient care. Our primary aim was to review our
recent management of this difficult condition. We per-
formed a comprehensive retrospective review of all cases
of deep extra abdominal fibromatosis managed within our
region. We report specifically on surgical management,
the use of adjuvant medical therapies, oncological out-
comes and recurrence rates.
Methods
We reviewed the cases of all resections or biopsies
coded as ‘Fibromatosis’ following histological examination
in our regional unit in the last 8 years. Patients were iden-
tified from 2 histopathological databases (one departmental
and one held by a specialist sarcoma pathologist) All intra-
abdominal, intra-thoracic, gynaecological and superficial
(ie palmar and plantar) cases were excluded. Cases of dis-
ease recurrence previously treated outside our region were
not included in our analysis. Cases referred for histological
opinion only, but not managed locally were also excluded.
Clinical notes, clinic letters, histopathological reports,
radiological images and reports and sarcoma multi-
disciplinary team (MDT) meeting minutes were reviewed
and cases cross-referenced.
Initial datasets included patient age at diagnosis, sex,
tumour sites and presenting specialities. We reviewed the
use of pre-operative imaging, regional sarcoma MDT
involvement, biopsy techniques (including histological
margins of resected tissues), oncological outcomes and
recurrence rates. Margins were defined as clear or not on
the basis of there being no tumour at the surgical resection
margin on the histological sections. We also report on our
use of adjuvant therapies. Data were stored in a password
protected Microsoft Excel Spreadsheet (Redmond, WA).
Results
47 eligible cases were identified (19 male: 28 female).
The mean age at diagnosis was 41.3 years (1e81 years).
Despite extensive attempts a lack of available clinical notes
meant collecting a full datasets for every case was not
possible and three cases in our series were lost to long
term follow up.
Disease location was varied and included the trunk
wall,
31
the limb or limb girdle,
10
the head or neck
2
and
the breast.
4
We recognise a high proportion of cases involve
the trunk wall. When further subcategorised these tumours
involved 3 main anatomical areas: the para-spinal region,
8
the chest wall
7
or the abdominal wall.
16
The speciality of the medical professional to which pa-
tients presented was determined in 45 cases and equally
varied. These included general surgeons,
14
orthopaedic sur-
geons,
6
breast surgeons,
7
primary care practitioners,
7
paedi-
atric surgeons,
6
cardiothoracic surgeons,
1
ENT surgeons,
2
plastic surgeons
1
and dermatologists.
1
Seventeen percent of cases (8 of 47) underwent no imag-
ing prior to biopsy. Using radiological investigations per-
formed following biopsy or histological reports it was
deduced that the average size of these 8 tumours was
1126 N. Eastley et al. / EJSO 40 (2014) 1125e1130
64.5 mm (defined as the largest single dimension on imag-
ing or specimen analysis). The remaining 39 patients under-
went a combination of ultrasound scans,
17
computed
tomography (CT),
15
magnetic resonance imaging (MRI)
23
and mammography.
5
The average size of imaged tumours
imaged was 62.2 mm (P¼0.89).
We determined the biopsy technique used to obtain a
primary tissue diagnosis in every case. Techniques were
varied and included core needle biopsy (CNB),
26
excisional
biopsy,
15
incisional biopsy
5
and endoscopic biopsy.
1
The
single case biopsied using endoscopic means was located
in the nasal cavity.
Only 81% of all cases (38 of 47) were discussed at the
regional sarcoma MDT at any stage. Thirty one of the 37
patients (84%) who underwent attempts at complete resec-
tion were discussed at MDT prior to surgery.
Following losses to long term follow up we were able to
accurately determine patient management in 44 cases. Five
cases were managed non-surgically; of these one patient’s
tumour was deemed inoperable (due to its proximity to
the carotid sheath) and treated with radiotherapy, two
recent cases were managed with a policy of active surveil-
lance and two with a combination of tamoxifen and Non-
Steroidal Anti-Inflammatory Drugs (NSAIDs.) One further
patient was awaiting surgery at the time of data collection.
The remaining 38 cases underwent some form of surgery
during our analysis period. One patient had disease which
was deemed radiologically unresectable in terms of cura-
tive intent and thus underwent planned debulking surgery.
The remaining 37 patients underwent attempts at complete
resection. Clear histological margins were achieved in 59%
of these cases (22 of 37 patients). There was no significant
difference in the size of those tumours completely and
incompletely excised (P¼0.66). Of the 22 cases
completely excised with clear histological margins (R0 re-
sections), 5 (23%) recurred locally. Of the 15 cases incom-
pletely excised with positive histological margins (R1
resections) 2 (13%) showed radiological progression
requiring treatment ( p¼0.68). When recurrence is defined
as radiological or clinically detectable disease at the site of
a previous R0 or R1 resection then the overall local recur-
rence rate in our series is 19% (7 of 37 cases.) There was no
significant difference between the size of those tumours
that recurred and those which did not (P¼0.63). Median
length of follow up was 4.9 years (1.9e9.7 years).
Following recurrence 3 patients underwent further sur-
gery (alongside adjuvant radiotherapy, chemotherapy or
treatment with NSAIDs); two underwent second attempts
at tumour resection (one requiring split skin grafting) and
one a forequarter amputation and chest wall resection.
Three of the remaining cases of recurrence were managed
medically; two received a combination of Tamoxifen and
NSAIDs, and one Tamoxifen, NSAIDs and chemotherapy.
The final case is currently being managed by surveillance.
Oncological outcome was determined for the 44 cases.
Twelve (27%) were alive with disease (AWD) three were
alive disease free (ADF) and 29 (66%) remained continu-
ously disease free (CDF) following treatment. Of those
cases alive with disease two were managed by an active sur-
veillance policy, three were managed with medical thera-
pies alone, one was awaiting surgery, one had undergone
an incomplete dubulking resection (and an MDT decision
made not to re-resect the tumour bed because of potential
functional impairment) and five patients had evidence of
local recurrence. None of our cases died of disease.
Discussion
DF affects a wide range of patients and carries a signif-
icant morbidity and recognised mortality. Many treatment
options are available for practitioners managing DF which
themselves carry significant morbidity ea factor of partic-
ular concern given that the optimal management of DF re-
mains controversial. These issues mean DF remains a
difficult problem to manage, and as a result we strongly
feel that detailed data reporting on the long-term outcome
of patients with this complex condition is key. Historically
management has been principally an attempt at complete
surgical excision of tumours. Several issues surround this;
Firstly post-operative recurrence rates as high as 77%
5
have dogged resections, leading to conflicting arguments
on the importance of clear histological margins.
24e26
Sec-
ondly the invasive nature of desmoid tumours often makes
their complete resection impossible without sacrificing
important adjacent structures resulting in significant func-
tional impairment. Thirdly, more recent observational
studies have highlighted the unpredictable natural history
of DF by showing that some cases will undergo sponta-
neous regression (allowing for sustained progression free
periods
19e21,27
) when simply observed.
As well as driving a movement to manage DF within
sarcoma teams, these issues have also led to clinicians
placing less emphasis on excision but more on the active
surveillance of patients.
28
Bonvalot et al. published their
own treatment algorithm consisting primarily of serial
MRI and a ‘watch and wait’ policy. Only if progression
occurred was medical therapy instigated (anti-hormonal
treatment and chemotherapy), and only following further
significant progression was surgery, isolated limb perfusion
and/or radiotherapy utilised.
18
Other groups have advocated
similarly conservative principles, recognising that
morbidity from attempts to eradicate tumours can often
result in symptoms worse than those attributable to the dis-
ease itself.
29
Despite a comprehensive search of medical
databases including Pubmed and Medline we found no pub-
lished evidence that adopting these observational ap-
proaches towards cases of extra-abdominal DF has had a
detrimental effect on oncological outcome. This is sup-
ported by our data. Of the 5 cases managed non-
operatively in our patient group only 1 underwent signifi-
cant radiological progression. This case was managed
with active surveillance alone.
1127N. Eastley et al. / EJSO 40 (2014) 1125e1130
Supporting these conservative principles further is the
emergence of strong evidence supporting the use of several
medical therapies for DF. Radiotherapy in isolation
24,30,31
or as an adjunct to surgery (regardless of margin clear-
ance)
24,32
has been shown to play a key role, particularly
for those cases at highest risk of recurrence
30,33
or in those
whom surgical resection would leave an unacceptable func-
tional or cosmetic outcome.
34
Several chemotherapeutic
agents
35,36
and non-cytotoxic drugs such as non-steroidal
anti-inflammatory drugs (NSAIDs) and hormonal agents
including Tamoxifen have also been recommended.
37e39
Tyrosine kinase inhibitors,
40
isolated limb perfusion and
cryoablation
18
may all also play therapeutic roles.
MRI and histological appearances do not help predict a
desmoid tumour’s natural history.
41,42
Until those naturally
indolent cases more likely to benefit from conservative
management can be identified from more aggressive tu-
mours better suited to more aggressive therapy we feel
practitioners managing DF require more guidance. There
are currently no NICE guidelines and no general consensus
on how patients with this challenging condition should be
managed. It is therefore perhaps unsurprising that our re-
view highlights variations and inconsistencies in current
care. There is potential for improvements in many aspects
of the management pathway including imaging, biopsy,
MDT involvement and management. Only 49% of cases
underwent MRI prior to biopsy, and 17% received no imag-
ing prior to attempts to obtain a tissue diagnosis. Although
the vast majority of these cases were small tumours on the
trunk wall, we are still disappointed by these figures.
Furthermore 21% of cases were not discussed with the
regional soft tissue sarcoma MDT at all. This necessitated
the use of two pathological databases to identify those pa-
tients who underwent surgical intervention or diagnostic bi-
opsy outside our sarcoma service. Tissue biopsy techniques
were also varied in our patient group and included CNB
(55%), excisional biopsy (32%), incisional biopsy (11%)
and endoscopic biopsy (2%).
We propose a framework to provide structure to the
diagnosis and management of DF. Although metastases
do not occur, we feel the propensity for desmoid tumours
to recur locally and their unpredictable behaviour means
that guidelines on sarcoma management are transferable
in some ways. Early, appropriate imaging is essential.
MRI has previously been labelled the imaging modality
of choice for superficial and deep DF and we support
this.
43,44
We recommend tissue biopsies are obtained using
appropriate techniques only. Image guided CNB is an accu-
rate, safe and relatively cost-effective method of diag-
nosing DF.
45,46
We do not believe excision biopsy
without imaging has any place in the management of DF.
We believe the absence of a clear gold standard manage-
ment for DF advocates centralised MDT care.
47
Although
emergency surgery for extra-abdominal DF is rarely neces-
sitated, we feel definitive treatment should only be started
after discussions with departments experienced in the
management of soft tissue tumours. Hopefully this will
introduce more consistency to care, increase diagnostic ac-
curacy and help reduce unnecessary surgery and the asso-
ciated morbidity. The differences between DF and
malignant soft tissue tumours may mean that designated
DF clinics may play a key role in the future management
of these patients.
The adverse effects of unplanned excisions not preceded
by appropriate biopsy, imaging or MDT involvement have
been reported in rare tumours.
48
Our work is novel in inves-
tigating this issue in DF. We retrospectively reviewed the
effects of biopsy technique/unplanned surgery and analysed
whether implementation of our proposed management
framework may alter excision margins and oncologic
outcome. In our series 22 patients diagnosed using CNB
underwent subsequent attempts at complete surgical resec-
tion. Of these 68% (15 of 22 cases) had clear histological
margins on the resection specimen. Fifteen cases were diag-
nosed by excision biopsy. Significantly fewer of these cases
(33%) were excised with clear margins (P¼0.05). This is
principally because an excision biopsy is a diagnostic rather
than an oncologic procedure. Only nineteen (43%) of the
total of 44 patients followed our proposed diagnostic
pathway. Attempts at complete surgical resection were
made in 17 (89%) of these cases. Of these, 14 (82%) had
clear histological margins. Conversely, 25 (57%) cases
deviated from our proposed strategy, of which 20 (80%) un-
derwent attempts at complete resection. In this group eight
(40%) patients had clear histological margins, significantly
higher number than in the non-standard pathway patients
(P¼0.018). Interestingly it should be noted that neither bi-
opsy technique nor overall adherence to our diagnostic
pathway appears to have a significant effect on oncological
outcome (see Table 1). We feel this fact emphasises the
complexity of DF, and the variation between DF behaviour
and standard surgical oncology rules, in turn adding
strength to our argument towards the centralisation of DF
care.
We recognise that the importance of histological mar-
gins following DF resection has been disputed. In this se-
ries we found no significant difference between those
cases which recurred following complete resection and
those cases which underwent radiological progression
following R1 resection (P¼0.68). Overall these figures
support suggestions that generally striving for clear margins
Table 1
Oncological outcomes of cases according to adherence to proposed diag-
nostic pathway. Alive with disease (AWD), continuously disease free
(CDF), alive disease free (ADF).
Oncological outcome AWD CDF ADF Total
Cases adherent
to strategy
712221
Cases deferred
from strategy
418123
1128 N. Eastley et al. / EJSO 40 (2014) 1125e1130
is not critical (disagreeing with recent prospective reviews
looking at DF,
16,49
), and should not be done at the cost of
impairing function in the majority of cases.
24e26
Conclusion
Our retrospective review confirms that the current man-
agement of DF is varied and inconsistent. This follows a
lack of formalised guidance for practitioners managing
this difficult condition.
The optimal treatment of DF remains controversial. A
general shift to a more conservative observational manage-
ment strategy has recently taken place with no apparent
detrimental effects in oncological outcomes. Our results
support this shift, and the reservation of surgical resection
for only the most aggressive or dangerously located
tumours.
We outline a diagnostic pathway which we feel will help
improve consistency of current DF care and reduce poten-
tially unnecessary surgery and associated morbidity, but
which significantly increases the rate of complete (R0) sur-
gical resections when surgery is appropriate, without signif-
icantly worsening oncological outcome. Although we
recognise the limitations of our data (specifically its retro-
spective, historical nature), we propose all patients in
whom a diagnosis of DF is considered should be imaged
appropriately (ideally with MRI), undergo a planned biopsy
(ideally by radiologically guided CNB) and be managed by
sarcoma units.
Conflict of interest statement
None of the authors have any financial and personal re-
lationships with individuals or organisations that could
inappropriately influence (bias) this work. RUA has
received funding from Sarcoma UK to support the develop-
ment of a DF registry.
Acknowledgements
The authors would like to thank Sue Carvell, Senior
BMS, for her invaluable help with the UHL pathology
database.
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