UNUSUAL MANIFESTATION OF EXTRAOSSEOUS
EWING SARCOMA: REPORT OF 3 CASES
Ioannidou M.1,*, Tsotridou E.1, Samoladas E.2, Tragiannidis A.1, Kouskouras K.3,
Sfougaris D.4, Spyridakis I.5, Foroulis C.6, Galli-Tsinopoulou A.1, Hatzipantelis E.1
*Corresponding Author: MD Maria Ioannidou, St Kiriakidi 1, Children’s and Adolescent’s Hematology-
Oncology Unit of 2nd Department of Pediatrics, AUTh, AHEPA University General Hospital,
Thessaloniki, Greece. Zip code: 54621. Tel.: 00306942067923. E-mail: ioannidou@auth.gr
page: 77
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DISCUSSION
EES was first described in 1969. It is a rare, malignant
mesenchymal tumor similar to its intraosseous counterpart
(14). Genetic studies have demonstrated reciprocal translocation
of t(11;22)(q24;q12) in about 95% of patients,
with a majority of the remainder demonstrating t(21;22)
(q22;q12) (15, 16).
Patients with EES often observe a rapidly growing soft
tissue mass, with about 1/3 being painful due to compression
of adjacent structures (1, 2, 17). They often occur in the
second decade of life with some studies reporting a mean
age of 15 years and with a mild predominance for males
and Caucasians. There is no evidence that familiar or environmental
factors influence the incidence of the disease (10,
11, 18). According to these findings, the main symptoms of
our patients included pain and swelling, while the presence
of other symptoms depended on the location of the tumor.
Our findings demonstrate the variability in primary
tumor sites. There are increasing reports of EES from
diverse sites whose origin has been attributed to ectopic
neural and neuroectodermal proliferations. Although the
paravertebral spaces, lower extremities, head and neck and
pelvis represent the most commonly affected sites, rare
locations of EES include the omentum (19, 20), kidney
(21), heart (22), uterus (23), lung (24, 25), chest wall (26)
and gastrointestinal track (27–29).
It is also noteworthy that EESs present metastases at
the time of diagnosis at a rate ranging from 10% to 25%
(11), depending on the study. This is a fact that complicates
the prognosis of the disease. In our study, one of
the patients was diagnosed with possible lung metastasis.
Localized tumors appear to have a survival rate of 75%,
while metastatic disease has an unfavorable prognosis (30).
The National Comprehensive Cancer Network
(NCCN) has published guidelines for the treatment of
bone cancers including ES, and the authors suggest that
any member of the Ewing tumor family can be treated
according to the same algorithm. The recommended treatment
is local therapy (surgery and/or radiotherapy) plus
chemotherapy (31, 32). The gold standard of treatment
for localized disease remains surgery, although the disease-
free survival rate in patients with sarcoma without
margin-negative surgery is worse (8, 9, 11). All 3 patients
in our report had first-line surgery with negative margins.
Post-surgery, the most appropriate course of chemotherapy
was planned. Regarding the use of chemotherapy, it plays a
critical role in the treatment of EES. Chemotherapy may be
provided after surgery to improve overall survival rates and
reduce the chance of the tumor recurrence (33). The third
approach to combating Ewing Tumors is the addition of radiotherapy
to the treatment plan. These malignancies have
been proven to be radiosensitive, however radiation hazards
in children and young adolescents are always present
and require careful consideration. Surgery also provides
better results in local control and survival. Indications for
post-operative radiotherapy are non-radical or marginal
resections and poor histological response (13,31). The
patients described were treated according to the EUROE.
W.I.N.G 99 chemotherapy protocol, without the need
for radiotherapy. A prevalent prognostic factor that is of
great interest in multiple studies is the surgical resection of
the tumor and the surgical margins. A complete resection
with surgical margins clear of any malignancy constitutes
a positive prognostic factor (8,9,11). All of our patients
had complete resection and negative surgical margins in
accordance with the above data and all are alive.
The improved understanding of EES molecular biology,
made possible by the recent advances in the field
of systems biology, has opened new perspectives for the treatment of this aggressive malignancy through the implementation
of precision medicine. Insulin-like growth
factor-1- receptor (IGF -1R), CD99 and the EWS-FLI-1
fusion product play key roles in malignant transformation
by inducing cell growth and inhibiting apoptosis and have
emerged as promising therapeutic targets (2, 34).
In conclusion, EES belong to a very rare and aggressive
tumor family. Despite the limitations of having a small
number of patients and the rarity of such tumors, the point
is that early recognition and treatment reduce morbidity
and mortality. Continuous research is needed to further
improve our understanding of the disease and to adjust the
treatment protocols in the hope of ameliorating prognosis.
Declaration of interest
The authors report no conflicts of interest. The authors
alone are responsible for the content and writing of
this article.
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