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



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
Ewings sarcoma of the sinonasal tract with orbital
extension: a rare case report and literature review
Omair Al Hussain
, Ahmed Aldandan
*, Abdulrahman Alkhatib
Ghaleb Alazzeh
, and Ali Almomen
Head of ENT Department, College of Medicine, Immam Mohammad Ibn Saud Islamic University, Riyadh,
Saudi Arabia,
Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia, and
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:
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.
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].
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
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
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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-
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
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.
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-
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.
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.
The authors declare that they have no conict of interest.
Informed consent was obtained from all individual participants
included in the study.
No funding obtained.
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Shetty J, Makannavar J. Rare case of extraskeletal ewings
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Ewings sarcoma of the sinonasal tract with orbital extension
... The diagnosis of ONB is extremely challenging as several sinonasal neoplasms must be excluded. The differential diagnosis is widely broad including rhabdomyosarcoma, lymphoma, undifferentiated carcinoma, neuroendocrine carcinoma, melanoma and Ewing' s sarcoma [11]. The differential diagnosis of ONB and neuroendocrine carcinoma was considered as ONB is nonreactive with CK and TTF-1. ...
... 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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Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent orbital protrusion is even rare. Grading ONB is extremely important as individualized treatment plans must be formulated according to tumor grade. We report the case of a 67-year-old female who presented with the chief complaints of persistent nasal congestion with intermittent epistaxis and unilateral proptosis over the past five years. Radiological imaging was suggestive of a large heterogeneous mass in the right superior nasal cavity with extensions into the right medial orbit, nasopharynx, the right maxillary sinus, the anterior cranial fossa, right ethmoidal, frontal and bilateral sphenoidal sinuses, as well as into the right frontal lobe. Assessment of the radiologic features revealed the diagnostic possibility of olfactory neuroblastoma. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Frontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. Evaluation of histopathological characteristics and immunohistochemical findings revealed a diagnosis of WHO grade IV olfactory neuroblastoma. Because of poor economic condition, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination. We report a huge ONB centered in the posterior right orbit with prominent orbital protrusion. Magnetic resonance image and computed tomography are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival.
... (10,12,14 ) La inmunohistoquímica de CD99, una glicoproteína de la superficie celular y un marcador de diagnóstico muy sensible pero poco específico para los SE, es fundamental en la evaluación diagnóstica, ya que el 95% de los SE lo expresa como un anticuerpo con marcaje en la membrana celular. (10,(12)(13)(14)(22)(23) Sin embargo, podríamos confirmar el diagnóstico con el apoyo de analisis moleculares que detectan el resultado de una fusión de la translocación del cromosoma 22 t(11:22) (q24:q12), el cual se encuentra en el 85%-95% de los casos de SE. (11,13,14,19 21, 22) A pesar de que en Honduras existen ciertas limitaciones en cuanto a estudios de inmunohistoquímica, se obtuvo apoyo externo para realizarlo y así diferenciar el Sarcoma de Ewing en cavidad sinonasal del resto de tumores de células pequeñas y redondas. ...
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El sarcoma de ewing es un tumor maligno de rápido crecimiento, con prevalencia de 1-5 casos por cada 1.000.000 habitantes, su forma extraesquelética en la cavidad sinonasal o senos paranasales es inusual. Objetivo: describir la localización atípica de esta neoplasia y la importancia de lograr un diagnóstico oportuno. Paciente femenina, con una masa en la cavidad nasal derecha de dos meses de evolución, cefalea y epistaxis. Con asimetría en región orbitaria derecha y deformidad del tabique nasal, senos paranasales con sintomas de obstrucción. La tomografía reveló una masa que invade senos paranasales. La biopsia mostró un sarcoma de Ewing. Se confirmó con CD99. La paciente recibió quimioterapia y plan de resección quirúrgica, pero falleció. El diagnóstico y tratamiento oportuno del sarcoma de ewing en cavidad sinonasal debe apoyarse con examenes tomográficos, histopatológicos, inmunohistoquímicos y de ser posible citogenéticos para llegar al diagnóstico definitivo en etapas tempranas del tumor.
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A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach. The soft mass was large and vascular. A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells. Mitotic figures were also present. Immunohistochemically, the tumor cells were strongly positive for CD99. Molecular studies using a PCR confirmed the chromosomal translocation of FLI1 (exon 6). These findings were considered diagnostic for Ewing's sarcoma. Postoperatively, the patient was treated with combined chemotherapy and radiotherapy. Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (total: 7 cycles) was commenced. He also received radiation therapy for local control (total dose: 50.4 Gy). The patient is currently alive without any evidence of recurrence or metastasis.
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Ewing’s sarcoma is a malignant tumor belonging to the group of small round cells tumors. Histologically similar to soft tissue neoplasms originally described as primitive neuro-ectodermal tumors (PNET), in the WHO classification, Ewing’s sarcoma and PNET are labeled together under the rubric of EWS/PNET. Rarely located in the nasal cavity and the para-nasal sinuses, we report three cases of Ewing’s sarcoma of maxillary bone and sinus. Our patients, 2 males and one female, were aged 20, 16 and 13years respectively. The chief complaint was a painful face swelling. The diagnosis was retained on histologic and immuno-histochemical results. In two cases, surgery was performed as primary treatment modality followed by chemo-radiotherapy, which was the only therapeutic modality in the remaining case. After a follow-up of 2, 3 and 8years (for each patient), we did not report local or distant failures.
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Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.
Introduction: Ewing sarcoma (ES) of the sinonasal tract and associated primitive neuroectodermal tumors are rare. To our knowledge, only 10 case reports of sinonasal ES of the nose or paranasal sinuses have been reported. Furthermore, there has been only 1 case of sinonasal ES arising from the middle turbinate. Recommended management of sinonasal ES stems from the management of its osseous counterpart, ES, but treatment with surgery, radiotherapy, and chemotherapy is varied. Five-year survival rates vary from 21% to 70%, with the lower rates representing patients presenting with metastatic disease. Case presentation: A 26-year-old man presented with persistent left-sided nasal obstruction. Endoscopy demonstrated a friable mass in the left nasal cavity originating from the middle turbinate with extension into the nasopharynx, confirmed with computed tomography and magnetic resonance imaging. There was no evidence of metastatic disease on positron emission tomography-computed tomography. Histopathologic results were consistent with sinonasal ES. The result of fluorescent in situ hybridization was positive for the EWS gene translocation. A multidisciplinary tumor board evaluated the patient. The patient then underwent neoadjuvant chemotherapy, followed by definitive endoscopic surgical resection and postoperative radiotherapy. Discussion: Our literature review found more involvement of the maxillary and ethmoid sinuses compared with the nasal cavity, and that the role of radiation and surgical approach was varied. ES of the sinonasal tract is a rare entity with high mortality, but few standardized treatment protocols exist. Further study and evidence-based treatment protocols are needed. This article outlines the role of relevant imaging, a multidisciplinary team approach, and the optimal timing of surgery, chemotherapy, and radiation.
The differential diagnosis for small round cell tumors in the sinonasal tract is diverse and as the body of literature documenting not only uncommon presentations but also availability of ancillary studies grows, so does the need for a reminder to take a conservative and thorough approach before rendering a diagnosis. Small tissue samples are particularly problematic, with limitations that include volume of tumor cells available for studies, lack of architectural context and a non-specific gross description. Incorporation of patient history and presentation, radiologic findings, clinical impression and concurrent studies often guide the course of studies performed by the pathologist. If these are non-specific, the pathologist may need to perform ancillary studies, including a broad panel of immunohistochemical stains and molecular studies. If tissue is limited, a precise classification may not be achievable. Although the expectation to render a definitive diagnosis is high, the pathologist should never feel compelled to go further with a diagnosis than the tissue itself supports.
Objective: The purpose of this paper was to review our experience with Ewing's sarcoma of the head and neck in children. Design: Retrospective chart review. Setting: The Hospital for Sick Children, Toronto, Ont., Canada. Methods: Between 1986 and 1996, 70 cases of Ewing's sarcoma were identified. The medical records, roentgenographic and pathology reports were reviewed retrospectively. The gender, age of presentation, location and clinical presentation of the tumor were noted in the cases involving the head and neck. The treatment and follow-up of these patients were recorded. Results: Of the 70 cases of Ewing's, five involved the head and neck (7.1%). The age of presentation ranged from 7.5 to 14 years. An enlarging mass in the mandible was the mode of presentation in three of the five children. Two patients had metastases at initial presentation. All patients received combination treatment regimens with chemotherapy initially, followed by adjuvant surgery and/or radiation. Follow-up ranged from 2 to 11 years. Three of five patients died of metastatic disease. Two are alive and well with no evidence of disease. Conclusions: Ewing's sarcoma occurs infrequently in the head and neck in children. An enlarging mass in the mandible is the most frequent mode of presentation. This tumor is treated systemically with high dose chemotherapy and locally with surgical excision where possible. In lesions that are initially unresectable and/or show a poor response to chemotherapy, radiation is used for local control. A good prognosis can be expected if the disease has not metastasized.
The Ewing's family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7-70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1-168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.
Progress in the treatment of Ewing's sarcoma, the second most common bone tumour in children and adolescents, has improved survival from about 10% in the period before chemotherapy was introduced to about 75% today for patients with localised tumours. However, patients with metastases still fare badly, and the therapy carries short-term and long-term toxicities. Multidisciplinary care is indispensable for these patients. Molecular techniques and new imaging modalities are affecting the diagnosis and classification of patients with Ewing's sarcoma. Cooperative group studies have led to chemotherapy regimens using the same drugs (vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide), although the exact regimens differ in Europe and North America. The EWS-ETS family of gene fusions and their downstream effects in Ewing's sarcomas provide opportunities for new approaches to treatment. These include the inhibition of the fusion gene or its protein product, and pathways related to IGF1 and mTOR. Inhibition of tyrosine kinases, exploitation of non-apoptotic cell death, and interference with angiogenesis are promising new approaches. With many new approaches and relatively few patients, it will be challenging to integrate new and established treatments through clinical trials.
Nasal fractures are a common complaint familiar to all otolaryngologists. Sinonasal primary Ewing's sarcomas are extremely rare. The case of a 9-year-old boy is presented whose nasal fracture and subsequent lateral nasal wall hematoma revealed an underlying Ewing's sarcoma. There are several unusual features in the history and clinical course of this patient. Following biopsies, immunohistochemistry proved essential in distinguishing a Ewing's sarcoma from other small cell tumours. It is important that a seemingly common condition can be the first presentation of a less common, more sinister pathology.
To evaluate the outcomes of patients with Ewing's sarcoma family of tumors (ESFT) treated with modern radiotherapy techniques with MRI along with optimal chemotherapy. The records of all 60 patients with ESFT who received radiation to the primary site between 1990 and 2004 were reviewed. All patients received chemotherapy, including vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Radiation was used as the sole modality for local control in 31 patients and was given either before (n=3) or after surgical resection (n=26) in the remainder. All patients had MRI and CT scan-based treatment planning, and 43% received intensity-modulated radiation therapy. Radiation doses ranged from 30 Gy to 60 Gy (median, 51 Gy), and 35% received hyperfractionated radiotherapy. Median age was 16 years (range, 2-40 years). Because of selection bias for radiotherapy, the majority of primary tumors were centrally located (72%): spine (n=18), pelvis (n=15), extremities (n=12), chest wall (n=5), head and neck (n=5), and other (n=5). Thirty-eight percent of patients presented with metastatic disease, and 52% of primary tumors were >or=8 cm. Actuarial 3-year local control was 77%. The presence of metastases at diagnosis was an adverse prognostic factor for local control (84% vs. 61%, p=0.036). No other predictive factors for local failure were identified. In patients without metastatic disease, 3-year disease-free and overall survival rates were 70% and 86%, respectively, whereas in patients with metastases they were both 21%. Follow-up of surviving patients was 6-178 months (median, 41 months). In this unfavorable cohort of ESFT patients, radiation therapy was an effective modality for local control, especially for patients without metastases. The presence of metastases at diagnosis is a predictive factor not only for death but also for local failure.