# Adult Head and Neck Soft Tissue Sarcomas: Treatment and Outcome

Authors: Rabindra P. Singh; Robert J. Grimer; Nabina Bhujel; Simon R. Carter; Roger M. Tillman; Adesegun Abudu
Journal: Sarcoma
 (2008)

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## Abstract

We have retrospectively analysed the experience of a musculoskeletal oncological unit in the management of adult head and neck soft tissue sarcomas from 1990 to 2005.
Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of
5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor.
Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.


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## Body

## 1. Introduction

Soft tissue sarcomas of the head and neck are rare mesenchymal
malignant neoplasms accounting for less than 10% of all soft tissue sarcomas
and approximately 1% of all head and neck neoplasms [1–5].  Nevertheless,
they represent an important group of tumours and are associated with
significant morbidity and mortality.There are several histological subtypes of sarcomas which present
with a variety of clinical characteristics and many often require treatment
with combination of surgery, radiotherapy, and chemotherapy. They are best
treated in specialist sarcoma units where expert multidisciplinary approach to
management is possible. In the UK,
surgical management of soft tissue sarcomas in the head and neck region is undertaken
by otolaryngologists, maxillofacial surgeons, as well as by musculoskeletal
sarcoma surgeons, depending on the location of the lesion.Reflecting the rarity of the disease, there is currently a scarcity
of studies in the literature and, to our knowledge, there is only one published
study that is based in a UK
hospital in the last 15 years [4]. Most of the series which are published have
reported outcome over a number of decades possibly to compensate for the rarity
of the disease and to increase the size of the study sample for meaningful
statistical analysis [6]. However, the spanning of studies over several decades
has an important drawback in that changes in the management of sarcomas and
their outcome are not always accurately reflected.We report on our experience of the management of adult head and neck
soft tissue sarcomas presenting to a regional sarcoma centre based at an
orthopaedic hospital over the past 20 years.


## 2. Methods

We have prospectively collected patient, tumour, treatment, and
outcome data on all patients with bone and soft tissue sarcomas for over 20
years at the Royal Orthopaedic Hospital (Birmingham, UK). We have identified all patients with soft
tissue sarcomas in the head and neck, defined as sites above the level of the
clavicle. We have included all patients seen at the unit between 1990 and 2005.We have made a number of observations related to patient
demographics, tumour variables, treatment modalities, outcome, and follow up
for patients with head and neck soft tissue sarcomas. Tumours were classified
as deep if they were deep to the investing fascia whilst they were superficial
if they lay purely in the subcutaneous tissues. The margins of excision were
classified according the method of Enneking [7] with a wide margin being one in
which a clear layer of normal tissue lay between the tumour and the excision
margin. A marginal  excision was when the excision plane passed
through the reactive zone around the tumour (clear but close) and an
intralesional excision was when tumour was incised at any part of the
operation, even if a subsequent wide excision was achieved. Survival was
estimated using Kaplan Meier survival curves and was determined for overall
5-year survival, and the log rank method was used to analyse the influence of
various prognostic factors on survival of the patients. For situations where no
events had arisen in one subgroup, chi-square testing was used to assess
possible significance. Institutional approval for this study was obtained.


## 3. Results

### 3.1. Patients

A total of 36 patients with head and neck soft tissue sarcomas were
seen during the study period, 24 of whom were treated at this unit. This is
approximately 2% of the total 1912 cases of all soft tissue sarcomas seen at
this unit during the same period. The median age of the patients was 46 years
with a range from 16 to 83 years. There were 24 male and 12 female patients
(M : F = 2 : 1).Thirty-five of the sarcomas were located in the neck and one was
located in the scalp. The average duration of symptoms experienced by patients
prior to diagnosis was 54 weeks (range 1–416 weeks) and
the most commonly reported symptom was the presence of a painless lump.The rest of the observations and analysis are based on the 24
patients who received treatment at this unit. We have excluded 12 patients who
were either referred for advice only (9 patients) and received definitive
treatment elsewhere or who presented with local recurrence and/or metastases
after previous failed treatment (3 patients). Various observations on tumours,
treatment, local control, and outcome are summarised in
Table1.Table 1
Patient
age, tumour factors, treatment, local recurrence, and outcome. MPNST:  malignant
peripheral nerve sheath tumour, DFSP:  dermatofibrosarcoma protuberans, MFH: malignant
fibrous histiocytoma, SEF:  sclerosing epithelioid fibrosarcoma, CT:  chemotherapy,
RT:  radiotherapy.
Case
no.Age
(yrs)DiagnosisSize (cm)DepthTrojani gradeDefinitive
treatmentSurgical marginLocal recurrence (months)Outcome (months)138Leiomyosarcoma0.6SubcutaneousHighExcisionWideAlive, 45222Ewing's sarcoma1.5DeepHighCT + excisionWideAlive, 208341DFSP1.7SubcutaneousIntermediateExcisionMarginalAlive, 20424DFSP2SubcutaneousLowExcisionWideAlive, 37519MPNST3SubcutaneousIntermediateExcisionWideAlive, 36620Ewing’s sarcoma4DeepHighCT + excision + RTIntralesionalAlive, 74741Spindle cell sarcoma4DeepIntermediateExcisionIntralesional8Alive, 21864Spindle cell sarcoma4DeepIntermediateExcision + RTMarginal16Died, 33939MPNST5DeepHighExcision + RTIntralesional26Died, 521053SEF5DeepIntermediateExcisionWide49Alive, 1391162Myxoid chondrosarcoma5DeepIntermediateExcisionMarginal96Alive, 1571277MFH6DeepHighExcision + RTIntralesional5Died, 221354Myxofiibrosarcoma6.5DeepHighExcision + RTMarginalAlive, 141422Synovial sarcoma7DeepHighExcision + RTIntralesionalAlive, 351538MPNST7DeepHighExcision + RTIntralesionalDied, 141650MPNST7DeepIntermediateExcision + RTMarginalAlive, 1911765Liposarcoma7DeepHighExcision + RTIntralesional12Died, 161832MPNST8DeepIntermediateExcision + RTMarginalAlive, 131968Spindle cell sarcoma9SubcutaneousHighExcision + RTWideAlive, 122073Liposarcoma9DeepHighExcision + RTIntralesional41Died, 512130Ewing’s sarcoma10DeepHighCT + excision + RTIntralesional12Died, 142248Synovial sarcoma10DeepIntermediateExcision + RTMarginalAlive, 112365MPNST10DeepIntermediateExcision + RTMarginalDied, 452433Liposarcoma13SubcutaneousLowExcisionMarginal8Alive, 51


### 3.2. Tumours

The median tumour size was 5.5 cm (range 0.6–13 cm) at
diagnosis. 18 of the sarcomas (75%) were deep to the investing fascia, the rest
superficial to the fascia. Two of the sarcomas were low grade (18%), the rest
intermediate (42%,n=10) or high grades (50%, n=12). All tumours which were
located deep to the fascia were of high or intermediate grade. Only one patient
(with a Ewing’s sarcoma) had lung metastases at
the time of presentation.Eleven different histological subtypes were identified, of which
malignant peripheral nerve sheath tumour (MPNST) was the commonest subtype
(25%,n=6). The histological subtypes are listed in Table 2.Table 2
Histological
subtypes. MPNST: malignant peripheral
nerve sheath tumour, DFSP: dermatofibrosarcoma protuberans, MFH: malignant
fibrous histiocytoma, SEF: sclerosing epithelioid fibrosarcoma.
SubtypesNo. of
patients (%)MPNST6
(25%)Ewing’s sarcoma3 (13%)Liposarcoma3
(13%)Spindle cell sarcoma3
(13%)Synovial sarcoma2
(8%)DFSP2
(8%)Myxofibrosarcoma1 (4%)Leiomyosarcoma1 (4%)MFH1 (4%)Myxoid Chondrosarcoma1
(4%)SEF1
(4%)


### 3.3. Treatment

The principles of treatment used at the Unit during this time
consisted of planned wide local excision followed by radiotherapy for all high-grade
tumours>5 cm or where there were close margins of excision.Eight patients were treated with surgery alone as initial treatment
for their sarcomas. 13 patients had surgery and postoperative radiotherapy. Two
of the three patients who had Ewing’s sarcoma received neoadjuvant
chemotherapy, surgery, and postoperative radiotherapy; and one received
chemotherapy and surgery. The documented margins of excision were intralesional
in 10 (42%), marginal in 9 (37%), and wide in 5 (21%).


### 3.4. Local Control

The patients were followed up for an average period of 50 months.
Local recurrence arose in 10 patients (42%) at a median time of 14 months
(range 5–96 months) following initial treatment. One of the two patients with
a low-grade superficial sarcoma developed local recurrence and 9 of the 22 with
high- or intermediate-grade sarcomas developed local recurrence. Local
recurrence was strongly related to margins achieved, arising in six of the ten
with an intralesional margin (60%), three of the nine with a marginal margin
(33%), and one of the five with a wide margin (20%).Of the ten patients who developed local recurrence, four were either
known to have systemic metastases already or were found to have synchronous
metastases at the time of restaging. All four received palliative treatment and
all died at a median of 9 months from diagnosis of the local recurrence. Six
patients with local recurrence had no evidence of metastases when they
developed the local recurrence and were treated aggressively with further
surgical excision and radiotherapy when possible, often requiring extensive
surgical reconstructions in order to obtain wide margins of excision. Two of these patients subsequently developed
metastases 6 and 12 months later, respectively, and both subsequently died. The
other four patients remained
disease-free at a mean of 52 months following their local recurrence.


### 3.5. Metastases

Ten patients (42%) developed metastatic disease at a median time of
17 months (range 0–139 months).
Eight patients developed lung metastases and two lymph node metastases. All of
the patients with concomitant or previous local recurrence subsequently died as
did two of the others without local recurrence. Two patients underwent surgical
resection of lung metastases and remained alive and disease-free at a median of 17 months.


### 3.6. Survival

Eight patients died at a median time of 2.3 years from diagnosis.
Overall survival was 48.6% at five years but with wide confidence limits (plus
or minus 13%) (Figure1). We investigated the following factors for possible
significance on survival.Figure 1
Overall 5-year survival.(i)
Size. Only 1 patient with a primary sarcoma >5 cm has yet survived more
than 5 years whilst the survival for patients with tumours ≤5 cm was 71% at 5
years (p=0.02) (Figure 2).(ii)
Grade. Neither of the two patients with low-grade tumours died but 8 of
the 22 with high- or intermediate-grade tumours died (P=.29) (chi-square).(iii)
Depth. None of the 6 patients with subcutaneous sarcomas died but 8 of the
18 with deep tumours died (P=.0455)  (chi-square).(iv)
Margin. Six of the 10 patients with intralesional and two of the 9 patients
with a marginal surgical margin died, however none of the 5 patients with a
wide surgical margin died (P=.04).(v)
Age. We could find no effect of age on survival.
Combining these factors revealed that all deaths arose
in the patients with high- or intermediate-grade deep tumours. When further
stratified by size, this showed that two of the seven patients with high-grade
deep tumours ≤5 cm died compared with 6 of the 11 with high-grade deep
tumours >5 cm (P=.04).Figure 2
Overall survival by size of sarcoma.


## 3.1. Patients

A total of 36 patients with head and neck soft tissue sarcomas were
seen during the study period, 24 of whom were treated at this unit. This is
approximately 2% of the total 1912 cases of all soft tissue sarcomas seen at
this unit during the same period. The median age of the patients was 46 years
with a range from 16 to 83 years. There were 24 male and 12 female patients
(M : F = 2 : 1).Thirty-five of the sarcomas were located in the neck and one was
located in the scalp. The average duration of symptoms experienced by patients
prior to diagnosis was 54 weeks (range 1–416 weeks) and
the most commonly reported symptom was the presence of a painless lump.The rest of the observations and analysis are based on the 24
patients who received treatment at this unit. We have excluded 12 patients who
were either referred for advice only (9 patients) and received definitive
treatment elsewhere or who presented with local recurrence and/or metastases
after previous failed treatment (3 patients). Various observations on tumours,
treatment, local control, and outcome are summarised in
Table1.Table 1
Patient
age, tumour factors, treatment, local recurrence, and outcome. MPNST:  malignant
peripheral nerve sheath tumour, DFSP:  dermatofibrosarcoma protuberans, MFH: malignant
fibrous histiocytoma, SEF:  sclerosing epithelioid fibrosarcoma, CT:  chemotherapy,
RT:  radiotherapy.
Case
no.Age
(yrs)DiagnosisSize (cm)DepthTrojani gradeDefinitive
treatmentSurgical marginLocal recurrence (months)Outcome (months)138Leiomyosarcoma0.6SubcutaneousHighExcisionWideAlive, 45222Ewing's sarcoma1.5DeepHighCT + excisionWideAlive, 208341DFSP1.7SubcutaneousIntermediateExcisionMarginalAlive, 20424DFSP2SubcutaneousLowExcisionWideAlive, 37519MPNST3SubcutaneousIntermediateExcisionWideAlive, 36620Ewing’s sarcoma4DeepHighCT + excision + RTIntralesionalAlive, 74741Spindle cell sarcoma4DeepIntermediateExcisionIntralesional8Alive, 21864Spindle cell sarcoma4DeepIntermediateExcision + RTMarginal16Died, 33939MPNST5DeepHighExcision + RTIntralesional26Died, 521053SEF5DeepIntermediateExcisionWide49Alive, 1391162Myxoid chondrosarcoma5DeepIntermediateExcisionMarginal96Alive, 1571277MFH6DeepHighExcision + RTIntralesional5Died, 221354Myxofiibrosarcoma6.5DeepHighExcision + RTMarginalAlive, 141422Synovial sarcoma7DeepHighExcision + RTIntralesionalAlive, 351538MPNST7DeepHighExcision + RTIntralesionalDied, 141650MPNST7DeepIntermediateExcision + RTMarginalAlive, 1911765Liposarcoma7DeepHighExcision + RTIntralesional12Died, 161832MPNST8DeepIntermediateExcision + RTMarginalAlive, 131968Spindle cell sarcoma9SubcutaneousHighExcision + RTWideAlive, 122073Liposarcoma9DeepHighExcision + RTIntralesional41Died, 512130Ewing’s sarcoma10DeepHighCT + excision + RTIntralesional12Died, 142248Synovial sarcoma10DeepIntermediateExcision + RTMarginalAlive, 112365MPNST10DeepIntermediateExcision + RTMarginalDied, 452433Liposarcoma13SubcutaneousLowExcisionMarginal8Alive, 51


## 3.2. Tumours

The median tumour size was 5.5 cm (range 0.6–13 cm) at
diagnosis. 18 of the sarcomas (75%) were deep to the investing fascia, the rest
superficial to the fascia. Two of the sarcomas were low grade (18%), the rest
intermediate (42%,n=10) or high grades (50%, n=12). All tumours which were
located deep to the fascia were of high or intermediate grade. Only one patient
(with a Ewing’s sarcoma) had lung metastases at
the time of presentation.Eleven different histological subtypes were identified, of which
malignant peripheral nerve sheath tumour (MPNST) was the commonest subtype
(25%,n=6). The histological subtypes are listed in Table 2.Table 2
Histological
subtypes. MPNST: malignant peripheral
nerve sheath tumour, DFSP: dermatofibrosarcoma protuberans, MFH: malignant
fibrous histiocytoma, SEF: sclerosing epithelioid fibrosarcoma.
SubtypesNo. of
patients (%)MPNST6
(25%)Ewing’s sarcoma3 (13%)Liposarcoma3
(13%)Spindle cell sarcoma3
(13%)Synovial sarcoma2
(8%)DFSP2
(8%)Myxofibrosarcoma1 (4%)Leiomyosarcoma1 (4%)MFH1 (4%)Myxoid Chondrosarcoma1
(4%)SEF1
(4%)


## 3.3. Treatment

The principles of treatment used at the Unit during this time
consisted of planned wide local excision followed by radiotherapy for all high-grade
tumours>5 cm or where there were close margins of excision.Eight patients were treated with surgery alone as initial treatment
for their sarcomas. 13 patients had surgery and postoperative radiotherapy. Two
of the three patients who had Ewing’s sarcoma received neoadjuvant
chemotherapy, surgery, and postoperative radiotherapy; and one received
chemotherapy and surgery. The documented margins of excision were intralesional
in 10 (42%), marginal in 9 (37%), and wide in 5 (21%).


## 3.4. Local Control

The patients were followed up for an average period of 50 months.
Local recurrence arose in 10 patients (42%) at a median time of 14 months
(range 5–96 months) following initial treatment. One of the two patients with
a low-grade superficial sarcoma developed local recurrence and 9 of the 22 with
high- or intermediate-grade sarcomas developed local recurrence. Local
recurrence was strongly related to margins achieved, arising in six of the ten
with an intralesional margin (60%), three of the nine with a marginal margin
(33%), and one of the five with a wide margin (20%).Of the ten patients who developed local recurrence, four were either
known to have systemic metastases already or were found to have synchronous
metastases at the time of restaging. All four received palliative treatment and
all died at a median of 9 months from diagnosis of the local recurrence. Six
patients with local recurrence had no evidence of metastases when they
developed the local recurrence and were treated aggressively with further
surgical excision and radiotherapy when possible, often requiring extensive
surgical reconstructions in order to obtain wide margins of excision. Two of these patients subsequently developed
metastases 6 and 12 months later, respectively, and both subsequently died. The
other four patients remained
disease-free at a mean of 52 months following their local recurrence.


## 3.5. Metastases

Ten patients (42%) developed metastatic disease at a median time of
17 months (range 0–139 months).
Eight patients developed lung metastases and two lymph node metastases. All of
the patients with concomitant or previous local recurrence subsequently died as
did two of the others without local recurrence. Two patients underwent surgical
resection of lung metastases and remained alive and disease-free at a median of 17 months.


## 3.6. Survival

Eight patients died at a median time of 2.3 years from diagnosis.
Overall survival was 48.6% at five years but with wide confidence limits (plus
or minus 13%) (Figure1). We investigated the following factors for possible
significance on survival.Figure 1
Overall 5-year survival.(i)
Size. Only 1 patient with a primary sarcoma >5 cm has yet survived more
than 5 years whilst the survival for patients with tumours ≤5 cm was 71% at 5
years (p=0.02) (Figure 2).(ii)
Grade. Neither of the two patients with low-grade tumours died but 8 of
the 22 with high- or intermediate-grade tumours died (P=.29) (chi-square).(iii)
Depth. None of the 6 patients with subcutaneous sarcomas died but 8 of the
18 with deep tumours died (P=.0455)  (chi-square).(iv)
Margin. Six of the 10 patients with intralesional and two of the 9 patients
with a marginal surgical margin died, however none of the 5 patients with a
wide surgical margin died (P=.04).(v)
Age. We could find no effect of age on survival.
Combining these factors revealed that all deaths arose
in the patients with high- or intermediate-grade deep tumours. When further
stratified by size, this showed that two of the seven patients with high-grade
deep tumours ≤5 cm died compared with 6 of the 11 with high-grade deep
tumours >5 cm (P=.04).Figure 2
Overall survival by size of sarcoma.


## 4. Discussion

Head and neck sarcomas are
rare and the paucity of studies about their management and outcome testifies to
this. Head and neck lumps are common and have a variety of diagnoses [2, 3].
Early detection and diagnosis is clearly essential. The National Institute for Health and
Clinical Excellence (NICE) guidance 2005 to all UK general practitioners emphasises
that “In patients with an unexplained
lump in the neck which has recently appeared or a lump which has not been
diagnosed before that has changed over a period of 3 to 6 weeks, an urgent
referral should be made” [8]. Whilst more general advice is also
given about lumps elsewhere in the body: “In patients presenting with a palpable lump,
an urgent referral for suspicion of soft tissue sarcoma should be made if the
lump is(i)
greater than about 5 cm in
diameter,(ii)
deep to fascia, fixed, or
immobile,(iii)
painful,(iv)
increasing in size,(v)
a recurrence after previous
excision.”
Our unit is a musculoskeletal unit that takes referrals of patients
with both proven or suspected sarcomas. In the case of the head and neck
tumours, some were referred directly to us for investigation and diagnosis
whilst others were referred after a biopsy or imaging had confirmed the
diagnosis of a sarcoma. We treated patients with sarcomas that were confined to
the superficial tissues or deep muscles of the head and neck and we have not included
patients with soft tissue sarcomas involving the facial skeleton or the
oropharynx which pose even greater challenges in treatment [6].Our management policy was based on principles used in treating soft
tissue sarcomas at other sites. The head and neck poses particular problems
however because of the proximity of so many important structures and the near
impossibility of obtaining wide surgical margins in many cases. Unlike limb
soft tissue sarcomas, there is no fallback option of doing an amputation if
local recurrence arises.The local recurrence rates for high-grade soft tissue sarcomas after
surgical excision have
been reported to be as high as 50% in the literature [3, 9, 10]. 42%
of patients developed local recurrence in our study, most arising within 2
years of treatment. Barker et al. in their study reported the median time to
local recurrence after treatment with surgery and/or radiotherapy to be 4
months, and Kraus et al. reported that patients who developed local recurrence
did so within 3 years [11, 12].The risk of local recurrence was higher with intralesional or
marginal surgical margins as has been shown by other authors [2, 12]. In view of
this, every effort should be made to maximise the margins that can be achieved
at the time of the first surgical procedure, if necessary by going back and
doing a further wide excision if the initial margins prove positive. All
patients should have their case discussed at a multidisciplinary team meeting
and the option of radiotherapy considered to try and decrease the risk of local
recurrence. Even if patients do develop
local recurrence, their case is not hopeless and further excision should be
considered. Clearly, however, initial wide surgical margins should always be
aimed for.The commonest site for metastases was the lungs which was also the
commonest cause
of death. Mendenhall et al. [3] suggested that patients should
undergo a chest CT before treatment and also suggested that in the absence of
pulmonary metastases, other distant metastases are highly unlikely. We concur
with this.The 5-year survival rate of 49% in our study (Figure1) is
comparable to a previous UK study by Eeles et al. [4] based at the Royal
Marsden Hospital of London. They analysed 103 cases seen over 44 years between
1944 and 1988 and reported 50% overall 5-year survival rate. This is similar to
the results reported by Bentz et al. [6] from Memorial Sloan Kettering Cancer
Centre. Most authors agree that the same
prognostic factors apply to sarcomas no matter where they arise—grade, size, and
depth. In the head and neck, however, local recurrence has more sinister
portents because of the difficulty of subsequent management [5, 13–18].Obtaining local control is paramount in managing these head and neck
sarcomas. Obtaining wide margins may often require a multidisciplinary team
consisting of a sarcoma surgeon, a head and neck surgeon and a reconstructive
surgeon. A clinical oncologist is an essential part of the team to advise about
radiotherapy usage. A metaanalysis published
in Lancet revealed that chemotherapy did not produce a survival benefit in the
treatment of soft tissue sarcomas [19]. The same analysis did, however, show a 10% benefit of chemotherapy on recurrence-free
survival. Adjuvant chemotherapy is not usually advocated for localised
soft tissue sarcomas but can be considered for metastatic disease as a
palliative treatment.Recent guidance from NICE, UK
[20] in the
management of patients with sarcomas has highlighted the importance of
referring all patients with soft tissue sarcomas to a sarcoma centre where they
can be managed by a multidisciplinary team (MDT). The guidance has also
emphasised the importance of close collaboration between these sarcoma MDTs and
site-specific head and neck surgeons and oncologists. This has been emphasised
by the recent paper of Harb et al. [21]. We recommend that all surgeons who
identify a suspected or proven soft tissue sarcoma of the head and neck should
refer that patient to a sarcoma MDT and that all head and neck cancer MDTs
should have close links with the local sarcoma MDT for management of these
cases.



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