Case Of Extraskeletal Ewing's Sarcoma Research Articles

Gastrointestinal Ewing Sarcoma: A Clinicopathological and Molecular Genetic Analysis of 25 Cases.

Occurrence of extraskeletal Ewing sarcoma (ES) in the gastrointestinal (GI) tract is extremely rare. Here, we report 25 cases of ES arising primarily in the GI tract with a focus on the clinicopathological and molecular features, differential diagnosis, and biological behavior. Thirteen patients (52%) were male, and 12 (48%) were female with age ranging from 9 to 59 years (mean: 36.2 years; median: 38 years). Twenty-one tumors (84%) occurred in the small intestine, 3 (12%) in the stomach, and 1 (4%) in the anal canal. At operation, 8/18 (44.4%) patients presented with abdominopelvic disseminated disease. Tumor size measured from 2 to 25cm (mean: 8.2cm; median: 6cm) in maximum size. Microscopically, the tumors were composed of infiltrative small round, ovoid, or short spindle cells arranged mostly in lobular and solid sheet-like patterns with a rich capillary vasculature. Focal formation of Homer Wright-type rosettes and pseudoalveolar architecture was noted each in 2 (8%) cases and 3 (12%) cases. Besides CD99 (25/25; 100%), Fli-1 (15/15, 100%), and NKX2.2 (14/16; 87.5%), the tumor cells also showed variable staining of CD117 (14/17; 82.4%). Of 25 cases, 23 (92%) demonstrated EWSR1 rearrangement by fluorescence in situ hybridization analysis. The 2 cases with negative fluorescence in situ hybridization results were found to harbor EWSR1::ERG and EWSR1::FLI1 fusion by further RNA sequencing, respectively, with a median follow-up of 12 months (range: 1 to 42 months), 5/19 (26.3%) patients developed visceral metastasis and 12/19 (63.2%) patients died of the disease (range:1 to 33 months; median: 9 months). This study showed that GI ES had a predilection for the small intestine, although other sites of the GI tract could also be involved. GI ES had a poor prognosis with a high rate of mortality, particularly in patients with abdominopelvic disseminated disease. In light of appropriate therapeutic strategies and prognostic considerations, it is essential not to misdiagnose GI ES as gastrointestinal stromal tumor owing to the expression of aberrant CD117.

Primary Extraskeletal Ewing Sarcoma of the Duodenum Revealed by 18 F-FDG PET/CT.

Extraskeletal Ewing sarcoma of the duodenum is a relatively uncommon entity tumor. We report a case of extraskeletal Ewing sarcoma in a 21-year-old woman. She complained of abdomen pain and melena. 18 F-FDG PET/CT showed an intense uptake by the duodenum mass along with multiple FDG-avid enlarged lymph nodes in the mesentery, which was pathologically proven as extraskeletal Ewing sarcoma.

A Rare, yet Aggressive, Intra-Abdominal Malignancy: Ewing Sarcoma of the Pancreas

Introduction: Ewing sarcoma (ES) is a malignant bone tumor of long-bones, which typically occurs in children. Extraskeletal Ewing sarcoma (EES) is a poorly differentiated soft tissue sarcoma within morphologic category of ‘small-round-cell’ tumors and accounts for less than 1% of soft tissue sarcomas. Only 24 cases have been reported in the literature. Herein, we present an interesting case of EES of pancreas. Case Description: 34-year-old male presented to clinic secondary to insidious onset, generalized abdominal pain for past 1-month. Physical exam revealed non-tender, moderately distended abdomen with a palpable mass above the umbilicus. Initial lab workup showed mild elevation of AST 91 (0-50 U/L) and alkaline-phosphatase 337 (38-126 U/L). CT abdomen showed lobulated heterogeneous mass with necrotic transformation in body and tail of pancreas, with multiple liver lesions. Resultantly, a working diagnosis of adenocarcinoma of pancreas with metastasis to liver was made. A CT-guided biopsy of pancreatic and hepatic mass showed small, blue, round cells with scant cytoplasm. Hence, the previous diagnosis of pancreatic adenocarcinoma was disregarded, and other differential diagnoses including EES, neuroblastoma, non-Hodgkin's lymphoma vs desmoplastic small blue round cell tumor were considered. Further testing with immunohistochemistry confirmed the diagnosis of EES. Subsequently, patient was started on chemotherapy with doxorubicin, cyclophosphamide and vincristine. After one round of chemotherapy, patient developed neutropenic fever and died after 1-month from a complicated hospital course. Discussion: This case highlights an extremely rare type of soft tissue sarcoma (EES) at an unusual location. The diagnosis of ESS is confirmed by immunohistochemistry as imaging modalities often remain nonspecific. Histologically, ES and EES are described as small, blue, round cells with hyperchromatic nuclei and scant cytoplasm, with molecular expression of MIC2, CD99 and antibody against FLI1 centered in nucleus. Though 85% of the ES cases are associated with chromosomal translocation t(11;22) and q(24;12), it remains non-specific. EES is an aggressive and malignant tumor with a 5-year survival rate of around 50%. Surgical excision, plus chemotherapy and radiotherapy is the main modality of treatment. Though EES is rare, physicians should be acquainted with and consider it in the differential diagnosis, as early recognition and intervention is very critical.1397_A.tif Figure 1: Necrotic mass (14.8cm x 5.6cm x 6.7cm) again visualized in the pancreas along with multiple hypo-attenuated liver lesions.1397_B.tif Figure 2: Small, blue, round cells with high NC ratio, dark chromatin pattern with scant cytoplasm. Tumor cells positive for vimentin; negative for CD45, CD117, chromogranin, synaptophysin, pan cytokeratin, smooth muscle actin, desmin, HMB-45, S-100 and CD138. Pattern is highly consistent with high grade soft tissue sarcoma- EES.

Primary cutaneous extraskeletal Ewing's sarcoma/PNET: possibility of better prognosis than deep ES/PNET

Extraskeletal Ewing sarcoma (ES) and Primitive Neuroectodermal Tumour (PNET) were first described by Angerval and Enzinger in 1975 [1]. Primary ES/PNET arising in skin is distinctly rare [2-6]. Unlike ES/PNET in deep soft tissue, which has a poor prognosis, cutaneous and subcutaneous ES seem to be associated with an indolent course and a favourable prognosis [3-5, 7]. We report a case of cutaneous ES (CES) in a young adult and additionally review the cases of CES which have been previously reported [...]

Extraskeletal Ewing’s Sarcoma Arising in the Larynx

Extraskeletal Ewing's sarcoma (EES), or primitive neuroectodermal tumor, is an uncommon neoplasm with low incidence in the head and neck. Occurrences in the larynx are even more exceptional with only two previous reported cases of EES arising from the larynx. We report the case of a 45-year-old woman with a laryngeal Ewing's sarcoma treated with chemotherapy with radiotherapy to follow. Here we describe the histology, molecular diagnosis and treatment of this unusual tumor.

Primary Extraskeletal Ewing's Sarcoma of Nasal Cavity

Extra skeletal Ewing's Sarcoma (EES) of nasal cavity is a rare soft tissue tumor, morphologically similar to the commoner Ewing's sarcoma (ES) arising from bone. We report a case of EES of nasal cavity in a nine year old male who presented with nasal obstruction. Contrast enhanced CT scan showed mildly enhancing soft tissue attenuating mass. Histopathologically examination showed round cell tumor which was confirmed by special stain and immunohistochemistry. PAS showed positivity for intracellular glycogen, Immunohistochemistry showed positivity for MIC-2,CK,S-100,EMA.

Extraskeletal Ewing's sarcoma of the thoracic epidural space: Case report and review of the literature

The occurrence of primary extraskeletal Ewing's sarcoma (EES) of the spinal epidural space has been rarely reported in the literature. The clinical, radiologic and pathologic features of a case of EES occurring in the thoracic epidural space are presented. A 37-year-old woman presented with a one-year history of back pain. Magnetic resonance imaging demonstrated an epidural mass at the T8-9 level. Laminectomy and partial resection of the tumor were performed. The differential diagnosis of a spinal epidural mass is broad. Histopathological and molecular cytogenetic examinations confirmed an EES arising from the thoracic epidural space. Despite receiving both chemotherapy and radiotherapy, the patient died of respiratory insufficiency due to medulla oblongata metastasis 22 months after the initial consultation. Awareness of this entity will allow this rare diagnosis to be considered and facilitate appropriate management. A review of the literature on spinal epidural EES is also presented.

Extraskeletal Ewing's Sarcoma of parapharyngeal space

Abstract Extraskeletal Ewing's Sarcoma (EES) of head and neck region, is a rare soft tissue tumour morphologically similar to the commoner Ewing's Sarcoma arising from bone. We report a case of EES in parapharyngeal space in a 6-year-old male; who presented with stridor. CT scan of neck showed soft tissue mass in right parapharyngeal space with intraorbital and intracranial extention. Histopathological examination showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. The patient responded very well to Concurrent Chemoradiation. An extensive review of English literature, to the best of our knowledge, did not reveal any previous case of EES in parapharyngeal space in children; although one similar case in a 53-year male is reported.

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

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.

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.

Extraskeletal Ewing's Sarcoma of the Hard Palate

Extraskeletal Ewing's sarcoma (EES) is a rare soft tissue tumor morphologically indistinguishable from the more common Ewing's sarcoma of bone. We report a case of EES arising in the hard palate of 34-yr-old male patient. Microscopically, the monotonous small round cells without neuronal differentiation showed membranous positive immunoreactivity for MIC2/CD99 and vimentin. Ultrastructurally, the tumor cells showed a few intracytoplasmic organelles without evidence of neurosecretory granules or neurofilaments. The EWS-FLI1 chimeric gene was identified using the nested reverse transcriptase-polymerase chain reaction.

Extraskeletal Ewing's sarcoma of the parapharyngeal space

Extraskeletal Ewing's sarcoma (EES) is rarely found in the head and neck region. We report here a case of EES of the parapharyngeal space in a 53-year-old man who presented with blurred vision, dysphagia, hoarseness and right facial numbness. CT examination showed a large, seemingly well-defined soft tissue mass in the right parapharyngeal space with skull base destruction and intracranial extension. The patient showed poor response to chemotherapy and radiotherapy and died 6 months after initial presentation. A review of the literature revealed no previous reports of EES occurring in the parapharyngeal space.

Extraskeletal Ewing Sarcoma of Cervical Epidural Region: Cases Report

Extraskeletal Ewing sarcoma is a rare malignant tumor found in children and young adults. It commonly occurs in deep soft tissue of the trunk, especially in the paravertebral region and extremities. We report two cases of extraskeletal Ewing sarcoma occurring as a cervical epidural tumor in elderly patients. The MRI and CT findings showed that paravertebral epidural tumors had invaded the spinal canal through the intervertebral foramen. At T1-weighted MR imaging, the masses were isointense to muscle, and at and T2-weighted images were hyperintense, and heterogeneous contrast enhancement was observed. Extraskeletal Ewing sarcoma, though quite rare, should be borne in mind in the differential diagnosis of paraspinal epidural tumors.

A CASE REPORT OF EXTRASKELETAL EWING'S SARCOMA OF THE CHEST WALL

We describe a rare case of extraskeletal Ewing sarcoma of the chest wall. The patient, a 30-yaer-old woman, was seen at the hospital because of right back pain and an abnormal shadow in the right chest wall on X-ray, CT, and MRI films. CT and MRI revealed a solid tumor attached to the posteroinferior aspect of the intrathoracic chest wall. Angiography of intercostal arteries disclosed feeding arteries from the sixth and seventh intercostal arteies, and a tumor stain. We suspected a sarcoma, and surgically resected a tumor in the sixth intercostal space with the sixth and seventh ribs en bloc. The tumor measured 4.0×2.5cm. Histologically, there was a little involvement of the ribs. Individual tumor cells were small and round-shaped, with scanty, ill-defined, PAS positive material in the cytoplasma. With these results, we made a diagnosis of extraskeletal Ewing's sarcoma. Combination chemotherapy with ADR, CPM, ACT-D and VCR was administered after the operation. Tegafur and PSK were orally administered after discharge. There has been no evidence of recurrence after two and a half years of follow-up.

後腹膜軟部組織に発生した Ewing 肉腫の1例

The patient was a female aged 18. Hematuria was discovered at an examination two years previously at which time the patient was complaining of right lumbar pain. The patient visited our hospital with a chief complaint of right abdominal tumor in June, 1988. A retroperitoneal tumor, 77 X 45 mm in size, was observed by CT and ultrasonic examination, and the right kidney was in hydronephrosis. An operation was performed in September, 1988, based on a diagnosis of retroperitoneal tumor and hydronephrosis. The tumor was seen to have involved the right ureter. Subsequently the tumor was surgically removed; right total nephroureterectomy and partial cystectomy were performed at that time. The removed tumor was 90 X 70 mm in size and 120 g in weight; it was histopathologically diagnosed as extraskeletal Ewing sarcoma. A systemic examination was carried out postoperatively, but no abnormalities were observed. As of September, 1990, the patient had had no recurrence and was being kept observation on an outpatient basis. There have been a total of 210 case reports regarding extraskeletal Ewing sarcoma published worldwide, including our case and 26 cases in Japan, so far as we know. However those of a lesion of retroperitoneal origin are extremely rare, numbering only 11 cases worldwide and 2 Japan. Herein we report our case of extraskeletal Ewing sarcoma with reference to the other 209 cases reported in the literature to date.