Open Journal of Clinical Diagnostics, 2011, 1, 1-4 OJCD
doi:10.4236/ojcd.2011.11001 Published Online June 2011 (http://www.SciRP.org/journal/OJCD/).
Published Online June 2011 in SciRes. http://www.scirp.org/journal/OJCD
Epithelioid sarcoma of the spine: a case report and review
Jeong Hyun Yoo1*, Sun Hee Sung2
1Department of Radiology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Korea;
2Department of Pathology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Korea.
Received 20 April 2011; revised 24 May 2011; accepted 8 June 2011.
ABSTRACT
Epithelioid sarcoma is a rare malignant soft tissue
neoplasm commonly involving extremities of young
adults and rarely occurring in the trunk area. It re-
sembles a chronic inflammatory process and mimics
benign reactive granuloma or other benign diseases.
Despite its indolence and slow growth, the prognosis
is poor with high recurrence and early lymph node
spreading and hematogeneous distant metastasis.
Involvement of the spine is extremely rare and diffi-
cult to diagnose correctly in clinical presentation and
pathology. We describe our experience a case of epi-
thelioid sarcoma involving lumbar spine. A pre-ope-
rative spine MRI showed a large solid and necrotic
paraspinal mass which ex tended into the spinal canal
with cord compression, and bone destruction. Final
correct diagnosis was confirmed by immunohisto-
chemical studies. When young adults present with a
spinal lesion that has unusual large necrotic soft tis-
sue mass, immunohistochemical studies are recom-
mended for promptly determining the possibility of
spinal epithelioid sarcoma.
Keywords: Sarcoma; Epithelioid: Spine;
Neoplasm: MR Image
1. INTRODUCTION
Epithelioid sarcoma is a rare malignant soft tissue neo-
plasm which occurs most commonly in the extremities in
young adults [1 -5]. The diagnosis of ep ithelioid sarcoma
has been considered difficult both for clinicians and pa-
thologists because of its relative indolence and slow
growth, as well as resemblance to other benign tumors
[2-4]. Despite its slow growth, the prognosis of epi-
thelioid sarcoma is poor because it has extreme aggres-
siveness and has a high tendency for local recurrence
and lymphatic or hematogeneous metastasis [1-3,5-8].
Spinal epithelioid sarcoma is extremely rare [9,10] and
is very difficult to diagnose correctly [2-4,8-10]. We
report a case of epithelioid sarcoma of the lumbar spine
with review of literature.
2. CASE PRESENTATION
A 29-year-old man presented with left leg weakness
which started 5 months earlier. He underwent curettage
and posterior fixation of the L1-3 vertebra in a different
hospital in response to a misdiagnosis of spondylitis.
Initial pathologic diagnosis was inflammatory reaction
with fibrosis (not shown). Symptoms did not improve
after original treatment. Paralysis and hypoesthesia of
the left lower extremity progressed, and a wound infec-
tion on his back worsened. The fixated devices were
removed with debridement and a tissue biopsy was ob-
tained for the correct diagnosis.
An initial lumbar spin e MRI performed at that outside
hospital revealed large soft tissue mass involving left L2
vertebral body and paralumbar muscle planes. The mass
showed iso- or subtle low signal intensity on the
T1-weighted image and heterogeneous slightly high or
bright signal intensity on the T2-weighted image. Con-
trast scan showed diffuse enhancement of the mass with
large necrotic non-enhancing portion. The lesion ex-
tended into the sp inal canal, compressing th e spinal cord
on the left. Destruction of the lumbar vertebra including
left pedicle and transverse process were also present
(Figure 1).
The histopathology showed plump and epithelioid
appearance of tumor cells, arranged in a granuloma-like
fashion around areas of necrosis (X400). On the immu-
nohistochemical stain, the tumor cells showed diffuse
strong positive reaction in EMA, as well as in cy-
tokeratin and vimentin. They showed focal positive re-
action on CD34. They also showed focal, weakly posi-
tive reaction to a smooth muscle actin. Desmin, s-100
protein, CD31, and HMB-45 were all negative. These
findings were consistent with epithelioid sarcoma with
marked necrosis (Figure 2).
Radical en-block resection of L2 vertebra and anterior
interbody fusion of L1-3 were performed with allogene-

J. H. Yoo et al. / Open Journal of Clinical Diagnostics 1 (2011) 1-4 2
Figure 1. Pre-operative initial MR imaging of lumbar spine. T1-, T2-weighted, and gadolinium enhanced axial (a-c) and sagittal (d-f)
scan, and T2-weighted coronal sequential images (g, h). Spine MR images show large soft tissue mass involving left L2 vertebral
body and paralumbar muscle planes. The signal intensities of the mass reveal iso- or subtle low signal intensity on the T1-weighted
image and heterogeneous slightly high or bright signal intensity on the T2-weighted image. Contrast scans show diffuse enhancement
of the mass with large necrotic non-enhancing portion. The lesions extend into the spinal canal compressing spinal cord on the left.
Destruction of the lumbar vertebra including left pedicle and transverse process is also noted.
ous bone graft. Radiation and systemic chemotherapy
were started. However, the patient’s symptoms persisted
and follow-up MR showed rapid progression of the dis-
ease, with the canal obstruction (not shown). A month
later, the patient developed cough and d yspn ea. Chest PA
and high resolution chest CT showed multifocal he-
matogeneous metastasis in both lung fields with substan-
tial pleural effusion (not shown). The patient also com-
plained of a left axillary mass, which was consistent with
metastasis in the aspiration cytology. The patient refused
continued treatment and was discharged against medical
advice on patient re q uest .
3. DISCUSSION
Spinal involvement of the epithelioid sarcoma is very
rare. [9,10]. To our knowledge, only two cases of spinal
epithelioid sarcoma have been reported. The first report
was described in S1 vertebr al body an d was mistaken for
inflammatory disease in clinical and pathologic diagno-
sis. However, in that case only post-operative image was
available without pre-operative radiologic findings. The
second case was one in the thoracic spine which showed
paraspinal soft tissue mass with spinal canal extension
and cord compression, similar to our case excep t without
osseous involvement.
Based on the radiologic findings, large soft tissue
mass with necrosis or hemorrhage is characteristic of
epithelioid sarcoma. MR signal intensity of the epi-
thelioid sarcoma is mostly isointensity to muscle on
T1-weighted image and hyperintensity on T2-weighted
image with strong enhancement, Bone invasion is a very
rare manifestation of epithelioid sarcoma [3,7,8]. If pre-
sent, it usually consists of localized demineralization or
cortical thinning rather than destruction [1,7,11].
Although the second reported thoracic spine case
showed paraspinal soft tissue mass with spinal canal
extension, it did not show definite tumoral necrosis and
bone involvement. MR of our case revealed characteris-
tic large soft tissue mass with necrotic change, although
this is very rare in the spine area. Furthermore, our case

J. H. Yoo et al. / Open Journal of Clinical Diagnostics 1 (2011) 1-4 3
(a) (b)
(c) (d)
Figure 2. Pathologic findings. Tumor cells were arranged in a granuloma-like fashion around area of necrosis. The tumor cells
showed plump and eosinophilic epithelioid appearance with nuclear atypical, vesicular chromatin, and numerous mitosis (X400) (a).
On the immunohistochemical stain, the tumor cells revealed diffuse strong positive reaction in EMA (b) and cytokeratin (c). CD34
showed focal cytoplasmic positive reaction (d).
also showed accompanying bone destruction, not just
demineralization, which is not a usual finding of epi-
thelioid sarcoma (Figure 1).
Epithelioid sarcoma is a malignant tumor with an al-
ready poor prognosis, aggravated by the commonly de-
layed diagnosis and resultin g inadequate treatment [2,3].
Therefore it is absolutely necessary to carry out immu-
nohistochemical studies for the correct diagnosis. Typi-
cal for the epithelioid sarcoma is immunohistochemical
positive for epithelial membrane antigen (EMA), cy-
tokeratin, and vimentin but is negative for carcinoem-
bryonic antigen and desmin [1-4]. Our case also revealed
typical positive reaction in EMA, cytokeratin, and vi-
mentin, although the initial pathologic diagnosis was
missed.
Even with the initial treatment of choice, radical exci-
sion of the primary lesion, there is a high rate of local
recurrence and lymphatic and pulmonary metastasis oc-
currence [1,4,6,8]. Many reports identify various factors
about the progno sis; 1) the size and dep th of the tumor, 2)
the number of mitotic figures, and 3) the presence of
hemorrhage, necrosis, and 4) vascular invasion [1,3,4,6,
8]. Three histology variables with vascular invasion,
tumor size of more than 5 cm, and more than 30% ne-
crosis were reported as statistically significant predicto rs
of a poor outcome [3]. According to these factors, the
prognosis of our case was considered to be poor because
of size greater than 5cm and more than 30% necrosis on
MR imaging and numerous mitosis and atypia in pa-
thology. Unfortunately, this patient subsequently fol-
lowed lymph node and rapid pulmonary metastasis in
spite of en-bloc wide resection an d adjuvant chemo- and
radiotherapy.
4. CONCLUSIONS
Spinal epithelioid sarcoma is extremely rare and difficult
to diagnose correctly. It also differs from other sarcoma
subtypes because of its relative common recurrence and
lymphovascular metastasis. When a spinal lesion has
large necrotic paraspinal soft tissue mass with extension

J. H. Yoo et al. / Open Journal of Clinical Diagnostics 1 (2011) 1-4 4
into the spinal canal in young adults, special immuno-
histochemical studies are recommended for determining
the possibility of spinal epithelioid sarcoma.
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