Monophasic synovial sarcoma of posterior

pharyngeal wall: a rare case report with

unique reconstruction using lateral
trapezius flap

Mudit Agarwal, Abhishek Singh, Adleeb

Abrari & Naveen Singh

European Archives of Oto-Rhino-

Laryngology
and Head & Neck

ISSN 0937-4477

Eur Arch Otorhinolaryngol

DOI 10.1007/s00405-016-4153-8

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Eur Arch Otorhinolaryngol
DOI 10.1007/s00405-016-4153-8
CASE REPORT
Monophasic synovial sarcoma of posterior pharyngeal wall: a rare
case report with unique reconstruction using lateral trapezius flap
Mudit Agarwal1 • Abhishek Singh1 • Adleeb Abrari1 • Naveen Singh1
Received: 29 February 2016 / Accepted: 14 June 2016
Ó Springer-Verlag Berlin Heidelberg 2016
Abstract Synovial sarcoma is a rare entity to be encoun-
tered in the head and neck region and is always a challenge
in terms of diagnosis, treatment planning and reconstruc-
tion of the surgical defect. In our case, we faced a similar
challenge for diagnosis and also have ventured for lateral
trapezius flap as a new reconstructive option for such bulky
tumour defects. We hereby present a 25-year old male
patient with monophasic synovial sarcoma of posterior
pharyngeal wall. The radiological and clinicopathological
features along with various diagnostic tests and treatment
options are discussed.
Keywords Cytogenetic analysis
Immunohistochemistry

Lateral trapezius flap
Neck dissection
Synovial sarcoma
Introduction
Synovial sarcoma comprises 8–10 % of all soft tissue
sarcomas. It commonly occurs in the extremities, followed
by the head and neck region which accounts for up to 10 %
of all cases [1, 2]. Most of these tumours manifest as
& Mudit Agarwal
mudit1000@yahoo.com
Abhishek Singh
innovateiconic@gmail.com
Adleeb Abrari
abrariand@gmail.com
Naveen Singh
dr_naveenks@yahoo.com
1
Head and Neck Surgical Oncology, Max superspeciality
Hospital, Patparganj, Delhi 110092, India
solitary retropharyngeal or parapharyngeal masses near the
carotid bifurcation. They have also been reported in the
soft palate, tongue, parotid gland and tonsil [1]. Rarely,
they have been seen in the medial canthus, submandibular
gland and mandible [3]. The first case of head and neck
synovial sarcoma which occurred in the pharynx was
described in 1954 by Jernstrom [2–5]. We here describe a
case of a monophasic synovial sarcoma of the posterior
pharyngeal wall with an update on the treatment modalities
available.
Case report
A 25-year old male patient reported to the Department of
Head and Neck Surgical Oncology with a chief complaint
of difficulty in breathing especially while lying down
accompanied by snoring since last 2 months. Patient also
gave a history of mouth breathing over the last 2 months
and dysphagia associated with taking solid foods over the
last 1 week.
An ultrasonography of the neck done 2 months back
was suggestive of a cyst in the posterior pharyngeal wall
following which biopsy and histopathological examination
revealed a benign mesenchymal tumour or a solitary
fibrous tumour. The disease progressed and a CECT scan
(contrast enhanced computerized tomography) of face and
neck was done following which patient reported to our
institute.
During initial clinical examination, on inspection, a
huge circumscribed oval growth approximate size
6 9 3 cm, covered by necrotic slough was seen intraorally
in the left tonsillar and pharynx region and crossing the
midline. On palpation the swelling was found to be firm,
non-fluctuant and non-tender.
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CECT-Scan of face and neck revealed a heterogeneous
enhancing mass in the left lower parapharyngeal region
with regular borders with a few interspersed radio-opacities
(Fig. 1).
Pet-scan done showed a hypermetabolicnaso-oropha-
ryngeal soft tissue lesion with associated thickening and
left parapharyngeal lymph nodes were likely to be
metastatically involved.
Additionally slide review and IHC (immunohistochem-
istry) panel (FLI-1, Chromogranin, CK5/6, Ki-67, Bcl2,
CK 18, SMA, CD34, p63, Vimentin, CK, S-100, CD99,
CK19 and EMA) was also done which suggested a synovial
cell sarcoma or a spindle cell carcinoma.
Based on clinical examination, histopathological
examination with IHC and radiological interpretation a
provisional diagnosis of soft tissue sarcoma of the posterior
pharyngeal wall was made.
Pre-operative workup including routine haematological
investigations, chest X-ray, liver and renal function tests
were normal.
Left mandibular swing, WLE (wide local excision) of
the pharyngeal mass along with left modified neck dis-
section (MND) type III, reconstruction using trapezius flap
and skin grafting under general anaesthesia was planned.
Skin preparation of face, neck, left shoulder and left
thigh was done with betadine followed by administration of
2 % xylocaine with 1:2,00,000 adrenaline for local infil-
tration. The tracheostomy was done at third tracheal ring
using Bjork flap and patient was given general anaesthesia.
A transcervical incision was placed extending from left
mastoid region to the hyoid bone level utilizing the second
neck skin crease and was joined with the chin sparing lip
split incision. The subplatysmal plane was followed up to
the lower border of the mandible and the marginal
mandibular nerve was identified traversing the facial ves-
sels. Routine MND (III) using open surgical approach was
done on the left side. The tumour was abutting the IJV
(internal jugular vein) and ICA (internal carotid artery).
ECA (external carotid artery) was ligated and transfixed.
Fig. 1 CECT-Scan of face and neck
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The hypoglossal, superior laryngeal and vagus nerves were
identified and preserved.
The lip split incision was extended intra orally till the
first premolar on the left side and full thickness mucope-
riosteal flap was raised taking care to preserve the mental
nerve. The lower left lateral incisor was extracted and the
planned stepped osteotomy line was marked. One 2 9 4
hole midgap straight plate (2 mm) was adapted subapically
in the left parasymphysis region and another single 2 9 5
hole continuous straight plate (2 mm) was adapted 5 mm
below the previous plate at the inferior border of the
mandible. These plates were secured with 2 9 8 mm
screws on each side of the intended osteotomy site and
were then removed. Osteotomy was carried out using
oscillating saw through the socket of the extracted lateral
incisor. The mandible was swung after giving a releasing
incision on the lingual mucosa and access to the pharyngeal
mass was achieved (Fig. 2). The tumour had two compo-
nents i.e., an extramucosal (5 9 4 cm) and a submucosal
(6 9 4 cm). The extension of tumour was inferiorly at the
level just above the arytenoids and superiorly just below
the basiocciput (nasopharynx apex). Deep prevertebral
muscles were removed, vertebral bodies were exposed and
superior and inferior extent of excision was marked with
metal clips. The tumour was removed with a 1 cm mucosal
margin all around the posterior pharyngeal wall, part of
base of tongue and left tonsil.
Frozen section was done to assess the margins which
were negative for malignancy.
The surgical defect (6 9 7 cm) was then reconstructed
using a left lateral trapezius myocutaneous flap, the portion
of the flap which was used for reconstruction of pharyngeal
defect was skin and subcutaneous tissue of the deltoid
region (Figs. 3, 4a). The flap was harvested and inset after
Fig. 2 Routine MND (III), mandibular swing and surgical exposure
of the tumour
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Fig. 3 Post resection defect (white arrow mandibular swing, black
arrow sternocleidomastoid muscle, black outlined arrow ligated
ECA, yellow outlined arrow hypoglossal nerve)
Fig. 4 a Lateral trapezius flap (white arrow de-epithelized portion of
the skin paddle, black arrow trapezius muscle part incorporated in the
flap, black outlined arrow spinal accessory nerve). b Lateral trapezius
flap harvested (white arrow de-epithelized portion of the skin paddle,
black arrow trapezius muscle part incorporated in the flap, black
outlined arrow spinal accessory nerve)
de-epithelizing the proximal portion (Fig. 4b). This flap is
based on transverse cervical vessels. It was then sutured to
the defect in a single layer with 3-0 vicryl. Left base of
tongue and left tonsillar mucosa was also sutured to the
Fig. 5 Fixation of the osteotomised mandible using miniplates and
monocortical screws
trapezius flap. The osteotomised mandible was fixed using
the same miniplates and eight 2 9 8 mm screws (Fig. 5).
Two holes were made along the inferior border of mandible
in the symphysis region and laryngeal suspension was done
bilaterally around the hyoid bone using 2-0 prolene.
The lingual mucosa was then sutured using 3-0 vicryl.
The donor site was closed partially by primary closure and
partially by skin grafting (using the de-epithelialized skin
of the same flap). Neck was closed after placing suction
drain in two layers. At the tracheostomy site endotracheal
tube was replaced by double lumen no. 8 cuffed tra-
cheostomy tube.
The specimen along with the nodes and submandibular
gland was sent for histopathological examination (HPE).
The entire specimen measured 11 9 8 9 2.5 cm in
dimensions, comprising of two components with the largest
polypoidal tumour mass measuring 6 9 4 9 3 cm and the
mucosa with adherent tumour mass measuring
6 9 4.5 9 3.5 cm.
Final histopathology revealed plump spindle to ovoid
shaped cells in sheets and intersecting fascicles. Zones of
calcification and necrosis were present. Mitoses focally
approaching up to 20/10 hpf were seen (Fig. 6). The lesion
was reported as a monophasic synovial sarcoma of the
posterior pharyngeal wall.
Cytogenetic analysis confirmed translocation of x:18
thus confirming the diagnosis of a monophasic synovial
cell sarcoma.
Post operatively, the patient complained of mild aspi-
ration. He was taught swallowing exercises. Patient was
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Fig. 6 H&E micrographs (anticlockwise). 1 Low power (910), tumour cells interspersed with blood vessels; 2 high power (940), atypical
spindle tumour cells, mitoses; 3 high power (940), polygonal ‘epithelioid’ tumour cells; 4 high power (940), dystrophic calcification

Fig. 7 Post-operative fibreoptic laryngoscopy showed a healthy flap and no aspiration

decannulated after 10 days and ryle’s tube removed after Discussion

2 weeks (Fig. 7). The treatment plan comprised of
sequential radiotherapy and chemotherapy. The patient
received IGRT (intensity gated radiation therapy) of
66 Gy. in 33# for 2 months. A regime of ifosfamide 3 mg
iv, mesna 600 mg at 0–4–8 h (to prevent bladder toxicity
due to ifosfamide) and Adriamycin 100 mg iv as D1, D2,
D3 every 21 days for 3 cycles was planned but the patient
developed neutropenia after one cycle, due to which the
regime was discontinued. The patient presently shows no
evidence of the disease and is on a semisolid diet.
Synovial sarcoma is an intriguing entity owing to its rarity,
controversial origin, challenging diagnosis and restricted
treatment protocols. It is frequently misdiagnosed as a
benign lesion due to its smooth margins, an associated cystic
component and lack of aggressive infiltration [6]. Its
microscopic resemblance to developing synovium was
hypothesized early in literature, but its origin from pre-
formed synovial tissues has never been substantiated [1, 7]. It
has been postulated to arise from the malignant degeneration

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Fig. 8 Immunohistochemistry micrographs (anticlockwise). 1 Cytokeratin, 2 BCL2, 3 EMA, 4 cytokeratin 19
of pluripotential mesenchymal cells near or even remote
from articular surfaces, tendons, tendon sheaths, juxta-ar-
ticular membranes, and facial aponeuroses [5, 8]. No benign
counterpart of the lesion has been described [8].
Although the term ‘synovial sarcoma’ is used to
describe this lesion, it has been suggested that it should be
replaced by ‘‘carcinosarcoma’’ of soft tissue, which more
accurately describes the essence of the lesion [1].
Synovial sarcomas of the head and neck show a male
predominance (3:2) and predilection for patients between
25 and 36 years of age [3, 4]. They are known to occur at
various sites in the head and neck region and it has been
said that they are commonly encountered in the
hypopharynx because it is the seat of numerous synovial
formations [5]. The patients present with a complaint of an
enlarging mass with difficulty swallowing and breathing,
alteration or loss of voice and hematemesis. Primary or
secondary involvement of nerves may cause pain, numb-
ness, and paraesthesia [1–3].
CT scan classically displays a multilocular tumour with
smooth margins and heterogeneous enhancement after
injection of contrast medium [5, 6]. The most striking
radiologic feature, found in 15–20 % of synovial sarcomas,
is the presence of multiple small, spotty radio-opacities
caused by focal calcification which was seen in our patient
as well (Fig. 1). Calcifications may range from fine stip-
pling to prominent radiopaque masses [1].
Histologically, the variants of synovial sarcoma include
a classic biphasic type, with distinct epithelial and spindle
cell components in varying proportions; the monophasic
fibrous type, which closely resembles fibro sarcoma and
MPNST, and at the other extreme of the morphologic
spectrum lies the monophasic epithelial type [1]. Wick
et al. stated that the monophasic synovial sarcoma is one of
the most frequently missed and misdiagnosed soft tissue
tumours [8]. A poorly differentiated synovial sarcoma may
superimpose on any of the variants and is more aggressive
with increased propensity for metastasis [1].
Synovial sarcomas, on cytogenetic analysis typically
harbours a t(X;18) (p11.2;q11.2) translocation with fusion
between SSX1 and SYT genes with a biphasic appearance
in two-thirds of cases whilst the remainder show a fusion
between SSX2 and SYT genes [1, 2, 7, 9]. We also found a
similar cytogenetic translocation in our case.
Immunoreactivity for epithelial markers is useful in
establishing the diagnosis [1]. Around 60–70 % of poorly
differentiated synovial sarcomas stain for cytokeratins 7, 8
and 19. EMA shows focal staining which confirms
epithelial nature and Bcl-2 shows a diffuse positivity. In
our case preoperative IHC showed neoplastic cells which
expressed diffuse strong Vimentin, weak to moderate Bcl2
and up to 40–50 % cells marked for EMA. Scattered cells
marked for CK (up to 5 %) and rare expressed CK19
(\1 %). FLI-1 was weak, but no CD99 expression was
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noted and SMA marked small number of scattered cells.
Chromogranin, CK5/6, CK18, CD34, p63, S-100 were
negative whereas Ki-67 was non-contributory (Fig. 8).
Synovial sarcoma of head and neck has an aggressive
nature and a guarded prognosis. A multidisciplinary
approach has to be formulated to limit recurrences and
prevent metastasis. Wide-local excision aimed at achieving
negative surgical margins is the mainstay of treatment for
synovial sarcomas. There is no indication for prophylactic
neck dissection except when palpable and enlarged lymph
nodes are present; we did it as was evident on PET CT scan
and also because these sarcomas have a tendency for nodal
metastasis [3, 4]. Metastasis is mainly to the lungs, fol-
lowed by lymph nodes and bone [2]. Most metastasis
originate from haematogenous dissemination, although up
to 20 % spread occur through the lymphatics to regional
lymph node [4].
Local excision is followed by high recurrence rates
(60–90 %), usually within 2 years. Daveau et al. study
supports the use of adjuvant radiotherapy as it improved
the overall survival rates with few recurrences in patients in
whom the primary tumour was treated with surgical
resection and radiotherapy [2, 4].
Overall, the 5-year survival rate in head and neck syn-
ovial sarcoma is around 40–60 % [2, 9]. Decreased sur-
vival rates are seen with large tumour size (greater than
5 cm), positive margin status, and high tumour grade
(Grade 3) [3].
Role of chemotherapy in the management of synovial
sarcoma is still debatable. A meta-analysis of all sarcomas,
including 10 % synovial sarcomas, found that doxorubicin
chemotherapy significantly delayed local recurrence and
remote metastasis and increased overall recurrence-free
survival [2]. Other studies have shown benefits with ifos-
famide based chemotherapy [9, 10]. Literature presents
with few cases of head and neck synovial sarcoma with no
universal consensus for the role of chemotherapy but as the
tumour size was more than 5 cm in our patient,
chemotherapy was considered as a treatment option.
Conclusion
A meticulous approach for preoperative diagnosis with use
of IHC molecular markers and cytogenetic analysis should
be done. The treatment should include surgery with nega-
tive margins, with radiotherapy and chemotherapy to
restrain the disease, thus contributing to better survival
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rates. Molecular analysis of the lesion should be encour-
aged which might reveal promising molecular therapeutics.
The use of lateral trapezius flap also adds a new dimension
to the reconstructive options for such large surgical defects
in the pharynx.
Acknowledgments We would like to acknowledge Dr. Roma
Ambastha, Dr. Abhishek Pareekh and Dr. Jyotika Jain for all their
help.
Compliance with ethical standards
Conflict of interest The authors declare that there is no conflict of
interest regarding the publication of this article.
Competing interests Nil.
Ethical approval Since it a case report, it is not applicable.
Patient consent Yes written patient consent has been obtained for
clinical photographs.
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