Extraskeletal Ewing's Sarcoma Research Articles

Extraskeletal Ewing's Sarcoma of the breast

Extraskeletal Ewing's Sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from the more common Ewing's sarcoma of the bone. Isolated cases of Extraskeletal Ewing's Sarcoma of various body parts have been reported. Here we report a case of Primary Extraskeletal Ewing's Sarcoma of the breast, that to best of our knowledge, has not been reported before.

Soft tissue sarcomas: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

The following recommendations apply to adult-type soft tissue sarcomas arising from limbs and superficial trunk. Recommendations on retroperitoneal sarcomas, desmoidtype fibromatosis, uterine sarcomas head and neck sarcomas and breast sarcomas are provided separately at the end of the chapter with regard to those main aspects by which they differ from more frequent soft tissue sarcomas. In general, the main principles of diagnosis and treatment may well apply to all soft tissue sarcomas, including the rarest presentations [e.g. visceral sarcomas other than gastrointestinal stromal tumours (GISTs)], which therefore are not specifically covered. Specific histological types, however, may deserve specific approaches, not necessarily covered hereafter, given the scope of these Recommendations. Extraskeletal Ewing sarcoma as well as embryonal and alveolar rhabdomyosarcoma are covered by other ESMO Clinical Practice Guidelines, inasmuch as they need completely different approaches. The same applies to GIST. Kaposi’s sarcoma is excluded from this chapter.

A rare case of intradural extramedullary Ewing's sarcoma with skip metastasis in the spine

Spinal cord tumors are rare and fortunately affect only a minority of the population. These tumors are classified based on their anatomic location in relation to the dura mater and spinal cord as epidural, intradural extramedullary or intradural intramedullary. Intradural extramedullary Ewing's sarcoma is extremely rare. Here, we are reporting a case of intradural extramedullary Ewing's sarcoma. This is the fourth such case to be reported in English literature. A 26-year-old gentleman presented to us with low backache and progressive paraparesis from a spinal tumor. Magnetic resonance imaging showed multiple intradural, extramedullary mass lesions extending from the T11 to S2 level. Neither osteolytic nor osteosclerotic changes were seen in the vertebral bodies. Extraskeletal Ewing's sarcoma was diagnosed histopathologically. He was treated by surgery, local radiotherapy and chemotherapy. Two months after treatment, he developed a new skip lesion in the spine at T6-T7 level. The new lesion was treated with local radiotherapy and chemotherapy. Presently, the patient has completed treatment and is clinically doing fine. Intradural extramedullary Ewing's sarcoma is a rare aggressive neoplasm with high propensity for skip metastasis.

CASE REPORT: Extraskeletal Ewing sarcoma of the abdominal wall

Ewing sarcoma is most commonly a bone tumour which has usually extended into the soft tissues at the time of diagnosis. Exceptionally, this tumour can have an extraskeletal origin. Clinical or imaging findings are non-specific and diagnosis is based on histology. We report a case of an extraskeletal Ewing sarcoma developed in the soft tissues of the abdominal wall in a 35-year-old woman who presented a painful abdominal wall tumefaction. Ultrasongraphy and computed tomography showed a large, well-defined soft tissue mass developed in the left anterolateral muscle group of the abdominal wall. Surgical biopsy was performed and an extraskeletal Ewing sarcoma was identified histologically.

Extraskeletal primary Ewing's sarcoma of the nasal cavity

Extraskeletal primary Ewing's sarcoma of the nasal cavity

Primary extraskeletal Ewing’s sarcoma of the maxilla with intraorbital extension

Extraskeletal Ewing's sarcoma is often described as a tumour involving the soft tissues of the lower extremities and the paravertebral region. Primary Ewing's sarcoma of the cranium is extremely rare, with only 17 cases reported so far [24]. Involvement of the paranasal sinus is a very rare entity. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. Primary Ewings sarcoma rarely arises in the facial skeleton and only occasionally in the maxilla. To date, there have been 22 cases of maxillary Ewing's sarcoma reported in the English-language literature [25]. We report here a new case of Ewing's sarcoma localized to the maxillary sinus, nose and the orbit, successfully treated by surgery, local high dosage radiotherapy and systemic chemotherapy.

Radiological findings of primary retroperitoneal ewing sarcoma

Ewing sarcomas are most commonly located in bone, while extraskeletal involvement of the retroperitoneum is extremely rare. We describe the radiologic and pathological findings in an adult patient with retroperitoneal extraskeletal Ewing sarcoma.

Extraskeletal primary Ewing's sarcoma of the nasal cavity

Summary Ewing's sarcoma (ES) and primitive neuroectodermal tumor represent the most frequent small and round cell bone tumors of childhood and adolescence. ES is the second most common primary malignant tumor of bone in children. In this article, a 1-year-old male patient with ES is presented and recent update on this entity is discussed. Computed tomography (CT) scans of the sinuses revealed a mass that appeared to be originating from the right inferior turbinate and filling the anterior part of the nasal cavity. The patient underwent complete removal of the mass with endoscopic nasal surgery under general anesthesia and was treated with adjuvant chemotherapy. Clinically and radiologically no recurrence of disease was noted during a follow-up period of 1 year. Although ES is extremely rare among nasal cavity neoplasms, it should be considered in the differential diagnosis.

Cervical extraskeletal Ewing's sarcoma: case report demonstrating radiological features and management

Extraskeletal Ewing's sarcoma is a rare tumour of mesenchymal origin, which is histologically similar to primary osseous Ewing's sarcoma. The majority of cases have been reported in the lower limb and paravertebral region, with a few cases reported in the neck. We report a patient with extraskeletal Ewing's sarcoma presenting with a right-sided neck mass, vocal fold palsy and T1 nerve root involvement. The detection of characteristic features on computed tomography and magnetic resonance imaging scans, in conjunction with histological analysis, was required to make the diagnosis. Radiological imaging demonstrated that the tumour was infiltrating through the neural exit foramina of the lower cervical nerve roots, with encasement of the vertebral artery. The patient was managed with combination chemotherapy for systemic control and irradiation for local control. Extraskeletal Ewing's sarcoma has a propensity to occur in a paravertebral location, being found rarely in the cervical region, and has a predilection to infiltrate through neural exit formina. Computed tomography and magnetic resonance imaging are helpful in the diagnosis of this rare tumour.

Spinal epidural extraskeletal Ewing sarcoma

A rare case of extraskeletal Ewing sarcoma, arising primarily in the spinal epidural space is reported. An 18-year-old male presented with a 2-month history of right shoulder pain progressing to complete paraplegia and urinary retention over the course of 2 days. Magnetic resonance imaging demonstrated an extradural mass extending from the C6 to T1 level. Histopathologic examination confirmed the diagnosis. The literature is reviewed and radiological differential diagnosis of this rare neoplasm is briefly discussed.

A single institution experience of combined modality management of extra skeletal Ewings sarcoma

BackgroundExtraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management.MethodsA retrospective review of the clinical features, treatment and outcome of patients with extraskeletal Ewings sarcoma who reported to a single institution between January 1992 – December 2003 is reported.ResultsA total of 19 patients with extraskeletal Ewings sarcoma were identified. Of these, 4 patients had metastatic disease at presentation and 15 patients with non-metastatic disease received combined modality treatment with primary combination chemotherapy followed by local treatment with radiotherapy or surgery. Disease free survival and overall survival for patients with non metastatic disease after combined modality treatment were 60% and 30% respectively. The significant predictors for prolonged disease free survival and overall survival were high haemoglobin(p = 0.002), low lactate dehydrogenase (p = 0.028), chemotherapy with Vincristine, Adriamycin, Cyclophosphamide, Ifosfamide and Etoposide regime (p = 0.008) and complete response to chemotherapy (p = 0.001).ConclusionAggressive combination chemotherapy followed by complete surgery or radiotherapy to a dose of more than 50 Gy is essential to confer optimal outcome for patients with extraskeletal ewings sarcoma.

Extraskeletal Ewing's Sarcoma of the Head and Neck Presenting as Blindness

Extraskeletal Ewing's sarcoma is rarely found in the head and neck regions. We report an unusual case of extraskeletal Ewing's Sarcoma of the parapharynx region in a 49-year-old man who presented with blindness. MRI examination showed marked enhancement of tumor thrombosis involving the superior sagittal sinus, straight sinus, transverse sinus, sigmoid sinus, and internal jugular vein. The final diagnosis was extraskeletal Ewing's sarcoma after biopsy of the internal jugular vein thrombosis by histopathological evaluation and immunohistochemical assay. In addition, the patient was diagnosed as having adenocarcinoma of the rectum by biopsy of the rectal mass. The patient was treated with systemic chemotherapy and showed improved response with durable remission. The patient's visual acuity, however, did not improve.

Primary Extraskeletal Ewing Sarcoma of the Thoracic Spinal Epidural Space: A Case Report

Cases of extraskeletal Ewing sarcoma (EES) originating primarily within the spinal epidural space, are very rare and have a very poor prognosis. There is no standard therapy for this disease. We report the case of a 23-year-old man presenting with symptoms of back pain and numbness of both legs for 10 days. Imaging studies revealed a dorsal soft-tissue, extradural mass at the T8–9 vertebral level. The patient underwent a laminectomy and complete excision of the tumor. The EES diagnosis was confirmed by histologic analysis including immunohistochemistry and by presence of the EWS-ERG due to the t (21: 22) (q22: q12) chromosomal translocation by a reverse transcriptase-polymerase chain reaction (RT-PCR). This is the first report of spinal epidural EES with presence of the EWS-ERG fusion transcript. Post-operatively, the patient received aggressive adjuvant chemotherapy and radiotherapy. At 63 months after surgery, the patient is without clinical or radiological evidence of recurrent or metastatic disease. Early discovery of EES and a complete resection followed by the aggressive treatment with radiation and chemotherapy may improve disease-free and overall survival.

Primary Pulmonary Ewing's Sarcoma/Primitive Neuroectodermal Tumor in a 67-year-old Man

Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma. We report a case of EES/PNET arising is the lung of a 67-yr-old man. Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin. The mass showed positive reactivity in the Periodic Acid Schiff (PAS) stain and MIC-2 immunoreactivity in immunohistochemical stain. Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement. The diagnosis was confirmed both pathologically and genetically. The mass lesion was resected, and the patient is currently undergoing chemotherapy.

Whole-body MRI for Detecting Metastatic Bone Tumor: Diagnostic Value of Diffusion-weighted Images

We assessed the diagnostic value of whole body magnetic resonance (MR) imaging (WB-MRI) using diffusion-weighted images (DWI) for detecting bone metastasis and compared it with that of skeletal scintigraphy (SS). Thirty patients with malignancies (breast cancer, 17 patients; prostate cancer, 9; and one patient each, thyroid cancer, liposarcoma, leiomyosarcoma, and extraskeletal Ewing sarcoma) underwent both WB-MRI and SS to detect bone metastasis. All patients were followed more than 6 months by MR imaging, SS, or computed tomographic (CT) examination. For WB-MRI, patients were placed in feet-first supine position with table-top extender and quadrature body coil. We acquired DWI (axial plane from lower neck to proximal femur) (single shot short TI inversion-recovery [STIR]: repetition time [TR] 6243/echo time [TE] 59/inversion time [TI] 180 ms; b value: 600 s/mm(2); 5-mm slice thickness; 112 x 112 matrix), T(1)-weighted fast spin echo (T(1)WI), and STIR (sagittal plane of total spine images and coronal plane of whole body images) images. Four blinded readers independently and separately interpreted images of combined MR sequences of T(1)WI+STIR (session 1) and T(1)WI+STIR+DWI (session 2). In 10 of 30 patients, we detected a total of 52 metastatic bone lesions; in the other 20, follow-up examinations confirmed no metastatic bone lesions. For these 52 lesions, for session 2, the mean sensitivity was 96% and the positive predictive value (PPV) was 98%. Those values were superior to those of session 1 (sensitivity: 88%; PPV: 95%) and those of SS (sensitivity: 96%; PPV: 94%). WB-MRI that included DWI was useful for detecting bone metastasis.

Extraskeletal Ewing Sarcoma Mimicking a Thyroid Nodule

ThyroidVol. 16, No. 10 Images in ThyroidologyExtraskeletal Ewing Sarcoma Mimicking a Thyroid NoduleCheng-Ho Chung, Chao-Hung Wang, Tao-Yeuan Wang, Jon-Kway Huang, and Yi-Shing LeuCheng-Ho ChungSearch for more papers by this author, Chao-Hung WangSearch for more papers by this author, Tao-Yeuan WangSearch for more papers by this author, Jon-Kway HuangSearch for more papers by this author, and Yi-Shing LeuSearch for more papers by this authorPublished Online:16 Oct 2006https://doi.org/10.1089/thy.2006.16.1065AboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail "Extraskeletal Ewing Sarcoma Mimicking a Thyroid Nodule." , 16(10), pp. 1065–1066FiguresReferencesRelatedDetailsCited byIntramedullary, periosteal, and extraskeletal Ewing sarcomas: retrospective study of a series of 126 cases in a reference center18 February 2022 | Skeletal Radiology, Vol. 51, No. 8Extraskeletal Ewing sarcoma of the buccal spaceClinical Imaging, Vol. 73Fifty years of thyroid pathology: concepts and developmentsHuman Pathology, Vol. 95Metastasis of Ewing's sarcoma to the thyroid gland: A case reportInternational Journal of Pediatric Otorhinolaryngology, Vol. 113Primary Ewing Sarcoma, ThyroidSarcoma de Ewing extraóseo en cabeza y cuello: revisión de la literatura a propósito de un casoRevista Española de Cirugía Oral y Maxilofacial, Vol. 39, No. 2Metastatic Neoplasms to the Thyroid24 June 2016Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient21 June 2014 | European Journal of Plastic Surgery, Vol. 37, No. 10Carcinoma of the Thyroid With Ewing Family Tumor Elements and Favorable Prognosis1 May 2013 | International Journal of Surgical Pathology, Vol. 22, No. 3Conservative Multimodal Management of a Primitive Neuroectodermal Tumor of the Thyroid1 June 2013 | Rare Tumors, Vol. 5, No. 2Extraosseous Ewing Sarcoma and Peripheral Primitive Neuroectodermal Tumor of the Thyroid Gland: Case Report and Review1 April 2013 | Ear, Nose & Throat Journal, Vol. 92, No. 4Extra-osseous Ewing sarcoma of the thyroid gland mimicking lymphoma recurrence: A case reportPathology - Research and Practice, Vol. 208, No. 6Extraosseous Ewing sarcoma of the thyroid gland19 August 2009 | Pediatric Radiology, Vol. 39, No. 12Primary Primitive Neuroectodermal Tumor of the Breast: a Case ReportKorean Journal of Radiology, Vol. 10, No. 4 Volume 16Issue 10Oct 2006 InformationCopyright 2006, Mary Ann Liebert, Inc.To cite this article:Cheng-Ho Chung, Chao-Hung Wang, Tao-Yeuan Wang, Jon-Kway Huang, and Yi-Shing Leu.Extraskeletal Ewing Sarcoma Mimicking a Thyroid Nodule.Thyroid.Oct 2006.1065-1066.http://doi.org/10.1089/thy.2006.16.1065Published in Volume: 16 Issue 10: October 16, 2006PDF download

Fulminant Thoracic Sarcoma Masked by Hemothorax

A 15-year-old female was admitted to the hospital with dyspnea and thoracic pain 1 month after trauma caused by a snowboarding accident. The patient was hit by another snowboarder and landed on the left hemithorax. She suffered immediate pain. The pain decreased gradually, but increasing dyspnea developed 2 weeks prior to admission. No evidence of other illness symptoms was recorded. Chest x-ray (Fig. 1) revealed left pleural effusion. Blood tests revealed a hemoglobin level of 9.9 gm/dl but were otherwise normal. Pleural drainage was performed based on clinical suspicion of traumatic hemothorax, and 2700 ml of blood-stained fluid was drained, with subsequent satisfactory radiological examination (Fig. 1) and immediate patient recovery. One month later, the patient was readmitted to the hospital with low hemoglobin (10.5 g/dl) and weight loss (7% of body mass). Except for a slight contusion to the left hemithorax at home, no other anamnestic event or new symptoms were recorded. After chest x-ray and CT scan (Fig. 2), the patient was operated on with the intention of performing hematoma debridement and lung decortication because of hemothorax. During thoracotomy, a cauliflower-like tumor filling the whole thoracic cavity and infiltrating the chest wall was found. There was minimal blood content in the thoracic cavity. Intraoperative biopsy diagnosis indicated “highly malignant neoplasm of unknown origin.” Taking into account extensive infiltration of the thoracic wall, the decision to abandon curative resection was made, and additional biopsies were taken before the thoracotomy was closed. The patient received four units of red blood cell concentrates after the operation. Histological examination revealed primitive neuroendocrine tumor, which belongs to the Ewing sarcoma family.1–3 The patient was subsequently treated with chemotherapy (ifosfamid, vincristin, doxorubicin, and cyclofosfamid) and died 8 months later from progressive disease. REFERENCES 1. Hadithi M, van Boxem TJ, Giaccone G, et al. Exercise-induced spontaneous hemothorax insinuates trauma; yet unmasks a lament disorder. Neth J Med. 2001;59:292–294. 2. Varsano S, Edelstein E, Gendel B, et al. Bilateral and unilateral spontaneous massive hemothorax as a presenting manifestation of rare tumors. Respiration. 2003;70:214–218. 3. Eroglu A, Kurkcuoglu IC, Karaoglanoglu N, et al. Extraskeletal Ewing sarcoma of the diaphragm presenting with hemothorax. Ann Thorac Surg. 2004;78:715–717.

Extra-skeletal Ewing's sarcoma of the submandibular gland

Extra-skeletal Ewing's sarcoma (EES) is an uncommon malignancy, especially in the head and neck region that may arise in various extra-osseous tissues. We report a 22-year-old male with an EES of the submandibular gland, which to the best of our knowledge, has not been described previously. The patient who underwent combined treatment with surgical resection and chemo-irradiation was disease free for 22 months but succumbed to multi-organ metastases 14 months later. This case highlights the combined diagnostic role of immunohistochemical, cytogenetic and radiological evaluation of EES. EES is an aggressive cancer that requires multidisciplinary management with wide surgical excision and adjunctive chemo-irradiation for the best outcome.

Extraskeletal Ewing’s Sarcoma Presenting With Multifocal Intrathoracic Mass Lesions Associated With Mediastinal Shift

Extraskeletal Ewing's sarcoma is an uncommon disease that predominantly involves the soft tissues of the trunk or the extremities. This article presents a patient with multifocal intrathoracic mass lesions involving the mediastinum and the lingula associated with mediastinal shift, eventually diagnosed as extraskeletal Ewing's sarcoma.

Establishment and Characterization of a Clonal Human Extraskeletal Ewing's Sarcoma Cell Line, EES1

Ewing's sarcoma, a small round cell sarcoma arising in soft tissue as well as the bone, is one of the most malignant tumors in children and young adults. Few established cell lines of extraskeletal Ewing's sarcoma (EES) have been reported, which made it difficult to examine the biological features of EES. Therefore, we have established a new clonal cell line of EES. We report its morphological characters, results of chromosomal and immunohistochemical analysis. A piece of tumor obtained from the 18-year-old female patient with EES was xenografted in a nude mouse. In vitro subcultured cells were then obtained from this xenograft. A clonal cell line was subsequently established by limiting dilution and designated EES1. EES1 cells had a doubling time of 24 hours. In the xenografted tumor, the cells expressed vimentin, CD99 (MIC2), neuron specific enolase (NSE) and cytokeratin. The original tumor cells also expressed vimentin, CD 99, and NSE, but was negative for cytokeratin. The morphological and immunohistochemical features of this cell line established, except for cytokeratin expression, were consistent with those of the primary tumor. Cytogenetic analysis of EES1 revealed chromosomal translocation of t(11; 12)(q24;ql2). The chimeric fusion of the Ewing's sarcoma gene in band 22q12 with the Friend leukemia virus integration-1 gene in band 11q24 was also demonstrated. Fluorescence in situ hybridization further confirmed the presence of translocation involving the Ewing's sarcoma gene in both the primary tumor and EES1 cells. In conclusion, we have established a human EES cell line EES1, which will provide a useful model for studying various aspects of human EES.