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Ewing’s sarcoma of the sinonasal tract with orbital extension: a rare case report and literature review

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Abstract

Ewing’s sarcoma is a rare and aggressive tumor which classified as peripheral primitive neuroectodermal tumor. It commonly arises in the long bones of the extremities and rarely in the head and neck region. Localization of the sinonasal tract is a rare occurrence thus the number of clinical studies published in the literature are limited. The diagnosis of this tumor requires a histopathological examination, immunohistochemistry and cytogenetic analysis. Ewing’s sarcomas are characterized by a CD99 positivity in immunohistochemistry stain and a t(11:22)(q24:q12) translocation in cytogenetic study. The treatment of choice is the multimodality treatment including surgery, radiotherapy and chemotherapy. This is a case report of sinonasal and orbital Ewing’s sarcoma in a 24-year-old male patient who presented with a history of right nasal obstruction, right eye pain and periorbital edema.
Journal of Surgical Case Reports, 2019;3, 13
doi: 10.1093/jscr/rjy362
Case Report
CASE REPORT
Ewings sarcoma of the sinonasal tract with orbital
extension: a rare case report and literature review
Omair Al Hussain
1
, Ahmed Aldandan
2,
*, Abdulrahman Alkhatib
3
,
Ghaleb Alazzeh
3
, and Ali Almomen
3
1
Head of ENT Department, College of Medicine, Immam Mohammad Ibn Saud Islamic University, Riyadh,
Saudi Arabia,
2
Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia, and
3
Department of ENT,
King Fahad Specialist Hospital, Dammam, Ministry of Health, Saudi Arabia
*Correspondence address. Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia. Tel: +966-54-364-9777; E-mail: drdandan94@gmail.com
Abstract
Ewings sarcoma is a rare and aggressive tumor which classied as peripheral primitive neuroectodermal tumor. It commonly
arises in the long bones of the extremities and rarely in the head and neck region. Localization of the sinonasal tract is a rare occur-
rence thus the number of clinical studies published in the literature are limited. The diagnosis of this tumor requires a histopatho-
logical examination, immunohistochemistry and cytogenetic analysis. Ewings sarcomas are characterized by a CD99 positivity in
immunohistochemistry stain and a t(11:22)(q24:q12) translocation in cytogenetic study. The treatment of choice is the multimodal-
ity treatment including surgery, radiotherapy and chemotherapy. This is a case report of sinonasal and orbital Ewingssarcomain
a 24-year-old male patient who presented with a history of right nasal obstruction, right eyepainandperiorbitaledema.
INTRODUCTION
Ewings sarcoma is a rare and aggressive tumor which classi-
ed as peripheral primitive neuroectodermal tumor [1]. It was
rst described by James Ewing, an American pathologist, in
1921 [2]. Ewings sarcoma commonly occurs in early childhood
and adolescence, but rarely in adulthood [3]. Most cases arise in
the long bones of the extremities [1,2,4]. Primary Ewings sar-
coma of the head and neck is uncommon, hence it accounts for
only 14% of all Ewings sarcomas [1,4,5]. Furthermore, sinona-
sal tract involvement is very rare and only few reported cases
have been published in world literature [2,4].
CASE REPORT
A 24-year-old male patient who presented with a history of
right nasal obstruction, right eye pain, lower eyelid swelling
and orbital swelling. Endoscopic examination revealed an
obstructive mass occupying the right nasal cavity (Fig. 1).
Cranial nerves were intact. The result of hematological and bio-
chemical investigations were within normal limits.
On radiological evaluation, CT scan with contrast of the
paranasal sinuses (PNS) revealed a mass involving the right
ethmoid sinus with medial wall and orbital oor extension
(Fig. 2). The subsequent magnetic resonance imaging (MRI)
revealed an inltrative soft tissue mass occupying the right
ethmoid sinus, eroding inferio-medial orbital wall and extend-
ing to the extracoanal space (Fig. 3). Positron emission tomog-
raphy (PET) scan demonstrated an ill-dened 4.5 ×4.2 cm
2
mass lesion in the right nasal cavity and ethmoid sinus extend-
ing to the right medial orbital oor (Fig. 4). The scan did not
reveal any associated lymphadenopathies.
Histological analysis of a biopsy from the mass revealed a
small rounded blue cell tumor suggestive of Ewingssarcoma/
primitive neuroectodermal tumor. Immunohistochemistry
Received: November 14, 2018. Accepted: March 13, 2019
Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019.
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1
showed the neoplastic cells are positive for CD99, S-100 pro-
tein and vimintin. However, neuron specic enolase (NSE),
smooth muscle actin (SMA), desmin, myogenin, CD45, synap-
tophysin and pan-cytokeratin were all negative. KI-67 index
was 3040%.
Subsequently, molecular study using uorescence in situ
hybridization (FISH) had shown rearrangement of EWSR1 gene in
100% of the analyzes nuclei that conrm the diagnosis of Ewings
sarcoma. The patient underwent endoscopic excision of the tumor
followed by chemotherapy and radiotherapy treatment. MRI 6-
months post treatment showed complete resolution of the disease
(Fig. 5). The patient remains symptom-free during 2 years follow-
upandmaintainsaverygoodqualityoflife.
DISCUSSION
Ewings sarcoma is an aggressive tumor that commonly arises
in the long bones or pelvis and less frequently in the soft tissue
Figure 2: Contrast enhanced computed tomography scan. Coronal CT scan with
contrast of PNS showing an enhancing soft tissue lesion in the right ethmoid,
eroding the medial and inferior orbital walls.
Figure 3: Magnetic resonance imaging (MRI) showing an enhancing soft tissue
mass in the ethmoid sinus, eroding inferio-medial orbital wall.
Figure 4: Positron emission tomography scan. PET scan showing an ill-dened
4.5 ×4.2 cm
2
mass lesion in the right nasal cavity, maxillary and ethmoid
sinuses extending to the right medial orbital oor.
Figure 5: Magnetic resonance imaging after treatment. MRI post treatment
showing total resolution of the tumor.
Figure 1: Nasal endoscopy image showing a soft tissue mass arising from the
right lateral nasal wall.
2
|
O.A. Hussain et al.
of the trunk and extremities [1,2]. It is a rare disease compris-
ing 46% of all primary bone tumors [1,5,6]. Ewings sarcoma
of the head and neck is uncommon, accounting for 14% of all
cases [1,4,5]. Moreover, Ewings sarcomas in the nasal cavity
and PNS are extremely rare [1,5]. Pediatrics and adolescents
are more susceptible to the disease [3]. About 80% of patients
are younger than 20 years of age with the highest incidence in
the second decade of life [2,3,6]. Ewings sarcoma is more com-
mon in white populations, and have a slight male predomin-
ance [4,5].
Clinically, majority of the patients are presented with enlar-
ging mass, nasal obstruction, rhinorrhea and epistaxis [4,7].
Other symptoms and signs are due to mass effect of the tumor
[4,7]. In particular, patients with periorbital extension may pre-
sent with proptosis, periorbital edema and decrease visual acu-
ity [7]. Approximately 1530% of the patients are presented
with metastasis at time of diagnosis [7]. The most common
sites of distant metastasis are the lungs and bones [7].
Microscopically, Ewings sarcomas are composed of uniform
small round cells with round nuclei containing ne chromatin,
scanty clear or eosinophilic cytoplasm and PASpositive intra-
cytoplasmic glycogen granules [2,7,8].
Diagnosing sinonasal Ewings sarcoma is extremely challen-
ging as several small round neoplasms must be excluded [1,2,9].
The differential diagnosis is widely broad including rhabdomyo-
sarcoma, lymphoma, undifferentiated carcinoma, melanoma and
olfactory neuroblastoma [2,6]. The diagnosis of Ewingssarcoma
can be established after a careful evaluation using radiological
study, histopathological examination, immunohistochemistry,
and cytogenetic analysis [2,9]. The immunohistochemical prole
usually shows CD99 and Fli-1 positivity [2,8]. In 2011, Hafezi
reviewed 14 cases of sinonasal Ewings sarcoma [6]. All cases
were positive for CD99 in the immunohistochemistry staining [6].
In the present case, immunohistochemistry revealed positive
staining for CD99, S-100 protein and vimintin. A denitive diagno-
sis of Ewings sarcoma can be achieved through molecular ana-
lysisthatdetectEWSR1/FLI-1fusion[
1]. This fusion causes a
specic t(11:22)(q24:q12) chromosomal translocation [1].
The prognosis depends on the site of the primary tumor, the
presence of distant metastasis at presentation, and the age of
the patient [2,3]. Researchers have found that patients younger
than 15 years old and patients with axial and sinonasal tract
diseases have a better prognosis [2,3]. Furthermore, a good
prognosis can be expected if the disease has not metastasized
[24]. Disseminated disease at time of presentation is asso-
ciated with 22% 5-year survival compared to 55% in patients
without metastases [24]. However, the survival rate in patients
without metastatic disease has improved to 86% [6,10]. This
increase in survival rate could be explained by the recent
advances in the treatment [6,10].
The treatment of choice in Ewings sarcoma is the multi-
modality treatment that includes surgery, radiotherapy and
chemotherapy [2]. In our case, based on the histological diagno-
sis, immunohistochemistry and cytogenetics conrmation, the
patient was treated with surgery followed by chemotherapy
and radiotherapy. The patient remains symptom-free and
maintains a very good quality of life after 2 years follow-up.
CONCLUSION
Ewings sarcoma originating from the sinonasal tract is very
rare. Although the diagnosis of the disease is challenging, it is
feasible using histopathological examination, immunohisto-
chemical study and cytogenetic analysis. Treatment includes a
multidisciplinary approach with surgery followed by chemo-
therapy and radiotherapy.
CONFLICT OF INTEREST STATEMENT
The authors declare that they have no conict of interest.
Informed consent was obtained from all individual participants
included in the study.
FUNDING
No funding obtained.
REFERENCES
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Ewings sarcoma of the sinonasal tract with orbital extension
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... In particular, ONB can be differentiated from intracranial immature teratoma for the absence of some increased substances including AFP, b-HCG, and PLAP in serum and cerebrospinal fluid [14]. The diagnosis of ONB can be established after a careful evaluation of radiological study, histopathological examination, immunohistochemistry, and cytogenetic analysis [11]. ...
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