Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case

A Italiano

A Srivasta

BA Guadagnolo

BE White

BV Palmer

CDM Fletcher

CM Mermigkis

Dibendu Betal

DS Ambani

FC Eilber

G Simon

H Marsh

H Schreiber-Facklam

J Alberty

J Hardie

JR Mullen

JR Parfitt

L Ramamurthy

L Saydam

L Tacconi

M Votruba

M Zenmyo

MC Aubry

PH Wright

R Artico

Ramesh Babu

RR Lawrence

S Frustaci

Sarcoma Meta-analysis Collaboration

SF Jones

T Iwata

Veysi Mehmet

WJ Harb

Y Mikami

English

PubMed

Springer - Publisher Connector

BioMed Central
International Seminars in Surgical 
Oncology
ssOpen AcceCase report
Monophasic synovial sarcoma of the pharynx: a case report
Dibendu Betal*, Ramesh Babu and Veysi Mehmet
Address: Department of Surgery, St Mary's Hospital, Isle of Wight NHS Trust, Parkhurst Road, Newport, Isle of Wight, PO30 5TG, UK
Email: Dibendu Betal* - ulogin2000@yahoo.co.uk; Ramesh Babu - Ramesh.Babu@iow.nhs.uk; Veysi Mehmet - Veysi.Mehmet@iow.nhs.uk
* Corresponding author    
Abstract
Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male
presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently
diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and
treatment of this case.
Background
Synovial sarcomas are named as they were believed to
originate from the synovium but less than 10% are intra-
articular. They are a rare form of soft tissue sarcoma after
liposarcomas, malignant fibrous histiocytomas and fibro-
sarcomas and make up 10% of all sarcomas. They nor-
mally involve large joints and lower extremities. They are
often small in size with a slow growth and well-circum-
scribed resembling benign lesions. This case demonstrates
a rare occurrence of a synovial sarcoma in the pharynx.
Case presentation
A 62 year-old gentleman presented to thyroid clinic with
a neck lump. It had been growing slowly over a six-month
period and became more noticeable. He did not report
any hoarse voice, dyspnoea or dysphagia. There was no
loss of appetite or weight. On examination there was a
palpable well circumscribed lump arising from the left
thyroid lobe measuring 5 × 3 cm. There was no tracheal
deviation and the lump moved on swallowing. There were
no other palpable lymph nodes and the patient was clini-
cally euthyroid.
His previous medical history of note includes a sub-arach-
noid haemorrhage ten years previously leaving the patient
with residual mild left sided weakness. He was a smoker
of ten cigarettes a day and was previously a heavy drinker.
Thyroid function tests were within normal limits and thy-
roid ultrasound demonstrated a lump that appeared
benign arising from the superior pole of the left lobe of
thyroid gland so the initial diagnosis was an adenoma in
the left lobe of thyroid.
During the operation it was noted that there was a cystic
lump that did not originate from the thyroid gland but
from the lower pharynx extending to the upper oesopha-
gus. The lump was found to be full of necrotic tissue. The
lump and left lobe of the thyroid were excised.
The histopathology demonstrated a normal left thyroid
gland. The 5 cm tumour was well circumscribed with
focally invasive spindle cells (Figure 1) with some areas
that were highly cellular with a fascicular fibrosarcoma-
tous like growth pattern. Elsewhere there was rather loose
fibrous and partly myxoid stroma with abundant mast
cells. In addition immunochemistry including EMA,
CD99 and BCL2 were all positive supporting the diagno-
sis of monophasic synovial sarcoma originating from the
lower pharynx.
Published: 31 March 2009
International Seminars in Surgical Oncology 2009, 6:9 doi:10.1186/1477-7800-6-9
Received: 23 December 2008
Accepted: 31 March 2009
This article is available from: http://www.issoonline.com/content/6/1/9
© 2009 Betal et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
International Seminars in Surgical Oncology 2009, 6:9 http://www.issoonline.com/content/6/1/9Staging CT-scan showed local disease recurrence with
infiltration of the strap muscles and trachea. He was
started on a course of radical radiotherapy. Follow-up CT-
scan revealed multiple pulmonary metastases although
the patient remains clinically stable.
Discussion
Synovial sarcomas [1] are named as it was believed that
they originate from synovial lining cells although there is
little evidence that the anatomical origin is within a joint.
They often present in adolescents or young adults with a
slight predominance of males. Clinically the features may
be non-specific and does not distinguish it from other sar-
comas. They often present as a slow growing well-circum-
scribed mass that mimics benign pathology. In rare
circumstances, patients present with symptoms secondary
to pulmonary metastases.
With regards to histopathology [2], up to two-thirds of
synovial sarcomas often have a biphasic appearance that
consists of a dual line of differentiation of tumour cells.
They are composed of a mixture of elongated basophilic
spindle cells and glandular structures made of columnar
epithelial cells. The spindle cell areas resemble fibrosar-
coma cells with haemagiopericytoma type vascular pat-
tern that often show small areas of calcification or
metaplastic bone. Up to one-third of cases are monopha-
sic in nature that are composed of only spindled cells or
very rarely, epithelial cells. However, lesions composed
solely of spindle cells may be misdiagnosed as fibrosar-
coma or malignant peripheral nerve sheath tumours.
Immunochemistry helps identify these tumours as they
show positive reactions for keratin and epithelial antigen
that differentiate them from other sarcomas. Specific
translocation has helped to classify synovial sarcomas as
they typically show a t(X;18) 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.
The first case reports of synovial sarcoma were reported in
knee joints [3-5] and the first literature review of cases was
published at the beginning of the 1900s [6]. There have
been a few publications relating solely to monophasic
synovial sarcoma and these have mainly been in the lung
[7-11], and fewer reports in the nerves [12,13], gastroin-
testinal tract [14], liver [15], vulva [16,17] and conjunc-
tiva [18,19]. Published cases of synovial sarcoma of the
pharynx are rare [20-22] and there are only two reports of
monophasic synovial sarcoma of the neck [23,24].
The treatment of choice is surgery with or without radio-
therapy and chemotherapy. The survival rate at five years
varies from 25% to 62% and at ten years range from 10%
to 30%. The common areas synovial sarcomas metastasise
to include the lung, skeleton and occasionally regional
lymph nodes.
Studies suggest that the use of adjuvant radiotherapy fol-
lowing surgery has a beneficial effect [25] however neoad-
juvant radiotherapy has yet to show any benefit [26]. The
role of neoadjuvant or adjuvant chemotherapy in primary
synovial sarcoma survival is inconclusive. Early meta-
analysis of 14 randomised controlled trials of doxoru-
bicin-based chemotherapy showed no benefit for overall
survival [27] and the French Sarcoma group showed no
survival benefit with ifosfamide-based neo-adjuvant or
adjuvant chemotherapy, however, radiotherapy signifi-
cantly improved local recurrence-free survival but not dis-
tant recurrence-free survival or overall survival [28]. The
Italian randomized cooperative trial showed that a com-
bination of anthracycline and ifosfamide showed a statis-
tical benefit for extremity and girdle sarcomas at median
follow-up of 59 months [29] however at the conclusion of
the trial after a median follow-up of 89.6 months there
was no statistical benefit [30]. A more recent study of ifos-
famide-based chemotherapy in primary extremity syno-
vial sarcoma showed benefit for disease-specific survival,
distance recurrence-free survival but not for local recur-
rence-free survival [31]. One study specific to head and
neck synovial sarcomas showed higher survival and lower
recurrence rates with patients treated with surgery and
adjuvant radiotherapy than with surgery alone or a com-
bination of surgery, radiotherapy and chemotherapy [32].
Conclusion
Synovial sarcomas are a rare form of soft tissue sarcomas.
This case represents a rare form of monophasic synovial
sarcoma of the pharynx. The gold standard of treatment is
complete surgical excision with adjuvant radiotherapy.
Monophasic spindle cellsFigure 1
Monophasic spindle cells.Page 2 of 3
(page number not for citation purposes)
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Controversy still remains regarding the use of neoadju-
vant and adjuvant chemotherapy however studies have
shown benefits with ifosfamide-based chemotherapy in a
select group of patients.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
DB Literature searches, writing the paper. RB Performed
surgical procedure. VM Performed surgical procedure and
supervised final revision of the article
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Monophasic synovial sarcoma of the pharynx: a case report

Crossref

A case of sarcoma of knee joint.

Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial.

AE: Monophasic synovial sarcoma of the liver. Arch Pathol Lab Med

AG: Monophasic synovial sarcoma arising in the vulva: a case report and review of the literature. Arch Pathol Lab Med

Alberto A: A case of monophasic synovial sarcoma presenting as a vulva mass. Gynecol Oncol

Bulut F: Synovial sarcoma of the pharynx: a case report. Ear Nose Throat J

CI: Synovial sarcoma of the pharynx.

Collaboration: Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: metaanalysis of individual data. The Lancet

DG: Primary monophasic synovial sarcoma of the brachial plexus: report of a case and review of the literature. Clin Neurol Neurosurg

Driman DK: Primary monophasic synovial sarcoma of the colon. Histopathology

EM: Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension. Head Neck

Exstirpation einer sehr grossen, mit dicken Stiele angewachsenen Kneigelenkmaus mit gluklichem Erfolge. Archiv fur Klin Chir

Gherlinzoni F: Ifosfamide in the adjuvant therapy of soft tissue sarcomas. Oncology

HJ: Synovial sarcoma of the pharynx and oesophagus.

Inoue A: Intraneural monophasic synovial sarcoma: a case report. Spine

JY: Neoadjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group. Ann Oncol

Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy.

Luthbert P: Primary monophasic synovial sarcoma of the conjunctiva.

Manabe T: Primary poorly differentiated monophasic synovial sarcoma of the lung. A case report with immunochemical and genetic studies. Pathol Res Pract

Monophasic synovial sarcoma of hypopharynx: case report and review of literature. Acta Otorhinolaryngol Ital

Monophasic synovial sarcoma of the neck in a 8-year-old girl resembling a thyroglossal duct cyst.

Monophasic synovial sarcoma: a case report. Plast Reconstr Surg

Primary monophasic synovial sarcoma of the duodenum with SYT/SSX2 type of translocation. Hum Pathol

Primary monophasic synovial sarcoma presenting as a pulmonary mass: a case report. J Med Case Reports

RC: Primary sarcoma of the lower end of the femur involving the synovial membrane: with a critical review of the literature of synovial sarcoma. Report of one case with complete pathological and radiographic examination. Ann Surg

S: Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg

Salter SC: Primary sarcoma of knee joint.

Soft tissue tumours.

Soule EH, Taylor WF: Synovial sarcoma.

Suehiro S: Metastatic monophasic sarcoma of the pleura. Ann Thorac Cardiovasc Surg

Tazelaar HD: Primary monophasic synovial sarcoma of the pleura: five cases confirmed by the presence of SYT-SSX fusion transcript.

Zagars GK: Synovial sarcoma outcome following conservation surgery and radiotherapy. Radiother Oncol

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Monophasic synovial sarcoma of the pharynx: a case report

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