Introduction: Synovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head & Neck. In the pediatric population, SS is an extremely rare head & neck malignancy.
Presentation of case: We present a case of sixteen years old boy diagnosed with SS situated of the hypopharynx treated by surgical excision and post operative radio-chemotherapy.
Discussion: This anatomical location brings additional functional challenges (swallowing, phonation, respiration), especially in the pediatric population. Pre-operative and even post-operative histopathological diagnosis of SS remains difficult. Optimal treatment of Head & Neck SS has to balance functional and oncologic aspects.
Conclusion: SS is an extremely rare head & neck malignancy in pediatric population. It has multifaceted challenges including pre and post-operative histopathological diagnosis and optimal modality of treatment. Clinical judgment, especially in the pediatric population, needs to balance tumor free margins and organ preservation in head and neck region

Aurélie Bornand

M. Becker

N.H. Alotaibi

Nicolas Dulguerov

Pavel Dulguerov

International Journal of Surgery Case Reports

PubMed

Synovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head &amp; Neck. In the pediatric population, SS is an extremely rare head &amp; neck malignancy

Alotaibi, Naif Hudaik K

Bornand, Aurélie

Dulguerov, Nicolas

Becker, Minerva

Dulguerov, Pavel

Archive ouverte UNIGE

Synovial sarcoma of the hypopharynx in a pediatric patient: Case report

Crossref

AbstractIntroductionSynovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head & Neck. In the pediatric population, SS is an extremely rare head & neck malignancy.Presentation of caseWe present a case of sixteen years old boy diagnosed with SS situated of the hypopharynx treated by surgical excision and post operative radio-chemotherapy.DiscussionThis anatomical location brings additional functional challenges (swallowing, phonation, respiration), especially in the pediatric population. Pre-operative and even post-operative histopathological diagnosis of SS remains difficult. Optimal treatment of Head & Neck SS has to balance functional and oncologic aspects.ConclusionSS is an extremely rare head & neck malignancy in pediatric population. It has multifaceted challenges including pre and post-operative histopathological diagnosis and optimal modality of treatment. Clinical judgment, especially in the pediatric population, needs to balance tumor free margins and organ preservation in head and neck region

Alotaibi, N.H.

Becker, M.

Elsevier - Publisher Connector 

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International Journal of Surgery Case Reports 28 (2016) 1–3
Contents lists available at ScienceDirect
International  Journal  of  Surgery  Case  Reports
journa l h omepage: www.caserepor ts .com
ynovial  sarcoma  of  the  hypopharynx  in  a  pediatric  patient:  Case
eport
.H.  Alotaibia,∗, Aurélie  Bornandb, Nicolas  Dulguerova,  M.  Beckerc, Pavel  Dulguerova
Department of Otorhinolaryngology – Head & Neck Surgery, Geneva University Hospital, 4, rue Gabrielle Perret-Gentil, 1204 Geneva, Switzerland
Department of Clinical Pathology, Geneva University Hospital, 4, rue Gabrielle Perret-Gentil, 1204 Geneva, Switzerland
Department of Radiology, Geneva University Hospital, 4, rue Gabrielle Perret-Gentil, 1204 Geneva, Switzerland
 r  t  i  c  l  e  i  n  f  o
rticle history:
eceived 13 July 2016
eceived in revised form 27 August 2016
ccepted 27 August 2016
vailable online 3 September 2016
eywords:
ynovial sarcoma
ead & neck
a  b  s  t  r  a  c  t
INTRODUCTION:  Synovial  sarcoma  (SS)  is uncommon  high  grade  soft  tissue  sarcoma,  accounting  for  less
than 10%  of all  head  and  neck sarcomas.  Also,  about  10%  of SS occur  within  the  Head  & Neck.  In the
pediatric  population,  SS  is an  extremely  rare  head  &  neck  malignancy.
PRESENTATION  OF  CASE:  We  present  a case  of  sixteen  years  old  boy  diagnosed  with  SS  situated  of  the
hypopharynx  treated  by  surgical  excision  and  post  operative  radio-chemotherapy.
DISCUSSION:  This  anatomical  location  brings  additional  functional  challenges  (swallowing,  phonation,
respiration),  especially  in  the  pediatric  population.  Pre-operative  and  even  post-operative  histopatho-
logical  diagnosis  of SS  remains  difﬁcult.  Optimal  treatment  of  Head  &  Neck  SS  has  to balance  functionalediatric
ase report
and  oncologic  aspects.
CONCLUSION:  SS  is an  extremely  rare  head  &  neck  malignancy  in pediatric  population.  It  has  multifaceted
challenges  including  pre and  post-operative  histopathological  diagnosis  and  optimal  modality  of  treat-
ment. Clinical  judgment,  especially  in  the  pediatric  population,  needs  to  balance  tumor  free  margins  and
organ preservation  in  head  and  neck  region.
 Publi
he  CC©  2016  The  Authors.
access  article  under  t
. Introduction
Synovial sarcoma (SS) is uncommon high grade soft tissue sar-
oma, accounting for less than 10% of all head and neck sarcomas.
lso, about 10% of SS occur within the Head & Neck. In the pediatric
opulation, SS is an extremely rare head & neck malignancy.
. Presentation of case
A sixteen years old boy was referred for a left neck mass and the
emaining clinical history was unremarkable. A ﬁrm 4 × 3 cm left
eck mass, not mobile, medial to the sternocleidomastoid muscle
as appreciated. Flexible endoscopy (Fig. 1) revealed a left sub-
ucosal lateral pharyngeal wall mass impinging on the larynx but
ith normal vocal cords mobility.
The head and neck magnetic resonance imaging studies showed
hat the mass measured 4.8 × 3.3 × 7.0 cm displacing the great ves-
els laterally and pushing the left hypopharynx and larynx medially
Fig. 2).
The patient underwent an excision of the mass thorough a left
ervicotomy. During surgery, the tumor was medial and easily dis-
Abbreviations: SS, synovial sarcoma.
∗ Corresponding author.
E-mail address: nlot@hcuge.ch (N.H. Alotaibi).
ttp://dx.doi.org/10.1016/j.ijscr.2016.08.043
210-2612/© 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing G
reativecommons.org/licenses/by-nc-nd/4.0/).shed  by  Elsevier  Ltd  on  behalf  of  IJS Publishing  Group  Ltd.  This is  an  open
 BY-NC-ND  license  (http://creativecommons.org/licenses/by-nc-nd/4.0/).
sected from the sternocleidomastoid muscle and carotid sheath. It
was covered laterally by a muscular layer and difﬁcult to dissect
from the posterior edge of the thyroid cartilage. Complete resec-
tion resulted in an elongated hypopharyngeal perforation which
was repaired without ﬂap usage. The post-operative course went
uneventful, without cranial nerve deﬁcit, and the patient resumed
oral feeding on day 7. At 8 months follow up, no recurrence was
detected (Fig. 3).
Histology revealed a biphasic Synovial sarcoma(SS), with malig-
nant spindle-cell proliferation (Fig. 4) and focal epithelioid cells,
showing vascular invasion. There were 35 mitoses per 10 high-
power ﬁelds (HPFs) and necrotic foci. The tumor was well-deﬁned
but not encapsulated. Immunohistochemical staining was focally
positive for EMA  (Epithelial Membrane Antigen), pancytokeratins,
CD56 and DOG1 (Discovered on GIST-1), and negative for S100,
smooth muscle actin, desmin, c-Kit (CD117) and CD34. RT-PCR
showed a t(X;18)(p11;q11) translocation, and ﬂuorescence in situ
hybridization (FISH) showed a rearrangement of the SS18 gene
(Fig. 5).
3. Discussion – synovial sarcoma of the hypopharynxSynovial sarcoma (SS) is uncommon high grade soft tissue sar-
coma, supposedly accounting for less than 10% of all head and neck
sarcomas [1]. About 10% of SS occur within the Head & Neck [1],
with a median age of presentation in the third decade1 making SS
roup Ltd. This is an open access article under the CC BY-NC-ND license (http://
CASE  REPORT  –  OPEN  ACCESS
2 N.H. Alotaibi et al. / International Journal of Surgery Case Reports 28 (2016) 1–3
Fig. 1. Endoscopic view of the larynx and pharynx. A lateral normal mucosa covered
mass displaces the larynx.
Fig. 2. The mass parenchyma showed T1 weighted signal isointense to skeletal mus-
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Fig. 3. Gadolinium enhanced T1 weighted image obtained after surgery shows
contrast enhancement along the outer border of the thyroid cartilage in the left
prelaryngeal strap muscles and along the left sternocleidomastoid muscle due to
inﬂammatory postoperative changes. Follow-up MRI  eight months later revealed
gradual decrease of contrast enhancement.
Fig. 4. Malignant spindle-cell proliferation. Hematoxylin-eosin, original magniﬁca-
tion, 20×.le and hyperintense signal on T2 weighted imaging with remarkable enhancement.
1 SPAIR sequence after gadolinium shown.
n extremely rare head and neck malignancy in the pediatric pop-
lation. About half of head and neck SS arise from the soft tissues of
eck, while the remaining half are related to aero-digestive track
tructures [1]. Therefore, a painless neck mass is the most frequent
resenting sign, the remaining symptoms depending on the exact
ocation of the tumor.
Although SS is named because of the histological resemblance
o the synovium, joints are rarely involved and the origin has been
ttributed to pluripotent mesenchymal cells [1]. Histologically two
orms are described: monophasic (containing only spindle cells)
nd biphasic, made off both spindle and epithelioid cells. Spindle
ells are arranged in sheets or fascicles with occasional herring-
one pattern. Nuclear palissading is rare, unlike leiomyosarcoma
nd malignant peripheral nerve sheath tumors. Epithelial cells form
landular structure or are arranged in solid nests or cords. There can
e some poorly differentiated areas showing an epithelioid pattern,
 small-cell pattern and/or a high-grade spindle-cell pattern. Many
S focally show a hemangiopericytic (vascular) pattern [2–4].
Fig. 5. Break-apart FISH. The red and green signals normally ﬂank the SS18 gene;
splitting of these signals indicates a rearrangement (arrow). Magniﬁcation 10×.
 –  O
rnal of
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N.H. Alotaibi et al. / International Jou
The differential diagnosis depends on the type of SS. A correct
nitial histopathologic diagnosis of monophasic SS is often difﬁ-
ult: the differential diagnosis including other spindle-cell tumors
uch as malignant peripheral nerve sheath tumors, leiomyosar-
oma, rhabdomyosarcoma, ﬁbrosarcoma, solitary ﬁbrous tumor,
nd spindle cell carcinoma. Biphasic SS is easier to identify, with
alignant peripheral nerve sheath tumors, carcinosarcoma, and
alignant mesothelioma being the differential diagnosis [3,4].
Immunohistochemistry is helpful, with most SS showing
ocal immunoreactivity for EMA  and cytokeratins, contrary to
alignant peripheral nerve sheath tumors, leiomyosarcoma and
habdomyosarcoma [2–4]. SS may  be focally positive for CD56 (up
o 80%) [4], S100 (up to 40%) [2,4], and DOG1 (15%) [5]. CD34 is
enerally negative (<5%), in contrast to solitary ﬁbrous tumor [2,4].
eiomyosarcoma is positive for desmin and smooth muscle actin,
hile rhabdomyosarcoma is positive for desmin and myogenin [3].
Most (90%) SS exhibit a translocation t(X;18)(p11:q11) between
hromosomes 18 (SYT gene) and X (SSX gene), resulting in a
YT-SSX-1 (biphasic SS) or SYT-SSX-2 (monophasic SS) fusion [6].
enerally we use break-apart FISH and RT-PCR to search the
ranslocation. This translocation is speciﬁc and diagnostic for SS
2–4].
Preoperative diagnosis of SS is challenging because of its non-
peciﬁc symptomatology, low clinical morbidity, lack of speciﬁc
adiologic features, and physicians’ unfamiliarity. The most fre-
uent preoperative diagnosis is neurogenic tumors and a recent
tudy of peripheral SS suggests that pain, short symptom duration,
eritumoral edema without a deﬁnite target sign on MR images
hould evoke SS [7].
Prognosis of synovial sarcoma has been related to tumor size
1,8], biphasic histology, [1,6,8] SSY-SSX2 gene fusion, and nega-
ive resection margins [1]. Since our preoperative diagnosis was a
chwannoma, the resection was less radical than possible and the
argins were focally positive, a frequent ﬁnding with SS. Others
ave performed extensive resections with total laryngopharyngec-
omy for margin clearance in similar case [9]. Clinical judgment,
specially in the pediatric population, needs to balance tumor free
argins and organ preservation. Besides surgery, radiation seems
o confer a local control and survival beneﬁt, while the role of
hemotherapy is controversial [1].
. Conclusion
SS is an extremely rare head & neck malignancy in pediatric
opulation. It has multifaceted challenges including pre and post-
perative histopathological diagnosis and optimal modality of
reatment. Clinical judgment, especially in the pediatric popula-
ion, needs to balance tumor free margins and organ preservation
n head and neck region.
onﬂicts of interest
The authors have no conﬂict of interest to declare.unding
None.
pen Access
his article is published Open Access at sciencedirect.com. It is distrib
ermits unrestricted non commercial use, distribution, and reproduct
redited.PEN  ACCESS
 Surgery Case Reports 28 (2016) 1–3 3
Ethical approval
Approval to publish case report is waived by the institution.
Consent
Written informed consent was obtained from the patient for
publication of this case report and accompanying images. A copy
of the written consent is available for review by the Editor-in-Chief
of this journal on request.
Author contribution
Naif Alotaibi. H, MD:  make substantial contributions to concep-
tion and design, acquisition of data,analysis and interpretation of
data.
Aurélie Bornand, MD:  participate in drafting the article and
revising it critically for important intellectual content.
Dulguerov Nicolas, MD:  participate in drafting the article and
revising it critically for important intellectual content.
Becker M,  MD:  participate in drafting the article and revising it
critically for important intellectual content.
Pavel Dulguerov MD:  give ﬁnal approval of the version to be
submitted and any revised version.
Guarantor
Pavel Dulguerov, MD.
Acknowledgements
Not applicable.
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Synovial sarcoma of the hypopharynx in a pediatric patient: Case report 

Synovial sarcoma of the hypopharynx in a pediatric patient: Case report

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