A 35-year-old female presented with pain and swelling of the distal left radius. A diagnosis of Ewing sarcoma was made and she underwent neoadjuvant chemotherapy and surgery. Macroscopic viable areas remained on the map of the surgical specimen; as such, she was classified as a poor responder and received high dose adjuvant chemotherapy. She remained disease-free for five years, until age 40. A vaginal polyp was then detected during a routine gynaecologic examination. It was removed and histopathology revealed metastatic Ewing sarcoma

A Zoubek

F Larbre

G Bacci

K Gündüz

L Astudillo

ME Mulligan

P Picci

RB Raney

RC Gaba

S Ferrari

WG McCluggage

English

PubMed

Noemie Vanel

Victoire Vierling

Jennifer Kreshak

Marco Gambarotti

Stefania Cocchi

Cristina Tranfaglia

Daniel Vanel

Crossref

Clinical Sarcoma Research

Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor

Springer - Publisher Connector

Vaginal metastasis of a ewing sarcoma five years after resection of the primary tumor

CASE REPORT Open Access
Vaginal metastasis of a Ewing sarcoma five years
after resection of the primary tumor
Noemie Vanel1, Victoire Vierling1, Jennifer Kreshak1, Marco Gambarotti1, Stefania Cocchi1, Cristina Tranfaglia2 and
Daniel Vanel1*
Abstract
A 35-year-old female presented with pain and swelling of the distal left radius. A diagnosis of Ewing sarcoma was
made and she underwent neoadjuvant chemotherapy and surgery. Macroscopic viable areas remained on the map
of the surgical specimen; as such, she was classified as a poor responder and received high dose adjuvant
chemotherapy. She remained disease-free for five years, until age 40. A vaginal polyp was then detected during a
routine gynaecologic examination. It was removed and histopathology revealed metastatic Ewing sarcoma.
To our knowledge, this is the first reported case of a vaginal metastasis of Ewing sarcoma.
Keywords: Ewing sarcoma, vaginal metastasis
Introduction
Ewing sarcoma (ES) is a small blue round cell tumor
belonging to the Ewing Family Tumour (EFT) together
with Primary Neuroectodermal Tumour (PNET) and
ASKIN tumor (of the thoracic wall). Eight hundred and
ninety six cases have been reported in our institute
since 1982. Ewing sarcoma has a distinct predilection
for males and occurs in the first two decades of life in
more than 75 percent of cases [1,2].
Metastases are frequent [3] and are mostly pulmonary
and osseous, but can be found in various other
locations.
We present here the first description of a vaginal
metastasis of Ewing sarcoma.
Case Report
A 35 year-old woman with no significant medical his-
tory presented with pain and swelling of the left wrist
over the past year. A radiograph and computed tomo-
graphy scan revealed a lytic lesion of the distal left
radius (Figure 1), with soft tissue extension on MR
examination (Figure 2, 3).
A biopsy was performed and histological examination
revealed a typical Ewing sarcoma. The diagnosis was
confirmed by FISH analysis, which demonstrated the
translocation t (11, 22).
Staging revealed a solitary non-specific pulmonary
nodule of the inferior right lobe that did not change
with time and was not considered metastatic.
The patient underwent neoadjuvant chemotherapy and
resection and allograft of the distal radius. She was con-
sidered as poor responder as macroscopic areas
remained on the surgical specimen, but all margins were
free of disease. High dose chemotherapy was then
performed.
After completion of her treatment, it was followed up
as per protocol and remained disease-free.
Five years later, during a routine gynaecologic exam, a
vaginal polyp was found and removed. Histology
revealed a ES metastasis (Figure 4), as confirmed by the
characteristic translocation t(11, 22).(Figure 5). The rest
of the evaluation (CT and bone scintigraphy) was
normal.
No treatment has been undertaken.
Discussion
Ewing sarcoma (ES) represents approximately ten per-
cent[1] of primary malignant bone tumours and one
percent of soft tissue tumours. It tends to arise in the
diaphysis or metaphyseal-diaphyseal portion of long
bones, although any bone may be involved.
Frequently, the first symptoms are pain and swelling.
* Correspondence: daniel.vanel@ior.it
1Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10,
40106, Bologna, Italy
Full list of author information is available at the end of the article
Vanel et al. Clinical Sarcoma Research 2011, 1:9
http://www.clinicalsarcomaresearch.com/content/1/1/9 CLINICAL SARCOMA RESEARCH
© 2011 Vanel 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.
Twenty percent of ES at diagnosis have radiographic
evidence of metastasis. Lungs and bone are the main
metastatic locations.
Radiologically, an aggressive osteolytic lesion is com-
monly observed.
ES is characterized by a morphologically uniform
round cell proliferation with round nuclei containing
fine chromatin.
CD99 is expressed in nearly all ES and thereby is a
highly sensitive immunohistochemical marker. Several studies have confirmed a characteristic 11, 22
(q24, q12) chromosomal translocation in 85 percent of
the cases [3]; the translocation t (21, 22) and three even
rarer translocations (t (7, 22), t(2, 22) t(17, 22)) have
also been found.
Necrosis has a strong prognostic value [4]. High dose
chemotherapy is used in poor responders [5].
Figure 1 Initial evaluation. CT: lytic lesion with partial cortical
destruction.
Figure 2 Axial T1W MR image after contrast medium injection:
the soft tissue extension is well studied.
Figure 3 Sagital T1W MR image, after contrast medium
injection. Medullary and soft tissue extensions are well evaluated.
Figure 4 The metastasis is made of homogeneous small round
cells.
Vanel et al. Clinical Sarcoma Research 2011, 1:9
http://www.clinicalsarcomaresearch.com/content/1/1/9
Page 2 of 3
We found 17 cases of primary ES involving the vagina
and/or vulva in the literature[6]. A few cases of primary
neuro ectodermal tumors (PNET) in the pelvis have also
been reported [7].
Unusual metastasic locations have been described, for
example, the breast [8], myocardial muscle [9], paranasal
sinuses [10], iris [11]), and pancreas [12]. That explains
why, even if a second primary cannot be completely
excluded in our case, the probability of a metastasis is
much higher. To our knowledge, this is the first case
ever reported of a vaginal Ewing metastasis.
Conclusion
This case exemplifies the idea that every new lesion in a
patient with Ewing sarcoma should be considered as a
possible metastasis.
Author details
1Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10,
40106, Bologna, Italy. 2Departement of Nuclear Medicine, San Orsola
Hospital, Via Giuseppe Massarenti, 940138 Bologna, Italy.
Authors’ contributions
NV wrote the article, VV checked the case, JK corrected the writing (English
and scientific content) MG checked and selected the histology, SC checked
and selected the FISH, CT checked the scientific content, DV proposed the
subject and directed the article. All authors read and approved the final
manuscript
Competing interests
The authors declare that they have no competing interests.
Received: 13 April 2011 Accepted: 1 August 2011
Published: 1 August 2011
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doi:10.1186/2045-3329-1-9
Cite this article as: Vanel et al.: Vaginal metastasis of a Ewing sarcoma
five years after resection of the primary tumor. Clinical Sarcoma Research
2011 1:9.
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Figure 5 Interphase FISH with the EWSR1 (22q12) break-apart
probe. Within a single nucleus fused red/green signals mark one
intact 22q12 region, whereas split red/green signals indicate the
presence of an EWSR1 gene rearrangement.
Vanel et al. Clinical Sarcoma Research 2011, 1:9
http://www.clinicalsarcomaresearch.com/content/1/1/9
Page 3 of 3


Acute cardiac failure caused by myocardial metastasis of an unrecognised Ewing sarcoma. Pediatrie

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C: Ewing family of tumours involving the vulva and vagina: report of a series of four cases.

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HM: Ewing’s sarcoma of soft tissue in childhood: a report of the Intergroup Rhabdomyosarcoma study,1972 to 1991. J Clin Oncol

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MJ: Ewing sarcoma metastatic to the iris.

Pancreatic metastasis from Ewing’s sarcoma. Clin Imaging

Smeland S: Nonmetastatic Ewing family tumors: high-dose chemotherapy with stem cell rescue in poor responder patients. Results of the Italian Sarcoma Group/Scandinavian Sarcoma Group III protocol. Ann Oncol

Tumors.

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Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor

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