# Delays in Referral of Soft Tissue Sarcomas

Authors: G. D. Johnson; G. Smith; A. Dramis; R. J. Grimer
Journal: Sarcoma
 (2008)

---

## Abstract

Introduction and aims. It is well established that soft tissue sarcomas (STSs) are more effectively treated in a specialist centre. However, delays in time taken for a patient to be referred to a specialist centre may lead to a poorer prognosis. This study aims to identify the length of these delays and where they occur. Patients and methods. Patients with a proven STS were included. They were recruited from both outpatient clinics and from the surgical ward of the Royal Orthopaedic Hospital (Birmingham, UK). A structured interview was used to take a detailed history of the patients' treatment pathway, before arriving at the specialist centre. Dates given were validated using the case notes. Results. The median time for the patient to present to a specialist centre from the onset of symptoms was 40.4 weeks. The median delay until presentation to a medical professional (patient delay) was 1.3 weeks. Median delay in referral to a specialist centre (service delay) was 25.0 weeks. Discussion. Medical professionals rather than patients contribute the greatest source of delay in patients reaching a specialist centre for treatment of STS. Adherence to previously published guidelines could decrease this delay for diagnosis of possible sarcoma. Steps should be taken to refer patients directly to a diagnostic centre if they have symptoms or signs suggestive of STS.


---

## Body

## 1. Introduction

Soft
tissue sarcomas (STSs) are a rare group of cancers with approximately 2200
diagnosed in the UK every year [1]. They account for approximately 1%
of all cancers and 2% of all cancer deaths [2]. Survival rates are
approximately 50% at 5 years and are related to grade, depth, and size of
tumour at diagnosis.Due
to the rarity and high mortality rate of STS, guidelines have been introduced
by the Department of Health for their early diagnosis.  The guidelines state thatany lump that is either: ≥5 cm in
size and/or deep to the fascia and/or painful and/or increasing in size should
be referred to a diagnostic centre for investigation and to a surgical centre for
management [3]. However, recent evidence suggests that these guidelines are not
well implemented, resulting in inadequate management of these tumours and
delays in referral [4–7].In
the Trent Cancer Registry region, median size at presentation to the sarcoma service
was 8 cm (range: 0.3–45 cm) [8] and data from the Scandinavian
Sarcoma Group Register shows a median size of 7 cm at presentation, varying
with age, with a larger median tumour size in older patients [9]. At our
centre, however, the median size of tumour seen at first presentation is 9 cm.
Thus, patients presenting to our centre have tumours nearly twice the size
recommended by the guidelines. This delay in presentation to specialist
services could be due to either unwillingness of patients to present initially
or to delays after inappropriate primary or secondary referrals.Due
to their rarity, referral patterns for STS are often circuitous and are manifested by delays in presentation and referral.
Lack of experience with these tumours is the oft-cited reason for delayed referral
practice and inadequate management. With an average General Practitioner (GP) seeing
only 1 STS in 20 years of practice, this claim is easily substantiated  [10]. Referral to a General or Othopaedic Surgeon
often increases delays to definitive treatment as, because of their rarity, a
malignant diagnosis is a low clinical suspicion. As a result, inappropriate
surgical techniques are often employed: lesions may be excised under the
assumption that they are benign, with inadequate surgical margins  [11, 12]. A recent study found that 50% of
patients referred to their Sarcoma Service had received some surgical
intervention at a nonspecialist centre [11].Several
papers have described delays in referral of patients with STS for treatment. These
found that medical professionals were the source of the greatest delay.
However, GPs and hospital consultants were not considered separately and all
the studies identified a time period which the authors thought constituted “delay,” only
going on to analyse those patients with delay  [4–6]. However, this fails to give an accurate
impression of the actual timescale.Current
guidelines in the UK
state that any patient with suspected cancer should be seen by a specialist
within 2 weeks of referral [13].
At present, there is no data on delay in the UK attributable to patients, and
medical professional delay is difficult to quantify, as the only UK paper on
the subject judged that delay was anything longer than 3 months [6]. This study
aims to bring clarity to the situation, which is essential to the development
of further training of medical professionals in the UK. Recent literature suggests that
obesity can cause a worse prognosis and delays in presentation in cancer
patients [14]; therefore we also plan to identify whether any patient factors
(BMI, Social Class and Literacy Levels) have any effect on delay in presentation
or size of tumour at diagnosis.


## 2. Patients and Method

A
total of 162 patients with a confirmed diagnosis of Soft Tissue Sarcoma were
recruited from a combination of the outpatient clinic and from the surgical
ward at the Royal Orthopaedic Hospital (Birmingham, UK) between January 2005
and May 2005. In order to maximise patient numbers in the study, a combination
of followup patients and new referrals were included. Appropriate ethical approval was gained.
Patients were consented by their doctors and the study was further explained to
them by a researcher at which point they were given an explanatory letter. A
structured interview was used to gather the study information, which was
completed by one member of the research team.


### 2.1. Demographics

We
collected standard demographic information about patient age, sex, histological
diagnosis, size of tumour at presentation and which features suggestive of
sarcoma from the guidelines the patient had, if any.


### 2.2. Time Delay

We
measured duration of symptoms before a patient presented to a medical professional
(MP), by asking the patients to recall when they first noticed the lump. We
asked the patients to recall the dates when they first saw a medical professional
(normally a GP) and when they were referred to someone, either a district general
hospital (DGH) or the Royal Orthopaedic Hospital (ROH). For those patients who
were referred to a DGH, we asked them to then recall when they were then
referred to the ROH.All
dates were confirmed with dates in the patients’ notes. Referral date to the
ROH was the only date that could be confirmed in all cases. This was therefore
used to calculate the accuracy of the dates given by each patient, by comparing
the ROH referral date they specified with the actual date recorded in the
notes.Patients
were asked to recall the management of the first medical professional they saw
and the subsequent clinicians. As a result, we calculated the number of
inappropriate
and inadequate procedures performed. We also recorded whether the
patient had any cross-sectional imaging or a biopsy when managed at a DGH.


### 2.3. Associations

Age,
sex, patients’ postal code, and the age at which they left full-time education were
recorded. Using the Townsend deprivation scale, we calculated social
deprivation from a patient’s postal code; and literacy levels were calculated
from the age they left full-time education.We
also recorded the patients’ height in centimetres and weight in kilos. Height
was recorded at interview, as this will not have significantly changed since
they first had their Soft Tissue Sarcoma. Weight was recorded from the operation
record to ensure that we recorded the weight of the patient as close to
diagnosis as possible. Using this information we then calculated each patient’s
body mass index.


## 2.1. Demographics

We
collected standard demographic information about patient age, sex, histological
diagnosis, size of tumour at presentation and which features suggestive of
sarcoma from the guidelines the patient had, if any.


## 2.2. Time Delay

We
measured duration of symptoms before a patient presented to a medical professional
(MP), by asking the patients to recall when they first noticed the lump. We
asked the patients to recall the dates when they first saw a medical professional
(normally a GP) and when they were referred to someone, either a district general
hospital (DGH) or the Royal Orthopaedic Hospital (ROH). For those patients who
were referred to a DGH, we asked them to then recall when they were then
referred to the ROH.All
dates were confirmed with dates in the patients’ notes. Referral date to the
ROH was the only date that could be confirmed in all cases. This was therefore
used to calculate the accuracy of the dates given by each patient, by comparing
the ROH referral date they specified with the actual date recorded in the
notes.Patients
were asked to recall the management of the first medical professional they saw
and the subsequent clinicians. As a result, we calculated the number of
inappropriate
and inadequate procedures performed. We also recorded whether the
patient had any cross-sectional imaging or a biopsy when managed at a DGH.


## 2.3. Associations

Age,
sex, patients’ postal code, and the age at which they left full-time education were
recorded. Using the Townsend deprivation scale, we calculated social
deprivation from a patient’s postal code; and literacy levels were calculated
from the age they left full-time education.We
also recorded the patients’ height in centimetres and weight in kilos. Height
was recorded at interview, as this will not have significantly changed since
they first had their Soft Tissue Sarcoma. Weight was recorded from the operation
record to ensure that we recorded the weight of the patient as close to
diagnosis as possible. Using this information we then calculated each patient’s
body mass index.


## 3. Statistical Analysis

Simple
descriptive statistics were performed on the data. Linear regressions and
correlations were performed to test for associations. It was predicted that the
data would not follow a normal distribution and therefore nonparametric tests were
used for comparisons (Mann-Whitney-U test). The median has been chiefly used as the method of
comparison because a number of patients had excessively long delays, which have
skewed the mean. However, the mean has been provided for completeness.


## 4. Results

Of the 162 patients included, the mean age at
diagnosis was 53 years (range 16–88). The sex distribution was 49.3% male: 50.7%
female. The median size of tumour at diagnosis was 8 cm (mean 8.91 cm, range 0–26 cm)
(see Figure1). Histological subtypes followed the expected distribution with
the commonest being Liposarcoma 24.1%, Myxofibrosarcoma 13.0%, and
Leiomyosarcoma 7.4%. A percentage of 55.5% of lumps were deep, 43.2% high-grade,
and 39.2% intermediate-grade. 88% of patients had at least one of the
features that are suspicious of sarcoma
at first presentation to a medical professional. Site of tumour also followed
the expected distribution,  with 42.5%
affecting the thigh, 20% the lower leg, 14.6% the forearm, 13% the upper arm,
9.2% the trunk, and 1.5% affecting the hand.Figure 1
Size of tumour at presentation to ROH.The median time for a patient to be referred to the
ROH from the onset of symptoms was 40.4 weeks (mean 112.3 weeks). Only 14.6%
were referred within 3 months of the onset of symptoms and 44.9% of patients
took longer than 1 year to be referred to the ROH from onset of symptoms (Table1).Table 1
Histological diagnosis.
DiagnosisFrequencyPercentageLiposarcoma3924.1Myxofibrsarcoma2113.0Leiomyosarcoma127.4Spindle cell sarcoma116.8Synovial sarcoma116.8Pleomorphic sarcoma74.3MFH63.7MPNST53.1Other5030.9Total162100.0The median time for a patient to initially present to
a medical professional from onset of symptoms was 1.3 weeks  (mean 28.6 weeks). 60.6% of
patients consulted a medical professional (91.6% of which were GPs) within 1
month. 72.5% had consulted within 3 months. 12.5% of patients waited longer than
one year before consulting; the longest a patient waited before seeking medical
advice was 674 weeks (13 years). From first presentation to a medical professional,
the median time for a patient to be referred to the ROH was 25.0 weeks (mean 83.1
weeks). Only 11.3% of patients were referred to the ROH within 1 month and 28.9%
within 3 months. 32.7% of patients took longer than 1 year to be referred to the
ROH for investigation and treatment (Table3).47% of patients were referred to a consultant
the first time the patient presented. 51.0% were referred within 1 month and 60.9%
within 3 months. 21.2% of patients took longer than 1 year to be referred. 9 of
the 162 patients were referred directly to the ROH. The remaining 153 were
referred to a local DGH and 37.3% of patients were referred to the ROH by the consultant
they saw within 1 month. 63.3% had been referred within 3 months. 12.7% of
patients managed by a consultant in a DGH took longer than 1 year to be
referred to the ROH.The 62 patients who were reassured by the first
medical professional consulted took a mean of twice as long to be referred to
another, more specialised, medical professional (P<.0001). The
median time for a patient who had been reassured to be referred increased nearly
16 folds, to 38.3 weeks. Although the number of patients is too small for a
statistically significant difference to be calculated (n=11), it appears that the
decision to obtain an X-ray was related to an increased time for referral from
the GP to hospital (median 6.7 weeks) (Table 2).Table 2
Tumour grade and depth.
GradeFrequencyPercentageHigh5443.2Intermediate4939.2Low2217.6Deep6155.5Subcutaneous4944.5Table 3
Summary of delays.
MeanMedianMin.Max.%
in 1 month%
in 3 months%
in 1 year%> 1 yearWeeksStage
128.61.30.0674.060.672.587.5100.0Stage
235.82.40.0552.451.060.978.8100.0Stage
35.62.20.0208.768.490.899.3100.0Stage
444.96.90.01065.337.363.387.3100.0MP
delay83.125.00.01083.311.328.967.3100.0Overall
time112.340.40.41089.33.814.655.1100.0Performing an MRI or biopsy was related to a
substantial decrease inmean time to refer to the ROH, although median time was slightly increased by performing biopsy. Performing surgery, whether
or not they had an MRI &/or biopsy, showed a highly significant (P<.0001) increase in the time taken for a patient to be referred to the ROH.
The 35 patients who had
surgery without biopsy took nearly 3 times longer to be referred to the ROH
than the study mean and median (P<.0001). The 4 patients who had surgery having already had a biopsy took a
median 15.2 weeks (mean 24.3 weeks) longer, longer to be referred to the specialist
centre. Only 3 patients were reassured by the DGH consultants so the difference
is not statistically significant, but the mean delay was nearly 7 fold longer
and median delay was nearly 27 fold longer (Table 2).The mean BMI of the patients studied was 26.6 (range: 18–45.8). The mean age that patients had left full-time education was 16.9 and
the mean Townsend score was −0.8373. No association between any of these
factors and either patient delay or medical professional delay was identified. No
association with BMI and size of tumour at presentation was identified. The
only factor that had any correlation with delay was the size of the tumour at
first presentation, where with increasing size of tumour, the time taken to
refer to the ROH decreased (P=.022). There was no significant
difference in time to presentation based on tumour site.


## 5. Limitations

The main limitation with this study is that it relies
largely on patient recall of significant dates in their history and this is
liable to bias. However, in order to minimise this effect, dates of referral
given by the patient were compared in all cases with those in the notes. We
found that there
was no significant difference between these dates.The population studied is an already biased
population, as we only studied patients diagnosed with sarcoma in a tertiary referral
centre. We are unable to take into account those who have had their sarcomas managed
elsewhere.


## 6. Discussion

Current
guidelines state that any patient with suspected cancer should be seen by a
specialist within 2 weeks of first presentation to a medical professional,
normally a General Practitioner. In the case of Soft Tissue Sarcoma, patients
should be referred to a specialist centre for further investigation and
management.The
most recent research on delays in the referral and diagnosis of STS has
identified significant delays due to medical professionals. However, these
papers set a fixed time period considered as “delay” being 1 month and 3 months
[4–6]. The analysis then considers only those who have been found to have a
delay, thereby giving an inaccurate impression of the actually timescale in the
study patients. For this analysis, no limit has been used to define “delay” and
all medians and means used contain all patients, including those who did not
suffer any delay.Nearly
73% of patients presented to a medical professional (91.6% GP) within 3 months
of the onset of symptoms, with a median time of 1.3 weeks (Table3). This shows
that, although some patients are more willing to tolerate symptoms, in general,
patients present quickly to a medical professional and therefore do not
contribute significantly to delay in reaching a specialist centre for
treatment.The
previous literature highlights general practitioners as the most common source
of delay in those patients who were defined as “delayed.” However, these studies identified only 20% [5] and
27% [4] of patients as delayed. Further
analysis of these patients revealed that GPs were responsible in the majority
of those cases. This study has demonstrated that, using median time as a method
of comparison, GPs are actually quicker at referring to someone more
specialised, with Figure 2 the median patient being referred within 2.4 weeks of
presentation, compared with 6.9 weeks for DGH consultants. However, when
examining the percentage of patients referred within 1 month and then 3 months,
consultants eventually appear to be as efficient as GPs. Although only 37.3% are
referred within 1 month compared to 50.1% for GPs, in a 3-month period 63.3% of
patients under the care of a consultant will be referred, compared to 60.9% of
patients under the care of a GP. Additionally, when comparing the percentage of
patients who took longer than 1 year to be referred, it appears that DGH
consultants are performing better than GPs, with 12.7% of patients taking
longer than 1 year, compared to 21.2% of GP patients (Figure 3). This difference
between GPs and DGH consultants is possibly due to the ordering of
investigations by consultants and waiting for followup appointments. However,
once the results of these investigations have been obtained, it seems that
referral is prompt; the 76 patients who were given an MRI by their DGH
consultant were actually referred slightly more quickly than those who were not
(6.7 weeks versus 6.9 weeks) (Table 2).Figure 2
The median patient.Figure 3
Flow chart of delay.It
seems that the majority of GPs are referring promptly, but they are referring
to the wrong specialists. However, a good proportion (40%) of GPs still takes over
3 months to refer to anyone, which is a problem that needs to be addressed.  The degree of delay is somewhat dependant on
the initial action of the GP.  The 62 patients who were initially reassured by
their GP took significantly longer to be referred than those who were not (Table4). If they
have been reassured that a lump is benign, it may then take patients a long
time to represent to the GP, thereby increasing time to referral.Table 4
Medical professional delay breakdown.
Actionn=MeanMedianMin.Max.% in 1 month% in 3 months% in 1 year%> yearWeeksTime to first referral if MP1Overall16235.82.40552.451.060.978.2100.0Reassures Patient6270.438.31.9552.46.524.254.8100Sends patient to A&E for X-ray1153.76.70405.145.563.681.8100Time until patient referred to the ROH if 2nd MPOverall16244.96.90.01065.337.363.387.3100.0Reassures3308185.379659.4000100Performs MRI7628.26.70991.339.563.293.4100Performs biopsy2019.17.430.9148.9356590100Peforms biopsy and MRI47.15.91.315.35075100100Performs surgery with prior biopsy469.222.12.1230.6255075100Performs surgery without prior biopsy4412018.32.61065.39.129.575100Performs surgery without prior biopsy or
MRI3212417.92.61065.39.437.571.9100The
figures for patients who have had a surgical intervention in a DGH are
particularly interesting. It has been widely documented that surgical
intervention and investigation that is not carried out in a specialist centre for
sarcomas is associated with a worse prognosis in terms of survival and local
recurrence. The interventions carried out are frequently incorrect, as the
consultants are unaware of the diagnosis; the so-called “whoops procedure.” 48
patients had surgery at a DGH, of which 4 had a biopsy before diagnosis. 44 had
no biopsy before excision, and 32 had no cross-sectional imaging. What was
particularly worrying was that these patients took a mean and median 3 times (P<.001) longer to be referred to the ROH than those who were not operated on (Table 2). Therefore, not only are these patients receiving inappropriate and
unnecessary surgery, but they are taking longer to be seen and treated by the
specialists of choice. In most cases, the possibility of malignancy had never
been considered, possibly increasing the waiting time for the operation.We
calculated BMI, recorded postal code to use the Townsend social deprivation score,
and recorded the age at which patient left full-time education. The hypothesis
was that patients with a higher BMI would take longer to present, as would
people living in more deprived areas or with earlier age of having left
full-time education. None of these factors were found to correlate with delay.
The only factor that was associated with delay was that of tumour size at first
presentation; as tumour size increased, time to referral to the Royal Orthopaedic Hospital decreased.In
terms of compliance with the recommended 2-week referral guidelines for
suspected cancer, only 3.8% of patients were seen at the ROH within those 2
weeks. All of these patients were referred directly to the ROH from the GP;
none took the typical route via a DGH. This
accounts for 6 patients of the 9 patients who were referred directly to the
ROH.


## 7. Conclusion

There
is considerable medical professional delay in the management of soft tissue sarcoma.
The fact that 88% of patients had at least one of the guideline features for
referral to a specialist centre at first presentation suggests that knowledge
of the guidelines is poor. Increased education, not only of hospital staff and general
practitioners but especially at medical schools, is essential to ensure that patients
are rapidly and correctly referred. The ideal pathway from GP to specialist centre
needs to be emphasised, so that patients do not get referred to a DGH, where
inappropriate and ineffective treatment is often used.



---

*Source: 378574-2008-02-14.xml*